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pmc-6151227-1 | A 40-year-old Caucasian man was admitted to our ward. He reported being a 6 pack/year smoker, occasional drinker, and had an otherwise unremarkable medical history. He presented with two accidentally self-inflicted wounds on the left arm caused by the mishandling of two work tools a week prior. One episode occurred while disposing of a metallic tool used during his morning work as a plumber and the second one in the afternoon with a metal needle used in the breeding of carps that he practices as a hobby. The day before admission, the patient presented at the Emergency Department (ED) of a hospital near his residence with redness and swelling in the left upper arm and fever. He was prescribed a seven-day course of amoxicillin/clavulanate 875 + 125 mg PO TID and discharged. He came back to the same ED the next day for excruciating pain in the entire arm, persistent fever, and extension of edema. A soft tissue ultrasound scan showed lymphedema of the left arm without signs of thrombosis. He was then referred to the Emergency Department of our hospital for further diagnostic investigations on 15/06/2017 which included an Infectious Disease consult who suspected necrotizing fasciitis (NF). A contrast-enhanced CT showed thrombosis and subcutaneous emphysema (). The patient was then started on a broad-spectrum empirical antibiotic therapy with metronidazole 500 mg IV QID, piperacillin/tazobactam 4.5 g IV TID, and doxycycline 100 mg PO BID. An orthopedic surgeon was consulted, and fasciotomy followed by surgical debridement was immediately performed. Blood cultures were obtained before the start of antibiotic therapy using two sets of bottles for aerobes and anaerobes, incubated in the automated system BactedTM FX (BD, Franklin Lakes, NJ, USA). Surgical samples from muscular bands and cutaneous flaps obtained during the fasciotomy were collected using an eSwabTM (COPAN Diagnostics, Murrieta, CA, USA) kit and cultured on different types of agar (blood, chocolate, McConkey, Mannitol, and Schädler). A GENbag anaer® (bioMérieux, Marcy l'Etoile, France) kit was also used to detect anaerobic bacteria. Antimicrobial susceptibility testing was carried out on Mueller–Hinton medium supplemented with 5% Fildes blood digest. The patient was then transferred to the Infectious Disease Department, and antibiotic therapy was restricted to piperacillin/tazobactam plus vancomycin, in accordance with the current IDSA guidelines []. A vacuum-assisted closure (VAC) therapy cycle was also started on 23/06/2017.
The cultures of eSwab samples and surgical samples became positive on 25/06/2017 and 26/06/2017. Peptostreptococcus anaerobius and Atopobium parvulum, two anaerobic pansensitive bacteria, were isolated and identified by matrix-assisted laser desorption ionization-time of flight (MALDI-TOF), using the Bruker database as reference for the microorganism identification process (Bruker Daltonics GmbH, Bremen, Germany). The isolates returned susceptible to piperacillin/tazobactam, and the patient continued his treatment (). Despite the use of appropriate antibiotic therapy and the use of advanced medications and VAC therapy, a large area of coagulation and necrosis remained in the affected area (). A new debridement was performed on 29/06/2017, and the patient continued the VAC therapy. Subsequently, granulation tissue began to form. Antibiotic therapy was changed to oral amoxicillin/clavulanate 1 g TID PO on 07/05/2017. The patient was discharged on 19/07/2017. He then underwent plastic surgery for autologous skin grafting, and after three months, the lesion had almost disappeared (). summarizes the events detailed in this case presentation. |
pmc-6151234-1 | A 79-year-old male with a medical history of cholelithiasis, for which he underwent cholecystectomy 11 years ago, hyperlipidemia, essential hypertension, and paroxysmal atrial fibrillation was admitted for mild acute pancreatitis. His presentation included severe epigastric pain radiating to the back, lipase of 1840 u/L, and no organ failure. He denied fever, chills, or body aches. The patient denied alcohol consumption and reported to have quit smoking 41 years ago. Liver chemistries, serum bilirubin, serum triglycerides, and immunoglobulin subclasses were within normal limits (). Abdominal CT scan demonstrated peripancreatic haziness consistent with noncomplicated acute pancreatitis (). Home medications included atorvastatin for several years and the addition of 160 mg fenofibrate six months prior to admission. These medications were held upon admission but resumed at discharge. Full medication lists on admission and at discharge are shown in (). The patient was managed with fluid resuscitation and pain control. The patient recovered well and was able to tolerate regular diet without any pain or nausea. The patient was discharged in a stable condition after three hospital admission days, and lipase level at the day of discharge was 307 u/l.
Three days later, the patient returned with similar symptoms. Lipase levels were >30,000 u/l (), and magnetic resonance cholangiopancreatography showed acute interstitial edematous pancreatitis (). The patient was treated conservatively with intravenous fluid resuscitation, pain control, and nothing per mouth until his symptoms resolved. Four days after his second admission, the patient recovered well and was discharged home in a stable condition. Fenofibrate and atorvastatin were discontinued ().
Two months later, the patient remained asymptomatic and returned for further workup to rule out an alternative cause to fenofibrate-induced pancreatitis. Endoscopic ultrasound (EUS) was done, which showed a 12 mm x 20 mm pancreatic head mass without pancreatic duct stenosis, strictures, or dilation. There were no common bile duct abnormalities. Fine needle aspiration was performed, and cytology was negative for malignant cells. Endoscopic retrograde cholangiopancreatography (ERCP) was performed to place a prophylactic pancreatic duct stent. Cholangiogram revealed a normal biliary system without dilation or strictures. The biliary tree was swept, and nothing was found. A prophylactic pancreatic duct stent was placed and a prophylactic sphincterotomy was performed. No recurrence of pancreatitis has occurred as of 6-month follow-up.
Given the course of the patient's illnesses in relation to fenofibrate usage, the timespan of drug initiation, and the fact that he had been taking atorvastatin for many years without previous signs or symptoms of acute pancreatitis, we hypothesize that his recurrent disease is probably due to the use of fenofibrate. |
pmc-6151238-1 | A male patient, aged 39 years, presented to a private clinic with a chief complaint of difficulty in mouth opening since one and half years. The patient had a habit of chewing gutka for the past eight years. It was observed that there is noticeable decline in mouth opening of 17 mm (intercanine distance) and tongue protrusion of 10 mm. On intraoral examination, generalized blanching of the oral mucosa with grayish black pigmentation was seen. And also, multiple vertical palpable fibrous bands with loss of elasticity and leathery in texture were noticed. OSMF was diagnosed, and the patient was treated with conventional intralesional steroid injections. Since the patient has been under similar treatment for over a period of time with no recognisable change or relief, he requested for an alternative therapy. Intraoral appliance therapy was considered, and prior consent was obtained from the patient. The patient was duly provided with necessary precautions regarding the usage of appliance and weekly follow-up without discontinuing the treatment. The treatment was carried out for a total period of 8 weeks and a follow-up of two months after completion of the therapy (). |
pmc-6151238-2 | A female patient, aged 56 years, presented to the private clinic with chief complaint of difficulty in mouth opening since one month. During her first visit, i.e., approximately a year back, she reported about the treatment that she received for trismus (due to OSMF) using intralesional injections. At that time, the patient had marginal relief from the symptoms. Again, she started developing trismus since one month and also had burning sensation in the oral cavity. Patient had restricted mouth opening of 30 mm (canine-canine distance) and tongue protrusion of 12 mm with all signs of OSMF (group 2 by Khanna et al.) in the oral cavity. As she was not able to tolerate any more pain from intralesional steroid injections, she was advised intraoral appliance therapy for 8 weeks. She was also instructed for weekly follow-ups with precautions during appliance position in the oral cavity. |
pmc-6151238-3 | A male patient, aged 40 years, with a history of chewing betel quid for the past 15 years, presented to the private clinic with reduction in mouth opening since one year. Patient had a restricted mouth opening of 35 mm (canine-canine distance) and tongue protrusion of 12 mm with all signs of OSMF (group 2 by Khanna et al.) in the oral cavity. The patient was then started with intralesional corticosteroids, which showed improved mouth opening till 42 mm (canine-canine distance), and then this treatment modality was discontinued due to pain arising from repeated punctures. The patient then requested for alternative therapy. He was advised appliance therapy and was instructed for weekly follow-ups for 8 weeks with precautions in positioning and usage of the appliance in the oral cavity. |
pmc-6151239-1 | The patient is a 43-year-old female who presented with sharp, substernal, nonexertional chest pain, and shortness of breath. Her past medical history included secondary APS (lupus anticoagulant positive) in the setting of SLE, with multiple, recurrent deep venous thromboses of the lower extremities. She was diagnosed with myopericarditis during a hospitalization one year prior when she presented with similar symptoms and an elevated troponin, after which a coronary catheterization demonstrated no significant coronary artery disease. Examination revealed a 2/6 systolic ejection murmur, without any radiation, gallops, rubs, or jugular venous distension. Auscultation of the lungs revealed clear breath sounds.
An echocardiogram was ordered when she was found to have an elevated troponin level of 0.209 ng/ml without any evidence of acute ischemic pathology on her electrocardiogram. Subsequently, a 2 cm mass was seen on the aortic valve with evidence of obstructive pathology. A follow-up transesophageal echocardiogram redemonstrated this mass, along with a smaller mass on the mitral valve associated with mild mitral regurgitation (). The aortic mass was consistent with fresh mobile thrombus, somewhat atypical for Libman–Sacks vegetations which usually have a more verrucous appearance. However, after blood cultures and an extensive workup for culture-negative endocarditis were negative, she was ultimately diagnosed with LSE and a recurrent flare of myopericarditis.
The patient's chest pain improved with colchicine, but her shortness of breath remained and was presumed to be from partial obstruction of the aortic valve by the large mass. After consultation with cardiothoracic surgery, medical therapy was initiated with a goal to avoid surgery if there were signs of improvement. She was already taking rivaroxaban when she originally presented, given that she had failed warfarin therapy in the past with persistently subtherapeutic INRs and recurrent DVTs, so she was started on therapeutic enoxaparin and aspirin. After a rheumatology consultation, hydroxychloroquine and prednisone were initiated as well. The patient remained stable with no new symptoms or signs of embolic events during her follow-up visits, and repeat transesophageal echocardiograms at 12 and 24 weeks demonstrated improvement in the size of both vegetations (). Her prednisone was gradually tapered down over a period of 9 months. |
pmc-6151243-1 | A 68-year-old male with a past medical history of schizophrenia and polysubstance abuse presented to the emergency department with a chief complaint of neck pain following minor trauma after sustaining a mechanical fall complicated by a television set then falling on him, striking him on the back of his neck. He denied loss of consciousness, paresthesias, or focal weakness but reported an increasing number of falls for the past two years. He endorsed daily intravenous heroin and methamphetamine use with social alcohol use and having previously quit smoking twenty years ago.
Vital signs were temperature 37.3°C, blood pressure 141/108 mmHg, heart rate 105 beats per minute, respiratory rate 24 breaths per minute, and oxygen saturation 97% on room air. Physical examination revealed a thin, cachectic African American male. He was noted to be without obvious signs of trauma to the head or neck. Cardiac examination revealed sinus tachycardia with no obvious murmurs, rubs, or gallops. Pulmonary examination was clear to auscultation in all lung fields. Abdominal examination was soft without evidence of distention or tenderness to palpation in all four quadrants. Skin examination showed no evidence of rashes, erythema, or trauma. Neurological examination was significant for cervical spinal tenderness to palpation but without evidence of step-off or gross deformity. Strength was noted to be diffusely diminished (4/5 throughout bilateral upper and lower extremities) without hyperreflexia or pathologic reflexes (negative Babinski and Hoffman tests). Anal wink was present. Sensation was preserved throughout. Laboratory results demonstrated white blood cell count 5540/mm3 with normal differential, serum creatinine 1.6 mg/dL (baseline 0.6 mg/dL), C-reactive protein (CRP) 25.2 mg/L, and erythrocyte sedimentation rate (ESR) 48 mm/hr, and urine drug screen was positive for amphetamines and opiates. HIV testing was negative. Hepatitis C testing revealed a positive antibody test but a negative viral load, suggestive of cleared prior infection. He denied prior treatment of Hepatitis C.
Computed tomography (CT) and magnetic resonance imaging (MRI) of the cervical spine revealed lytic destructive changes involving the C5 and C6 vertebral bodies, concerning for osteomyelitis. Due to clinical stability, antibiotics were held and he was admitted to the hospital for planned CT-guided aspiration. Five sets of blood cultures drawn over two consecutive days while off antibiotics were negative. Transthoracic echocardiography revealed a 3 × 4 mm echodensity attached to the noncoronary cusp of the aortic valve that was not independently mobile and without associated aortic regurgitation. In addition, there was a 3 × 5 mm echodensity attached to the anterior leaflet of the mitral valve that was not independently mobile and without associated mitral regurgitation.
During his hospitalization, the patient remained afebrile and hemodynamically stable but began to report tingling in his right thumb and index finger and decreased dexterity in terms of handwriting and grip strength, resulting in dropping objects. Due to new neurologic symptoms, the patient went to the operating room on hospital day #3 and underwent C5-C6 corpectomy, C4-C7 anterior fusion with allograft, and C3-T1 posterior fusion with allograft. Intraoperative findings demonstrated soft bone from the inferior portion of C4 to the superior portion of C7 with granulation tissue noted at the posterior longitudinal ligaments. There was no obvious evidence of tissue necrosis or purulent material. Postoperative vancomycin and piperacillin/tazobactam were started. Deep operative cultures subsequently grew Burkholderia cepacia complex, identified by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). Susceptibilities determined by the Kirby–Bauer method revealed susceptibility to ceftazidime, trimethoprim/sulfamethoxazole, and minocycline, with intermediate resistance to levofloxacin.
His antibiotics were narrowed to intravenous ceftazidime 2 g every 8 hours. He was evaluated by the Improving Addiction Care Team (IMPACT) and started on medication-assisted treatment for his opioid use disorder with Suboxone while being an inpatient. He was discharged to a skilled nursing facility where he completed 6 weeks of ceftazidime. At the end of his intravenous antibiotic therapy, his CRP was noted to be 0.7 mg/L, and his ESR was 39 mm/hr. He was prescribed indefinite minocycline 100 mg by mouth twice daily due to vertebral hardware placement. He subsequently missed his follow-up clinical and opiate counseling appointments and was ultimately lost to follow-up. |
pmc-6151332-1 | A 30-year-old female Caucasian was referred by her urologist because of persistent asymptomatic microhematuria and a suspicious finding on CT scan. The patient was naive to medication except oral contraception. Family history for malignancies in the upper and lower urinary tract, as well as for stone disease, was negative.
She was normotensive without pathologic findings in the physical examination. Laboratory findings were within normal range. Urinalysis demonstrated blood in the urine without proteinuria or signs of infection. A pregnancy test was negative.
Renal- and bladder ultrasonography as well as the cystoscopy were without pathological findings.
CT urography revealed a 5 mm filling defect in the left caudal calix (), so she was referred to our department to undergo retrograde pyelography and ureterorenoscopy to rule out upper urinary tract tumor.
The patient underwent cystoscopy with left-sided retrograde pyelography after sampling a urine cytology from the left renal pelvis. The urine cytology revealed papillary formations with nuclear atypia without malignancies (PAP III). The retrograde pyelogram showed the previously described contrast-filling defect in the caudal calix of the left kidney (). Because of a stenosis of the distal ureter, we placed a 7F Double-J stent and performed fURS 2 weeks later. The fURS was performed with a 9.9F video ureteroscope (Olympus, Tokio, Japan) and showed pathologic findings neither within the ureter nor within the renal pelvis. However, in two calices of lower pole, nodular calcified papillary structures were found (), which were both biopsied endoscopically and a 7F Double-J stent was placed again.
A second urine cytology from the left renal pelvis was taken during fURS, with similar results as obtained at the initial intervention. The histology analysis (including hematoxylin and eosin staining, Berliner Blau and CK20) of the biopsies showed a reactive urothelial hyperplasia without any malignancies.
After thorough communication with the patient about the benign cause of the AMH, we removed the Double-J stent without a subsequent surgery (e.g., ablation with a holmium-YAG laser). After 3 months, the patient was still asymptomatic and no changes were noted on CT scan with respect to contrast enhancement or the size of the lesion. |
pmc-6151368-1 | A total of 376 patients diagnosed with pHPT were operated on in our service from January 2000 to December 2013.
We present the case of a 30-year-old male patient who referred to our clinic with the diagnosis of primary hyperparathyroidism. The patient had a history of stage IV-B non-Hodgkin lymphoma diagnosed in 2005 and treated with radiochemotherapy, currently in remission. Our patient presented with polydipsia and polyuria without associated bone pain. The laboratory tests showed a serum calcium of 12.7 mg/dl (laboratory range 8.7–10.4 mg/dl), phosphorus 2.4 mg/dl (2.4–5.1 mg/dl), hormone intact parathyroid (PTH) 216 pg/ml (11–80 pg/ml), 25-hydroxyvitamin D 23 ng/ml (30–100 ng/ml), alkaline phosphatase 102 U/l (45–129 U/l), 980 mg calciuria in 24 hours, and preserved renal function. Cervical ultrasound performed preoperatively showed a hyperechogenic nodule of 9 mm adjacent to the left lower thyroid pole. A scintigraphy with Tc99m sestamibi (MIBI) was performed in which a pathological hypercaptation was observed at the level of the left inferior thyroid.
In view of these findings, the patient underwent an exploratory cervicotomy using a classic Kocher incision. During surgery, a normal-appearing thyroid parenchyma without nodules was observed. A slightly enlarged left lower parathyroid gland that was excised under the suspicion that adenoma was evidenced. The left superior gland was of normal appearance and macroscopic characteristics although a biopsy was performed. The intraoperative PTH levels did not decrease (PTH at the beginning of the surgery of 333 pg/ml, PTH after 20 minutes of its extraction of 366 pg/ml). The intraoperative result of both biopsies was of parathyroid tissue without histological alterations. It was decided to explore the right side finding, an upper and lower gland of normal macroscopic appearance and orthotopic localization. The left inferior thyroid lobe was mobilized, finding an extracapsular supernumerary gland adjacent to the common carotid artery with a maximum diameter of 15 mm that was excised (). The decrease in PTH to 22.1 pg/ml and biopsy confirmed the diagnosis of a paratiorial adenoma.
The patient presented an immediate postoperative period normal, with evidence of a decrease in PTH and normalization of calcium levels. During twenty months of follow-up, the patient has not shown data of recurrence or persistence of hyperparathyroidism with calcemia and PTH within laboratory ranks. |
pmc-6151369-1 | A 42-year-old woman was admitted to our ward from the emergency room (ER), complaining of persistent gastrointestinal discomfort for nearly 3 weeks; she also had fever and severe lower abdominal pain.
The patient did not have any underlying diseases or specific past history, except for infertility and having recently received in vitro fertilization (IVF) treatment. The patient had undergone transvaginal sonography before receiving IVF treatment. Sonography revealed a teratoma-like ovarian mass on the left side, sized approximately 6 × 4 × 4 cm3 and several uterine myomas. The IVF-embryo transfer program included the use of a gonadotropin-releasing hormone antagonist, and ovulation was induced using one dose of recombinant human chorionic gonadotropin (250 µg). After 35 hours, she underwent ultrasonographically guided TVOR without any apparent complications and frozen embryo transfer was performed 3 days later. However, pregnancy test 2 weeks later was negative, and patient's failure to conceive in this cycle of treatment was confirmed.
After undergoing TVOR, the patient experienced dull lower abdominal pain, which persisted for 3 weeks before she decided to visit the ER. Moreover, other symptoms, including fever up to 38°C, severe lower abdominal pain, and watery diarrhea several times a day, had developed 3 days prior to her arrival. The following parameters were recorded upon her arrival at the ER:Vital signs: body temperature, 38.5°C; pulse rate, 84 bpm; respiratory rate, 16 bpm, blood pressure, 92/51 mmHg. Physical examination: abdominal distension and diffuse lower abdominal tenderness, particularly on the left side. Laboratory data: elevated C-reactive protein (CRP) level (11.32 mg/dL), without other specific abnormalities. Sonography: cyst and abscess formation on both sides of pelvis with fluid accumulation. Computed tomography (to exclude other related diseases): a mixed-density mass, sized approximately 4 × 4 (cm), with calcifications but with a discontinuous border over the left adnexa, and diffused abdominal ascites with mesentery edema; the findings were compatible with clinical suspicion of a ruptured teratoma caused by puncture (of a teratoma) during TVOR (). Chest X-ray: no specific findings (e.g., no pleural effusion)
Under the impression of peritonitis due to ruptured teratoma, the patient was admitted for further examination and surgical treatment.
Unfortunately, her condition deteriorated rapidly 1 day after admission, with unstable vital signs: pulse rate, 108 bpm; respiratory rate, 46 bpm; blood pressure, 80/57 mmHg. Her urine output also decreased. Physical examination demonstrated diffuse abdominal tenderness, and sonography revealed results similar to those of the previous day. The laboratory data showed some abnormalities, including leukocytosis with WBC count of 34440/µL, neutrophil fraction of 95.4%, CRP level of 34.37 mg/dL, and creatinine level of 1.2 µg/dL. Chest X-ray reports before and after presentation at the ER did not differ considerably. Severe sepsis and accompanied acute kidney injury was initially suspected. We changed the antibiotics from cefazolin + gentamycin + clindamycin to flomoxef + metronidazole, immediately followed by surgical treatment.
The patient received exploratory laparoscopy later that day. Upon entering her abdomen, severe adhesions as well as the presence of purulent fluid were found in her peritoneal cavity. Near the left adnexa, a ruptured teratoma and its spilled contents, including sebaceous substances, were noted (). Because of severe adhesions caused by active inflammation, it was very difficult to even visualize or to remove the ruptured cyst. Left oophorectomy was also considered at the time but ultimately not performed because of the extent of peritonitis and adhesions. We did, however, remove as much tissue, adhesions, and purulent fluid adjacent to the left adnexa as possible. We also performed washing cytology, arranged intra-abdominal lavage with antibiotics, and inserted bilateral drains. Pathological studies showed that the fibrinopurulent exudate was composed of fibrin, neutrophils, mononuclear inflammatory cells, and cell debris, but without any ovarian tissues ().
After the surgery, patient complained of occasional shortness of breath. Bilateral pleuritis was noted on chest X-ray. Drainage with pigtail insertion was subsequently arranged. Cytological findings included an increased number of neutrophils, histiocytes, inflammatory cells, and reactive mesothelial cells on the fibrinoid as well as a bloody background on both sides, which was suspected to be the exudate fluid caused by the chemical irritation of the spilled contents of the teratoma. For the next 2 weeks, patient continued to have intermittent fever, mild abdominal pain, and sporadic shortness of breath, which explained her long stay at the hospital. We continued to administer antibiotics and made the necessary adjustment according to the result of the pus/blood culture (Escherichia coli growth was observed in her pus culture, but not in her blood culture). Eventually, patient's condition improved steadily, and she was discharged 28 days after admission. |
pmc-6151676-1 | A 26-year-old male graduate student presented to the emergency department with a three-day history of nausea, vomiting, and abdominal pain. He additionally complained of dark urine with sediments, dehydration, and scleral icterus. Laboratory evaluation revealed an aspartate aminotransferase (AST) of 13,527 U/L, alanine aminotransferase (ALT) of 8,745 U/L, and ferritin of >40,000 ng/mL. His total bilirubin was 5.5 mg/dL and international normalized ratio (INR) was 1.95, suggesting a chronic liver injury (). The patient reported drinking four to five beers the night before the onset of symptoms but denied taking acetaminophen, ibuprofen, or other drugs. He was given intravenous saline and admitted to the hospital for further evaluation.
Given his presentation and significant transaminase elevation, drug-induced hepatitis or viral hepatitis was suspected, while ischemic hepatopathy was unlikely. Right upper quadrant ultrasound was unremarkable. Acetaminophen, salicylate, and tricyclic levels were negative. Mononucleosis, human immunodeficiency virus, cytomegalovirus, antinuclear antibody, and viral hepatitis panel were also negative. Herpes simplex and varicella zoster IgG were positive, likely due to prior infection or immunization.
He remained stable during hospitalization and his liver enzymes began to trend downward, so he was discharged two days after admission. At six-week gastroenterology follow-up visit, his liver enzymes had returned to normal and he reported complete resolution of symptoms. The remainder of the pending laboratory evaluation (smooth muscle antibodies and hemochromatosis testing) returned negative.
