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A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 67-year old female presented with a smooth, solitary lump on scalp in the right occipital region (Figure ).\nOn examination, a 3 x 3 cm smooth, firm, non-mobile lump was palpable with overlying scalp hair. The initial impression was that of a sebaceous cyst and she was advised to wait and watch for any changes to the features of the lesion or development of any new symptoms. On review after six months, the size of the lesion increased to 4 x 4 cm and the patient reported progressively worsening itching on the overlying skin. A decision was made to completely excise the cyst. Histopathological analysis from the lesion confirmed a mucinous eccrine carcinoma of skin (Figures , ).

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 70-year-old male noticed a swelling near the the left canthus since one-and-a-half years. The swelling was gradually increasing in size. On examination, a raised, freely mobile, firm skin mass of approximately 3.0 × 2.5 cm was present on the lateral canthal region on the left eye . The patient's extraocular eye movements were unaffected. Examination revealed no other suspicious skin lesions or palpable lymph nodes. The case was clinically diagnosed as fibroma. Excision biopsy was done and the specimen was sent for histopathological examination. Gross examination revealed a well-circumscribed, spherical soft tissue mass, partially skin covered, measuring 2 cm in diameter. The cut surface of the tumor had a gray-brown gelatinous appearance []. Microscopically, the sections revealed a tumor in the dermis composed of tumor cells arranged in nests, glands, and cribriform patterns []. The individual tumor cells were columnar and had a hyperchromatic nucleus. The cells were seen floating in large pools of mucin separated by thin fibrovascular septa []. The mucin was periodic acid schiff (PAS) positive and diastase-resistant. [] Further investigations, including a colonoscopy, ultrasonographic examination of the abdomen, and computerized tomography (CT) scans of the chest, abdomen, and pelvis were found to be normal. Thus, the lesion in the skin was reported to be PMC of the skin.

An 82-year-old male consulted MDACC with a right infraorbital ulcerated subcutaneous nodule and a right nontender neck mass. He had history of SRCC arising in the right orbit diagnosed 13 years prior to presentation at MDACC, followed by radiation therapy, chemotherapy, and right orbital exenteration. His family history revealed lung cancer in one brother, and one sister died of stomach cancer. A CT scan of the maxillofacial area and neck confirmed soft tissue thickening along the right infraorbital region () and metastases to the right intraparotid and right level II lymph nodes. A CT scan of the chest, abdomen, and pelvis showed no evidence of possible second primary disease or metastases. Revision of the right orbital exenteration, parotidectomy, and right neck dissection were performed, and positive resection margins were identified by intraoperative frozen section analysis. The indurated mass involving periorbital tissue and right upper cheek measured 5.3 × 4.5 cm. Microscopically, multifocal cords of signet ring cells arising in close proximity to eccrine dermal ducts were observed (), extending into soft tissue and surgical margins of resection. The patient underwent chemoradiation and passed away 5 years after the final treatment (18 years from initial presentation). The case was previously reported by Requena et al. [].

Write a detailed clinical case vignette based on the following key phrases: Mucinous Carcinoma, Skin Cancer, Eyelid Tumors

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 52-year-old woman, a previously diagnosed case of cryptogenic cirrhosis, presented with progressive worsening of jaundice and unrelenting right upper quadrant abdominal pain. Previously, her work-up for known etiologies of chronic liver disease (including hepatitis viral serology, autoantibodies such as antinuclear and antismooth muscle antibodies, serum ferritin, and ceruloplasmin levels) were negative. At present admission, her liver biochemistry revealed total, direct, and indirect bilirubin of 7.8, 4.1, and 3.7 mg/dL, respectively. Serum alkaline phosphatase was elevated at 264 IU/L, whereas alanine aminotransferase and aspartate aminotransferase were only marginally raised. Ultrasonography of abdomen revealed biliary dilatation with suspicion of an intraductal calculus impacted at the ampulla. Interestingly, subsequent magnetic resonance cholangiopancreatography (MRCP) revealed duplication of the extrahepatic bile duct []. The duplicated bile ducts were running parallel and were seen to reunite into a common channel at the level of the head of the pancreas consistent with type-Va double common bile duct according to Choi et al. Additionally, a filling-defect suggestive of an obstructive calculus was seen in the common channel just above the ampulla causing obstruction and upstream biliary dilatation (arrow). No associated anomaly of the gallbladder was evident. The cystic duct was seen inserting into the right-sided bile duct. The biliopancreatic junction was unremarkable.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