The etiology of this patient's hepatitis remained unknown until many months later he admitted to inhaling chloroform. He described recreational huffing, to the point of unconsciousness, twice weekly over several months. This information was not volunteered at the time of presentation because the patient was embarrassed and did not feel comfortable discussing substance abuse with his physicians. |
pmc-6151684-1 | A 50-year-old African-American female with diabetes-mellitus type 2, hypertension, and cardiomyopathy presented with sharp abdominal pain in the right upper quadrant and epigastric region for 4 weeks and an episode of hematemesis with bright red blood and clots.
A computed tomography (CT) scan of the abdomen/pelvis with contrast () showed a partially exophytic lobular mass along the superior wall of the gastric fundus measuring 4.5 × 4.3 × 2.0 cm, seven lesions within the liver with the largest measuring 2.2 × 2.0 × 2.5 cm, and a metastatic lymph node anterior to the GE junction. EGD showed a large polypoid fundic mass (). H&E staining demonstrated characteristics of gastric carcinoma (), with final pathological findings of a sarcomatoid carcinoma which was positive for cytokeratin, SMA, desmin, and GATA3. The patient was discharged to follow up with an oncologist.
She presented again with nausea and vomiting in a couple of months and a repeat CT scan of the abdomen/pelvis () showed an increase in the gastric mass to 8.7 cm with ulceration and invasion of the left lobe of the liver. There was a massive increase in the regional and hepatic metastases with the largest measuring 10 cm, and there was metastasis to the right adrenal gland with associated ascites.
GATA3 is more common in breast or urothelial carcinomas, so a mammogram was performed and reported as BIRADS-1. Also, there was no evidence of any urothelial mass/neoplasm on the earlier CT scans. The patient was transferred to a tertiary care hospital but died before starting therapy. |
pmc-6151688-1 | A 40-year-old female, a known case of hypothyroidism, was discovered to have incidental pancytopenia while she was being managed for vocal cord papilloma at a tertiary care center. Extensive invasive workup for pancytopenia could not be ensued because of patient refusal. Almost a year later, the patient at 30 weeks of gestation presented to the hospital with severe fatigue. Her laboratory results revealed pancytopenia (white blood cell (WBC) count) of 2.8 × 109/L, hemoglobin (Hb) of 9.5 g/dL, platelet (Plt) count of 150 × 109/L, serum folate of 26 nmol/L, and serum B12 of 160 pg/mL along with normal renal/liver function profile and normal viral hepatitis serology reports. Peripheral blood morphology was performed, showing severe neutropenia and toxic granulation. Bone marrow biopsy demonstrated reduced cellularity (10–15%), reduced erythropoiesis, granulopoiesis, and megakaryocytes. Fluorescence in situ hybridization and flow cytometry studies revealed no evidence of bone marrow infiltration or leukemia.
Two months after uncomplicated normal vaginal delivery, the patient presented to the hospital with aplastic anemia, evident by her complete blood count: WBC of 1 × 109/L, absolute neutrophil count (ANC) of 0.05, Hb of 7.8 g/dL, and Plt of 5 × 109, and the bone marrow biopsy revealed markedly reduced cellularity with many lymphoid aggregates and no clear dysplastic changes or evidence of malignancy. Molecular study (BCR-ABL by PCR), cytogenetic study (Karyotyping), and chromosome breakage study were performed. The molecular study and chromosome breakage study came negative, and there was no growth on the karyotyping by G-banding.
The patient was kept under observation in the hospital while tests were performed on her family to search for a HLA-matched donor. Her brother was subsequently found to be a full HLA-match. The preparative regimen for transplantation consisted of cyclophosphamide (total dose: 200 mg/kg) and along with antithymocyte globulin (ATG; thymoglobulin Genzyme©).
The patient developed a fever on the 10th day of transplantation with skin redness and swelling on the intravenous line sites. She was administered cefepime and vancomycin, which was later switched to meropenem (blood cultures revealed positive Pseudomonas aeruginosa colonies). Furthermore, ultrasonography was performed, showing edema with mildly increased vascularity in accordance with inflammation and no signs of abscess. Skin biopsy was done, indicating dermal edema and dermal RBC extravasation. She also developed cyclosporine-related hypertension which was managed by amlodipine administration. The patient was started on granulocyte colony stimulating factor (G-CSF) at 300 mcg daily.The patient's cell counts did not rise significantly (WBC of 0.18 × 109/L, Hb of 9.8 mg/dL, and Plt of 2 × 109/L) ten days after administration of G-CSF; therefore, the dosage of G-CSF was further increased to 5 mcg/kg/day. Chimerism studies at day +30 after transplantation indicated 25% donor lymphoid cell engraftment on peripheral blood, and bone marrow (BM) biopsy showed a cellularity of 5%. The patient was eventually declared as graft failure.
The patient's state of health was discussed in the BMT didactic conference; the team decided to consider the patient for salvage haploidentical HCT after conditioning. It is worthwhile in general to wait until day +60 for engraftment failure before taking a major step like a second allogeneic transplant; however, the patient's risk of infections was significantly high, and thus waiting for an extra month would have possibly led to fatal infections. Thus, workup for second allogeneic transplant started around 5 weeks from the first transplant. The conditioning regimen comprised of antithymocyte globulin (ATG), total body irradiation (TBI), and fludarabine. ATG brand THYMOGLOBULIN (Genzyme) at a dose of 9 mg was used in conditioning. Same brand was used for both transplants. DSAs before the second transplant to the selected donor were found in DRB1 and DQB1 as follows: anti-DRB1 mean fluorescence intensity (MFI) = 3154 and anti-DQB1 MFI = 2141.
The patient received haploidentical stem cells from her other sibling. She received CD34+ count of 11.12 × 106/kg after conditioning with the aforementioned conditioning regimen. Total time difference between the 2 transplants was 65 days (first allogeneic transplant: 20th December 2016; second allogeneic transplant: 23rd February 2017). After transplantation, the patient remained asymptomatic with no signs or symptoms suggestive of acute GVHD or infections in acute posttransplant phase. Her laboratory results demonstrated ANC > 0.5 and platelet >200 × 109/L at day +10 after transplantation. The patient was further managed with intensified immunosuppressive therapy, which includes tacrolimus and mycophenolate. The patient was discharged on pertinent medications and was advised to follow up with her primary care physician.
Five months after transplantation, the patient presented to the hospital with fever and diarrhea for 3 days; it was initially managed with antibiotics. However, her condition deteriorated without significant improvement. On performing further investigations, she was found to have Clostridium difficile (C. diff) positive stool, asymptomatic urinary tract infection (UTI) evident by E. coli positivity in urine culture report, acute kidney injury (AKI) possibly due to dehydration, and elevated tacrolimus blood levels. The patient's condition gradually improved after administration on antibiotics (metronidazole and ciprofloxacin for C. diff and UTI, respectively) and was eventually discharged.
In order to rule out GVHD, colonoscopy was performed; it demonstrated moderate ulcerative colitis, which came out negative for mucosal architectural distortion and ulceration, microorganism viral cytopathic effect, granuloma, and adenomatous dysplastic change. Typical morphological features of acute or chronic GVHD were not evident on colonic biopsy; a microbiological correlation was suggested to rule out the possibility of infectious colitis. Her laboratory results revealed the following: white blood cell (WBC) of 5.9 × 109/L, hemoglobin (Hb) of 13.2 mg/dL, platelets (Plt) of 189 × 109/L, and absolute neutrophil count (ANC) was normal. Renal function displayed improvement showing a precipitous decline in blood creatinine levels from 300 µmol/L to 74 µmol/L, GFR turned out to be greater than 60 mL/min/1.73 m2, and potassium levels were 4.4 mm/L. Tacrolimus levels were found to be therapeutic (serum level: 10.9 ng/mL).
Her dose of tacrolimus was reduced, keeping in consideration the blood levels of tacrolimus. The patient was subsequently discharged on pertinent medications and was instructed to follow up with her primary care physician. |
pmc-6151845-1 | An 8-year-old, 8.1 kg, neutered male miniature Poodle presented with suspected spinal trauma after being hit by a car 4 days prior to referral. The dog had been medically managed at a local emergency clinic with concerns of a T2-T4 injury with cervical component. Thoracic radiographs taken at the time of presentation to the emergency clinic revealed possible vertebral injury or intravertebral disc compression at T2-T3; the remaining thoracic images were unremarkable.
On initial presentation, the dog was tachypneic and laterally recumbent with a body condition score of 5/9. The dog vocalized on palpation of the neck and pain was assessed at a 2/4 using the Canine Acute Pain Scale []. The remainder of the examination was unremarkable.
On neurologic examination, the dog was laterally recumbent, quiet, and alert. Cranial nerve function was grossly intact. The dog was nonambulatory with paresis of the thoracic limbs and plegia of the pelvic limbs. Muscle tone was increased in the thoracic limbs. When supported in standing position, a right head turn was noted and conscious proprioception was decreased in the thoracic limbs and absent in the pelvic limbs. Segmental reflexes were intact. The panniculus reflex was absent caudal to L1 bilaterally. No pain was elicited on spinal palpation; cervical range of motion was not evaluated. Neurolocalization was determined to be T3-L3 with suspected C6-T2 involvement.
Notable blood work findings included an elevated alanine aminotransferase (185U/L; reference, 10-125U/L), normochromic normocytic anemia (hematocrit 35.7%; reference, 40-56%), leukocytosis (15.69K/uL; reference, 5-13 K/uL), neutrophilia (10.7K/uL; reference 2.7-8.9K/uL), monocytosis (2K/uL; reference, 0.1-0.8K/uL), and hyperfibrinogenemia (500mg/dL; reference, 100-400mg/dL).
Abdominal ultrasound revealed mild retroperitoneal effusion adjacent to the left kidney consistent with trauma with no other significant findings.
The dog was placed under general anesthesia for advanced imaging with propofol (2mg/kg IV) and midazolam (0.3mg/kg IV) and maintained on isoflurane. Maropitant (1mg/kg SQ) and atropine (0.01mg/kg IV) were administered as needed.
Magnetic resonance imaging (MRI) of the entire spine revealed a T2-T3 vertebral luxation with focal spinal cord compression, characterized by a right lateral displacement of T3. Intramedullary spinal cord changes noted on T2 weighted image (T2-WI) and short tau inversion recovery (STIR) were suggestive of edema and/or hemorrhage, with suspected adjacent epaxial myositis. Multifocal noncompressive bulges of the C2-C7 intervertebral discs were noted ().
On noncontrast computed tomography (CT), a vertebral luxation of T2-T3 was confirmed, with a 4.5mm right displacement of the T3 vertebral body. A T2 caudal endplate fracture was noted with associated T2-T3 disc space collapse. A single mineralized 4mm fragment was located within the vertebral canal at the level of T2-T3 (). The dog recovered from general anesthesia uneventfully and surgery was scheduled. Due to the severity of the spinal cord injury, the clients were counseled regarding the potential for the dog to have permanent pelvic limb deficits.
Prior to surgery, the dog was managed on methadone (0.2mg/kg IV bolus, then 0.1mg/kg IV bolus, then IV CRI at 0.025-0.05mg/kg/hr), acepromazine (0.01mg/kg IV once), and maropitant (1mg/kg IV once) and maintained at 40% O2.
It was elected to stabilize C7-T4 and the dog was premedicated with methadone (0.3mg/kg IV) and lidocaine (2.5mg/kg IV) and induced with propofol (3mg/kg IV) and ketamine (3mg/kg IV). The dog was maintained on isoflurane and received atropine (0.4mg/kg IV), a combination of hydromorphone, lidocaine, and ketamine (end total 1.32mg, 131mg, and 44mg IV CRI, respectively), dopamine (total 2mg IV CRI), cefazolin (22mg/kg q90min), and packed red blood cells (100mL IV). A ventral cervical approach was extended caudally into a median sternotomy to gain adequate exposure. The mediastinum and brachycephalic trunk were dissected bluntly to approach the ventral spinal musculature. A pericardial sling was employed to allow caudal and ventral retraction of the heart and great vessels away from the area of interest. Similarly, retractors were placed near the thoracic inlet and caudally along the brachycephalic trunk to increase visualization of the spine and prevent vital structures from becoming entrapped with the various power instruments utilized (). The longus coli muscles were elevated from the ventral aspect of C6-T5. Manual reduction of the luxation was attempted; however due to the presence of fibrous tissue, complete reduction of the lesion was not achieved. An 8-hole 2.0mm String-of-Pearls (SOP) plate (OrthoMed, West Yorkshire, UK) was placed along the ventral aspect of C7-T4 vertebral bodies and temporarily stabilized with 0.89mm Kirschner wires (IMEX Veterinary, Longview, Texas) placed through a node on both sides of the T2-T3 disc space (). Plate location and pin depth were evaluated with fluoroscopy (Insight II; Hologic, Marlborough, Massachusetts). A 6-hole 2.0mm SOP plate was contoured to the vertebral surfaces of C7-T3 and placed adjacent to the first plate. 2.0mm cortical screws (DePuy Synthes, West Chester, Pennsylvania) were placed in holes engaging C7, T1, and T3 in the 6-hole plate (). Cortical screws were placed in the 8-hole plate engaging C7 and T2-T4 following removal of the Kirschner wires (). Based on the String-of-Pearls plate design as a locking construct, the screws were placed with the intention of being monocortical; however second cortex was breached in the T3 and T4 vertebrae. A 5 Fr MILA chest tube (International, Erlanger, Kentucky) and subcutaneous soaker catheter (MILA International, Erlanger, Kentucky) were placed and closure was routine. Postoperative radiographs indicated apparent appropriate implant placement, though the T2-T3 disc space remained collapsed ().
The dog was managed postoperatively with fentanyl (1-5mcg/kg/h IV CRI), naloxone (0.04mg/kg IV once), bupivacaine (1mg/kg q8h through soaker catheter), dexamethasone SP (0.2mg/kg IV once), prednisone (0.6mg/kg PO q24h), and cefazolin (22mg/kg IV q8h). The day following surgery, tramadol (3mg/kg PO q8h) was initiated; fentanyl and cefazolin were discontinued. The dog's neurologic status remained unchanged immediately postoperatively. Three days later, the dog was transferred to his primary veterinarian for long-term care.
The dog re-presented eight weeks after surgery for follow-up. Physical examination was unremarkable. Neurologic examination revealed an ambulatory patient with moderate to marked pelvic limb paraparesis and proprioceptive ataxia. Conscious proprioception was absent in pelvic limbs bilaterally. Segmental reflexes and panniculus were intact. No pain was elicited on spinal palpation. The lesion was neurolocalized to T3-L3 and markedly improved from prior exam.
CT of the cervical and thoracic spine was performed under sedation using dexmedetomidine (14mcg/kg IM) and reversed with atipamezole (0.25mg/kg IM). The intervertebral disc of T2-T3 remained collapsed, the luxation of T2-T3 was consistent with initial postoperative radiographs, and the T2 caudal endplate fracture was ill-defined. The two most distal screws protruded into the vertebral canal but spinal cord compression was not suspected (). |
pmc-6151924-1 | We present a case of a 77-year-old Caucasian man with past medical history of stage I adenocarcinoma of transverse colon status post laparoscopically assisted segmented transverse colectomy in April 2014. Other medical history included type 2 diabetes, hypertension, hypothyroidism, and benign prostatic hypertrophy. Medications at the time of diagnosis included aspirin, metformin, lisinopril, and levothyroxine. His family history included lung cancer in his father who was a tobacco smoker. Our patient was a former tobacco smoker but denied history of alcohol or drug abuse and had no history of occupational or chemical exposure. He presented for follow-up screening colonoscopy approximately 2 years later in July 2016 at which time he was asymptomatic. His Eastern Cooperative Oncology Group (ECOG) performance status was grade 1. On clinical examination he was afebrile, mildly hypertensive with blood pressure 146/81, heart rate 78, respiratory rate of 16 with oxygen saturation of 96% on room air. He had normal cardiac rate and rhythm, and no abnormal breath sounds on respiratory examination. His abdomen had normal bowel sounds on auscultation, was soft and non-tender without distension. A neurologic examination demonstrated normal neurologic function without sensory deficits and normal muscle strength.
On colonoscopy, he was found to have an abnormal-appearing appendiceal orifice which was biopsied; pathology was suggestive of mucinous adenocarcinoma with signet ring cell features versus a goblet cell-type carcinoid tumor of the appendix (Fig. ). The appendiceal orifice appeared normal on previous colonoscopies in March and December of 2014. Pre-colonoscopy complete blood count (CBC) revealed white blood cell (WBC) count of 5.7 103/uL (reference range 4–11), hemoglobin 13.9 g/dl (reference range 14–17) with mean corpuscular volume (MCV) of 82.3 fL (reference range 80–94), and platelet count of 171 K/mm3 (reference range 150–400). Pre-colonoscopy basic chemistry including sodium, potassium, chloride, bicarbonate, and creatinine were all within normal limits.
On histological examination the tumor was present as infiltrative small nests and clusters of cells with small nuclei compressed by abundant cytoplasmic mucin vacuoles, giving a signet ring appearance (Figs. and ). Given the location of the lesion at the appendiceal orifice, the diagnosis of goblet cell carcinoid was strongly suspected, but definitive diagnosis was deferred to complete resection. Further laboratory workup with tumor markers and neuroendocrine markers revealed carcinoembryonic antigen (CEA) of 3.1 ng/ml (reference range 0.0–3.1) and chromogranin A, and 24-hour urine 5-hydroxyindoleacetic acid (5-HIAA) within normal limits. A computed tomography (CT) scan of his chest, abdomen, and pelvis showed a thickened appendix (12 mm) without evidence of fat stranding (Fig. ). There was no significant lymphadenopathy, no colonic masses seen, and no evidence of distant metastatic disease.
After surgical evaluation, he underwent a right hemicolectomy in August 2016. Both specimens from colonoscopy and right hemicolectomy were sent for expert consultation. On pathologic review, the bulk of the tumor involved the appendix, essentially obliterating the lumen, with diffuse spread into the mesoappendix and serosal adipose tissue. Both perineural and lymphovascular invasion were noted. Six of 14 lymph nodes harbored metastatic carcinoma. In areas of the appendiceal wall, the nests of signet ring cells coalesced into pools of mucin containing “floating” cells, indicating frank mucinous carcinoma, so-called adenocarcinoma ex-goblet cell carcinoid (Fig. ), Tang group B. Immunohistochemistry for synaptophysin highlighted scattered occasional peripheral endocrine cells, as is characteristic of goblet cell carcinoid (Fig. ). The final pathologic staging of the patient's tumor was pT3 N1 M0, stage III as per American joint committee on cancer staging manual, 7th edition [].
Postoperatively, we discussed treatment options including adjuvant chemotherapy; our patient was initially against adjuvant chemotherapy due to prior experiences with family members, but he agreed to it after the rationale was explained. He was given adjuvant capecitabine with a goal of eight cycles. Given his age, the first four cycles of capecitabine were given at a 25% dose reduction of 1500 mg twice daily for days 1–14 every 21 days. As he tolerated therapy well, the dose was increased to 2000 mg twice daily for days 1–14 every 21 days for cycles five to eight. He completed eight cycles of capecitabine and tolerated treatment well other than mild hand and foot syndrome which developed during the last two cycles. A follow-up CT scan at 6 and 12 months after completion of adjuvant chemotherapy showed no evidence of recurrent disease. A repeat colonoscopy at 1 year from original diagnosis was also negative for any malignant-appearing lesions. We are continuing surveillance with history and physical with CEA every 3 months, and CT of his chest, abdomen, and pelvis every 6 months for the first 2 years and then annually for up to 5 years. At the current time he remains disease free at 2 years from time of diagnosis. |
pmc-6152398-1 | A 76-year-old Danish male presented to the emergency department at Aalborg University Hospital (Aalborg, Denmark) in May 2017 with right-side otalgia throughout the previous week, and onset of fever and confusion within the previous 24 h. Upon admission, the patient was otherwise healthy, and he used no daily medication. Fifty-three days prior to admission, the patient returned from a 16 day holiday on the east coast of Peninsular Malaysia. Before the journey, he was re-vaccinated against diphtheria, tetanus and hepatitis A. He had not used malaria prophylaxis during his vacation.
On examination, the patient had altered mental status with a Glasgow Coma Score of six, neck stiffness and fever (40.0 °C). In accord with national guidelines on the management of suspected bacterial meningitis, the patient had blood cultures performed and was started on high-dose intravenous (iv) benzylpenicillin (1.8 g every 4 h), cefotaxime (3 g every 6 h) and dexamethasone (10 mg every 6 h). A lumbar puncture of the patient was performed, following a computed tomographic scan of the cerebrum, which had proved normal. Laboratory tests showed a C-reactive-protein level of 273 mg l−1, procalcitonin of 10.8 µg l−1 and white blood cells of 19.9×109 l−1. Cerebrospinal fluid (CSF) analysis revealed pleocytosis with white blood cells of 741×106 l−1 (636 polynuclear and 105 mononuclear), a slightly decreased glucose ratio (CSF : serum) of 0.38, an elevated protein level of 1.34 g l−1 and lactate of 7.3 mmol l−1, and the patient was transferred to the Intensive Care Unit (ICU).
Overnight culture of the patient’s CSF yielded Gram-negative, non-motile, oxidase- and catalase-positive rods. Moreover, a simultaneous positive blood culture (BD BACTEC; Becton Dickinson) had similar findings. MALDI-TOF MS (matrix-associated laser desorption ionization-time of flight MS) (MALDI Biotyper 3.1, Bruker Daltonics Microflex LT, MBT 6903 MSP Library) could not distinguish the colonies between either E. meningoseptica (score 2.215) or E. miricola (score 2.101), whereas E. anophelis was not present in the MALDI library. API 20 E v5.0 (bioMérieux) gave an identification of E. meningoseptica with a score of 71.6 % identity (numerical profile: 0042004). The isolate was multidrug resistant and positive for metallo-β-lactamase by using the MBL Confirm kit (Rosco Diagnostica). Antimicrobial-susceptibility testing (AST) was performed by using Etests (bioMérieux) according to the European Committee on Antimicrobial Susceptibility Testing guidelines (). We used the pharmacokinetics/pharmacodynamics (non-species related) breakpoints except for trimethoprim/sulfamethoxazole, in which Stenotrophomonas maltophilia breakpoints were used, and for gentamicin and colistin we used Pseudomonas species breakpoints, as performed by Eriksen et al. []. The isolate was susceptible to moxifloxacin (MIC 0.125 mg l−1) and trimethoprim/sulfamethoxazole (MIC 0.25 mg l−1); intermediately susceptible to amoxicillin/clavulanic acid (MIC 6 mg l−1); and resistant to ciprofloxacin (MIC 0.75 mg l−1) and all other drugs tested including: ampicillin, cefuroxime, ceftazidime, meropenem, gentamicin, colistin and tigecycline. An Etest for vancomycin showed a MIC of 12 mg l−1, but we did not make an interpretation of susceptible or resistant. Etests were not available for piperacillin/tazobactam and rifampicin, but the disc diffusion zone (Neo-Sensitabs; Rosco Diagnostica) for piperacillin/tazobactam was 19 mm and for rifampicin was 24 mm, but as for vancomycin we did not make an interpretation of susceptible or resistant.
To determine the identity of the isolate to the species level, we performed sequencing with the Illumina MiSeq instrument producing 2×300 bp paired-end reads by using a Nextera XT library preparation kit (Illumina). Reads were assembled using CLC Genomics Workbench (version 11) (QIAGEN Bioinformatics) into 105 contigs, N50 (497, 160), total sequence length 4 047 726 bp, with a G+C content of 35.6mol %.