The patient is a 79-year-old white man who had a laparoscopic converted to open cholecystectomy performed approximately 3 weeks before transfer to our facility. Details of the original operation noted a gangrenous perforated gallbladder requiring an open conversion. The cholecystectomy was performed using a retrograde fundus-down approach, with inability to obtain the critical view. A tubular structure, thought to be a duct of Luschka, was also identified and ligated in addition to the cystic artery and duct. A single drain was placed, and the procedure was completed in an otherwise standard fashion.\nPostoperatively, the patient had persistent high-output bilious drainage. He was discharged on postoperative Day 6 with a drain in place. He returned to the outside facility approximately 4 days later with jaundice, weakness, fatigue and pain. A computed tomography (CT) scan of the abdomen and a nuclear cholescintigraphy scan confirmed a biliary leak with free intra-abdominal fluid consistent with biliary peritonitis (Fig. ). The patient was transferred to our critical care unit for further management.\nThe patient was placed on broad spectrum antibiotics and treated for atrial fibrillation with rapid ventricular response. Laboratory workup revealed a bilirubin level of 17 mg/dl, as well as profound protein–calorie malnutrition with an albumin of 1.9 gm/dl. A percutaneous cholangiogram was performed confirming a hilar ligation of his hepatic duct with inability to pass a wire or catheter distally. A right posterior and right anterior percutaneous cholangiogram catheter was placed for definitive biliary diversion (Fig. ). The patient was then managed with high calorie, high protein enteral nutrition through a nasogastric Dobhoff tube for approximately 7 days until able to tolerate appropriate oral intake for discharge. He was scheduled for follow-up in 2 weeks.\nAt follow-up, the patient had improved substantially. Repeat CT scan confirmed appropriate drainage of all abdominal fluid collections. His bilirubin improved to 1.2 mg/dl with an albumin of 3.1 gm/dl and prealbumin of 17.3 gm/dl. At this visit, he was scheduled for definitive biliary reconstruction approximately 2 months after his initial operation.\nDuring this operation, the porta was dissected and the common and proper hepatic arteries were identified and preserved. We were unable to palpate the previously placed percutaneous biliary catheters due to the high placement above the hilum at the level of multiple clips. We transected the CBD distally and removed numerous clips, finally noting bile drainage, but were unable to identify a cholangiogram catheter. We then identified a second tubular structure more lateral to the duct. We elected to transect this tissue, identifying a second extrahepatic bile duct. The anterior percutaneous catheter was identified proximally within the duct. An on-table cholangiogram with fluoroscopy was performed noting two separate extrahepatic biliary systems, draining the right and left lobes of the liver, respectively (Figs and ). Both distal ducts were ligated to definitively close the orifice to the duodenum and prevent spillage. A Roux limb of jejunum was created and anastomosed in a retrocolic fashion to the two separate hepatic ducts at the level of the hilum. A drain was placed, and there was no evidence of bile leakage.\nThe patient was discharged on postoperative Day 6 with normal hepatic function, normal bilirubin and no further complications. A follow-up cholangiogram at his postoperative clinic visit showed normal intrahepatic ducts with no evidence of stricture or leak, signifying successful repair of his injury with bypass of his duplicated biliary anatomy (Fig. ). His cholangiogram catheters were removed, and he was discharged from the clinic.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 61-year-old man was admitted to our hospital because of abdominal discomfort since 3 days. He had been in good health without any previous medical history of note. About 23 years ago, he had undergone primary repair of bowel rupture. Physical examination did not show any abnormalities. Initial laboratory data showed elevated serum total bilirubin 1.48 mg/dL, serum aspartate aminotransferase 980 IU/L, serum alanine transaminase 274 IU/L, and serum C-reactive protein 10.8 mg/L. Serum amylase and lipase were within normal limits. Serum was negative for hepatitis B and C markers. Abdominal computed tomography (CT) scan showed multiple CBD stones with dilated bile duct and MPD. On esophagogastroduodenoscopy, the major papilla of Vater was not found in the second duodenal portion. It was finally located in the duodenal bulb (). Endoscopic retrograde cholangiopancreatography (ERCP) was successfully performed via cannulation of the ectopic opening in the duodenal bulb. Dilated CBD with multiple stones was directly visualized radiographically () by injecting contrast media via the orifice. The patient underwent dilatation of the papilla with a balloon catheter (CRE wire-guided balloon dilator 12 to 15 mm; Boston Scientific, Marlborough, MA, USA), and a basket was used to remove the stones (). He was discharged without complications after the procedure.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 63-year-old woman from rural Nepal presented with a history of right upper abdominal pain, epigastric discomfort, bloating, and dyspepsia for 4 years, followed by intermittent fever and jaundice for a month. She had an increase in severity of pain, yellowish discoloration of body, vomiting, and fever for 1 week but had no history of anorexia or weight loss. She had no significant past medical and surgical history. On general examination she had icterus. Her vital parameters were within the normal range. An examination of her abdomen revealed a non-distended soft abdomen with negative Murphy’s sign and her gall bladder was not palpable.\nBiochemical parameters showed hemoglobin 13.4 gm/dl, total leukocyte count 12,600/mm3, platelets 158,000/mm3, total bilirubin 7.9 mg/dl, direct bilirubin 5.4 mg/dl, alanine aminotransferase (ALT) 196 U/L, aspartate aminotransferase (AST) 146 U/L, alkaline phosphatase (ALP) 273 U/L, serum amylase 50 U/L, and lipase 100 U/L. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive (Fig. ).\nDue to radiological findings that were suspicious of double CBD (DCBD), our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cm2, and cystic duct opening into the right duct (Fig. ). So the diagnosis of type Va DCBD with cholelithiasis and choledocholithiasis was made. Following this, cholecystectomy was done, the common duct was explored and the stone was extracted. The right duct was divided just proximal to the opening of the cystic duct and the left duct was also divided at the same level. After closing distal common bile duct stump, reconstruction was done by hepatic ductoplasty (joining two ducts) with hepaticojejunostomy in Roux-en-Y fashion. Her postoperative period was uneventful and she was discharged after 1 week and was doing well at 6-month follow-up.\nHistologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 57-year-old man was referred to our hospital for further evaluation of pneumobilia. He was complaining of nausea for 1 month. He had no past medical history of note. On admission, physical examination was unremarkable. Initial laboratory findings were normal. On abdominal CT, diffuse pneumobilia was evident, and a communication was suspected between the duodenal bulb and the extrahepatic bile duct. For confirmation, ERCP was performed. ERCP showed an ectopic opening of the major papilla of Vater in the duodenal bulb (). We cannulated the ectopic opening and injected contrast media via the orifice. We found that the CBD and MPD were communicating with the duodenal bulb (). After 9 months, abdominal CT scan showed focal nodular wall thickening with enhancement of the gallbladder (). Consequently, the patient underwent radical cholecystectomy with hepaticojejunostomy as gallbladder cancer was suspected. Histopathological examination revealed a moderately differentiated adenocarcinoma arising from the gallbladder and extending into the perimuscular connective tissue (). After the operation, he was discharged without complications.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

An 81-year-old woman presented with a 2-day history of chills and fever. She had been admitted two years ago with jaundice and fever. On a CT scan, the distal CBD, a large gallbladder (GB) and cystic duct stones were revealed. Urgent endoscopic retrograde cholangiopancreatography (ERCP) to remove the distal CBD stone was performed. Multiple CBD stones were extracted using a basket and balloon sweep. After the stones were removed, there was no filling defect on the CBD during balloon cholangiography. The patient refused cholecystectomy. She was not followed up after discharge from the hospital. On the next presentation, the laboratory tests revealed a slightly elevated aspartate aminotransferase level (45 IU/L) and an increased value of C-reactive protein (15.3 mg/I). The total bilirubin level, the alkaline phosphatase level, the gamma-glutamyltransferase level and the WBC count were all within the normal ranges. The presumed diagnosis was recurrent CBD stones, and an ERCP for recurrent CBD stones was planned. Transabdominal ultrasonography demonstrated a dilated extrahepatic bile duct filled with echogenic lesions that showed posterior acoustic shadowing, which was suggestive of bile duct stones (). On the more medial view of the sagittal sonogram, another mildly dilated extrahepatic bile duct was seen, but no stone was noted (). The sonographic findings led us to presume that the stone-filled aberrant right hepatic duct drained into the common hepatic duct. A contrast-enhanced CT scan was performed using a 64-MDCT scanner (LightSpeed VCT, GE Healthcare, Milwaukee, WI) and it showed two separate extrahepatic bile ducts (). Multiple stones were noted in a posterolaterally located bile duct and these stones extended to right intrahepatic bile ducts. There was a large stone in the GB and the cystic duct joined to a posterolaterally-located extrahepatic bile duct. The MinIP images were obtained on a postprocessing workstation (Advantage Windows workstation [version 4.3], GE Healthcare, Milwaukee, WI). The MinIP image (coronal oblique with a 5.3 mm thickness slab) revealed double extrahepatic bile ducts that combined to create a CBD in the pancreatic head portion with a communicating channel in the hilar portion (). MRCP performed with a 1.5-T Intera scanner (Philips Medical Systems, The Netherlands) failed to depict the precise anatomy of this anomaly due to the impacted stones (). There was no AUPBD on the MRCP. ERC was subsequently performed to remove the multiple stones in one of the double extrahepatic bile ducts. An ERC image also disclosed duplication of the extrahepatic bile duct with multiple stones in the posterolaterally located duct and the right intrahepatic ducts (). However, we failed to gain access to the right CBD and to remove the CBD stones.\nTo remove the remnant stones, duct exploration with insertion of a T-tube and cholecystectomy was performed. On a cholangiogram obtained during the subsequent operation, there was no evidence of remnant stones in the bile ducts (). The patient's postoperative recovery was uneventful.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

A 54-year-old male presented to a primary care physician with a complaint of indigestion. He underwent gastrofiberscopy, which revealed a small circumferential ulceroinfiltrative ill-defined mucosal lesion at the anterior wall of the antrum. Biopsy specimens were taken from the ulceroinfiltrative lesion, and showed adenocarcinoma. He was diagnosed with gastric cancer and referred to our hospital for an operation. We didn't detect any abnormal findings physically or in the laboratory. Abdominal CT scan showed mild gastric mucosal thickening in the anterior wall of the gastric antrum. We decided to perform laparoscopic assisted distal gastrectomy. During the surgery, we incidentally detected bile leak from the tubular structure around the hepatoduodenal ligament, and therefore converted to open surgery (). We performed intraoperative cholangiogram by cannulizing into the tubular duct, and confirmed that the tubular duct was the accessory bile duct with an ectopic drainage into the stomach, which was connected with the proximal common bile duct and linked with the left intrahepatic duct (). In the specimen, the ACBD was independent of the cancer lesion (). The ACBD was resected afterward. The patient was discharged without complications, and is doing well until now.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 74-year-old male presented with jaundice for one month associated with generalized pruritus, epigastric pain, anorexia, and weight loss. On clinical examination, the patient was thin built and icteric with palpable gall bladder.\nLiver function test was consistent with obstructive jaundice with total bilirubin of 154 μmol/l, direct of 152 μmol/l, SGOT of 147 U/l, SGPT of 67 U/l, and ALP of 731 U/l.\nTransabdominal ultrasonography revealed hepatomegaly, moderately dilated IHBD and CBD, and distended gall bladder.\nContrast-enhanced CT of the abdomen with pancreas specific protocol revealed enhancing soft tissue density in the distal common bile duct with upstream dilatation of the CBD, CHD, and IHBD.\nWith the diagnosis of distal cholangiocarcinoma, the patient underwent Whipple's pancreaticoduodenectomy. After dividing the common hepatic duct, two openings were appreciated indicating that the division of common hepatic duct occurred at the confluence. However, on further evaluation, it was appreciated that the confluence was intact and it was the septum in the CHD that was giving the false impression. On detailed evaluation of the resected specimen, a septum extending from the hilum with cystic duct opening on the right side () to the distal end of the bile duct was appreciated, suggesting duplication of the common bile duct. However, the septum was not extending up to the papilla ().\nThis anatomical variation was not appreciated in the imaging preoperatively. A retrospective evaluation of the cross-sectional imaging revealed an incomplete septum extending from the hilum to the distal end of the common bile duct (Figures and ).