Analysis of the 16S rRNA gene, as well as a k-mer based distance measure, compared to the publicly available strains of E. meningoseptica and E. miricola showed a clear identification of the isolate as E. anophelis (data not shown). Breurec et al. [] and Perrin et al. [] reported a clear division of E. anophelis into 15 sublineages, including 1 associated with the large outbreak of E. anophelis infections that occurred in Wisconsin (USA) in 2015–2016. To subtype our strain and define its sublineage, we used the core-genome multilocus sequence typing (cgMLST) strategy, using the subset of 1546 genes families that are highly conserved within E. anopheles []. The cgMLST profile of our isolate was compared to those publicly available in the Elizabethkingia cgMLST database on the Institut Pasteur server (). Cluster analysis based on cgMLST profiles, see , showed that the isolate belonged to sublineage 11, which was defined with strain CIP 60–59 (CDC 3375; ATCC 13255; NCTC 10586; CCUG 4321; LMG 12873) as a reference. Strain CIP 60–59 was isolated from the CSF of a premature infant who died []; however, the two strains of sublineage 11 have different alleles at 299 loci out of 1513 loci called in both strains. This result clearly shows that AAUH 98722 (our isolate) and CIP 60–59 are genetically distinct. The assemblies were submitted to ResFinder 3.0 () to analyse them for the presence of antimicrobial-resistance genes []. The isolate proved positive for two metallo-β-lactamase genes, namely blaGOB-3 (located on contig 69; hit length 756; 100 % ID; accession no. AF189291), and blaB-3 (located on contig 21; hit length 750; 100 % ID; accession no. AF189299), as well as an extended-spectrum β-lactamase-encoding gene, blaCME-1, (located on contig 41; hit length 784; 100 % ID; accession no. AJ006275). This is consistent with the known conservation of carbapenemase and β-lactamase-encoding genes within E. anophelis [].
After the preliminary identification of Elizabethkingia species, the antimicrobial therapy was changed to iv vancomycin combined with iv rifampicin, 600 mg two times a day. However, as MICs values were available the day after, the definite treatment was changed to iv moxifloxacin, 400 mg once per day, combined with iv rifampicin 600 mg two times a day for a total duration of 14 days. The patient improved and after 10 days at the ICU he was transferred to the infectious disease ward for further treatment and rehabilitation, and he was finally discharged after 3 weeks of hospitalization. The only sequela was a partial hearing loss. Due to the severity of infection, the patient was examined for immunodeficiency in an outpatient setting and was found to have a sustained and elevated level of IgM of approximately 14 g l−1 (normal range 0.39–2.1 g l−1) during the following months. Seven months after the meningitis episode, his bone marrow was further investigated, and he was finally diagnosed with lymphoplasmacytic lymphoma (Waldenström macroglobulinaemia). |
pmc-6152400-1 | A 21-year-old male was admitted to the Centre Henri Becquerel, Rouen, France, with asthenia, fever (39 °C) and icterus on July 15 2016. The patient reported having had these symptoms for 2 weeks. Laboratory investigations indicated pancytopenia (polynuclear neutrophils=0.6 g l−1; platelets=6 g l−1; haemoglobin=12.5 g dl−1), acute cytolytic and cholestatic hepatitis, and inflammatory syndrome. Viral serology was negative. A myelogram revealed low bone marrow density and an absence of megakaryocytes. Medullary biopsy confirmed medullary aplasia without fibrosis. The karyotype was normal, and the patient was negative for paroxysmal nocturnal haemoglobinuria clones and Fanconi disease. The patient, who worked in car body repair, had no relevant medical history. In the absence of another aetiology, a diagnosis of aplastic anaemia was established.
The same day, empirical antibiotherapy with piperacillin/tazobactam (4 g/0.5 g every 8 h) i.v. was initiated. A physical examination on July 21 2016 did not reveal the source of the fever, and abdominal ultrasound and echocardiography were normal. Six blood cultures were taken between July 14 and 25 2016. Two (July 23 and 25) were positive for yeast. Cyberlindnera jadinii was identified from aerobic cultures by MS. (The MICs are shown in ).
Caspofungin therapy was initiated on July 26 2016 (70 mg on day 1, followed by 50 mg day−1). The same day, the patient presented a diffuse, purplish maculo-papular eruption. Cutaneous fungal infection was suspected, but the cutaneous biopsy was inconclusive. On July 29 2016, the patient displayed persistent fever despite the caspofungin therapy, so caspofungin was replaced by intravenous (i.v.) liposomal amphotericin B (3 mg kg−1 day−1).
On August 1 2016, the patient still presented with a continuous fever (40 °C), and the cutaneous eruption was extensive. Pulmonary computed tomography showed alveolar and nodular opacities with fuzzy contours evoking a fungal infection. Due to the patient’s worsening condition and the computed tomography results, combined therapy with caspofungin (50 mg every day i.v.) and i.v. liposomal amphotericin B (3 mg kg−1 every day) was decided. The patient continued to be febrile until August 4 2016, so tazocillin was replaced by imipenem (1000 mg every 8 h i.v.) and trimethoprim/sulfamethoxazole (2400 mg/480 mg every day) to treat uncertain pulmonary pneumocystosis. A new cutaneous biopsy was performed and, after 9 days of culture, Malassezia sp. was found. A non-pathogenic species was suspected.
Bronchoscopy on August 8 2016 did not reveal mucosal lesions. However, macrophages and amphotericin B-sensitive Candida kefyr were found in the bronchoalveolar lavage while the patient was receiving combined antifungal therapy.
Because of persisting skin lesions and fever, on August 9 2016, a new cutaneous biopsy was performed, revealing an inflammatory perivascular reaction of the dermis and a remodelling of hypodermic fat with mononuclear mobilization. The patient was transferred to an intensive care unit on August 11 2016 for septic shock and probable invasive candidaemia. While there, the patient received i.v. noradrenaline for a few hours. The patient became hypothermic, so external warming was performed. Because of the patient’s prolonged neutropenia, vancomycin (2 g day−1 i.v.) was introduced. Because his clinical condition improved, vancomycin was stopped on August 25 2016. The patient continued to have cockade-like skin lesions and no evidence of viral aetiology, with negative PCR for cytomegalovirus, parvovirus B19, Epstein-Barr virus and herpes simplex virus.
The patient was transferred back to the Centre Henri Becquerel on August 15 2016. He continued to present night fever, and the cutaneous eruption gradually improved.
Aspergillus galactomannan antigenaemia was consistently negative. In agreement with the Cyberlindnera jadinii-positive blood culture, positivity to β-d-glucan (146 pg ml−1; negative <60 pg ml−1), assessed September 5 2016, confirmed a fungal infection.
To manage the aplastic anaemia, haematopoietic stem cell transplantation (HSCT) was considered. While awaiting a compatible donor, the patient received classical doses of granulocyte-colony stimulating factor and erythropoietin to treat the anaemia. The patient’s 24-year-old brother and other related donors, however, were not HLA-identical. Although the patient did not meet the criteria for HSCT in aplastic anaemia (age <40 years and no HLA-identical familial donor) [], given the severity of the patient's illness and the absence of a related or unrelated HLA-identical donor, haploidentical HSCT using bone marrow from the patient’s brother was indicated. The patient was A negative and his brother was O negative. Both the patient and his brother were positive for Epstein-Barr virus, and the patient was immunized for cytomegalovirus.
On September 9 2016, 6 days before peripheral stem cell infusion, a non-myeloablative conditioning regimen and prophylaxis for graft-versus-host disease (GVHD) were initiated (). Prior to the transplant, the patient was clinically stable with only temporary (12 h) fever. Bone marrow transplantation was performed on September 15 2016.
No immediate toxicity of the conditioning regimen or graft was found. On day 1, the patient remained febrile with a temperature of ≥39 °C. Thoracic computed tomography revealed nodular opacities evoking a mucosal infection. Voriconazole (4 mg/kg evey day i.v.) was added to the continued antifungal therapy with caspofungin and liposomal amphotericin B. Bronchoscopy and bronchoalveolar lavage were negative.
The patient became afebrile on day 8. Voriconazole was stopped 48 h later because the patient tested negative for aspergillus, and all antibiotics were progressively terminated. Caspofungin was replaced by fluconazole (200 mg every day i.v.) on day 22. Starting on day 19, the patient received oral cyclosporine (2 mg/kg evry day i.v.) to prevent GVHD, and on day 27, the patient received valganciclovir due to cytomegalovirus reactivation.
The last platelet transfusion was performed on day 16 and the last blood transfusion on day 17. On day 18, the patient was no longer aplastic, and on day 26, platelets were >20 g l−1. The patient did not display acute GVHD. The whole blood chimerism was 100 % 7 months after the transplantation.
One-year post-transplantation, the patient decided to stop all treatments. In January 2018, the patient was in complete remission without GVHD. |
pmc-6152401-1 | A 68-year-old -man with a history of coronary bypass grafts and stable Crohn’s disease, last treated with infliximab 11 months prior to presentation, was diagnosed with transitional cell carcinoma of the bladder and underwent a transurethral resection of the bladder tumour. He subsequently received six weekly intra-vesical instillations of BCG. He received maintenance therapy 3 months later, and on his second instillation for maintenance there was difficulty inserting a catheter and frank haematuria was noted following catheterization. After 11 days he presented with fevers of 39.1 °C, sweats, shivers, generalized myalgia and reduced appetite. On admission he also complained of shortness of breath on exertion, with no cough or sputum production initially, and right upper quadrant pain. On examination his chest was clinically clear and his oxygen saturation was 95 % on air. He had a palpable liver edge. A full neurological examination was normal. He was commenced on intravenous piperacillin/tazobactam.
Abnormal results on initial investigations included a thrombocytosis of 507×109 l−1, alkaline phosphatase 150 U l−1 (normal range 40–130), an arterial blood gas with a pO2 of 10.2 kPa on air, C-reactive protein raised to 90 mg l−1 (normal <10) and a chest X-ray with micronodular shadowing in the mid- and lower zones bilaterally (). A subsequent computed tomography scan showed fine miliary shadows widespread in the lungs, consolidation at the right base and slight enlargement of the liver ().
There was no improvement with intravenous piperacillin/tazobactam and with the above results he was diagnosed clinically as having probable disseminated BCGosis infection. Subsequently he developed a productive cough and sputum. Blood and early morning urine samples were sent for mycobacterial cultures, prior to commencing rifampicin, isoniazid, ethambutol and pyridoxine 4 days after admission. On this treatment his temperature and cough settled. However, later all his mycobacterial cultures proved negative. Fourteen days after admission he began to develop an unsteady gait. On examination he had new reduced pin-prick sensation from the thigh downwards on both lower limbs and also in his fingertips. He had reduced proprioception in both toes and ankles. His reflexes were absent in his lower limbs and diminished in his upper limbs. He developed significant neuropathic pain in his legs which was relieved by amitryptilline. He also had postural hypotension with his systolic blood pressure falling from 128 mmHg lying to 105 mmHg on standing. |
pmc-6152402-1 | A 41-year-old male living in New Caledonia without significant past medical history and no recent travel, presented to his general practitioner with fever (38.8 °C), headache, sinusitis and exanthematous maculopapular rash over neck, thorax and upper limbs. The symptoms had started 2 days prior and a diagnosis of an arbovirus infection was first considered by the physician due to the current DENV outbreak and a confirmed diagnosis of the patient’s wife of dengue infection by RT-PCR the week before. A few days later a decline of health was observed and the patient was sent to the emergency department, 6 days post the onset of symptoms. On admission, the patient presented with a temperature of 40.5 °C, a spO292 %, a blood pressure of 110/50 mm Hg, dehydration, a weight loss of 6 kg, an erythroderma on trunk and face and an axillary and a cervical lymphadenopathy (<1 cm). A complete blood count showed a lymphopenia (lymphocytes count 0.68×109 l−1; reference range 1−5×109 l−1) with associated stimulated lymphocytes, and hepatic cytolysis (aspartate aminotransferase 288 IU l−1; reference value <34 IU l−1, and alanine aminotransferase 485 IU l−1; reference value <55 IU l−1). The patient was placed on intravenous paracetamol at a dose of 1 g and 10 mg of metoclopramide. The blood sample tested 3 days prior for dengue, chikungunya and Zika by real time RT-PCR was negative for the three arboviruses. With regard to the exanthema, the high fever, a recent administration of ibuprofen, amoxicillin and clavulanic acid and the cytolic hepatitis, a drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome was first considered and the patient was admitted to the internal medicine and infectious disease ward. |
pmc-6152403-1 | A 14 year old girl developed diarrhoea and vomiting and presented to hospital four days after symptom onset following a fainting episode. She had also experienced sharp colicky pains in her abdomen. She had been on holiday in the United Kingdom prior to the episode.
The patient had developed nephrotic syndrome at age 13 with a renal biopsy showing focal segmental glomerulosclerosis. She also had mild asthma, menorrhagia and pulmonary stenosis (diagnosed on echocardiogram).
Her medications included tacrolimus, enalapril, atrovastatin, omeprazole, penicillin-V, levothyroxine and ferrous fumarate. The tacrolimus had been stopped a day before the start of illness as it had not produced any improvement in renal function or reduction in proteinuria. The tacrolimus had been commenced 14 months earlier at a dose of 5 mg twice daily. A tacrolimus level had last been assayed 2 months before onset of her illness. This was 7.3 µg l−1 i.e. within the normal therapeutic range.
On admission, she was felt to be fluid-depleted with mild dehydration and after initial treatment with a bolus of 500 ml of normal saline in the emergency department, was commenced on intravenous fluids in view of abdominal pain and vomiting. Her renal function was deranged with an acute kidney injury score of 2. Urea was 22.8 mmol l−1, previously having being in the range of 6.5–11.8 mmol l−1 and creatinine was raised to 157 µmol l−1, previously having being in the range of 70–95 µmol l−1. Liver function tests were normal. White blood cell count was normal (6.1×109 l−1). Enalapril was stopped in view of these results. She improved after 24 h of maintenance IV fluid therapy with 0.9 % saline and 5 % dextrose, renal function returning to previous levels (urea 16.7 mmol l−1, Creatinine 94 µmol l−1) and she was discharged. Subsequently, Cryptosporidium was detected on stool microscopy. This was confirmed to be Cryptosporidium hominis by PCR at the national reference laboratory. The sample was negative for all other pathogens tested for, including Escherichia coli 0157, Campylobacter, Salmonella and Shigella. Following discharge she continued to have some episodes of diarrhoea but no vomiting. She re-presented 19 days later with significant abdominal pain and infrequent small vomits which were non bilious, her diarrhoea had resolved.
On examination she had epigastric tenderness but no hepatomegaly. Her perfusion was adequate with a capillary refill time <2 s, pulse rate of 89, blood pressure was 104/58, both within the normal range for her age. However, her peripheral pulses were poor. She was commenced on maintenance intravenous fluids with 0.9 % saline and 5 % dextrose. Liver function tests were noted to be deranged with raised aspartate transaminase (451 IU l−1), alanine transaminase (267 IU l−1), gamma-glutamyl transferase (115 IU l−1) and lactate dehydrogenase (853 IU l−1). Her renal function showed raised urea (10 mmol l−1) and creatinine (72 µmol l−1) which were above baseline but within her usual range. Her liver function continued to deteriorate over the next 24 h (peak aspartate transaminase 1062 IU l−1, alanine transaminase 1292 IU l−1, lactate dehydrogenase 882 IU l−1, gamma-glutamyl transferase 209 IU l−1). An ultrasound showed a thickened gall bladder but otherwise normal liver and bile duct.
After discussing the history and results with specialist paediatric hepatologists who, in view of the facts, that her stool was positive for Cryptosporidium hominis, she had worsening liver function tests and ultrasound evidence of cholangitis, recommended a three day oral course of nitazoxanide 500 mg twice daily. Following this, her liver function tests rapidly improved, returning almost to baseline after 4 days. Her appetite returned to normal and her abdominal symptoms resolved. |
pmc-6152477-1 | A 77-year-old woman, current smoker, was diagnosed with stage IV lung adenocarcinoma and no known genetic driver mutations (EGFR, ALK, ROS1). She was treated initially with a biweekly regimen of carboplatin plus gemcitabine []; however, despite an initial partial response, the disease progressed after ten cycles of chemotherapy. A second-line treatment with erlotinib was administered with disease progression as best response after two months of treatment []. A high expression (85%) of PD-L1 by immunohistochemical staining(SP263) was detected in archival tumor samples (Figure ).
The patient was in good general physical condition with Eastern Cooperative Oncology Group performance status (ECOG PS) 0. The use of nivolumab became available at our center, and a compassionate use of third-line nivolumab was approved by our institution.
After 4 cycles (8 weeks from start of treatment), computer tomography (CT) scan evaluation showed a partial response in lung tumor mass, lymphatic nodes and hepatic metastasis (Figure ). Nivolumab was well tolerated and no toxicity was observed during the seven initial cycles.
Before the eighth cycle (14 weeks) was started, a blood test showed an elevation in creatinine (2.39 mg/dL, previously ranged from 0.71 to1.22 mg/dL). Because an irAE (nephritis) was suspected, nivolumab treatment was stopped and methylprednisolone treatment was started (1 mg/kg/day). A 24-hour urine test dismissed nephrotoxic syndrome. Two weeks later (16 weeks), creatinine levels were lower (1.24 mg/dL) and nivolumab treatment was restarted with reduced corticoids (0.5 mg/kg/day) (Figure ).
Before the fourteenth nivolumab cycle (week 30), a blood test showed increased levels of liver function metrics: aspartate aminotransferase (AST), 317 UI/L (normal value <32); alanine aminotransferase (ALT), 462 UI/L (normal value <33); and bilirubin, 0.62 mg/dL (normal value <1.2), with normal values of creatinine (0.95 mg/dL) (Figure ). The patient was still receiving methylprednisolone at low doses (0.25 mg/kg/day) at this time. A CT scan was negative for dilatation of the biliary conduct, but a right axillary lymph node was observed, with maintained partial response of the other target lesions in the lung and liver (dissociated response) (Figure ). Previous hepatic function had been normal, even with known hepatic metastases, which were in near radiological complete response (Figure ). Thus, a new irAE (hepatitis) was suspected. Nivolumab treatment was stopped, methylprednisolone dosage was increased (1 mg/kg/day) and the patient was admitted for daily monitoring of liver function. During the hospitalization period, the elevation in transaminases and bilirubin (common terminology criteria for adverse events, grade 3 and 2, respectively) had good evolution, permitting discharge at week 33. During hospitalization, methylprednisolone treatment was increased up to 3.5 mg/kg/day and the patient was discharged at a dose of 2 mg/kg/day. The patient remained completely asymptomatic (ECOG PS 0) during this time.
During an ambulatory visit at week 36, the patient reported progressive dyspnea. No relevant alterations were observed in chest radiography, and empirical antibiotic therapy (amoxicillin and clavulanic acid) was started. The patient was receiving 1 mg/kg/day methylprednisolone at this time. No clinical improvement was achieved after 5 days of antibiotics, so a new thoracic CT scan was performed, which revealed the presence of bilateral diffuse ground glass opacity suggesting nonspecific pneumonitis (data not shown). Autoimmune pneumonitis was suspected. The patient was admitted for treatment with oxygen, methylprednisolone was increased to 2 mg/kg/day, and trimetoprim/sulphametoxazol treatment was administered. To complete the study, a bronchoscopy was performed and cultures of bronchial washings were positive for Pseudomonas and Candida. An ultrasound-guided fine-needle aspiration of the right axillary node was performed during admission, which revealed malignant cells with PD-L1 expression (SP263) of 80% in immunohistochemical staining (Figure ). The symptoms resolved after 2 weeks treatment and the patient was discharged on 0.75 mg/g/day methylprednisolone.
At week 40, a blood test reported elevated bilirubin and transaminases, and the patient was readmitted for study. The patient developed clinical worsening (ECOG PS 2), with flapping tremor, ascites and disorientation. Ultrasound study of the liver suggested miliary liver metastases, which were confirmed by a CT scan where multiple thoracic lymph nodes showed progression in addition to hepatic flare disease progression (initial lung metastases remained in partial response) (Figure ). As the patient was in a poor clinical state and in confirmed progression, best supportive treatment was initiated according to the patient and family. A graphical summary of the patient´s evolution is provided in Figure . A written informed consent was obtained from the patient for publication of this case report and any accompanying images. |
pmc-6153089-1 | A 25-year-old primigravid female presented to the general surgery outpatient clinic with intermittent epigastric pain, which radiated to the left hypochondrium and left shoulder. This pain was moderate in severity and had persisted for around two months. It was associated with projectile vomiting upon ingestion of solid food materials, as well as watery diarrhea. The patient complained of anorexia and subjective abdominal distension focused in the epigastrium. At that time, the patient had reached her second trimester of pregnancy with no complications in that regard. She reported she was pregnant with twins.
Upon examination, a large nontender mass was palpated in the epigastric region, extending to the left hypochondrium and umbilical region. It was firm in consistency, immobile, and dull to percussion. Auscultation revealed normal bowel sounds. It did not seem to be attached to the overlying skin and the margins could not be palpated superiorly. The rest of her physical examination was unremarkable, with the exception of the expected gravid uterus with twin foetuses.
After further questioning, it became apparent that she had a remote positive history of trichophagia and onychophagia during her childhood. She ate her own hair till the age of 10 and her nails till the age of 17. She had a previous history of iron-deficiency anemia at the age of 18 but had sought treatment with complete resolution.
As the mass was large and the extent of it was unknown, abdominal magnetic resonance imaging (MRI) was performed as an initial investigation to explore the provisional diagnosis of a bezoar. The MRI revealed gastric distension with the presence of a soft tissue mass extending throughout the stomach (Figure ). This confirmed the diagnosis of gastric bezoar, and the patient was admitted as an in-patient and advised for immediate surgery.
Subsequent to assessment of risk factors and initial blood-work, the patient was scheduled for open laparotomy and surgical bezoar removal five days later. After sterile precautions were taken and appropriate draping was performed, an upper midline incision was made, the muscle layers were retracted, and the stomach was opened between two vicryl sutures. A large trichobezoar was removed, which had taken the exact shape of the distended stomach with a small extension into the duodenum. At the time of removal, its weight was 1.2 kg (Figure ). The stomach was sutured, and the abdomen and skin were closed in layers. With an unremarkable postoperative stay, the patient was discharged at the sixth postoperative day, with a follow-up visit scheduled in the surgery clinic. |
pmc-6153090-1 | A 68-year-old male with past medical history of ESRD on peritoneal dialysis, hypertension, hyperlipidemia, seizure disorder, left craniectomy was sent from his nursing home in 2013 for abdominal pain, decreased appetite, lethargy, and low oxygen saturation. Examination revealed a cachectic male with tender distended abdomen. Peritoneal dialysis catheter was visualized and the site was clean. He was suspected to have subacute bacterial peritonitis secondary to peritoneal dialysis and was empirically treated with vancomycin and cefepime. Computed tomography (CT) of the abdomen and pelvis showed a small amount of free fluid in the peritoneal cavity (Figure ).
However, the peritoneal fluid analysis did not grow any organism. Peritoneal dialysis was temporarily discontinued and a temporary hemodialysis catheter was placed. He was later discharged on peritoneal dialysis after his symptoms improved.
The patient presented with similar complaints in 2014 and a single-photon emission computed tomography (SPECT) gallium scan revealed abnormal activity in the right lower quadrant of abdomen and pelvis, suspicious for peritonitis. CT abdomen showed pneumoperitoneum and ascites, peritoneal fluid again did not grow any organisms. Due to elevated leukocyte count and fever, the patient was empirically treated with antibiotics. He was readmitted in 2015 with similar complaints when interventional radiology (IR) guided hemodialysis catheter was placed and peritoneal dialysis was permanently discontinued.
The patient presented two years after the discontinuation of peritoneal dialysis with massive abdominal distension, abdominal pain, and vomiting. He was receiving hemodialysis at this time. Repeat CT scan of abdomen and pelvis revealed massive abdominal and pelvic ascites with encapsulated complex pseudocyst arising from the peritoneal membrane impinging on the liver (Figure ).
Ultrasound of the abdomen and pelvis showed multiple fluid-filled loculations present within the cyst (Figure ).
The pseudocysts were suspicious for malignancy; however, IR guided drainage revealed chocolate brown fluid that was negative for malignant cells or organisms.
He presented three more times in the following four months with similar complaints. Surgical management with pseudocyst removal was considered but deferred due to the loculated nature of the cyst and patient’s comorbidities. Medical management with peritoneal drainage drained large amounts of fluids although the procedure was limited by the loculations. The abdominal swelling decreased and the patient experienced temporary relief after every drainage. CT guided subcutaneous peritoneal drainage catheter was temporarily placed and the patient continued to improve. He continues to have occasional episodes of abdominal distension secondary to fluid collection in the pseudocysts although the frequency of recurrence drastically reduced. |
pmc-6153093-1 | A 24-year-old African American male with no significant past medical history presented to the emergency department with a five-day history of severe weakness, generalized muscle pains, and decreased urine output. He denied any trauma, exertional activity, or prolonged immobilization. He also complained of a sore throat and mild diarrhea. He was taking naproxen 375 mg twice a day, prescribed for generalized pain four days before the admission. He denied any sick contacts or illicit drug use but admitted to being sexually active with multiple male partners. Physical examination was unremarkable except for oral thrush and reduced muscle strength in all extremities.