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 71-year-old female presented with right upper quadrat (RUQ) pain associated with vomiting. She has no significant past medical or surgical history. Laboratory results revealed a normal white cell count and liver function tests but a raised C-reactive protein.\nComputerized tomography demonstrated an enhancing soft tissue thickening in the gastric pylorus with soft tissue extension into the left lobe of the liver, invasion of the left intrahepatic bile ducts with obstruction and dilatation of the left sided intra- and extrahepatic biliary tract (). Oesophagogastroduodenoscopy (OGD) showed a pre-pyloric fistula ().\nFurther examination led to a diagnosis of DCBD with a separate left intra- and extrahepatic bile duct with ectopic drainage into the stomach. Magnetic resonance cholangiopancreatography (MRCP) revealed marked left intrahepatic duct (IHD) dilatation. The left IHDs were draining via an aberrant extra-hepatic bile duct into the pre-pyloric region. The left and right hepatic ducts did not communicate. It also demonstrated pancreatic divisum and multiple side branch intra-ductal papillary mucinous neoplasms (IPMN) (). Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the MRCP findings but also revealed an intraluminal-filling defect in the left IHD ( and ).\nShe underwent a left hemi-hepatectomy and a wedge excision of the antrum of the stomach. Intraoperative examination confirmed the radiological findings (–). There were an atrophic left liver lobe and a dilated ACBD draining segments 2–4. The left portal vein crossed anteriorly to the ACBD with the left hepatic artery (LHA) lying medial to it. An accessory LHA was also present within the hepato-gastric ligament. Postoperative recovery was uneventful. Histology showed intraductal papillary neoplasm of the bile duct (IPN-B) with low-grade dysplasia involving both the intra- and extrahepatic bile ducts.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 64-year-old female presented to the surgical emergency unit with a day's history of right upper quadrant pain. Her physical examination revealed right upper quadrant tenderness with no rebound tenderness or muscular guarding. Other systemic findings were normal. Laboratory test showed leukocytosis of 17 × 10³/µL and elevated C-reactive protein of 51 mg/L (0–5 ml/L). Other laboratory values including serum amylase, bilirubin and transaminases were normal. Abdominal ultrasound revealed thickened gallbladder (GB) wall (4.7 mm) with a 25 mm gallstone. A diagnosis of acute calculous cholecystitis was made and intravenous fluid and antibiotic treatment were initiated. Laparoscopic cholecystectomy was considered after 48 h of observation. During surgery a standard 4-port technique was used after pneumoperitoneum using the Veress needle. GB wall was found to be inflamed with omental adhesions. Using the critical view of safety approach, the cystic artery and cystic duct were separately identified, clipped and transected. After careful dissection of the GB from the liver bed small amount of bile was observed oozing from a spot proximal to the ligated cystic duct stump. Irrigation of the spot revealed a small tear at what was thought to be the common hepatic duct. A 5-Fr drainage catheter (50 cm long, 1.4 mm in diameter) was placed into the opened duct and exteriorized through the mid-clavicular 5 mm port site for post-operative drainage. A tube cholangiography done on post-operative Day 7 showed duplication of the common hepatic duct proximal to the cystic duct stump with the injury occurring on the right-sided duct (Fig. ). A magnetic resonance cholangiopancreatography (MRCP) was taken which confirmed the presence of the duplicated common hepatic duct with the cystic duct stump distal to the duplication (Fig. ). No PBM was observed. Her post-operative course was uneventful with an average biliary drainage output of about 300 ml. Drainage tube was clamped on post-operative Day 9 and finally removed 4 weeks after surgery. No complication was observed and patient is currently doing well.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 60-year-old Middle Eastern male patient with a past medical history of benign prostatic hyperplasia, hypertension, gastroesophageal reflux disease and type 2 diabetes mellitus presented to the emergency department complaining of crampy lower abdominal pain associated with an unintentional 40 pounds weight loss over 4 months. A magnetic resonance imaging (MRI) of the abdomen with intravenous contrast demonstrated a heterogeneous solid pancreatic uncinate mass (3.9 × 3.2 cm) with a duplicated common bile duct ().\nA subsequent endoscopic ultrasound (EUS) confirmed a large heterogeneous hypoechoic mass with irregular borders and cystic areas in the uncinate process of the pancreas. The distal CBD appeared mildly dilated with two extrahepatic bile ducts consistent with type II accessory common bile duct (ACBD). The accessory bile duct provided a conduit for the deposition of bile in the pancreatic head where it terminated. Subsequent inflammation progressed to metaplastic changes confirmed by a fine needle aspiration (FNA) via trangastric approach. The atypical cells of metaplasia found in the pancreatic head were confirmed to be pancreatic adenocarcinoma. Magnetic resonance cholangiopancreatography (MRCP) confirmed the above EUS findings along with multiple enhancing foci also present in the liver. A triple phase computerized tomography (CT) also re-demonstrated an ACBD () with multiple hypervascular hepatic lesions and a soft tissue mass in the pancreatic uncinate process () encasing the superior mesenteric artery (SMA). The patient was eventually discharged for further staging and subsequent chemotherapy at a different institution.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

A 35-year-old female presented to a local hospital with a 3-month history of intermittent abdominal pain. Initial abdominal ultrasonography revealed cystic dilatation of the extrahepatic bile duct. A suspicion of choledochal cyst was made and was referred for surgical management. Her past medical history was unremarkable. She weighed 83 kg and was 164 cm tall, with body mass index of 30.9 kg/m2. Apart from mild right upper quadrant tenderness, her physical examination was unremarkable. Laboratory tests including liver and renal function tests, serum and urine amylase levels were normal. A magnetic resonance cholangio-pancreatography (MRCP) done showed a cystic fusiform dilatation of the common bile duct (CBD) extending into the right and left hepatic ducts (). No PBM was observed. The MRCP findings suggested type Ic CC. Without any further imaging, patient was scheduled for complete cyst excision with cholecystectomy and hepaticoenterostomy surgery. At laparotomy, the CBD was found to be fusiformly dilated. Bile samples were taken from the cyst and the gallbladder (GB) for amylase level essays. The GB was dissected from the liver bed and the cystic duct opened into the midportion of the cyst. After careful dissected of the cyst from surrounding vascular structures, the distal part entering the head of pancreas was noticed to be unaffected (). The distal part was transected just at the level of the head of pancreas and samples were taken for frozen section. Dissection at the hepatic hilum revealed two separate dilated ducts originating from the confluence of the left and right hepatic ducts and both entering into the cyst (). The two ducts were transected just distal to their origin from hepatic confluence to avoid confusing them as true right and left hepatic ducts (). The hepatic confluence was then opened to expose the true left and right hepatic ducts (). The dilated portions of the both hepatic ducts were transected and samples were taken for frozen section. A right and left hepaticojejunostomy reconstruction was done. No intraoperative cholangiogram was performed. Frozen section results of both the distal and proximal surgical margins were negative for malignancy. The amylase levels of both the cyst and GB were 47 031 and 44 770 U/L, respectively (normal serum amylase level: 25–110 U/L). Patient was discharged on postoperative Day 6. She is well with no evidence of malignancy after 23 months of follow-up.

A 77-year-old woman presented to the outpatient department of our hospital complaining of recurrent epigastric pain.\nPatient’s symptoms started a month ago with recurrent episodes, with no history of fever, chills, or vomiting.\nThe patient had a history of laparoscopic cholecystectomy for cholelithiasis 2 years earlier at another hospital. She had undergone endoscopic retrograde cholan-giopancreatography (ERCP) twice for choledocholithiasis before undergoing cholecystectomy.\nThe patient did not have a history of drinking or smoking. There was no remarkable family medical history.\nPhysical examination did not reveal tenderness or signs of peritoneal irrigation, and the bowel sounds were normal. At the outpatient clinic, her temperature was 36.7 °C, pulse rate was 70 beats/min, and blood pressure was 122/82 mm Hg.\nBlood analysis showed normal complete blood cell count, liver function test and C-reactive protein.\nComputed tomography (CT) imaging revealed a dilated CBD that harboured multiple choledocholithiasis (Figure ). After admission, ERCP was subsequently performed, and the stones were extracted using a Dormia basket (MTW Endoskopie, Wesel, Germany) (Figure ) after endoscopic papillary balloon dilatation by a CRETM balloon dilatation catheters (Boston Scientific Corporation, Natick, MA, United States). A 5 Fr endoscopic nasobiliary drainage (ENBD) catheter (ENBD-5, Cook Medical, Bloomington, IN, United States) was inserted at the end of the procedure. A day after the ERCP, tubography via the ENBD catheter was performed to confirm the presence of residual stones in the bile duct. However, no definite filling defects were observed on tubography (Figure ). The patient was discharged without any complications of ERCP. A day after discharge, she visited the emergency department with complaints of severe epigastric pain and fever. Her temperature was 38.2 °C, pulse rate was 79 beats/min, and blood pressure was 149/85 mm Hg. Laboratory findings were suggestive of cholestasis [aspartate aminotransferase, 210 U/L (normal: 0–35 U/L); alanine aminotransferase, 134 U/L (0-35); alkaline phosphatase, 201 U/L (30-120); gamma glutamyl transferase, 184 U/L (9-64); total bilirubin, 2.77 mg/dL (0.3-1.2); direct bilirubin, 1.32 mg/dL (0-0.2); and C-reactive protein 6.91 mg/dL]. A CT scan demonstrated another dilated extrahepatic bile duct draining the right lobe of the liver, which also contained stones in the distal portion (Figure ). An urgent ERCP was performed as the stones were impacted at the ampulla (Figure ), and the stones with pus were retrieved using a balloon catheter (Escort II® Double Lumen Extraction Balloon, Cook Medical, Bloomington, IN, United States). A 10 Fr, 5 cm endoscopic retrograde biliary drainage tube (Cotton-Leung Biliary Stent, Cook Medical, Bloomington, IN, United States) was then placed in the CBD.\nMagnetic resonance cholangiopancreatography (MRCP) was performed to evaluate the delicate structures of the CBD and to check for any remnant stones. MRCP revealed DCBD independently draining the left and right lobes of the liver, that created a short segment intrapancreatic CBD (Figure ) without a communicating channel, that was consistent with the DCBD Type Va as classified by Choi et al[,]. Residual choledocholithiasis was also noted in the right CBD. There was no evidence of anomalous union of the pancreaticobiliary duct on MRCP.