On initial laboratory investigations, serum creatinine, phosphorus, calcium, and uric acid levels were 5.7 mg/dL, 11.9 mg/dL, 5 mg/dL, and 13.6 mg/dL, respectively. Urine myoglobin was positive and creatine phosphokinase (CPK) was more than 200,000. The HIV-1/2 Antigen/Antibody screen was positive, Western blot was negative for both HIV 1 and 2 antibodies, HIV-1 ribonucleic acid polymerase chain reaction was detected above 10,000,000 copies/mL and CD4 count was 170 cells/mm3, all of which were consistent with early HIV infection before seroconversion. Serological tests for hepatitis B, hepatitis C, cytomegalovirus, Epstein-Barr virus, and urine drug screen were all negative. The patient developed anuria and fluid overload and was dialyzed through the right internal jugular catheter. He was started on a regimen of abacavir, lamivudine, ritonavir, and darunavir. About five days after the start of antiretroviral therapy, the patient’s symptoms and his CK level improved significantly (Figure ). His urine output gradually increased and he was discharged home with scheduled outpatient dialysis. |
pmc-6153094-1 | A 26-year-old female patient was admitted for a one-month-old abdominal pain in February 2012. The initial evaluation was consistent with a 10-cm type II hydatid cyst in the liver, and she was, therefore, referred to our center. Abdominal computed tomography (CT) in our center revealed a 17x12x17 cm mass with solid and cystic components in the left lobe of the liver (Figure ).
The tumor size and the location and cystic nature of the mass suggested biliary cystadenocarcinoma as a possible diagnosis. A biopsy of the mass showed an inflamed myxoid stroma with necrosis and fine needle aspiration cytology of the cystic fluid revealed necrosis with minimally atypical glandular epithelial cells.
The patient’s physical evaluation was normal, except for the palpable mass in the epigastric region. Laboratory findings, liver function tests, and tumor markers (CA 19-9, CEA, and CA-125) were all normal.
The patient's surgical procedures and outcomes were as follows: nonanatomic liver, cholecystectomy, and extrahepatic biliary tract resection were performed. The right lobe anterior segment hepatic duct was anastomosed to the jejunum in Roux-en-Y fashion. The right lobe posterior segment hepatic duct was anastomosed to the common bile duct.
Bile leakage developed after the operation. Postoperative magnetic resonance imaging and magnetic resonance cholangiopancreatography revealed a leakage from the hepaticojejunostomy. Furthermore, intrahepatic bile duct dilatation was observed secondary to stenosis in both anastomoses (Figure ). The patient was referred to the interventional radiology unit. First, the infected bile collection was drained under ultrasonography and fluoroscopy guidance. Following the resolution of the collection, percutaneous transhepatic cholangiography (PTC) was performed. PTC revealed that the leakage was healed. Eight FR external biliary drainage catheters were placed for each anastomosis separately (Figure ). Next, a guide wire was inserted through the stenosis at the hepaticojejunostomy site and balloon dilatation was performed. An internal-external biliary drainage catheter was placed in this position (Figure ). However, stenosis located at the anastomosis between the right lobe posterior hepatic duct and the common bile duct could not be managed percutaneously. Therefore, open surgery was planned. Under intraoperative fluoroscopy, a guide wire was directed through the patients’ external biliary drainage catheter. This wire was guided to puncture the biliary tract proximally from the stenosis and through the liver parenchyma into the abdomen. The blind loop of jejunum was moved toward the visible catheter and a mucosal graft was applied on the catheter through a small opening made on the jejunum segment. A 10 FR internal-external biliary drainage catheter was placed at the site of this anastomosis (Figure ).
A single drain was placed near the newly created anastomosis. This time, the postoperative period was uneventful, and the patient was eventually discharged successfully. Three months later, control cholangiography revealed that both of the anastomoses were healed perfectly. Both biliary drainage catheters were consequently removed. A postoperative contrast-enhanced CT image is presented in Figure .
The excision specimen consisted of a 25x19x10 cm well-circumscribed mass with small remnants of non-neoplastic, non-cirrhotic hepatic parenchyma at the periphery. The lesion was gray-tan in color and was made up of multiple areas of cystic degeneration, fragile fibrous septae, areas of hemorrhage, and myxoid change (Figure ). Necrosis was not prominent on a macroscopic examination. A microscopic examination revealed widespread cystic degeneration, hemorrhage, and necrosis along with small hypercellular nodules and myxoid areas harboring neoplastic cells. Neoplastic cells displayed moderate pleomorphism and nuclear hyperchromasia. Although the lesion seemed well-circumscribed macroscopically, microscopy showed tumor cells infiltrating between hepatocyte cords (Figure ). Immunohistochemically, neoplastic cells were strongly and diffusely positive for CD56 (Figure ) and focally positive for vimentin (Figure ), CD10, WT1 (cytoplasmic), Factor VIII, and desmin (Figure ). Pancytokeratin (CK) (Figure ), ER, PR, CD31, lysozyme, smooth muscle actin, CD68, myoD1, HepPar1, and CD34 were negative in tumor cells. Rare PAS-positive, diastase-resistant, intracytoplasmic material was detected in some neoplastic cells (Figure ).
The proliferation index (PI), as demonstrated by Ki67 immunohistochemistry, was approximately 30% (not shown). In some cells that lined the multicystic-degenerated areas, rare intracytoplasmic mucin droplets were noted. These cells expressed CK19 and were considered to be remnants of trapped bile duct elements (not shown).
After surgery, the patient received the taxol-cisplatin-ifosfamide chemotherapy protocol (Table ). The patient is currently disease-free after six years of follow up. |
pmc-6153392-1 | A 25-year-old woman presented to the ENT clinic on April 2017 with complaints of generalized weakness and difficulty walking due to progressive worsening of leg pain. The leg pain had started several months before and was initially mild at onset, however, now caused the patient significant pain. Her past medical history included Bartter’s syndrome (diagnosed at age two), ESRD (2014), secondary hyperparathyroidism, previous pulmonary embolism (2016) and anemia of chronic disease. She was currently taking iron, thiamine, zinc, vitamin c, mag-ox, aspirin, lovenox and KCl. On physical examination- height was 158 cm, weight was 80.5 kg, vital signs were within normal limits. No other significant findings were noted.
The PTH level was 1849 pg/mL during the initial visit, therefore a Sestamibi scan was performed, however the scan did not show a localized adenoma. shows a Sestamibi scan for a patient with secondary hyperparathyroidism.
The patient was immediately scheduled for resection of all four parathyroid glands based on several factors; including her PTH level, complaints of generalized weakness and worsening bone pain. The procedure was performed under general anesthesia, with no complications. Sections of the parathyroid glands were taken and sent for pathology. The PTH level intraoperatively was noted to be 71 pg/mL. The patient was then admitted to the ICU in stable condition following surgery. Goals that were set for the patient following surgery- 8.0–8.5 mg/dL in serum calcium and 4.5 mg/dL for ionized calcium.
On postoperative day (POD) one, the nephrologist was put on consult and became part of the patient’s care team till her discharge. They helped to determine daily amount of calcium administered and whether any adjustments were needed. Other recommendations made by the nephrologists: daily weights, hemodialysis twice per week, vitamin D supplements, and close observation of magnesium and phosphorus levels.
The development of hypocalcemia in the patient was rapid and progressive. On POD 1, the patient had a PTH level of 12 units/l, serum calcium level of 7.3 mg/dL and an ionized calcium of 2.9 mg/dL. However, she was asymptomatic. POD 2, the patient was stable and still in ICU, but complained of pain at the intravenous (IV) site on her right hand.
She was scheduled for hemodialysis with high-calcium bath twice a week. The patient’s weight decreased 25 kg (from 80.5 kg to 55.4 kg) within a span of four days post-surgery. The baseline weight of the patient being unknown it can be assumed that the 80.5 kg was substantially higher than her norm and could potentially be attributed to the patient’s missed dialysis sessions as well as the bilateral lower extremity edema. Once the patient was able to tolerate oral calcium supplements she was transferred from the ICU to the internal floor. The patient received 11.37 g of elemental calcium, consisting of 27.5 g of calcium carbonate and 4 g of calcium gluconate, on her first day of oral calcium replacement. The concentrations of elemental calcium increased substantially thereafter, from 11.37 g to 17 g → 30 g → 35.9 g. The highest amount of calcium was provided on POD 11 where 28 500 (x6) g of calcium citrate was given to the patient, resulting in 35.9 g of elemental calcium. During the 11 days of treatment her total serum calcium maintained at an average of 7.8 mg/dL and ionized calcium at 4 mg/dL, while these levels are considered low they did not indicate the indicate to start the patient on intravenous calcium. illustrates the patient’s changes in serum/ ionized calcium and PTH levels. The amount of calcium which this patient received has never been reported or recommended for any patient with HBS. The current recommendations are between 6–12 g/day []. The patients POD 7 laboratory values are presented in .
The treatment was stopped abruptly due to the patient’s request. She was discharged with 2 days of medication and given prescription for another 14 days. The importance of maintaining her bi-weekly dialysis appointments was discussed with the patient, where her serum calcium, ionized Ca, BMP and Alkaline phosphatase will be closely monitored. Calcium levels on discharge were - serum calcium of 9.5 mg/dL and ionized calcium of 4.9 mg/dL. Had the patient not requested discharge, the medical team wanted to continue the ongoing treatment plan. That plan involved providing the necessary amount of calcium, closely monitoring her serum levels as well as her weight for an additional few days (, ).
The discharge medications included: calcium citrate 30 g/QID; calcitriol 5 mcg/daily; calcium citrate 200 mg/QID; epoetin alfa 10 000 units/mL. The oral calcium supplement (ca citrate) was prescribed to help increase and maintain the serum calcium levels. Epoetin alfa is a synthetic erythropoietin analog, which stimulates the bone marrow to produce more red blood cells and calcitriol was prescribed to function as an active form of Vitamin D. |
pmc-6153900-1 | A 47-year-old woman visited our outpatient clinic complaining of progressive blurred vision in both eyes. In 1994, she had undergone refractive surgery for RK in both eyes at the age of 25 years, and her preoperative refractive error values were −6.5 D in the right eye and −8.5 D in the left eye. Her uncorrected visual acuity was 0.4 logMAR in both eyes, and her autorefraction (Topcon RM6000, Japan) was +3.75 DS/−1.50 DCX129° in the right eye and +4.00 DS/−1.25 DCX82° in the left eye. Her best corrected visual acuity (BCVA) with spectacle correction of +3.50 DS/−1.50 DCX130° in the right eye and +3.25 DS/−1.00 DCX80° in the left eye was 0.2 logMAR in the right eye and 0.3 logMAR in the left eye. Her keratometric readings were 35.75 D/36.75 D at 74° in the right eye and 35.25 D/36.25 D at 61° in the left eye.
Slit lamp examination revealed a clear cornea with eight radial incisional scars in both eyes; both lenses were clear. Her optic zone after the RK procedure measured 2.4–2.5 mm in the right eye and 2.2–2.3 mm in the left eye. A fundus examination was unremarkable. The intraocular pressure was 19 mmHg in both eyes. The corneal topography of both eyes was obtained using the Oculus Pentacam (Oculus Optikgeräte GmbH, Wetzlar, Germany; Figs. , ); corneal central flattening with refractive powers of 27–30 D was noted (Figs. , ). The patient was therefore diagnosed as having hyperopia with astigmatism following RK.
Prompted by an intolerance of glasses, the patient asked for treatment using contact lenses. At first, we applied an RGPCL. However, we noted marked decentration and poor fitting due to central corneal flattening, because the lens did not have a sufficiently flat base curve. Next, we applied a conventional PSSCL, which is thick in the center and can therefore correct hyperopia and low-grade astigmatism simultaneously. The conventional PSSCL showed slightly inferior decentration, with good movement, and the patient was satisfied with her corrected visual acuity. Subsequently, after ascertaining the patient’s living habits, we decided that a daily disposable contact lens would most meet her needs. The final prescription was a Johnson & Johnson® daily disposable PSSCL (+3.50 D/base curve, 8.5 mm/diameter 14.2 mm in the right eye; +3.75 D/base curve 8.5 mm/diameter 14.2 mm in the left eye). The patient was satisfied with her corrected visual acuity of 0.0 logMAR in the right eye and 0.0 logMAR in the left eye. The daily disposable PSSCL-corrected visual acuity was stable during the 10-month follow-up period. |
pmc-6154401-1 | A 62-year-old man was referred to our clinic mainly for central visual disturbance and metamorphopsia in his right eye lasting more than 2 months. The patient had no significant history of systemic disease other than hypertension. At his initial visit, the best corrected visual acuity (BCVA) was 20/29 in his right eye and 20/17 in his left eye. The vertical and horizontal M-CHARTS (Inami & Co., Ltd., Tokyo, Japan) scores were 0.9° and 0.5° in the right eye, respectively. No distortion was detected by M-CHARTS in the left eye. A slit-lamp examination of the anterior segments revealed mild cataracts (grade I according to the Emery-Little classification) in both of his eyes. A fundus examination of his right eye showed a slightly reddish macula with a macular hole-like conformation (Fig. ).
As described in a previous report [], the morphological features of the macula were identified by utilizing two swept-source OCT (SS-OCT) imaging methods (DRI OCT-1 Atlantis, TOPCON Corporation, Tokyo, Japan): radial B-scan imaging and en face imaging (Fig. ). The radial B-scan image confirmed the presence of partial-thickness defect of the macula with degenerative retinal cleavage as well as LHEP at the surface of the retina (Fig. ). The central retinal thickness was 156 μm, and the ellipsoid zone was almost continuous but showed an irregular reflection intensity. En face imaging revealed a membrane structure on the macula, but no retinal fold was observed (Fig. and ). There was a retinal cleavage from the level of the ILM to the level of the outer nuclear layer (Fig. ). Based on these findings, we diagnosed the patient as degenerative LMH with LHEP.
To treat the case patient, we performed both LHEP embedment into the retinal cleavage as well as ILM inversion. Briefly, after performing phacoemulsification with intraocular lens implantation and a 25-gauge micro-incision vitrectomy, the LHEP was centripetally peeled off of the retina using intraocular forceps and was left attached to the edge of the LMH (Fig. , and ). After trimming the LHEP using a vitreous cutter (Alcon Laboratories, Inc., Fort Worth, Texas) to fit the size of the retinal cleavage, the remnant LHEP was gently massaged centripetally over the LMH and thus embedded into the retinal cleavage (Fig. , and ). Next, the ILM was visualized with indocyanine green and then peeled from the periphery towards the LMH. During this peeling, the ILM was not completely removed from the retina but was instead left attached to the edge of the LMH. The ILM was then inverted from upper to lower so that it completely covered the LMH with the embedded LHEP (Fig. and ) []. During the ILM inversion, 1% low molecular weight hyaluronic acid (Opegan; Santen Pharmaceutical Co. Ltd., Osaka, Japan) was used to control the direction of the ILM flap inversion. Thereafter, sodium hyaluronate-chondroitin sulphate (Viscoat; Alcon Laboratories, Inc., Fort Worth, Texas) was placed on the inverted ILM in order to stabilize the flap. At the end of surgery, fluid-air exchange was performed, and the vitreous cavity was filled with 20% SF6 gas. Small amount of these viscoelastic substances (also known as ophthalmic viscosurgical devices) were intentionally left on the inverted ILM flap. The patient remained in the prone position for 3 days after the surgery. [See Additional file 1].
One month after the surgery, both B-scan and en face OCT images showed that most of the retinal cleavage had disappeared (Fig. ). B-scan imaging showed the presence of embedded LHEP and inverted ILM, although it was difficult to distinguish these two from each other because they appeared to be integrated (arrowheads, Fig. ). Three months after the surgery, the foveal contour had further improved (Fig. ), and the ellipsoid zone had recovered (Fig. , arrows). Furthermore, BCVA had improved to 20/20 and the vertical and horizontal M-CHARTS scores in the right eye had improved to 0.8° and 0°, respectively. |
pmc-6154412-1 | A 47-year-old previously healthy male patient presented with a paroxysmal and progressively scrotal ache and a bilateral inguinal region with a burning sensation. He was admitted to the local hospital 4 h after onset. During admission, a suspected diagnosis of scrotitis was made, and the patient then underwent emergent and extensive surgical debridement. Past-operative histology showed chronic suppurative inflammation. However, despite the treatment, the skin of the scrotum continued to necrotize, and he was transferred to our hospital immediately. Physical examination showed a total scrotal skin defect, extensive left and right inguinal region skin defects (4 × 10 cm and 5 × 15 cm, respectively) and a purulent necrotizing tissue covering, which was accompanied by swelling and erythema of the surrounding skin. Bilateral testes were exposed. Five drainage tubes were visible(Fig. ). The patient remained afebrile with stable vital signs. Blood chemistry showed a white blood cell count of 10.35 × 10ˆ9 /L (neutrophil percentage 86.5%), a red blood cell count of 1.18 × 10ˆ9 /L, an albumin count of 26.8 g/L, and an erythrocyte sedimentation rate count of 84 mm/h. An ultrasound scan showed that the necrotic scrotal wall was thickened with edema, internal echo heterogeneity, a hydrocele of the right tunica vaginalis, and normal blood supply to the testes and epididymis. Cultures of the exudates from the scrotal wound grew Pseudomonas aeruginosa. The admission diagnosis was perineum necrotizing fasciitis. There was no history of trauma or symptoms of dysuria or haematuria. The patient had no history of diabetes, high blood pressure, or other chronic diseases; his past surgical history was unremarkable, and he was not on any regular medications.
After hospitalization, comprehensive therapy was managed by the patient. The patient then presented with fugacious pyrexia (37.8 °C), further erythema and swelling that spread to the right lower abdominal wall, which was hardened on palpation 5 days after admission. Because the patient’s condition had worsened, immediate surgical debridement was necessary. Histology confirmed the preoperative diagnosis. Cultures of exudates from the scrotal wound grew Pseudomonas aeruginosa and Klebsiella pneumoniae, and the antibiotic treatment was adjusted according to the drug sensitivity results. On the 14th day of admission, the erythema and swelling continued to extend to the right outside the groin region and 2 cm down to the perianal region. There was no other discomfort. Although the lesion might have extended, we continued to strengthen the conservative treatment rather than use surgical debridement. After extensive drug-resistance developed, the antibiotic was withdrawn and conventional moist gauze therapy was applied after a shower once or twice daily. When the wound bed had been prepared, STSGs from the patient’s scalp were grafted to the scrotum, perineum, inguinal region, and perianal region. We gave the patient intravenous Cefuroxime for 3 days after his STSG, to which he was sensitive. To secure the STSGs and improve graft survival, a vacuum sealing drainage dressing (WEGO) was placed to cover the wound and the anus, and a negative sucker was placed upon the anus for 5 days. While using the NPWT, the patient did not defecate but passed gas normally (Fig. ). For economy, NPWT was used only after STSGs to help with wound healing. During this time, the patient did not complain of any pain or bleeding, which are common complications of NPWT. The dressing was changed every 3 days, after which the patient was able to defecate normally. After the grafted skin had survived, semi-exposure therapy was continued to facilitate wound healing. The patient had a urinary catheter placed throughout the treatment process.
After the treatment, graft take was 95%; local flaps were then grafted to cover the residual wound and the testis. The wound had decreased to two pea-sized areas (Fig. ). The patient was discharged and received conventional moist gauze therapy to close the residual wound at his local hospital. A follow-up by telephone 1 month later showed that both wounds had healed and that the patient was satisfied with the outcome. |
pmc-6154430-1 | A 38-year-old Gravida 3 lady at 36/6 weeks of gestation presented to our labor and delivery unit for a renal colic. Her prenatal course was unremarkable. She had no past surgical or medical history, but she was presenting acute pelvic pain, related to renal colic. Her vital signs were as follows: blood pressure 115/68 mmHg, HR 73 bpm, respiratory rate 17 breaths per minute, and temperature 36.7 °C. Her physical exam was unremarkable. Maternal baseline ECG was normal. Baseline electrolytes and maternal thyroid panel were within normal limits. There was absense of fever and of blood and urine infection indexes. After ruling out threatening labor, fetal ultrasound showed grossly normal fetus with an estimated weight of 2,783 g and amniotic fluid index 14.6 cm. The anatomical survey including fetal cardiac evaluation was found to be within normal limits. The 4-chamber view of the heart was normal. No signs of hydrops fetalis were noted. Electronic fetal HR monitoring showed a sustained baseline fetal heart of 140 bpm with minimal to moderate variability, with absent accelerations or decelerations and not relevant uterine contractile activity. Middle cerebral artery Doppler ultrasonography was also within normal.
Spasmolytic therapy (Butylscopolamine) did not resolve colic pain. The following day, although her physical exam was unchanged, a plan was made for administration of betamethasone series for lung maturity and morphine for colic pain control. Immediately after morphine intravenous administration (0.1 mg/kg), electronic fetal HR monitoring showed a sudden sustained increase of baseline fetal heart from 140 bpm to over the upper range limits cardiotocography (CTG) registration (200 bpm). Fetal echo on M mode revealed a 1:1 atrial ventricular rate of 240 bpm consistent with SVT (Fig. ). Conversely, heart rate, blood pressure, respiratory rate, and temperature of the mother were substantially unchanged.
After 4 h fetal HR was sustained at 240 bpm. Since the fetus was at a reasonable maturity stage, immediate delivery was decided. A baby boy was delivered following a caesarean section, with a 2920 g birth weight, APGARs of 10 and 10 at 1 and 5 min, umbilical artery cord blood pH 7.37, BE-1.7. He was admitted to the regular nursery. Pre-ductal saturation was 99%. Mean blood pressure 38 mmHg. Tachycardia was noticed at birth 240 bpm, and the HR progressively decreased starting at 60 min after birth and reached normal levels during the following 3 h.
Postnatal adaptation was uneventful and exclusive breastfeeding was carried on. After a 2-days period of neonatal cardiac monitoring (including echocardiography) the mother and her baby were discharged home in stable condition. The neonate progressed well. His weight increased normally and there was no evidence of cardiac rhythm disturbance up to the 1-year follow-up as outpatient in the pediatric cardiology department. |
pmc-6154744-1 | A 75-year-old man was admitted to our hospital on June 15, 2016 with a chief complaint of coughing and blood-stained sputum for over 1 month. He presented chest stuffiness and shortness of breath and required continuous oxygen inhalation. The patient also presented coronary arteriosclerosis. There were no symptoms of dizziness, chest pain, fever, and vomiting. He had a smoking history of over 60 years and smoked up to 30 cigarettes a day. Tumor markers carcinoembryonic antigen and neuron-specific enolase were within the normal range. Chest computed tomography (CT) revealed a soft mass (63 × 48 mm) and signs of pneumonia in the left lung (Figure ). Swollen lymph nodes above the left clavicle were evident on color Doppler ultrasound images. CT-guided lung biopsy was conducted. The mass was histologically diagnosed as sarcomatoid carcinoma, which was staged as IV according to the 8th edition of the AJCC/UICC TNM staging system for lung cancer. Supraclavicular lymph node metastasis was detected. Immunohistochemistry showed that tumor cells were positive for cytokeratin (CK) 7, CK8, and thyroid transcription factor 1 (TTF-1) and negative for CK5/6, p63, p40, CD56, and synaptophysin. Of note, the tumor was abundantly vascular, with strong immunostaining for smooth muscle actin (SMA), CD31, and CD34 (Figure ). As determined by next-generation sequencing, this patient displayed 4 mutations in NF1 (p.Q347Sfs*29), CDKN2A (p.G23V), ERBB3 (p.V104L), and TP53 (p.V157F) genes (Table ).