Write a detailed clinical case vignette based on the following key phrases: Bile Duct Anomalies, Choledocholithiasis, ERCP

Patient 3, a 10-year-old boy, was born by normal delivery. His parents did not show cutaneous anomalies. He was admitted for consultation due to episodes of generalized tonic-clonic seizures lasting several minutes: seizures had started at age 2 years. He showed neurodevelopmental delay from his first months of life: his current school performance is poor. An electroencephalographic (EEG) examination revealed spike and wave discharges in the temporal and parietal regions. Treatment with sodium valproate substantially reduced the frequency of seizures. At physical examination, he weighted 34 kg, with a height of 141 cm (75th percentile). The hypopigmented lesions were localized in the thorax and in the back, mainly on the left side. The diameter of the sural region of the lower left limb was 8 cm larger than the contralateral, and the diameter of the biceps region of the left limb was 3 cm larger than the contralateral. The left lower limb was 2 cm longer than the right. The asymmetry involved also the left hemiface. A lower-limb MRI showed increased growth in both long bones and soft tissues. Mild thoracolumbar right kyphoscoliosis was noticed. Brain MRI and ophthalmologic examination were both normal.

Patient 8, a 25-year-old male, had no family history of cutaneous anomalies. His cutaneous anomalies were localized over the trunk and the limbs, particularly on the right (Figure ). The stature was 181 cm (75th percentile). The right upper limb diameter was 2 cm larger as compared with the left; the right lower limb diameter was 4 cm larger. Lower limb length discrepancy was 5 cm (the right side was longer). There was mild involvement of the right hemiface. Left kyphoscoliosis was present (20 degree of Cobb). The man attended school with good scores. No seizures had been recorded.

Write a detailed clinical case vignette based on the following key phrases: Hypopigmented lesions, Body asymmetry, Developmental delays

A 2-month-old girl was referred to the Department of Dermatology for the evaluation of extending hypopigmented skin lesions. At birth, she had multiple linear erythematous erosive skin defects, which became linear, hypopigmented patches with irregular border on the face, scalp, trunk, and both extremities (). We received the patient's consent form about publishing all photographic materials. The patient had a history of intrauterine growth retardation as a prenatal problem. There was no specific family history, and her two siblings were normal.\nThe patient also presented multiple congenital anomalies. Physical examination revealed patchy alopecia of the scalp, hypoplastic nails, and irregular gingiva with conical teeth. Ocular examination showed bilateral aniridia, moderate to severe microphthalmia, and unilateral corneal central opacity of the left eye (). The retinal vessels of both eyes were uncertain on fundus examination. Musculoskeletal examination revealed syndactyly of toes (), hypoplasia and clinodactyly of fingers, and coccyx deviation. Chromosomal analysis on peripheral blood lymphocytes showed normal karyotype (46, XX) (), and no mutation was found in IKBKG gene test. Therefore, the patient was diagnosed with HI with multiple congenital anomalies.\nTo manage the associated musculoskeletal anomalies, the patient was referred to the Department of Pediatric Orthopedic Surgery and underwent surgical treatment of toe syndactyly. For proper management of the other anomalies, she had been undergoing pediatric, ophthalmologic, and dermatological regular check-up.

Patient 8, a 25-year-old male, had no family history of cutaneous anomalies. His cutaneous anomalies were localized over the trunk and the limbs, particularly on the right (Figure ). The stature was 181 cm (75th percentile). The right upper limb diameter was 2 cm larger as compared with the left; the right lower limb diameter was 4 cm larger. Lower limb length discrepancy was 5 cm (the right side was longer). There was mild involvement of the right hemiface. Left kyphoscoliosis was present (20 degree of Cobb). The man attended school with good scores. No seizures had been recorded.

Write a detailed clinical case vignette based on the following key phrases: Hypopigmented lesions, Body asymmetry, Developmental delays

Patient 4, a 15-year-old girl, was the only daughter of nonconsanguineous parents. Both her parents were healthy with no cutaneous anomalies. The girl was born at 40 weeks by normal delivery, with a birthweight of 3300 g, a height of 50 cm, and a head circumference of 35 cm. She first came to our observation at the age of 2 years, due to psychomotor delay. The parents noticed the presence of cutaneous spots at about 3 months of age. Since her first year of life, the girl showed mild body asymmetry. At physical examination, at age 2 years, the girl showed typical hypopigmented lesions along the lines of Blaschko, mainly in the trunk and the upper and lower limbs (particularly evident over the right part of the body). During these years, the girl manifested moderate cognitive delay, poor school performance, and generalized tonic-clonic seizures, with a frequency of 6 to 8 episodes per year despite valproate treatment. At the age of 6 years, clobazam was added to the valproate regimen and the frequency of seizures decreased. An EEG revealed generalized spike and wave discharges. Currently, her physical examination reveals a weight of 70 kg and a height of 174 cm (both falling in the 90th percentile). Her school performance remained poor. The diameter of the biceps region of the right limb was 3 cm larger than the contralateral. Her right thigh diameter was 66 cm and her left thigh diameter was 57 cm (Figure ): the sural right lower limb diameter was 43 cm, compared with the left, which measured 40 cm. The right lower limb length was 103 cm, whereas the left was 100 cm. Facial asymmetry was also present, but to a milder degree. She currently walks with unstable equilibrium with frequent falls down. A color Doppler ultrasound examination of her limbs showed normal laminar flow, with a wider diameter of the arteries in the affected right limb (Figure A and B). Brain MRI was normal as her ophthalmological examination. Spinal x-ray examination revealed mild left kyphoscoliosis. Skin biopsy was not performed.

Patient 8, a 25-year-old male, had no family history of cutaneous anomalies. His cutaneous anomalies were localized over the trunk and the limbs, particularly on the right (Figure ). The stature was 181 cm (75th percentile). The right upper limb diameter was 2 cm larger as compared with the left; the right lower limb diameter was 4 cm larger. Lower limb length discrepancy was 5 cm (the right side was longer). There was mild involvement of the right hemiface. Left kyphoscoliosis was present (20 degree of Cobb). The man attended school with good scores. No seizures had been recorded.

Write a detailed clinical case vignette based on the following key phrases: Hypopigmented lesions, Body asymmetry, Developmental delays

Patient 1 was a 4-year-old male who first came to our observation at the age of 3 years, due to episodes of febrile seizures. The parents and the youngest sister were healthy and did not show cutaneous anomalies. He was born by Caesarean section with a birth weight of 3100 g. Psychomotor development was regular. At his first physical examination, he weighted 17 kg, with a height of 101 cm and a head circumference of 51 cm (all within the 50th percentile). The hypopigmented lesions, in the form of whorls, were localized on the upper side of the right trunk and showed a linear pattern in the right lower limb. Limbs were asymmetrical: the asymmetry involved the right lower limb, which was larger than the left of 1 cm in diameter, and of 0.5 cm in the right upper limb compared with its counterpart, the right lower limb was 1 cm longer than the left. No facial asymmetry was present. Apart from the hemi-body asymmetry, the patient did not show other anomalies. Neither cognitive delay nor spine anomalies were present. The right femoral bone age was 1 year older than the left side. No skin biopsy to check for genetic mosaicism was carried out.

Patient 8, a 25-year-old male, had no family history of cutaneous anomalies. His cutaneous anomalies were localized over the trunk and the limbs, particularly on the right (Figure ). The stature was 181 cm (75th percentile). The right upper limb diameter was 2 cm larger as compared with the left; the right lower limb diameter was 4 cm larger. Lower limb length discrepancy was 5 cm (the right side was longer). There was mild involvement of the right hemiface. Left kyphoscoliosis was present (20 degree of Cobb). The man attended school with good scores. No seizures had been recorded.

Write a detailed clinical case vignette based on the following key phrases: Hypopigmented lesions, Body asymmetry, Developmental delays

A 5.5-year-old girl was brought to our hospital with a complaint of disproportionate overgrowth and lengthening of the right leg accompanied by marked hypopigmented lesions over the trunk and legs, which reportedly had become noticeable by 2-weeks postpartum and gradually increased and spread. She was born to non-consanguineous parents and had been under monitoring for delayed speech. On examination, her height and weight were 110 cm (10th to 25th percentile) and 23 kg (75th to 90th percentile), respectively. The dermatological examination was remarkable for hypopigmented patches following the Blaschko lines over the trunk and both legs, more prominently on the right leg (, ), which was thicker and longer (~2 cm) than the left (). Apart from these findings, the systemic examinations, routine haemogram and biochemical tests, and magnetic resonance scans of the cranium and lower extremities were normal. When the Denver Developmental Screening Test was administered for speech delay, it was graded as ‘delayed’, correlating with the age of 3.5 years. After consultation with the dermatology department, a skin biopsy was considered unnecessary.

Patient 8, a 25-year-old male, had no family history of cutaneous anomalies. His cutaneous anomalies were localized over the trunk and the limbs, particularly on the right (Figure ). The stature was 181 cm (75th percentile). The right upper limb diameter was 2 cm larger as compared with the left; the right lower limb diameter was 4 cm larger. Lower limb length discrepancy was 5 cm (the right side was longer). There was mild involvement of the right hemiface. Left kyphoscoliosis was present (20 degree of Cobb). The man attended school with good scores. No seizures had been recorded.

Write a detailed clinical case vignette based on the following key phrases: Hypopigmented lesions, Body asymmetry, Developmental delays

Patient 5 was a 18-year-old female who first came to our observation at the age of 24 months due to language delay. Her psychomotor development was also delayed. Convergent strabismus was present, with no anomalies at fundoscopy. Mild dysmorphic features were present, including epicanthic folds and hypotelorism. Physical examination revealed that her height was 175 cm (in the 90th percentile). The hypopigmented lesions were widely diffused in the trunk, particularly on the right side. The right upper limb diameter was 4 cm larger than the left side, and the right lower limb diameter was 7 cm larger and 3 cm longer than the left. Also, the right hemiface was involved by the disturbed growth. EEG and brain MRI were unrevealing. Left kyphoscoliosis was recorded with 10 to 15 degree of Cobb scale.