The patient had an ECOG performance status of 3 and thus was precluded from chemotherapy. After receiving informed consent, he was given apatinib (250 mg) orally once per day from June 27, 2016. Haemoptysis symptoms disappeared and chest stuffiness was relieved 5 days after apatinib treatment. At 10 days after treatment, he restored normal breathing. Follow-up CT at 1 (Figure ), 3 (Figure ), and 9 (Figure ) months showed that the tumor regressed to 54 × 43 mm, 41 × 40 mm, and 36 × 28 mm, respectively. Until drafting the manuscript (14 months after therapy), tumor regression was observed. There was no evident complication associated with apatinib therapy. |
pmc-6154794-1 | A 3-year-old male patient (case 17) was diagnosed with speech delay. He started to walk in his first year and began speaking by using three words at two and half years of age. At neurological examination, head circumference was 48 cm (10th percentile), height was 93 cm (10th–25th percentile), and weight was 13 kg (10th–25th percentile). Hypertelorism, broad nasal bridge, micrognatia were observed. Hyperactivity and poor eye contact were detected. No pyramidal system involvement was present, and reflexes were normoactive. Cerebellar system examinations were normal. Serum electrolytes, electroencephalography, and abdominal ultrasonography findings were within normal limits. He had stereotypical behaviors, deficits in communication, and autism spectrum disorder, which was diagnosed at 3 years of age. Denver developmental screening test showed one-year delay in speech and social skills. CMA revealed 2.9-Mb de novo deletion at 18q22 region. RTTN, SOCS6, CBLN2, NETO1 genes were located at the deleted region. |
pmc-6154823-1 | A 56-year-old male patient with no known comorbidities presented to the emergency department with history of fever and headache for past 2 days with sporadic episodes of projectile vomiting, irrelevant talk, and poor sensorium for a day. On admission, the patient manifested fever (39 °C), a heart rate of 104 beats per minute, blood pressure of 130/60 mmHg, respiratory rate of 30/min, and capillary blood glucose of 140 mg/dl. His Glasgow Coma Scale (GCS) score was 9, with respective scores of 5, 2, and 2 for motor, eye, and verbal responses. There were no signs of meningeal irritation or focal neurological deficit. Clinical examination was otherwise normal except for a scar over the anterior bi-frontal region which was a surgical incision. On further inquiry, he had an accidental fall from a two-story building 23 years ago, causing a persistent CSF leak from the nose (rhinorrhea). He underwent complete repair for the CSF leak without any residual defect. There were no further hospitalizations for the past 23 years with symptoms suggestive of meningitis.
His computerized tomography (CT) scan showed cystic gliosis changes in the left frontal lobe which was communicating with the frontal horn of ipsilateral lateral ventricle. CSF analysis of the patient revealed hypoglycorrachia (< 20 mg/dl with corresponding blood sugar of 140 mg/dl), elevated protein > 300 mg/dl, and neutrophil-predominant pleocytosis (total cells 360/mm3 with 96% polymorphs) suggestive of acute pyogenic meningitis. He was started empirically on ceftriaxone 100 mg/kg/day in two divided doses, amoxycillin 100 mg/kg/day in four divided doses, and acyclovir 45 mg/kg/day in three divided doses. Initial peripheral blood WBC count was 14,760 with 85% neutrophils and serum lactate levels of 5.35 mmol/l. His renal and liver function tests were normal. Antibiotics (ceftriaxone, amoxycillin) were continued, and acyclovir was stopped as there were filamentous gram-negative rods in gram staining of the CSF, which was a rare morphology to be identified (Fig. ). Further, the sample was plated onto blood agar, MacConkey agar, and chocolate agar and incubated aerobically at 37 °C. Non-hemolytic colonies were formed on blood agar (Fig. ) which were lactose-fermenting colonies on MacConkey agar (Fig. ). The organism was identified to be Escherichia coli by Vitek 2 Compact (Biomerieux), and spectrum of antibiotic sensitivity was described in Table .
In accordance with the culture and sensitivity pattern, ceftriaxone was continued and amoxycillin was stopped. He was afebrile for the next 48 h, and his sensorium improved. Further work up was done to identify the source of seeding of E. coli to the meninges. Urine culture and blood cultures that were sent prior to initiation of antibiotics did not show any bacterial growth, and stool microscopy was negative for ova and cysts. CT scan of the abdomen revealed a non-obstructive renal calculus of 6 mm × 6mm at interpole of the left kidney without any signs of pyelonephritis. After the initial improvement, patient again had a drop in sensorium without any localizing signs. Magnetic resonance imaging (MRI) of the brain with contrast study was done to rule out secondary complications associated with meningitis like vascular infarcts or hydrocephalus. There was post-contrast enhancement of leptomeninges and subtle enhancement of ventricular wall and posterior fossa cisternal spaces in post-contrast T1 sequence, and the same findings along with exudates in the ventricles in T2 fluid attenuated inversion recovery (FLAIR) images, which were suggestive of ventriculitis (Figs. and ). CT cisternogram was performed to check for residual CSF leak at the past surgical site, in which there was an opacified density in the left frontal region representing a porencephalic cyst communicating with the left frontal horn. There was no CSF leak into the paranasal sinuses or nasal cavity (Fig. ).
In the CSF analysis repeated on day 7 of antibiotic therapy, there was improvement in cell count (total cell count of 26 with 100% of lymphocytes) and cultures were sterile. He was discharged from the hospital on day 10 and advised to continue intravenous antibiotics for 6 weeks in view of ventriculitis. On further follow-up, the patient has improved, with no neurological deficit and antibiotics have been stopped at the end of the sixth week. |
pmc-6154829-1 | A 47-year-old male admitted to our department with pain and swelling on his right calf. The patient was diagnosed with RA 5 years ago. He had no history of knee trauma. On physical examination, tenderness of wrists and elbows, swelling on the left wrist and contracture of right elbow were found. McMurray test was negative bilaterally. There was no swelling in the left knee. Right knee flexion was limited and Ballotman test was positive. There was bilaterally knee joint tenderness with palpation.
He had painful swelling and redness on his right calf (Fig. ). There was a persistent itching on the skin of the right calf. Homans test was positive on the right. Plain radiographs showed mild degenerative changes at knee joints. Erythrocyte sedimentation rate was 22 mm/hour, C-reactive protein was 24.7 mg/L, WBC count was 14350, rheumatoid factor was positive. Complete blood count except for WBC and biochemical laboratory tests were within normal limits.
These findings on the calf were similar to deep vein thrombosis. We used ultrasonography for differential diagnosis of deep vein thrombosis. Ultrasonography revealed massive fluid collection within the fascial compartments and gastrocnemius muscle layers (Fig. ). There were two septas between fluid collections. Color Doppler ultrasonography showed normal flow pattern in popliteal veins. According to these findings, the patient was diagnosed as Pseudothrombophlebitis syndrome due to rupture of Baker’s cyst. 280 cc inflammatory fluid was aspirated from three different points under the guidance of ultrasonography. After the intervention, the swelling on the right calf decreased. The patient was relieved and elastic bandage was applicated to his calf. After 1 week follow-up, the swelling on his calf repeated and another intervention was performed. Approximately 100 cc inflammatory fluid was aspirated and 1 cc steroid (betamethasone) was injected into right knee joint. After 1 month follow-up, we found that the pain and redness on his calf was clearly decreased. The patient’s pain visual analog scale (VAS) value was decreased from 8 to 2. We observed that the patient continued to improve at follow-up visit 3 months later; VAS value was 0 and there was no swelling or redness on his calf. |
pmc-6154876-1 | A 75-year-old male patient was referred to our hospital for the detailed investigation of elevated serum hepatobiliary enzymes. The patient had a history of hypertension, type 2 diabetes mellitus (DM) with diabetic nephropathy, malignant otitis externa, and an operative history of emergent coronary artery bypass grafting (CABG) 10 years before for myocardial infarction. During cardiac surgery, the right gastric artery, which was bifurcated from the common hepatic artery, was anastomosed to the posterior descending artery by the ante-gastric route. The height and weight of the patient was 1.62 m and 65 kg, respectively. The performance status of the patient assessed by the Eastern Cooperative Oncology Group was Grade 1. The results of preoperative laboratory testing were as follows: white blood cell count, 5,900 cells/μL; red blood cell count, 478 × 104 cells/μL; serum hemoglobin concentration, 10.3 g/dL; serum platelet count, 16.6 × 104 platelets/μL; serum aspartate aminotransferase, 40 IU/L; serum alanine aminotransferase, 32 IU/L; serum alkaline phosphatase, 639 IU/L; serum gamma glutamic transpeptidase, 297 IU/L; total serum bilirubin, 0.66 mg/dL; serum albumin, 3.91 g/dL; serum C-reactive protein, 0.05 mg/dL; prothrombin time (%), 114%; hemoglobin A1c, 7.6%; indocyanin green retention rate after 15 min, 4.0%. The levels of serum carcinoembrionic antigen and carbohydrate antigen 19–9 were elevated at 2.5 ng/mL and 35.8 U/mL, respectively.
Endoscopic retrograde cholangiography and 3-dimensional computed tomography cholangiography found abrupt narrowing of the common hepatic duct that was 20 mm in length including the right and left hepatic ducts, with involvement of the right anterior and posterior bile duct bifurcations (Fig. ). Adenocarcinoma was evident by the cytological findings, and this was confirmed by brush cytology. Contrast enhanced computed tomography (CECT) showed no distant metastases or lymph node metastases. We diagnosed these findings as Bismuth type 4, T2N0M0 Stage II hilar cholangiocarcinoma. According to the preoperative imaging findings, the left bile duct was completely occluded by cancer, there was narrowing of right hepatic duct and anterior sector bile duct, and only the right posterior bile duct was free from the margin. Therefore, we planned left trisectionectomy, extrahepatic bile duct resection, and Roux-en-Y choledochojejunostomy.
CECT also showed that the right hepatic artery was bifurcated from the supra mesenteric artery, and the left hepatic artery was dominant from the celiac artery. The bile duct, which was involved in the cholangiocarcinoma, and the RPHA were not adjacent (the distance between them was 8 mm) because the pattern of confluence of the right posterior hepatic duct was of the supra-portal type, although the narrowing of the RPHA was 5.6 mm in length. Because this patient had multiple cardiovascular risk factors including a history of CABG, we suspected that the narrowing of the RPHA was a benign stricture caused by atherosclerosis. Arterial imaging with 3-dimensional multidetector computed tomography (MDCT) (Fig. ) and curved planner reconstruction (CPR) of MDCT also showed stricture of the RPHA (Fig. ). We performed digital subtraction angiography (DSA) and intravascular ultrasonography (IVUS). DSA showed stricture of the RPHA (Fig. ), and thick plaques in the arterial intima were evident on IVUS (Fig. ). According to these findings, we diagnosed stricture of the RPHA due to atherosclerosis, and we performed percutaneous balloon arterioplasty followed by bare-metal stent placement to prevent postoperative biliary complications (Fig. ). His clinical course after stent placement was uneventful, and we performed left trisectionectomy, extrahepatic bile duct resection, and Roux-en-Y choledochojejunostomy 51 days after stent placement.
Since this patient developed bile leakage postoperatively, percutaneous drainage was performed. The bile leakage was successfully controlled without stenosis of the choledochojejunostomy (Fig. ), and the patient was discharged 3 months after surgery. Unfortunately, 4 months after hepatectomy, he was re-hospitalized with multiple pyogenic liver abscesses (Fig. ). Klebsiella pneumoniae was identified from the liver abscess, and we performed intensive multimodal treatment for the pyogenic liver abscesses. Although we stabilized the disease (Fig. ), we eventually lost this patient due to liver failure 14 months after surgery. |
pmc-6154888-1 | The proband is a 10-year-old Sri Lankan girl of Sinhala ethnicity born to healthy non-consanguineous parents with two other older, healthy sons. She had an uneventful pregnancy and birth history and there was no family history of ophthalmological or neurological diseases. Although her parents noted delayed development, they had not sought medical attention for this. She started walking unaided at around 9 years and continues to have a significantly unsteady gait. Her hearing was not affected but speech was delayed (first word at 3 years and currently speaking 3–4-word complex sentences). Her parents reported photophobia since infancy.
On examination aged 10 years, her height, weight and occipito-frontal circumference were 132 cm (10th–25th centile), 26 kg (25th–50th centile) and 51 cm (25th–50th centile) respectively. She had no dysmorphic features. A pigmented macule with a serpiginous border (22 cm × 10 cm), which may represent a blaschcoid pattern suggestive of a cutaneous mosaicism, was noted on her right thigh (Fig. ). Neurological examination revealed an ataxic gait, hypotonia, dysdiadochokinesia, intention tremor, horizontal nystagmus and dysarthria, all consistent with cerebellar disease. No pyramidal or extrapyramidal signs were identified. She had bilateral pes planus (Fig. ). Ophthalmological examination revealed her pupils to be fixed and dilated and slit lamp examination revealed bilateral partial aniridia (Fig. ) with a scalloped edge of the irises, normal fundi and impaired pupillary light reflexes. Pupillary membrane remnants were not seen and the cornea and lens were clear. Visual acuity was reduced (bilateral 6/30). Intelligence Quotient (IQ) assessment revealed moderate learning disability with better verbal IQ (verbal comprehension, working memory and processing speed scales) than non-verbal IQ (Test of Nonverbal Intelligence – TONI version 3- score 70). Her short-term memory was within normal limits although she had deficits in long-term memory.
Magnetic resonance imaging (MRI) of the brain revealed cerebellar hypoplasia especially affecting the vermis and white matter changes (Fig. ). Cervical spine x-ray, echocardiogram and ultrasound abdomen were normal. Mutation analysis by region-of-interest targeted sequencing (NM_001168272.1/ENST00000302640 coding exons 46 and 52–56, which encode the region spanning Glu2094 and the entire calcium ion channel domain, respectively) was performed as previously described []. This identified a previously described pathogenic heterozygous variant in the ITPR1 gene, namely c.7786_7788delAAG p.(Lys2596del). The clinically unaffected parents’ DNA have not been tested for this variant due to lack of availability of samples. |
pmc-6154911-1 | A 5-year-old Iranian boy with a neurodegenerative disorder was referred for genetic testing. His clinical symptoms were speech problems, lack of concentration, walking disability at age of 4 years leading to quadriplegia, spontaneous laughing and crying because of hidden seizure, clumsiness, psychomotor delay, and vision deterioration at age of 5 years which could be the consequence of macular dystrophy. Brain magnetic resonance imaging (MRI) and electroencephalogram (EEG) showed bilateral white matter signal change with preservation of white matter (Fig. and Fig. ). There are no available histopathological studies or skin biopsy for this patient.
The organic acids in his urine were determined using gas chromatography–mass spectrometry (GC-MS) of the trimethylsilyl ethers and esters of the ethyl acetate extract from acidified urine after preparation of the ethoxime derivatives. The organic acid in the urine of this patient showed a normal pattern with no evidence for metabolic disorders. There is no evidence for tyrosinemia, glutaric aciduria, methylmalonic aciduria, Canavan disease, propionic aciduria, isovaleric aciduria, and other organic aciduria known in Iran. Neonatal screening, clinical chemistry, and metabolism assays showed a normal pattern and these analyses were unremarkable (Table and Table ).
Our patient’s parents are first cousins and had experienced three gestations, the first one aborted spontaneously before 4 weeks of pregnancy. The second one is the male reported here and the third one is a 2-year-old girl who does not manifest any signs and symptoms yet and seems to be normal. The pedigree is shown in Fig. .
Deoxyribonucleic acid (DNA) was extracted from peripheral blood of our patient and his healthy parents. Whole exome sequencing (WES) was performed on all three samples as following. Approximately 37 mega base pairs (Mb; 214,405 exons) of the Consensus Coding Sequences (CCS) were enriched from fragmented genomic DNA by > 340,000 probes designed against the human genome (Nextera Rapid Capture Exome, Illumina) and the generated library sequenced on an Illumina HiSeq 4000 platform (Illumina) to an average coverage depth of more than 100 × (Table ). An end-to-end bioinformatics pipeline including base calling, primary filtering of low quality reads and probable artifacts, and annotation of variants was applied. All disease-causing variants reported in HGMD® or ClinVar (class 1) as well as all variants with minor allele frequency (MAF) of less than 1% in Exome Aggregation Consortium (ExAC) database were considered. The evaluation focused on exons and intron boundaries +/− 20. All relevant inheritance patterns were considered and clinical information was used to evaluate eventually identified variants. Relevant variants identified by WES were validated by Sanger sequencing in forward and reverse direction. By applying different filtering steps mentioned in the method part we ended up with only two novel variants in MFSD8 and AFF2 genes (Table ).
The c.325_339del (p.Val109_Ile113del) variant in the MFSD8 gene was a previously unreported variant and found to be homozygous in our patient whereas his parents were heterozygote carriers (Table ). Segregation analysis of this variant was done in the affected index, his parents, and relatives. It is a deletion of 15 base pairs (bp), which causes the loss of five residues. The evolutionary conservation of amino acid residues in the region of deletion estimated by ConSurf tool [] is shown in Fig. .
The c.259A>G (p.Asn87Asp) variant found in AFF2 was a hemizygous change in our patient; his mother was also heterozygous whereas his father was negative for this variant. This variant had not been reported in the public databases at the time of this publication (Table ). |
pmc-6154944-1 | A 44-year-old male was referred to our hospital 26 days after an accidental chemical injury in his left eye with 50% sodium hydroxide solution. The patient irrigated his left eye with tap water immediately after the injury and was subsequently treated at a local clinic. At the time of presentation to our hospital, his visual acuity was hand movements in the left eye and 20/20 in the right eye. Slit-lamp examination revealed an inferior corneal epithelial defect involving 2 clock hours along with diffuse corneal epithelial and stromal edema (Fig. ). The patient was treated with 0.3% ofloxacin four times a day, pranoprofen 1% four times a day, 0.1% prednisolone acetate eye drops four times a day, 1% atropine sulfate eye gel twice daily and oral 2000 mg vitamin C per day. The patient was advised to follow-up in our clinic on a weekly basis. The epithelial defect resolved at one week follow up, when the best-corrected visual acuity (BCVA) improved to 20/400. Six weeks after the initial injury, DMD was noted in the inferonasal quadrant on slit-lamp examination (Fig. ) and the BCVA was 20/200. AS-OCT scan confirmed a localized DMD in the inferior quadrant. The detached Descemet membrane was thick and adherent to the underlying iris tissue. The iris was pulled anteriorly (Fig. ). Confocal scanning microscopy failed to detect the corneal endothelium. On the following day, 0.1 ml of 12% perfluoropropane (C3F8) gas was injected into the anterior chamber. However, the detached Descemet’s membrane persisted postoperatively (Fig. and ). The central cornea clarity improved gradually and the BCVA of the left eye recovered to 20/100 and 20/50 at two and four months after the initial injury. |
pmc-6154944-2 | A 28-year-old male presented to our hospital after ocular chemical injury with 50% sodium hydroxide. The patient irrigated his eyes with tap water immediately after the injury and presented an hour later to our hospital. At the time of presentation, the BCVA was 20/40 in the right eye and 20/200 in the left eye. There was extensive corneal edema along with limbal ischaemia (Fig. ). Treatment was commenced in the form of topical levofloxacin 0.5% four times a day, topical pranoprofen 1% four times a day, topical atropine 1% gel nocte and oral vitamin C 2000 mg per day. Five weeks later, slit lamp examination revealed a DMD, which had not been present during the initial examination. AS-OCT showed a localized DMD in the inferior quadrant. Similar to the first case, the detached Descemet membrane was thick, adherent to the underlying iris tissue and pulled the iris anteriorly (Fig. ). Topical prednisolone acetate 1% eye drops and sodium chloride 5% eye drops were administered every 2 h; however, the DMD persisted at the end of one week and one month (Fig. and ). There was severe corneal neovascularization (Fig. ) and the BCVA of left eye dropped to hand movements at one-year follow-up visit. He received penetrating keratoplasty at another institute and the BCVA improved to 20/200 postoperatively. |
pmc-6155969-1 | A 46-year-old woman who had received radiation therapy for anaplastic oligoastrocytoma and who was taking temozolomide presented to our hospital with cough and palpitations. Her vital signs on admission included blood pressure 115/83 mm Hg, heart rate 117 beats/min, body temperature 36.5°C, and SpO2 96% (O2 2 L/min cannula). A 12-lead electrocardiogram showed sinus tachycardia and SIQIII TIII, with negative T in V1–3 (Fig. A). An ultrasound cardiogram (UCG) showed a distended right ventricle, D-shape (+) (Fig. B), moderate tricuspid valve regurgitation, and moderate to severe pulmonary hypertension (tricuspid valve regurgitation pressure gradient max 59 mm Hg). Chest X-ray showed distended pulmonary arteries and interstitial lung infiltrate (Fig. A). Pulmonary embolism was suspected and the patient underwent emergency computed tomography (CT). However, enhanced chest CT showed no signs of thrombus in the major pulmonary arteries, but did show nodular opacities with tree-in-bud pattern (Fig. B). Abdominal CT showed a slightly distended gastric wall with some lymphadenopathies. Laboratory data showed the following: decreased platelets, 71 × 103/μL; activated partial thromboplastin time, 30.7 seconds; prothrombin time-international normalized ratio, 1.19; fibrinogen, 100 mg/dL; and increased D-dimer, 20.08 μg/mL. No signs of infection were detected (white blood count 7450/μL, C-reactive protein 0.24 mg/dL, βd-glucan <2.4 pg/mL). The patient was hospitalized with suspected temozolomide-induced interstitial pneumonia complicated with disseminated intravascular coagulation and right heart failure. Steroid pulse therapy was initiated (methylprednisolone 1 g/d) for interstitial pneumonia, and recombinant thrombomodulin and continuous heparin infusion were administered for her hypercoagulable state.
The patient's condition deteriorated the following morning; her systolic blood pressure decreased to 40 mm Hg and her SpO2 was unmeasurable. She was moved to the intensive care unit (ICU) and intubated, and catecholamine administration was initiated. Her systolic blood pressure was 40 mm Hg and her arterial lactate level increased to 16.1 mg/dL on a regime of noradrenaline 0.3 μg/kg per min, dopamine 10 μg/kg per min, dobutamine 10 μg/kg per min, and vasopressin 2 U/h. However, UCG showed that the D-shape of her heart had worsened, and the patient suffered cardiac arrest. Although we suspected PTTM because of the rapid deterioration of her right heart failure, no definite diagnosis was made at this point, and VA-ECMO rescue therapy was initiated. The VA-ECMO conditions included a 22-Fr drainage catheter from the right femoral vein to the right atrium, and a 20-Fr arterial catheter for the left femoral artery. The pump was started as 2400 rpm and the resulting blood flow was about 3 L/min. After starting VA-ECMO, the patient's blood pressure increased to 80 mm Hg and her lactate level decreased.
The patient's hemodynamic status stabilized on VA-ECMO. She started to be weaned off VA-ECMO and catecholamines the following day, and VA-ECMO was ceased on ICU day 3. The patient underwent gastroesophageal endoscopy on ICU day 4 to screen for possible gastric cancer, as a potential cause of her PTTM, and a gastric tumor (Borrman type IV) was detected (Fig. ). Biopsy showed a poorly differentiated adenocarcinoma with signet ring cells. Her carcinoembryonic antigen (CEA) level was 9.1 ng/mL and CA-19-9 was 44,793 U/mL. The patient suddenly arrested again on ICU day 5 and could not be resuscitated.
Autopsy revealed a gastric tumor in the greater curvature of the stomach and distended bilateral hilar lymph nodes. Microscopic examination of the gastric tumor showed a poorly differentiated adenocarcinoma, and blood vessels filled with the adenocarcinoma. Anti-CEA immunostaining was positive for tumor cells. Although no lung nodular lesions were detected macroscopically (Fig. A–C), histological examination revealed fibrocellular stenosis of the pulmonary arterioles (Fig. D) and fibrocellular intimal proliferation in the pulmonary vessels (Fig. E and F). The lung-infiltrating tumor cells were positive for CEA by immunostaining (Fig. G and H). These findings confirmed a diagnosis of PTTM caused by gastric cancer. Blood vessels in other organs, including the liver, pancreas, uterus, and ovaries, were also filled with tumor cells and showed intimal proliferation. |
pmc-6155972-1 | A 12-year-old female presented with intermittent lumbosacral pain for 2 years and worsening walking instability for half a year. This study was approved by the Institutional Review Broad of Honghui hospital, and the patient provided signed informed consent. She was admitted to the local hospital 2 years ago and underwent lumbar and pelvic frontal and lateral radiographs, which suggested sacral deformity. Over the past 6 months, she presented aggravating sedentary lumbar pain, gait instability, and lumbar tilt. She came to our institution for further medical advice. The patient was in good health before without any trauma or operation history. She was of full-term normal delivery without birth trauma or asphyxia, and her physical and mental developments were appropriate for her age.
Physical examination revealed that the patient had an abnormal gait and asymmetric pelvis, with the left iliac crest higher than the right. Left spinal rotation, scoliosis, and left buttock atrophy could be observed. Motion and sensation of limbs appeared intact, and bilateral physiologic reflexes were normal. X-ray of her spine suggested lumbar scoliosis from L1 to L5 and left sacrum agenesis with 10° Cobb angle (Fig. A). Computed tomography (CT) reconstruction revealed sacral wing agenesis from S1 to S5 with rotational displacement of left sacrum and sacroiliac dissociation (Fig. B). In the standing position, the patient's left sacrum was higher than the right, with obvious pelvis tilt (Fig. C). The diagnosis of congenital sacral agenesis combined with spinopelvic dissociation and spinal scoliosis was made. The preoperative scoliosis research society-22 (SRS-22) scores in terms of function/mobility, pain, appearance, and mental health were 10, 6, 13, and 6, respectively, with an average score of 6.2. The postoperative SRS-22 scores were 18, 14, 16, and 13, with an average score of 13.8. |
pmc-6155973-1 | An 8-year-old girl was admitted to the Emergency Department of our hospital on April 10, 2015 because of accidental aspiration of a plastic bead 7 hours ago. A transient bout of coughing and labored breathing appeared, which spontaneously resolved without subsequent breathing difficulty. She had cough, transient throat wheezing, and intermittent cough. Examination at admission showed that the child was in a general good condition. No throat wheezing was present. There was no cyanosis and the inspiratory 3-concave sign was negative. The child had no breathing difficulty and hoarseness. Bilateral lung sounds were coarse on auscultation and chest expansion was largely symmetrical. No slap sound by a foreign body was heard. Chest computed tomography scan revealed a round shadow 1.2 cm in diameter in the right main bronchus, but was otherwise unremarkable. A diagnosis of foreign body in the right main bronchus was entertained.
Emergency bronchoscopy was carried out upon admission, and the child underwent rigid bronchoscopy (STORZ) under general anesthesia for retrieval of the foreign body. A blue plastic bead was visualized directly under the rigid bronchoscope and was found to be closely impacted on the opening of the right main bronchus (Fig. ). The bead was 1.0 cm in diameter with a central hole 1 mm in diameter and was not readily mobile and cannot be retrieved by forceps. A fiberoptic bronchoscope (Olympus BF-P260, Olympus medical corporation, Tokyo, Japan the external diameter 4.0 mm) was advanced through the laryngeal mask airway for re-exploration and a balloon-tipped catheter (lacrosse balloon catheter 4.0 mm × 20 mm) was entered at the same time via the maneuvering channel. The balloon was advanced through the central hole in the plastic bead and released and inflated after passing beyond the hole. The inflated balloon was 4 mm and after it was secured, it was pulled out along with the plastic bead (Fig. ). The child had an uneventful recovery.
We searched PubMed for published cases of bronchial aspiration of plastic beads using the keywords “bead,” “endobronchial foreign bodies,” “foreign body bronchus,” “Fogarty catheter,” and “balloon.” A total of 4 cases (including our current case) of bronchial aspiration of plastic beads published between January 1975 and November 2017 were identified in PubMed (Table ). One report described the retrieval of the foreign body by surgery in a child in a critical condition.[ Their age ranged from 6 to 12 years. These children had varied manifestations. Chest X-ray findings were positive in most patients, including atelectasis, hyperventilation, and shadow of a foreign body. Except in the surgical case, all the plastic beads were removed using the “balloon bead technique.” Three cases underwent successful retrieval of the foreign body on the first attempt. In the remaining child, the balloon was improperly positioned and improper force was used to pull out the balloon, leading to rupture of the balloon. The plastic bead was retrieved on the second attempt under C-arm guidance. All the children recovered uneventfully after foreign body removal.[ |
pmc-6156004-1 | A 44-year-old right-handed man visited the clinic because of radial-side pain associated with the left wrist, which was aggravated during the previous 10 days. Pain in the left wrist started 4 months ago, during exercise in a gym. He reported that he was self-employed and played golf and underwent weight training. With regard to the wrist pain, the patient was diagnosed with de Quervain's disease at another clinic, and was treated with physical therapy, nonsteroidal anti-inflammatory drugs and steroid injections twice, with temporary pain relief. The patient had no evidence of systemic diseases or trauma history.
Upon examination, the patient's wrist showed mild swelling on the radial side, severe tenderness associated with the radial styloid process and moderate tenderness on the mid-dorsal aspect of the radio-carpal joint, which was occasionally swollen and inflamed. He yielded positive results in Finkelstein's test and displayed limited range of motion of the left wrist. Motion of the thumb and wrist aggravated the pain. The left-wrist grip strength was 18 kg (29 kg in the right) and thumb pinch strength was 5 kg (8 kg in the right). The visual analog scale (VAS) score for pain was 10 at rest. Initial plain left-wrist radiograph showed no abnormalities. Magnetic resonance imaging (MRI) of the left wrist at another clinic suggested mild tenosynovitis at the third extensor compartment and intersection syndrome. However, the clinical symptoms failed to match the MRI findings. Therefore, we clinically diagnosed him with de Quervain's disease and focal synovitis of radio-carpal joint. We decided to perform diagnostic wrist arthroscopy for the radio-carpal joint and retinacular release of the first extensor compartment for de Quervain's disease. Wrist arthroscopy using standard portals under general anesthesia yielded the following arthroscopic findings: dorsal synovitis, scapho-lunate and lunato-triquetral instability. Upon completion of the arthroscopic procedure, a zig-zag skin incision was made on the radial styloid process (Fig. ). The superficial radial nerve was isolated followed by volar retraction and release of the retinaculum of the first extensor compartment. The APL tendon presented in 3 slips and the condition of APL tendon was not bad relatively, although we failed to find EPB tendon. Therefore, we incised the skin more distally, followed by location of another tendon ulnar to the APL tendon at a more distal site, separated by another retinaculum (Fig. ), which was released. The operative findings revealed stenosing tenosynovitis with partial tearing (Fig. A). We initially considered the tendon to be the EPB tendon. However, the course of the tendon was unusual, warranting a further skin incision proximally, but not to the Lister's tubercle, to trace the course of the tendon. Hard bony protrusion instead of a septum separated the 2 tendons clearly (Fig. B). We partially removed the hard bony protrusion. Retraction of this tendon extended the thumb interphalangeal joint, suggesting that this tendon was the EPL tendon instead of EPB tendon.
Upon completion of the operation, we reviewed the MRI of the patient. It revealed that the oblique EPL tendon originated from the ulnar side of the forearm to the radial styloid at the radial and proximal sites of Lister's tubercle (Fig. A and B). No EPB tendon was found. During the follow-up, we could not find the anatomical snuff box in his left wrist. However, in his healthy right wrist, the EPL tendon ran ulnar to Lister's tubercle in a normal course and the anatomical snuff box was recognized on physical examination (Fig. ).
At 12 months follow-up, the patient was completely asymptomatic at the radial styloid process and able to resume full daily activities. |
pmc-6156021-1 | A man in his early 70s underwent upper endoscopic examination during a health checkup; during the examination, a submucosal tumor measuring 20 mm was detected in the greater curvature of the middle body of the stomach (Fig. ). Following this, the patient visited our hospital, and endoscopic ultrasound-guided fine-needle aspiration (FNA) of the lesion was performed. Cytology revealed many epithelial cells showing sheet-like clusters or mildly overlapping clusters and isolated scattered cells in a background of inflammatory cells and mucin. The cytoplasm of the epithelial cells contained granules that stained light green or had abundant mucus (Fig. ). Because a mild increase in the nuclear chromatin and a clear nucleolus were observed, the possibility of a proliferative lesion was considered; however, it was difficult to confirm malignancy because the cells did not show distinctive atypia. Thus, endoscopic submucosal dissection (ESD) was performed. On assessment of the ESD specimen, a tumor measuring 23 × 15 mm was observed only in the submucosal layer (Fig. ). Histology revealed that atypical cells, which showed a morphology similar to that of foveolar epithelium, mucous gland, and fundic gland cells, proliferated and formed large and small irregular glands (Fig. ). No tumor or scar was detected in the lamina propria above the tumor. Immunohistochemically, the tumor showed scattered positivity for pepsinogen I and H+/K+-ATPase and strong positivity for MUC6. These findings indicated tumor differentiation into a gastric fundic gland (Fig. A–C). Moreover, the tumor showed positivity for MUC5AC, indicating its differentiation into gastric foveolar epithelium (Fig. D). Staining for MUC2, CD10, and chromogranin A was negative. The tumor showed focal and weak positivity for p53, and the Ki-67 labeling index was 14%. On the basis of the above findings, the tumor was diagnosed as GA-FGM.
Because the margins of the ESD specimen were positive, total gastrectomy and lymph node dissection were additionally performed. No residual tumor or lymph node metastasis was observed; however, numerous heterotopic gastric glands (HGGs) were observed in the stomach wall (Fig. ). In these HGGs, gastric foveolar and fundic gland- or pyloric gland-like epithelia were observed (Fig. ). In the background of the gastric mucosa, atrophy along with intestinal metaplasia was generally observed. After treatment, no recurrence was observed during a 1-year follow-up period.
Ethical approval was not required for this case report, as it did not relate to the patient's privacy or treatment. Informed consent for the publication of this case report has been obtained. |
pmc-6156028-1 | A 60-year-old man underwent a gastroscopy in a local hospital due to a pain and distention in his upper abdomen and melena for 15 days. Gastroscopy revealed a mass measured 3.5 cm diameter on the front wall of the gastric antrum. The lesser curvature and prepyloric region were involved. It had an irregular surface and a large and deep ulcer in the center. The pathologic diagnosis was a poorly differentiated adenocarcinoma. He then came to our hospital for further treatment. Further examination revealed that the AFP level was 1683.33 ng/mL (<9.0). Abdominal computed tomography (CT) revealed a thickening in the front wall of the gastric antrum (Fig. ). The radial line was about 16 mm. There was no obvious abnormality in the liver, except multiple cysts. The patient was treated with R2 radical gastrectomy. Postoperative pathology revealed a hepatoid gastric adenocarcinoma with NED (Figs. and ). It was invading the muscular layer of the stomach. The tumor could be found in the vessel but not in the epiploon and incisal edge. One lymph node was positive on the greater curvature (1/11), and all were negative on the lesser curvature (0/9). TNM staging was T2N1Mx. Immunohistochemical analysis showed AFP, Hepatocyte, synaptophysin (Syn) and chromogranin A (CgA) positive, and Ki67 60% positive (Figs. –).
The AFP level was 189.98 ng/mL (<9.0) 10 days after the surgery. The patient refused chemotherapy and left the hospital 12 days after the surgery. The patient came back to the hospital half a year after the surgery. The AFP level was >2000 ng/mL (0–8.78). There was no obvious abnormality from CT. The patient refused positron emission tomography computed tomography (PET-CT) and left the hospital. |
pmc-6156029-1 | A 64-year-old right-handed retired male teacher was referred to our clinic with an inability to flex the interphalangeal (IP) joint of his left thumb. One week previously, he was gardening. He had flexed his thumb around the stem of a firmly embedded weed, and as he pulled hard, he felt a sudden pop in his left thumb and found he could not flex his left thumb. Although he often played saxophone, he could not recall any specific episode of pain or discomfort within the left thumb. There was no evidence of any history of rheumatoid or other inflammatory arthritis and his rheumatoid serology was normal.
Upon examination, no specific painful swelling in the left thumb was detected. However, the patient was unable to actively flex his thumb at the IP joint (Fig. ). He had full passive range of motion of IP joint.
Initial plain left thumb radiographs revealed no bony abnormalities resulting in secondary tendon rupture. Magnetic resonance imaging (MRI) revealed complete FPL tendon rupture at the level of the distal one-third of the proximal phalanx and the proximal end of the ruptured tendon was retracted to the level of the metacarpophalangeal (MP) joint (Fig. ).
With the patient under general anesthesia, the FPL tendon was explored through a volar zig-zag incision. During the operation, the FPL tendon was found to be ruptured completely. Gross examination revealed a slightly yellowish denaturated tissue at the distal end of the ruptured tendon and the gap of the ruptured ends was interposed by a fibrous tissue (Fig. ). No bony prominence was observed throughout the excursion of the tendon. We excised the denaturated tissue from the distal end of the ruptured tendon and sent it for histological examination. FPL tendon was repaired primarily via modified Becker method (Fig. ). Histopathological examination revealed normal vasculature in the tendon tissue and degenerative changes associated with lipid deposits in the tendon tissue (Fig. ).
Postoperatively, the left thumb was immobilized in a below-elbow plaster splint with extension block for 1 week, followed by dynamic splinting recommended for another 6 weeks and unrestricted full active motion at week 7.
At 12-month follow-up, the patient was completely asymptomatic and had excellent IP joint range of motion (0°–40°) in his left thumb. The wrist grip strength was 30 kg (28 kg in the Rt.) and the thumb pinch strength was 5.7 kg (4.7 kg in the Rt.). The Quick DASH score was 0 (Fig. ). |
pmc-6156038-1 | A 44-year-old woman with no remarkable family history was admitted to our hospital with the following medical history: after undergoing modified radical mastectomy for cancer of the right breast (papillotubular carcinoma, T3N1M0) in March 2009 at another hospital, the patient received postoperative chemotherapy (4 courses of docetaxel+trastuzumab followed by 14 courses of trastuzumab). However, in March 2011, local excision was performed because of a recurrence that appeared at the site of the surgery, and adjuvant chemotherapy was administered. No relapses were observed thereafter.
When the patient was admitted to our hospital, the patient was of moderate build, was well nourished, had no yellowing of the bulbar conjunctiva, had no conjunctival pallor, and displayed no unusual finding in the heart or lungs. The abdomen was flat; the liver and spleen were not palpable. No remarkable finding was observed. An elastic, hard tumor approximately 20 mm in diameter with relatively distinct boundaries was felt in the left C region. The tumor was observed to be not fixed to the pectoral muscle, and it had not infiltrated the skin. No abnormal nipple discharge was observed, and axillary lymph nodes were not palpable. Peripheral blood and blood biochemistry test results were normal.
By mammographic examination, a high-density tumor with irregular margins and internal calcification was observed and was classified in Category 4. Because the imaging system at our institution has been changed and all previous imaging data were deleted, there are no mammograms to display here. Ultrasonographic analysis revealed that a 10 × 11 × 9 mm hypoechoic area was observed in the C region of the left breast. The lesion had a Tsukuba elasticity score of 2 and internal blood flow, and malignancy was suspected (Fig. ). By positron emission tomography and X-ray computed tomography (PET-CT), an area of greatly increased uptake was observed in the upper outer quadrant of the left breast (SUV max: 8.2→9.77) (Fig. ).
Based on the above characteristics, the patient was given a preoperative diagnosis of T1cN0M0 Stage I cancer of the left breast, and left partial mastectomy and axillary lymph node dissection were performed.
The tumor was a solid light gray nodular lesion with distinct borders and measured 27 × 23 × 23 mm. Histologically, the tumor was a well-differentiated squamous cell carcinoma with prominent keratinization, and there was prominent inflammatory cell infiltration, necrosis, and fibrosis. These findings were consistent with primary pure squamous cell carcinoma of the breast. The specimen was ly(−), v(−) and did not appear to have any clear sign of vascular invasion. The lymph node was pN0 (0/9): Level I-0/8, Level II-0/1, and no lymph node metastasis was observed (Fig. ). Immunohistological staining revealed that hormone receptors were negative, with estrogen receptors (ERs) at ≤1% and progesterone receptors (PgRs) at ≤1%. The HER2 score was 1+ but is only a reference value.
Based on the above findings, the final diagnosis was cancer of the left breast, T2N0M0 Stage IIA.
The postoperative clinical course of the patient was favorable, and the patient was administered 4 courses of FEC100 and 4 courses of DTX as postoperative adjuvant therapy. Observation was continued in the outpatient department, and no sign of relapse was observed. However, the patient moved away from the area to another hospital in March 2014, and eventually died due to relapse in January 2016. |
pmc-6156038-2 | A 58-year-old woman with no remarkable medical and family history was admitted to our hospital. When the patient was admitted to our hospital, the patient was of moderate build, was well nourished, had no yellowing of the bulbar conjunctiva, had no conjunctival pallor, and displayed no unusual finding in the heart or lungs. The abdomen was flat; the liver and spleen were not palpable. No remarkable finding was observed. An elastic, hard tumor approximately 30 mm in diameter with relatively distinct boundaries was felt in the left C/D region. The tumor was observed to be not fixed to the pectoral muscle and had not infiltrated the skin. No abnormal nipple discharge was observed, and axillary lymph nodes were not palpable. Peripheral blood and blood biochemistry test results were normal.
By mammographic examination, a local asymmetric shadow was observed in the left M area, and the tumor was classified in Category 3 (Fig. ). Ultrasonographic analysis revealed that a hypoechoic area measuring 18 × 31 × 24 mm with partially calcified areas and a hyperechoic margin was observed in the C/D region of the left breast. The lesion had a Tsukuba elasticity score of 2 and internal blood flow, strongly suggesting malignancy (Fig. ). By CT examination, a thoracoabdominal CT scan did not reveal anything unusual, such as enlarged axillary lymph nodes or distant metastases in other organs.
Based on the above findings, the patient was given a preoperative diagnosis of T2N0M0 Stage IIA cancer of the left breast, and left modified radical mastectomy and axillary lymph node dissection were performed.
The tumor was a solid, light gray nodular lesion with distinct borders and measured 32×22 mm. Histologically, the tumor was a squamous cell carcinoma with prominent keratinization exhibiting an infiltrative growth pattern. The tumor had no connection to the epidermis and partially transitioned into the atypical ductal epithelium (atypical metaplasia) in the area surrounding the focus. These findings were consistent with primary pure squamous cell carcinoma of the breast. The resection margin tested negative. The specimen was ly(−), v(−) and did not appear to have any clear sign of vascular invasion. The lymph node was pN0 (0/20): Level I-0/18, Sentinel-0/2, and there was no lymph node metastasis (Fig. ). Immunohistological staining revealed that hormone receptors were negative, with ERs at ≤1% and PgRs at ≤1% (both tested positive). The HER2 score was 1+, but it is only a reference value.
Based on the above observations, the final diagnosis was cancer of the left breast, T2N0M0 Stage IIA.
As skin necrosis was observed at the site of surgery, debridement was performed to control necrosis. As the patient did not wish to receive postoperative adjuvant therapy, she was only observed. The patient is periodically examined using full-body or local diagnostic imaging in the outpatient department and currently, 4 years after surgery, no relapse has been observed. |
pmc-6156069-1 | The proband was a 21-year-old Chinese female (Han ethnicity). On April 1, 2016, she suffered from severe abdominal pain with nausea and vomiting after having some ice cream for lunch. She was given supportive treatment against “gastroenteritis” and soapy-water enema therapy against “incomplete intestinal obstruction” by local hospitals. On April 7th, she had an epileptiform seizure accompanied by hyponatremia (Na: 103 mmol/L, normal: 130–150 mmol/L), hypokalemia (K: 2.85 mmol/L, normal:3.5–5.5 mmol/L), and hyperbilirubinemia (total bilirubin: 68.19 μmol/L, normal: 2–20 μmol/L; indirect bilirubin: 58.66 μmol/L, normal: 0–14 μmol/L). In the ensuing days, she manifested consciousness disorder (drowsiness, illusion, and confusion), persistent lower limb weakness and pain, sinus tachycardia, and hypertension in addition to recurrent abdominal pain. The imaging examination of her brain and abdomen showed normal results, except for intestinal tympanites and cholecystolithiasis (Fig. A, B). However, monitoring of her blood testings indicated further deterioration, including anemia (hemoglobin: 76 g/L, normal: 115–150 g/L), hypohepatia (alanine aminotransferase: 111 U/L, normal: 0–40 U/L), and elevated pancreatic enzyme (lipase: 682 U/L, normal: 0–190 U/L). On April 12th, the patient was transferred to our hospital. In the following 20 days, her condition was gradually improved after water intake restriction. In the meanwhile, she manifested brown urine (Fig. C), indicating diagnosis of AIP, which was finally confirmed by the elevated level of urine PBG and the negative result for urine lead. On December 30th, she presented in our hospital again with drastic abdominal pain and mild consciousness disorder. After 4 days’ treatment with intravenous glucose (at least 250 g per day), her symptoms were alleviated.
The patient was born from a nonconsanguineous family. She has a history of constipation from childhood and shortened menstrual cycles. Clinical characters of her family members are showed in Table . Of 9 family members, 6 had refractory constipation, 3 had recurrent abdominal pain. Almost all the female members had irregular menstruation. |
pmc-6156118-1 | A 51-year-old female with a past medical history of type 2 diabetes mellitus presented with complaints of chest pain for three days and vomiting for one day. She had episodes of palpitation, sweating, and weakness for the last three years. These episodes were self-resolving and last for 10-15 minutes. On admission, her blood pressure (BP) was 130/80 mmHg, pulse was 117/min, respiration was 24/min, and oxygen saturation (SpO2) was 100% in room air. While in the hospital she had episodes of orthostatic hypotension with systolic BP ranging from 60 to 130 mmHg and diastolic BP 30-90 mmHg. These episodes were associated with sweating, palpitation, and anxiety and resolved spontaneously. Her pulse remained high ranging between 110 and 120/min. Her electrolyte panel revealed persistently low magnesium, calcium, and potassium levels despite aggressive replacement. She also had persistent hyperglycemia requiring an insulin drip. Computed tomography (CT) angiogram was performed to rule out pulmonary embolism. Incidentally, a large suprarenal hypoattenuating mass was seen pressing on the right kidney. The CT scan of the abdomen with contrast and magnetic resonance imaging (MRI) confirmed it as 11 cm x 11 cm right suprarenal heterogeneous mass as shown in Figure .
Further workup along with a normal blood urea nitrogen (BUN), serum creatinine, and parathyroid hormone levels is listed in Table :
We made a diagnosis of pheochromocytoma and scheduled her for surgery. Preoperative management consisted of doxazosin 1 mg once daily, propranolol, and IV fluids. Orthostatic hypotension was abated. Three weeks later right adrenalectomy was performed. The pathology revealed a large tumor and a diagnosis of pheochromocytoma. The gross appearance and histology are shown in Figures -.
Postoperatively, she remained hemodynamically stable with no hypotensive episode. Her electrolytes and blood glucose remained normal. Laboratory data taken six weeks after surgery show normal 24-hour urine metanephrine and normetanephrine and normal serum potassium, magnesium, and calcium levels. |
pmc-6156119-1 | Patient X is a 78-year-old, right-handed man who was seen at the emergency department, presenting with sudden-onset left hemiparesis persisting for five hours. Prior to this, he complained of loss of vision temporarily in his right eye, which he described as a “film coming over his eye.” Swallowing and speech remained intact. Patient X’s medical history consisted of a transient ischaemic attack (TIA), which occurred three weeks ago, hypertension, ischaemic heart disease (coronary artery bypass surgery in 2008), and chronic kidney disease secondary to obstructive uropathy. His social issues revealed that he is also a smoker with a 31-pack-a-year history and drinks one pint a week. His family history showed that his father died at the age of 46 from a myocardial infarction. Patient X lives alone but has a partner who is able to provide support. He is retired but previously worked loading and driving lorries. Prior to the presentation, he was independent and able to carry out all his activities of daily living.
A general examination revealed a carotid bruit and a blood pressure of 151/60 mmHg. On neurological examination, he was alert and orientated. Left-sided power was reduced to 2/5 in the upper limb and 4/5 in the lower limb. There were a mild left-sided facial droop and a left-sided homonymous hemianopia. Reflexes were difficult to elicit. Sensation and coordination were intact.
Investigations
Initially, a computed tomography (CT) scan of the head was noted to be unremarkable (Figure ) but a further scan (two days later) depicted a right-sided hypodense area, likely a right-sided parietal infarct (Figure ).
The electrocardiogram (ECG) showed a sinus rhythm. A pattern of left ventricular hypertrophy was noted.
A carotid scan, five days post-presentation showed a large calcified plaque at the origin of the right internal carotid artery. A subsequent CT angiogram, 10 days post-presentation demonstrated calcified plaques in both carotid bifurcations, which appeared larger in the right internal carotid artery compared to the left
(Figure ).