Patient 8, a 25-year-old male, had no family history of cutaneous anomalies. His cutaneous anomalies were localized over the trunk and the limbs, particularly on the right (Figure ). The stature was 181 cm (75th percentile). The right upper limb diameter was 2 cm larger as compared with the left; the right lower limb diameter was 4 cm larger. Lower limb length discrepancy was 5 cm (the right side was longer). There was mild involvement of the right hemiface. Left kyphoscoliosis was present (20 degree of Cobb). The man attended school with good scores. No seizures had been recorded.

Write a detailed clinical case vignette based on the following key phrases: Hypopigmented lesions, Body asymmetry, Developmental delays

Patient 7, a 22-year-old male, had no family history of cutaneous lesions. At physical examination, his height was 182 cm (75th percentile). The hypopigmented lesions were widely diffused over the trunk and with greater prevalence on the right side (Figure ). Left upper limb diameter was 2.5 cm larger than the right, whereas left lower limb diameter was 6 cm larger. The difference in the lower limb length was 4 cm (left > right). Right hemiface was importantly involved. Cognitive delay was present. An EEG revealed generalized spike and wave discharges, and the patient experiences epileptic seizures of the generalized tonic-clonic type. Fundus examination was normal. Convergent strabismus was noticed. Short and broad nose, macrocrania, and left kyphoscoliosis (20 degree of Cobb) were also recorded. Brain MRI was normal.

Patient 8, a 25-year-old male, had no family history of cutaneous anomalies. His cutaneous anomalies were localized over the trunk and the limbs, particularly on the right (Figure ). The stature was 181 cm (75th percentile). The right upper limb diameter was 2 cm larger as compared with the left; the right lower limb diameter was 4 cm larger. Lower limb length discrepancy was 5 cm (the right side was longer). There was mild involvement of the right hemiface. Left kyphoscoliosis was present (20 degree of Cobb). The man attended school with good scores. No seizures had been recorded.

Write a detailed clinical case vignette based on the following key phrases: Hypopigmented lesions, Body asymmetry, Developmental delays

Patient 8, a 25-year-old male, had no family history of cutaneous anomalies. His cutaneous anomalies were localized over the trunk and the limbs, particularly on the right (Figure ). The stature was 181 cm (75th percentile). The right upper limb diameter was 2 cm larger as compared with the left; the right lower limb diameter was 4 cm larger. Lower limb length discrepancy was 5 cm (the right side was longer). There was mild involvement of the right hemiface. Left kyphoscoliosis was present (20 degree of Cobb). The man attended school with good scores. No seizures had been recorded.

Patient 6, a 20-year-old male with no noticeable familial cutaneous anomalies, was first referred for a diagnostic work-up regarding his cutaneous manifestations, which were diffuse, as patches, and primarily found on his left side. He had been first referred at age 3 years due to his severe psychomotor delay and for generalized tonic-clonic seizures. The EEG, at that age, revealed spike and wave discharges, prevalent in the frontal regions. Moderate cognitive delay was present. At his most recent physical examination, his stature was 175 cm (50th percentile): his left upper limb was 3 cm larger than the right, and the left lower limb 6 cm larger than the right. The left lower limb was 2.5 cm longer than the right. The ears were bilaterally large and cupped with an enlargement of the left hemiface. Mild right kyphoscoliosis was recorded. Brain MRI was normal. Skin biopsy was not performed.

Write a detailed clinical case vignette based on the following key phrases: Hypopigmented lesions, Body asymmetry, Developmental delays

Patient 2, a 6-year-old girl, was the first born to healthy parents (both parents did not have cutaneous anomalies). The asymmetry was first noticed at birth. Psychomotor development was within normal ranges. At physical examination, she weighted 24 kg (90th percentile), with a height of 115 cm (50th percentile) and a head circumference of 49 cm (50th percentile). The hypopigmented patterns, in the form of whorls and V-shaped arrangements, were more evident in the trunk and in the back, with a linear configuration in the right lower limb. The diameter of the right lower limb was 1 cm larger than the lower left limb; the difference in the upper limb was less pronounced (0.4 cm). The right lower limb was 0.8 cm longer than the left. No hemiface involvement, cognitive delay, epilepsy, or spine anomalies had been recorded. The right femoral bone age was advanced of 1 year compared with the left.

Patient 8, a 25-year-old male, had no family history of cutaneous anomalies. His cutaneous anomalies were localized over the trunk and the limbs, particularly on the right (Figure ). The stature was 181 cm (75th percentile). The right upper limb diameter was 2 cm larger as compared with the left; the right lower limb diameter was 4 cm larger. Lower limb length discrepancy was 5 cm (the right side was longer). There was mild involvement of the right hemiface. Left kyphoscoliosis was present (20 degree of Cobb). The man attended school with good scores. No seizures had been recorded.

Write a detailed clinical case vignette based on the following key phrases: Hypopigmented lesions, Body asymmetry, Developmental delays