Treatment
Patient X didn’t present within the time window for thrombolysis, therefore, he was started on aspirin 300 mg daily for two weeks to reduce the risk of stroke []. The patient was transferred to an inpatient rehabilitation unit. Due to the scan findings, the patient underwent a right carotid endarterectomy (12 days post-admission). This procedure also aimed to reduce the risk of stroke [].
Outcomes and follow-up
Two days following the right carotid endarterectomy, the patient developed dysphagia. A swallow test was conducted, which showed food getting stuck in the esophagus and then being regurgitated. A discussion followed with the patient about using PEG for feeding. It was explained that the dysphagia was a result of irreversible nerve (CN IX) damage secondary to the carotid endarterectomy. The patient was deemed to have capacity and was thus posed the option of choosing PEG (long-term) or NGT (short-term). Patient X refused PEG and instead opted for feeding via an NGT. Patient X also started receiving physiotherapy in an attempt to strengthen his throat muscles. Doctors are doubtful this patient will be able to regain the ability to swallow normally.
Four days following the surgery, patient X developed healthcare-acquired pneumonia, confirmed by an X-ray (Figure ). This was managed by intravenous antibiotics and chest physiotherapy.
Subsequently, patient X has recovered from pneumonia although his dysphagia is still present (one month postoperatively). He is currently admitted to the stroke unit for further rehabilitation. |
pmc-6156688-1 | A 33-year-old man presented with a 4-month history of abdominal pain and weight loss. On examinations, all parameters were within normal limits. Therefore, an abdominal CT scan showed the presence of retroperitoneal enlarged lymph nodes without other pathological findings. Retroperitoneal lymph node biopsy by the laparoscopic approach was then performed because of the suspicion of lymphoproliferative disease, and the pathological result was nonspecific adenitis.
Because of persistent abdominal pain, in association with left inguinal pain, a further abdominal CT scan was performed. This showed a large left retroperitoneal fluid collection () that caused slight left hydronephrosis and retroperitoneal lymph node enlargement in the interaortocaval, precaval, and right common iliac artery territories. In the presence of these findings, percutaneous drainage of the collection was carried out. With the patient in the supine position, under local anesthesia (10 mL of 2% mepivacaine), the lesion was punctured with a 21-gauge Chiba needle under ultrasonography and fluoroscopic guidance, leaving an 8.5F pigtail drainage catheter (Ultrathane® drainage catheter set; Cook, Inc.) without complications.
The drainage recovered 1100 mL of milky liquid. Analysis revealed high levels of protein and triglycerides compatible with chyle, raising suspicion of cisterna chyli injury because of a history of retroperitoneal operation. Conservative management with a low-fat diet, medium-chain triglycerides, and octreotide was established, but the pigtail output was maintained at 1500 to 2000 mL per day for 2 weeks. For these reasons, the decision was taken to use surgical management.
During surgical planning, lymphangiography was performed after gaining direct access to the inguinal lymph node under ultrasonography guidance using a 22-gauge spinal needle. Iodixanol 270 mg/mL contrast (Visipaque™; GE Healthcare) was injected and the radiographic image showed perfect opacification of the lymphatic afferent and efferent vessels of the punctured lymph node and contrast leakage at the level of the fourth lumbar spine, within the retroperitoneum (inferior to the cisterna chyli) (). A laparoscopic transperitoneal approach to the retroperitoneum was used. Trocar disposition was as follows: paraumbilical (10 mm), hypochondrium (5 mm), iliac fossa (12 mm), and anterior axillary line (5 mm). Dissection of the white line of Toldt was carried out until access was gained to the retroperitoneum. After identification of the chyle collection (), the interventional radiologist performed intraoperative lymphography through direct inguinal lymph node puncture (as described previously) to corroborate the findings and then proceeded to the injection of 5 mL methylene blue in the cannulated lymph node. Four minutes later, leakage of methylene blue in the retroperitoneum () was identified, with localization of the leakage point. Selective ligation was performed using a 3-0 polyglactin suture, encompassing the area of leakage, and n-butyl cyanoacrylate was applied after stitching. Pathological lymph nodes were identified and extirpated for pathological analysis.
After operation, the drainage output diminished from ∼1500 to 2000 mL per day to <500 mL per day. Pathological evaluation of the adenopathies resulted in a diagnosis of seminoma. Testicular ultrasonography was carried out without pathological findings and the diagnosis of extragonadal retroperitoneal seminoma was considered. Chemotherapy using a cisplatin plus etoposide regimen was instituted; in addition, the patient remained under conservative treatment for chyle leakage, with gradual reduction of pigtail output to zero at 2 weeks after operation. |
pmc-6156839-1 | A 16-year-old boy presented to eye casualty having noticed a brief episode of flashing lights followed by acute inferior hemi-field visual loss in the left eye while walking to class. The visual loss persisted, and the event was completely painless. Past medical history consisted only of migraine which the patient was not experiencing at the time of visual field loss. He denied any history of smoking, illicit drug use, alcohol consumption or sexually transmitted infections.
Examination revealed a normal visual acuity of 6/6 in both eyes. A clear inferior altitudinal defect was evident when visual fields were tested to confrontation and a supra-temporal wedge of retinal pallor with associated arterial attenuation was visualized on slit lamp ophthalmoscopy. Colour retinal photography confirmed evidence of a superior branch retinal artery occlusion (BRAO) (ref. Fig. ) and visual field testing confirmed an inferior altitudinal field defect in the left eye (ref. Fig. ). Aspirin was commenced at this point and the patient was referred for urgent review with the local stroke team. Trans-thoracic echocardiography revealed a bicuspid aortic valve but reassuringly no vegetations that may have produced emboli. MRI and MR-angiogram of brain and neck were unremarkable. Haematological investigations revealed a moderate-severely raised homocysteine level (68.0 μmol/L, normal range 5.5–13.6 μmol/L), vitamin B12 deficiency (108 ng/L, normal range 191–663 ng/L) and a borderline folate deficiency (4.6 μg/L, normal range 4.6–18.7 μg/L). Following these findings, genetic testing was completed, revealing a C677T subtype homozygous mutation for the gene encoding methylenetetrahydrofolate reductase (MTHFR), a vital enzyme in homocysteine metabolism. The patient was commenced on vitamin B12 and folate replacement (with resultant normalization of homocysteine levels) and continued on aspirin. Subsequent visual field testing at two months revealed slight improvement in the visual field defect (ref. Fig. ) and no further vaso-occlusive events were noted. |
pmc-6156853-1 | The patient is a 67-year-old man with a history of localized prostate cancer treated with prostatectomy. He initially presented with a 22 cm lytic mass of the distal femur. Core needle biopsy revealed grade 2 conventional chondrosarcoma. After resection, final pathology showed grade 3 conventional chondrosarcoma (Fig. ).
Nine months later, he developed innumerable, biopsy-proven pulmonary metastases (Fig. and Fig. ). Due to the general lack of efficacy of cytotoxic chemotherapy for conventional chondrosarcoma [], the patient’s inability to travel to participate in clinical trials and following extensive discussion with the patient, he initiated nivolumab 240 mg (flat dose) intravenously every 2 weeks on a compassionate use basis. After 4 doses, the pulmonary nodules increased in size and number (Fig. ). Therapy was stopped, with plans to enroll in a clinical trial that was not available at our site at the time of nivolumab initiation.
Three months later, pre-trial CT scans revealed a near complete response in his pulmonary nodules, with most nodules resolving. One remaining index nodule previously measuring 16x17mm decreased to 5 mm and another measuring 11 mm decreased to 4 mm. Rather than enrolling on trial, he reinitiated nivolumab therapy and continues with improvement in his few remaining pulmonary nodules (Fig. ).
We conducted analyses to understand the underlying pathogenetic mechanisms operative in this case. NextGen sequencing (NGS) revealed a mutation in exon 4 of IDH2 and a variant of unknown significance in exon 11 of BRCA2. The tumor was microsatellite stable by sequencing and demonstrated no loss of expression of mismatch repair proteins (MLH1, MSH2, MSH6, and PMS2) when assessed by immunohistochemistry. PTEN expression was retained. The tumor did not express PD-1 and was 1% positive (2+) for PD-L1. Tumor mutational burden was low (4 mutations/Mb). |
pmc-6156856-1 | A 68-year-old woman was admitted by her general practitioner (GP) with chronic pain situated in the epigastrium lasting for several years. No complaints of nausea, vomiting, body weight loss, or other symptoms were noted. The patient had no history of oedema of the lower limbs, varices, and trauma. Her only prior surgery was a Fothergill-Manchester operation in 2004 due to genital prolapse and urinary incontinence. The GP performed an abdominal ultrasound revealing a fluid-filled tumour (5.5 × 5.5 cm) situated between the common iliac arteries (Fig. ).
No pathology was noted during the complete gynaecological examination. Subsequently, computed tomography (CT) of the abdomen and pelvis was performed revealing an enormous mass in the presacral space. The CT image clearly suggested the presence of a neoplasm in the abdominal cavity (Figs. and ).
After the CT, the patient was admitted to the Department of General and Vascular Surgery and Angiology. An arterial and venous Doppler ultrasound was performed that indicated proper arterial flow and no deep vein thrombosis in the lower limbs. The Doppler ultrasound was followed by a direct phlebography that revealed a large venous aneurysm of the right common iliac vein with an adhering thrombus and a large collateral circulation (Fig. ). All of the imaging studies excluded the presence of arteriovenous fistula. The D-dimer value was in the normal range. No other pathology was noted. The patient was offered surgical treatment but refused. Anticoagulation therapy with rivaroxaban was administered.
She received anticoagulant treatment for 2 years, and no thromboembolic complications or aneurysm growth has been noted thus far. |
pmc-6156871-1 | A 44-year-old Japanese man presented to the Maxillofacial Surgery Clinic at the Tokyo Medical and Dental University, Tokyo, Japan, with reports of a hard mass and dull pain in the left mandible. His medical history was generally unremarkable, though his mandible had been accidentally smashed against his young son’s head at the age of 40. No fracture was detected at the time, and no treatment was received.
Clinical examination revealed slight swelling in the left lower molar region and swelling of the left mandible. There was no disturbance of sensitivity in the left lower lip or chin, and he could move his lips normally. Radiographic examination showed large, irregular, multilocular radiolucency of the left area of mandible extending to the premolar and molar area, with no evidence of any impacted tooth (Fig. ). A computed tomography (CT) image revealed a 45 × 30 × 30 mm multilocular cystic mass in the mandible (Fig. ). An axial CT image showed thinning or partly resorption site of the buccal cortical plate. The coronal section images of the CT scan showed the mass lesion displacing the mandibular canal downward, near the inferior border. The roots of the adjacent teeth were resorbed, changing their shapes (Fig. ). The location of the teeth was not changed.
The initial clinical impression was an ameloblastoma, myxoma, keratocystic odontogenic tumor or another tumor type lesion.
The lesion was removed surgically with the buccal cortical bone and buccal mucosa in contact with the mass of the tumor. The mass fully excised intraorally under general anesthesia, and the inferior alveolar nerve in contact with the mass was preserved.
The resected specimen of the primary tumor was a soft, fragile, yellowish-white mass. Microscopically, tumor tissues were composed of plexiform or cord-like nests of tumor cells with round to oval nuclei and eosinophilic cytoplasm in a matrix with prominent myxoid change (Fig. ). On the other hand, the component of solid sheets of tumor cells was limited (Fig. ). In some areas, tumor cells surrounded small blood vessels (Fig. ). Moreover, tumor cells manifested a uniform cell morphology, poor dysplasia, and inconspicuous mitosis.
Immunohistochemical staining was positive for vimentin (Fig. ), muscle-specific actin/HHF35 (Fig. ), and Calponin (Fig. ), a protein responsible for binding the actin-binding protein. A hairline-shaped area of positive staining for type IV collagen surrounding the tumor cells was also observed (Fig. ). VEGF and D2–40 was also focally positive in tumor cells. In contrast, staining for alpha-SMA, cytokeratin (AE1/AE3), cytokeratin (CAM5.2), CK19, CD34, CD68, p63, S-100, Factor VIII, and desmin, CD56, chromogranin A, synaptophysin was all negative in the tumor cells. Moreover, CD31 was also negative, but we observed many blood cells intervened between tumor nests. The Ki-67 labeling index was almost 1%. No necrosis or tumor invasion into the neurovascular channel was observed. The primary tumor was diagnosed as a glomus tumor based on these findings.
The patient remained symptom-free and manifested no signs of recurrence. However, a recurrent tumor was detected in a panoramic radiograph during an 8-year follow-up. The panoramic radiographs taken earlier, after excision of the primary tumor, showed normal healing process, bone regrowth, and increased radiopacity. The follow-up panoramic radiograph 8 years later depicted the recurrence as a radiolucent expansion in the lower area of the mandible. A CT exam showed an expanding lesion exiting the lower site of the mandible and a thinning buccal cortical bone in contact with the tumor (Fig. , ). In magnetic resonance imaging (MRI), the recurrent lesion showed a lower or compatible signal intensity compared to the muscle in T1-weighted images and a low-to-high inhomogeneous signal intensity in T2-weighted images. The apparent diffusion coefficient in the glomus tumor area was 2.0 × 10− 3 mm2/sec, suggesting a low cellular density (Fig. -).
The recurrent tumor mass and related teeth were removed under general anesthesia, and the bone surface was shaved. The recurrent tumor had the same microscopic, morphologic, and immunohistochemical features as the primary tumor (Fig. ). The tumor invaded the medullary cavity of the mandible, involving spongiosa and resorbing compressively the cortex bone around tumor.
The patient has no symptoms of recurrence as of this writing, 2 years after the second operation, and will be followed for the long term to promptly detect any signs of new tumor growth.
The patient is satisfied that the glomus tumor was regulated without resecting the mandibular region. He is pleased that there was no functional deterioration that he was worried about before treatment and there was no aesthetic damage.
Timeline of patient diagnosis and treatment.
1. 4 years before the first visit, the patient had been smashed against his young son’s head.
2. At first visit, the symptom was slight swelling of the left mandible. There was no disturbance of sensitivity. The examinations were performed with X-lay and CT.
3. First surgery was performed and started clinical follow-up after the surgery.
4. 8 years later from first surgery, the recurrence was found by panoramic radiograph. The examinations were performed with CT and MR.
5. Second surgery was performed, and it was passed 2 years with no recurrence from second surgery. |
pmc-6156902-1 | A 53- year old gentleman presented to the emergency eye service with a 1 week history of right visual disturbance with constant multi-coloured photopsia and reduced visual acuity. There was no significant past ocular or medical history. He was involved in a road traffic accident (RTA) 3 weeks earlier when his car was hit from the back but he sustained no injuries at that time. He was otherwise systemically well.
The best-corrected visual acuity (BCVA) was 6/12 in the right and 6/6 in the left eye. There were no anterior chamber or vitreous cells and no vitreous haze. In the right eye a large white deep retinal lesion, with an opaque appearance, was identified in the temporal retina, extending into the macular area and transecting the fovea (Fig. ). The lesion had a unique shape and margins: the nasal aspects of the lesion had a geometric like shape, with defined linear straight and curved edges, and a pronounced bright white border; the remaining border was less defined and homogenous with the lesion. Spectral domain optical coherence tomography (SD-OCT, Topcon Medical Systems Inc., Newbury, UK) within the lesion showed hyper-reflective sub-retinal change (Fig. ). Beyond the observed lesion edge, the ellipsoid zone (EZ) was disrupted in the nasal macular (Fig. ) Small, well-defined creamy white sub-retinal lesions were also evident in the superior macular area of the right eye (Fig. ). The left eye was unremarkable. (Fig. ).
The history of RTA led to consideration of commotio retinae, secondary to presumed whiplash injury from the car accident. Investigations for inflammatory and infective pathologies were negative including normal inflammatory markers, autoimmune screen, serum angiotensin enzyme, chest x-ray and syphilis testing.
Six weeks later, the patient reported full resolution of symptoms, including photopsia but the right BCVA remained the same. The area of retinal whitening and corresponding hyper-reflective sub-retinal OCT changes had resolved. Partial restoration of the EZ in the nasal macular was noted compared to earlier OCT. Multiple small macular sub-retinal pigment epithelium (RPE) deposits had appeared, with corresponding hyper-autofluorescence on fundus autofluorescence (FAF). The left eye remained normal.
The patient was lost to follow-up and re-presented 4 months later with recent onset blurred vision. The BCVA had deteriorated to 6/15 right and 6/19 left. There was no cellular activity in the vitreous but the posterior vitreous face appeared condensed and thickened. New retinal lesions were evident in the maculae of both eyes: in the right, a long, vertical lesion with ill-defined edges and different orientation, shape and margin appearance to the original lesion, was present; (Fig. ) in the left, a similar, ill-defined white retinal lesion inferior to the fovea was noted (Figure ). OCT within these lesions demonstrated hyper-reflective sub-retinal change, similar to the original lesion (Fig. and ). More numerous bilateral sub-retinal and sub-RPE deposits were seen. (Fig. ).
One week later, significant evolution of the right lesion morphology was observed: the temporal margin appeared more defined with re-emergence of a bright linear edge; the lesion had increased in size; new sub-retinal deposits were identified (Fig. ) and the lesion appeared to have migrated supero-nasally across the peri-papillary area, maintaining the outline shape temporally of the earlier lesion. Within the previously involved area, the sub-retinal lesion clinically appeared to have resolved and OCT demonstrated resolution of the hyper-reflective change with the RPE intact. (Fig. , ) In the left, the lesion inferior to the fovea had evolved and was replaced by multiple small defined sub-retinal and sub-RPE deposits. (Figs. , ) Fundus fluorescein angiogram (FFA) and indocyanine green angiography (ICG) showed multiple hypofluorescent spots corresponding to the sub-retinal/ sub-RPE deposits. (Fig. ) These lesions were more numerous than those clinically detectable. On FAF, there were hypo- and hyperautofluorescent spots corresponding to sub-retinal and sub-RPE deposits respectively. (Fig. ) The patient was referred to the uveitis service for opinion.
Prior to review, the patient developed acute confusion and impaired neurological function. Magnetic resonance imaging (MRI) of the brain, lumbar puncture and urgent brain biopsy confirmed the diagnosis of primary CNS diffuse large B-cell lymphoma.
He underwent MATRix (methotrexate, cytarabine, thiotepa, rituximab) chemotherapy and brain and orbital radiotherapy. During treatment the patient noticed a significant and improvement in his visual symptoms. His last recorded BCVA, at 12 months follow up, was 6/7.5 right and 6/6 left. Clinical examination and repeat imaging demonstrated resolution of the large retinal lesions and reduced number of sub-RPE lesions in the both eyes with complete restoration of the outer retinal architecture on OCT (Fig. to ). |
pmc-6156949-1 | A 60 year old female presented with a 2-year history of retinal detachment in the right eye. A right eye PVR inferior bullous retinal detachment involving the macular reduced the vision to hand motion. A chorioretina scar was present in the nasal mid peripheral retina, while a large retina break was noted in the temporal periphery at about 10 o’clock position. The Left eye was satisfactory at this time.
The patient had a right eye uneventful vitrectomy and silicone oil injection on the 29th of April 2011. The retina was entirely reattached under the silicone oil.
Post operatively, by the first day through to the first month she received topical steroid and antibiotic preparations with the vision initially improving to 6/60. She however developed ptosis, erythema and significant keratic precipitates (KPs) and the silicone oil became opaque with an absence of the previously seen retina view. A diagnosis of ocular inflammation due to delayed onset post vitrectomy endophthalmitis was made (Fig. ).
Silicone oil was removed on the 12th of August 2011.
Microbiology study: of the silicone oil was reported as;
“Gram stains could not be done, as the stains could not adhere to the silicone oil. Also no growth was seen after 40 h of culture”.
Hypopyon in the immediate postoperative stage was managed with frequent topical antibiotics and steroids (moxifloxacin and dexamethasone).
Cataract surgery was performed on the 4th of November 2011 facilitating view of the retina. A generalized PVR process occurred, vision deteriorating from hand motion to light perception and IOP of 2 mmHg. The inflammation was controlled with use of topical medications though a phthisical globe was the eventual outcome.
The left eye developed a para papillary choroidal neovascular membrane and intravitreal antiVEGF therapy was given. |
pmc-6156949-2 | A 34-year old female bilateral myope, presented with a 2-month history of right eye retinal detachment. A macular involving inferior retina detachment and multiple inferior retina breaks reduced the visual acuity to 6/60 + 1. The left eye had a prophylactic retina laser to peripheral retina breaks and lattice degeneration.
Uneventful right eye vitrectomy and silicone oil exchange were performed on the 1st of July 2011 with a fully reattached retina post operatively. Visual acuity was counting fingers on the first postoperative day and improved to 6/36 by the first week. The patient had significant ocular pain and conjunctiva hyperemia that were beyond normal expectation. The patient’s vision deteriorated to hand motion with complaints of increasing ocular pain and increased hyperemia. The patient developed a ptosis and the fundal view became hazy. The diagnosis was a post pars plana vitrectomy endophthalmitis.
The patient was initially treated with frequent topical moxifloxacin, dexamethasone, atropine and intravitreal injections of vancomycin and ceftazidime (as per the EVS protocol).
On the 9th of August 2011, the silicone oil was removed and cataract surgery was also performed. There was a hypoypon on post operative day 1.
The retina re-detached postoperatively and silicone oil was re-injected into the vitreous cavity on the 20th of August 2011 after further retina reattachment surgery (Fig. ).
Microbiology study: of effluent from the vitreous cavity and silicone oil yielded; gram-negative bacilli and Pseudomonas species were isolated, sensitive to ceftazidime and co-trimoxazole, but resistant to amikacin, gentamicin and ciprofloxacin.
The patient was then placed on oral co-trimoxazole and continuous topical moxifloxacin and dexamethasone with complete resolution of the inflammation and severe PVR reducing vision in this eye to hand motion and IOP of 2 mmHg. |
pmc-6156949-3 | A 34-year old male bilateral high myope presented with a long-standing right eye retina detachment. Fundal examination revealed an inferior PVR macular involving retina detachment reducing the visual acuity to counting finger. The patient had multiple retina breaks in the inferior retina periphery and received prophylactic retina laser to the affected eye.
On the 8th of July 2011, the patient had a right eye combined vitrectomy with an encircling band and silicone oil injection. Post operatively the retina was reattached and the vision remained counting fingers. He was prescribed postoperative topical dexamethasone and moxifloxacin preparations. At the first post operative week there was considerable conjunctiva hyperemia and lid edema, visual acuity was noted to be hand motion.
Within the first month, he complained of significant tearing and vision had deteriorated to light perception. The eye was still hyperemic with significant chemosis, cornea opacity, hypopyon, an evolving cataract and posterior synechia. A corneal stromal abscess was noted during his subsequent visit. The diagnosis was an acute type post vitrectomy infectious endophthalmitis.
This was managed with frequent topical moxifloxacin and dexamethasone.
On the 31st of August 2011 a combined silicone oil removal and cataract surgery was performed.
Microbiology study: silicone oil mixed with fluid from the vitreous cavity yielded gram-negative bacilli and the organism isolated was Burkholderia (Pseudomonas) cepacia. It was sensitive to ceftazidime and co-trimoxazole, but resistant to ciprofloxacin, amikacin and gentamicin.
Topical medications as before were continued. The vision deteriorated to no light perception, a phthisical globe and corneal opacity as shown on Fig. . The left eye remained normal. |
pmc-6156949-4 | A 43-year old male who had previously undergone a left eye vitrectomy with silicone oil and a 360-degree encirclement buckle for an inferior PVR retina detachment involving the macular, had reduced vision in the eye to 6/36. Surgery was performed on the 20th of May 2011. He suffered a post operative hyphema with bleeding into the silicone oil and vision reduced to hand motion in the post operative period. As the hemorrhage into the silicone oil did not clear. On the 8th of July 2011, the hemorrhagic silicone oil was removed and fresh silicone oil was re-injected.
Post operatively, the patient’s vision remained limited to hand motion. The patient developed ocular inflammation with symptoms of ptosis, lid swelling, hyperemia, chemosis, cornea ulcer and flare in the anterior chamber as in case three.
At the first post operative week there was significant hyperemia and by the subsequent visit the patient presented with a hypopyon. He was placed on frequent topical moxifloxacin and dexamethasone. Intravitreal injections of vancomycin, ceftazidime (as per the EVS protocol) and dexamethasone were given. The patient went on to develop rubeosis, which was managed with intravitreal antiVEGF.
The silicone oil was removed on the 15th of September 2011.
Microbiology study: silicone oil mixed with vitreous effluent reported on microscopy numerous pus cells.
Bacterial culture: Burkholderia (Pseudomonas sp) cepacia, sensitive to ceftazidime and co trimoxazole but resistant to amikacin, ciprofloxacin and gentamicin.
He developed significant PVR with hypotony and a vision of hand motion. |
pmc-6156949-5 | A 63-year old male who presented on the 18th of September 2012 with reduced left eye vision to 6/36 as a result of a 6-month period of retina detachment. Upon fundus examination an inferior macular involving chronic retinal detachment was observed (Fig. a). The patient was scheduled for a left eye vitrectomy and had an uneventful surgery on the 21st of September 2012. On the 1st post operative day his visual acuity was noted to be counting fingers with a reattached retina. At the next visit 1-week post surgery, he complained of pain in the left eye and vision remained limited to counting fingers but improved marginally to 6/60 with pinhole. At a subsequent visit he complained of reduced vision and foreign body sensation. His vision was reduced to hand motion, and he developed hyperemia and keratic precipitates. Posterior segment examination revealed opaque silicone oil. A faint retinal view could be seen. At his next visit few days later, there was severe conjunctival hyperemia, increasing number of anterior chamber cells and hypopyon. Due to this presentation and a heightened index of suspicion for SORE, the patient was scheduled for immediate silicone oil removal. On the 5th of October 2011, the silicone oil was removed. The procedure involved extraction of the silicone oil, multiple irrigation of the vitreous cavity with vancomycin and ceftazidime (in a concentration of 1 mg/0.1 ml and 2 mg/0.1 ml respectively) into a fluid filled eye. After this irrigation of the vitreal cavity, multiple air fluid exchange was performed. Then, 0.1 ml each of vancomycin, ceftazidime and dexamethasone was injected into the vitreous cavity. Intravitreal 10% C3F8 was used as tamponade after which the three sclerostomy sites were securely closed using 8–0 vicryl sutures.
Microbiology study: silicone oil and fluid mixture yielded heavy growth of gram-negative bacilli, which was isolated and identified as P. aeruginosa. It was sensitive to ceftazidime and co-trimoxazole, but resistant to amikacin, ciprofloxacin, levofloxacin, and gentamicin.
The patient was commenced on oral co-trimoxazole, frequent topical moxifloxacin and dexamethasone.
The intraocular inflammation settled completely with this treatment.
An uneventful left eye cataract surgery (phacoemulsification with implantation of posterior chamber intraocular lens) was performed on the 18th of December 2012. Post operatively, final vision settled to 6/9 with correction of − 0.50DS/− 3.00DC × 80. The patient’s eye remained quiet and retina attached with no PVR formation or macular edema (Fig. b, c). His fellow eye remains satisfactory (Fig. d).
A summary of the clinical presentation of SORE is seen in Table . |
pmc-6156974-1 | Thirty-one-year-old male.
Anorexia, general weakness, prostration, and mild dizziness for several days.
No specific findings.
Current smoker (15 pack-years) and social drinker.
The patient had worked for 5 years in Yeosu National Industrial Complex as a non-destructive testing worker and had no job before. He performed radiographic testing using gamma radiation. In January 2017, the Korea Information System on Occupational Exposure (KISOE) found that his personal thermoluminescent dosimeter (TLD) badge indicated that he had exceeded the exposure limit. The patient’s TLD badge indicated that in December 2016, the patient’s radiation exposure dose for the month was 120 mSv. The patient’s radiation exposure dose was 1191 mSv according to the chromosome aberration test by South Korea’s Nuclear Safety and Security Commission (NSCC) []. The patient visited our hospital via the emergency room, and underwent a complete blood count test and bone marrow biopsy. It was revealed that the patient performed non-destructive tests without radiation shielding when working in high places of the large pipe surface.
When the patient came to our hospital, he was clearly conscious with a blood pressure of 140/80 mmHg, temperature of 37.5 °C, pulse rate of 104 beats/min, and respiration rate of 20 breaths/min. He was anemic. The venous injection areas had bruises and bleeding tendency. There were no abnormal findings in the cornea and lens of the eye. We tried to perform semen analysis but failed due to the patient’s condition. Subsequently, semen analysis could not be performed because of refusal by the patient.
In a pre-placement medical examination conducted 6 months before the accident, all blood parameters were in the normal range: white blood cell count: 4920 cells/mm3, absolute neutrophil count: 2410 cells/mm3, absolute lymphocyte count: 1880 cells/mm3, hemoglobin: 14.7 g/dL, and platelet: 217 × 103/mm3. A blood test performed in the hospital after the symptoms appeared showed severe pancytopenia: white blood cell count: 1360 cells/mm3, absolute neutrophil count: 500 cells/mm3, absolute lymphocyte count: 680 cells/mm3, hemoglobin: 7.1 g/dL, and platelet: 14 × 103/mm3. The laboratory results showed a slight recovery after 26 days from the date of prohibition of work: white blood cell count: 2200 cells/mm3, absolute neutrophil count: 760 cells/mm3, absolute lymphocyte count: 1080 cells/mm3. After a 13-month follow-up on the blood test, pancytopenia improved over time; however, it did not recover to the level before the accident (Table , Fig. ).
The patient’s radiation exposure dose was assessed using lymphocyte depletion kinetics. The patient’s lowest absolute lymphocyte count was 0.65 × 109 cells/L, and the radiation exposure dose based on this count was 1.4 Gy (95% confidence limits: 1.1–1.6) [, ]. The patient’s radiation exposure dose was 1191 mSv in the chromosome aberration test by South Korea’s NSCC []. |
pmc-6157056-1 | This study was conducted according to the Declaration of Helsinki, and was approved by the local institutional review board guidelines (number: 54306316.4.0000.5514).
The patient, a 66-year-old Brazilian male sought the Coloproctology Division at USF, Bragança Paulista, São Paulo, Brazil due to a complaint of an anal lump for 3 years, and fistula with absolute hyperemia and decreased caliber of the stool accompanied by increased constipation with occasional rectum bleeding. The colonoscopy showed the presence of intense perianal rash with xeroderma, peeling skin, warty lesions and a severe stenosis of the anal orifice (Fig. ). Also, no changes in the rectal mucosa and absence of polyps in the colon were observed.
Perianal surgical biopsies were performed with segments of skin resection and subcutaneous tissue at areas where there was a lack of skin irregularity and hardened consistency. Large biopsies fragments of four quadrants were removed. Post-treatment biopsies were performed using the same technique. Perianal biopsy showed the atypical Paget cells suggesting PPD or balloon cells melanoma (Fig. ). For the differential diagnosis, Melan-A, CDX-2, cytokeratin 20 (CK20), cytokeratin 7 (CK7), p63, and human epidermal growth factor receptor 2 (HER2) immunostaining markers panel was analyzed. Positive staining only for cytokeratin 20 (Fig. ) and CDX-2 (Fig. ) markers was observed. No PPD diagnosis secondary to a colorectal origin was confirmed. The anal orifice stenosis was treated by sphincterotomy.
Local excision is considered the first-line therapy in PPD cases and most of them requiring also an abdominoperineal amputation of the rectum with a permanent colostomy. The patient refused that option. Then, since there was no dermis invasion and no associated colorectal carcinoma, the PPD treatment choice was Imiquimod 5% cream for 12 months. Every 4 months a new colonoscopy revealed skin condition improvement (Fig. ) and perianal biopsy showed no residual PPD (data not shown).
After the first Imiquimod treatment cycle, two more surgical biopsies were performed and a new Imiquimod cycle was prescribed. After 3 months a third biopsy revealed no sign of lesion. The patient is currently being closely followed every 3 months. The patient remains without signs of recurrence to date. If no lesion is observed, the follow-up may include biopsy from the old perianal lesion once a year and colonoscopy once every 2 years.
It is interestingly to note that patient has presented pain and irritation of the perianal skin during the period of Imiquimod application and the inflammatory process completely improved after the first 45 days with remaining residual local pruritus. |
pmc-6157120-1 | MG is a 60-year-old Arabic lady diagnosed with scleroderma, also known as systemic sclerosis. She is under annual observation without systemic medication by a Rheumatologist, having previously required high dose oral corticosteroids. Her past ocular history is positive for right amblyopia, which can now be attributed to a macular scar of unknown aetiology. In her adult life, she had been diagnosed with chronic open angle glaucoma, undergoing a trabeculectomy & three needlings in the right eye.
The left eye also had a failed trabeculectomy, which was then managed by a Baerveldt tube (BVT) insertion. Eight months after this primary surgery, she developed anterior uveitis with hypopyon, with both intra-vitreal and anterior chamber samples revealing no growth. Unfortunately, this was further complicated by conjunctival erosion, tube exposure, leak around the sclerostomy site and hypotony. She was taken back to theatre for tube revision, including using a tube extender and a single layer amniotic membrane transplant (AMT) over the exposed area.
She was referred to the Western Eye Hospital (WEH) to answer a specific question: what should we do about the tube exposed once again through the conjunctiva in the left eye despite the revision with AMT? After much deliberation and lengthy discussions with the patient, we explored the techniques which would minimise further erosions and delivered the following options:Completely abort the premise of conjunctival drainage by removing the BVT and either doing:○ Endocyclophotocoagulation (ECP), hence prohibiting aqueous production. ○ Assess the integrity of the angle, by completing a minimally invasive glaucoma surgery such as an iStent, hence encouraging drainage through the irido-corneal angle. ○ This was unacceptable to MG, since she wanted to avoid staged procedures such as sequential ECP’s and potentially eventual tube revision. Move the existing tube into the ciliary sulcus, +/− iridotomy to observe its position○ Although this would not address the issue of friability of the conjunctiva overlying the tube Retract the tube, with a right angle clip and pass this existing tube through the pars plana○ This would circumvent completely discarding the tube, although the plate would have to be retracted back away from the precarious limbal area. The clip would extend the tube enough for it to reach the vitreous cavity. ○ The disadvantage of this technique is this clip has a high profile and would potentially put undue stress on an already compromised conjunctiva by tenting it up. Move the plate to the supero-nasal quadrant and enter anterior chamber (AC) Take out original tube and put in a pars plana tube (PPT)
We proceeded with revision involving gluing double layered pericardial tissue (tutoplast) and autologous conjunctival graft harvested from the infero-nasal quadrant of the ipsilateral eye, sutured over the graft. Despite careful follow-up, the tube was observed to be eroding through the conjunctiva once again at 2 months (Figs. and ). Further surgical intervention became inevitable and she underwent a tube extraction, pars plana tube plate stabilisation, pars plana vitrectomy (PPV), pars plana tube insertion (Fig. ), phacoemulsification and intra-ocular lens insertion, jointly with the vitreo-retinal surgeons and with high dose prednisolone cover both pre- and post-operatively. This satisfied her request of having a definitive procedure including refractive correction and had an advantage over the right angle clip of possessing a flatter profile, as well as the plate being suitably distal from the limbus.
Pre-operatively, we had multi-disciplinary team meeting to discuss the best way to circumvent future conjunctival erosion. Whilst not in the literature, we decided to harvest the capsule which had grown over the end plate of the original tube. We sutured this over the new tube, specifically over a single layer of tutoplast prior to conjunctival closure (see Fig. ). The closure itself took 2 h to complete and we considered the role of fibrin glue in achieving this, as we wanted to circumvent the adherence and subsequent fibrosis it would lead to, in case a further revision would be necessary. We did proceed using glue with the premise that it assisted the dispersal of pressure, minimizing the tension on the conjunctiva with sutures alone. |
pmc-6157124-1 | An 8-year-old girl presented in August 2015 to the Royal Belfast Hospital for Sick Children’s Accident and Emergency department with headache for 2 months. These had been increasing in intensity and frequency for the preceding 3 weeks requiring her to take paracetamol on a daily basis. At times the headache was associated with redness and watering of her left eye. She attended her optician due to the headaches who noted an abnormal appearance of her left fundus. She denied any visual loss or any other visual symptoms. There were no previous eye problems known.
Systemic enquiry revealed she had occasional nosebleeds. There was no foreign travel except for a family holiday in Spain twelve months previously. As her grandfather was from South Africa, she had received the Bacillus Calmette-Guérin (BCG) vaccine. Her family had a pet dog that lives outside. There were no cats and she did not live on a farm. There was no history of tick bites or cat scratches. She reported no recent viral illness or vaccinations and no recent pyrexia, fatigue, cough or sore throat. She had no known underlying medical conditions. She was not on any medications except for paracetamol as required for her headaches.
Aged 7, she had previously been admitted for investigation of pyrexia and left knee swelling. At that time, mum described her as having a bruise-like rash around the affected left knee. She was extensively investigated as a Magnetic Resonance Imaging (MRI) scan showed abnormal changes in and around both knees. Bilateral bone marrow trephine of each posterior iliac crest and bone morrow aspirate and biopsy of the affected distal femur were all negative. Haematological malignancy was excluded. Of note, at that time her C-Reactive Protein (CRP) was 69 mg/L and Erythrocyte Sedimentation Rate (ESR) was 110 mm/hr. but otherwise blood testing did not identify any specific underlying diagnosis for her peculiar presentation.
The examination findings at presentation are shown in Table and Fig. .
She was admitted with findings of left posterior uveitis and mild vitritis for further investigation. A differential diagnosis of unilateral chorioretinitis included infectious chorioretinitis such as toxoplasma, toxocara, tuberculosis, syphilis, borriella burgdorferi (B.burgdorferi) and post-streptococcal syndrome and inflammatory chorioretinitis such as sarcoidosis, multifocal choroiditis and other white dot syndromes. An infiltrative cause was also considered.
Extensive investigations, as presented in Table , failed to identify a specific cause for her unilateral chorioretinitis.
Over the following 4 months, without treatment, her vision increased in the left eye to 6/12 unaided and the fundal appearance improved showing reduced sub-retinal infiltration; however, a focal circular area of chorioretinitis developed along the superotemporal arcade (Fig. ). One month later, the circular area of chorioretinitis had faded but a new active area of chorioretinitis was noted superior to the disc. Within this area, an elongated, white, glistening nematode was identified with an estimated size of 1500 μm when compared with the optic disc diameter (Fig. ). Ocular coherence tomography horizontally through this new area of active chorioretinitis indicated a nematode with tapered end curling upwards from subretinal space into deep retinal layers (Fig. ). This led to a working diagnosis of diffuse unilateral subacute neuroretinitis (DUSN).
Following diagnosis of DUSN, diode laser was performed under general anaesthetic to the superior area of active chorioretinitis and presumed nematode. The patient was also commenced on oral albendazole 200 mg twice daily for 1 month and a reducing course of oral prednisolone starting at 25 mg daily and tapered over 30 days. After another month, her visual acuity had improved further to 6/9 and the treated area of chorioretinitis superior to the optic disc had disappeared. However, new areas of chorioretinitis had appeared temporal to the superotemporal arcade and in the inferonasal fundus (Fig. ). A further course of oral albendazole 200 mg twice daily and oral prednisolone 7.5 mg was commenced and planned for 30 days but was discontinued by the patient after 2 weeks. After a further 2 months, the fundal appearance had changed once again and new areas of chorioretinitis had appeared in the superotemporal retina with resolution of the areas inferonasally and temporal to the superotemporal arcade (Fig. ). Further laser was administered to the new superior lesion where a nematode was suspected. Despite additional anti-helminthic treatment with ivermectin, recurrence of active chorioretinitis lesions continued and electrophysiology testing indicated significant left retinal dysfunction.
A specialist uveitis opinion was sought from a tertiary centre in London that concurred with the diagnosis of DUSN; but it was thought that a secondary immune-mediated inflammatory response might be contributing to the clinical picture. Another tapering course of oral prednisolone along with mycophenolate mofetil was trialled without success. At last review, 19 months from initial presentation, the fundal appearance continued to change and a new area of focal chorioretinitis had appeared at the temporal macula (Fig. ). |
pmc-6157125-1 | A 19y old male with relapsed and refractory ALL was admitted under haematology with pyrexia of unknown origin. At the time of his admission, he reported sequential, bilateral visual impairment that had started 2 days previously. There was no accompanying redness, pain, photophobia, photopsia or floaters. His past medical history involved total body irradiation, chemotherapy and, 4 months previously BMT to treat the ALL. There was no previous ocular or family history of note. His medication included cyclosporine to prevent BMT rejection, and prophylactic posaconazole and acyclovir. At the time of eye clinic review his bloods were Hb- 8.2 g/dL, plt-18 × 10 9 /L and WCC- 0.1 × 109/L. PCR from peripheral blood for viral DNA tested negative for EBV, CMV and ADV.
On examination Snellen acuity was reduced to 6/36 on the right and 6/24 on the left. The anterior segments were normal. There was no anterior chamber (AC) activity or vitritis and the intraocular pressures were normal. Dilated fundus examination showed bilateral and symmetrical retinopathy with cotton wool spots and retinal haemorrhages across the posterior pole with relative sparing of the peripheral retina. Cystoid macular oedema in both eyes, with sub-retinal fluid in the right eye, was noted on optical coherence tomography (OCT) imaging. The central sub-field thickness (CST) was 557 μm and 603 μm in the right and left eyes. Imaging findings are shown below in the colour fundus pictures and OCT images (Fig. ).
Given the pyrexia of unknown origin and the above fundus findings an urgent AC tap was arranged to exclude viral retinitis. This was negative for CMV, HSV, VZV and EBV DNA. Given the persistent visual impairment but without an obvious infective aetiology, cyclosporine was stopped under haematology guidance, as this can be retinotoxic, and 40 mg of oral steroids commenced.
Vision subjectively started to improve over the subsequent 3–4 days despite no resolution of cystoid macular oedema. Two weeks after presentation, the CST was 815 μm in the right eye and 1016 μm in the left eye despite an improvement in VA. Oral steroids were slowly tapered down and stopped over a period of 4 weeks. The cyclosporine was not restarted. The vision continued to improve to 6/9 in the right and 6/6 in the left accompanied by the resolution of macular oedema over several months. This improvement did not coincide with haematological normalisation.
Sadly over the course of the subsequent few months, the patient’s systemic health deteriorated and he died as a result of pulmonary complications. |
pmc-6157128-1 | We present a case of acute angle closure due to spontaneous suprachoroidal haemorrhage secondary to loss of anti-coagulation control.
A 67-year-old man, who recently returned from a holiday abroad, presented with a one-day history of worsening right visual acuity and 4 day history of increasing right retro-bulbar pain not relieved with simple analgesia.
He had a past medical history of essential tremor managed with Propranolol, Atrial Fibrillation on anticoagulation with Warfarin 4 mg daily – target International Normalised Ratio (INR) 2.5. Possible confusion with his tablets in the week leading up to the start of his symptoms.
Our patient was also known to have normal tension glaucoma (NTG) managed with Latanoprost. He had Selective Laser Trabeculoplasty (SLT) to the right eye 12 months before to improve his intraocular pressure control. His last recorded visual acuity (VA) was 6/6 in both eyes.
On examination the patient was found to have reduced VA in the right eye 6/12 with an injected conjunctiva, cloudy cornea and a mid-dilated pupil with a very shallow anterior chamber (AC) and closed irido-corneal angle on gonioscopy (Figs. and ). Fundus exam revealed a large supero-nasal suprachoroidal haemorrhage not involving the macula. His right intra-ocular pressure (IOP) was 42 mmHg. The left eye had a VA of 6/6 with a deep AC and IOP of 12 mmHg (Fig. ). He was therefore diagnosed with acute angle closure secondary to spontaneous suprachoroidal haemorrhage. His INR measured at > 8. The patient was given 1 mg of Vitamin K to reverse his INR, which quickly came down to 5.1. Advice was taken from the general physicians’ team and no further Vitamin K doses were given.
He was started on maximal topical and systemic IOP lowering treatment including G. Apraclonidine 1% TDS, G. Latanoprost 0.005% ON, G. Brinzolamide/Timolol (Azarga®) and Oral Acetaolamide 250 mg QDS as well as cycloplegia with G. Atropine 1% OD.
After 12 h the IOP was 27 mmHg and INR 3.1. But unfortunately, in the following 12 h, the patient had a second bleed, and his IOP went up to 42 mmHg and VA was down to finger counting. There was no view of the fundus due to corneal edema. B-Scan Ultrasound showed an extension of the suprachoroidal haemorrhage, covering 360 degrees and involving the fovea (Fig. ).
For the next 7 days the patient’s remained on the same medical treatment and his IOP was stable in the high 20s. A decision was taken to perform a combined phacoemulsification and lens implant, pars-plana vitrectomy and suprachoroidal haematoma drainage under general anesthesia. (Additional file 1).
Six weeks post operatively the patient had a wide-open angle with a central IOL and a flat retina (Figs. and ). Intraocular Pressure without IOP lowering treatment was recorded at 20 mmHg with VA 6/24. He was restarted on IOP lowering topical treatment (G Brinzolamide/Timolol BD). |
pmc-6157136-1 | A 36-year-old female patient presented with a 4-year history of right ankle pain. She had significate loss of weight and started physical activity just before the symptoms appeared four years ago. No history of trauma was reported. First diagnosis was synovitis, for which the patient underwent physical therapy with torpid outcome. She experienced paraesthesia along the posterior and medial aspect of the ankle and foot. Symptoms got worse at the end of the day, with cramping episodes in the foot. Physical examination revealed a positive dorsiflexion-eversion test provoking numbness of the foot. Tinel's sign was positive. Ankle range of motion was limited due to pain, scored 8 using the Visual Analog Score (VAS). The patient had a preoperative score of 40 points, according to the American Orthopaedic Foot and Ankle Society (AOFAS). Bilateral electromyography (EMG) was performed, disclosing abnormal adductor hallucis and adductor digiti quinti neurophysiologic parameters in comparison to the asymptomatic side.
Initial plain radiographs showed a considerable (1.8 cm) posterior bone fragment in relation with the talus (). CT scan was then performed, which showed the presence of an articulated accessory bone. At this point, talus bipartitus (TB) diagnosis was suspected (). MRI disclosed the presence of an inflammatory process, bone fragment covered with cartilage, and what appears to be a degenerative synchondrosis as a consequence of posteromedial impingement ().
In this scenario, a surgical procedure was indicated due to increasing symptoms and several conservative treatment trials with no response.
Preoperatory assessment included three-dimensional CT scan images to get full awareness of the bone fragment's specific anatomy. Anatomic relationship between the tibial nerve, posterior tibial artery, flexor hallucis longus, flexor digitorium longus, and tibialis posterior tendon and the bone fragment was assessed with the help of MRI. Though fixation of the accessory bone has been previously reported, in the index case, this was not an option because of its particular shape, with a medial protuberance invading the tarsal tunnel.
Excision of the bone fragment was performed. A medial approach was used. Both tibialis posterior tendon and neurovascular bundle were identified and carefully preserved. The bone fragment was removed and the tunnel was released (). Postoperative care included cast immobilization and a 30-day weight-bearing restriction. Postoperative radiographs showed the complete absence of residual bone fragment (). Histological findings disclosed mature bone with articular cartilage surface. After 6 months of follow-up, the patient had a VAS score of 2 and an AOFAS score of 87. At a 2-year follow-up, VAS was 1 and AOFAS was 96. Patient had no residual pain and no recurrence of neurological symptoms. |
pmc-6157168-1 | A 26-year-old female patient complained a growing mass in the hard palate since 12 months ago. This mass causes swallowing and closing mouth difficulty. She also complained about changes in the shape of her finger bones, mandible, and ribs. The decrease in height of 9 cm and weight loss of 10 kg were observed within 2 years. The patient experienced end stage of renal failure and has been treated with hemodialysis 2 times per week for seven years since 2009.
The physical examination showed a mass in the palatum durum and mandible, measured 4 × 6 × 2 cm and 3 × 4 cm in size, respectively. The mass has rough surface, hard-solid consistency, with no tenderness. A mass in the right neck was measured 3 × 5 cm in size. The mass has smooth surface and solid consistency ().
The result of the palatum and mandible biopsy was compatible with ossifying fibroma or fibrous dysplasia. Neck ultrasonography showed a solid mass in the right thyroid, bilateral cystic nodule in the thyroid lobes, and small cystic nodule in the thyroid isthmus. Bone survey showed the diffused decreasing bone density, fractured right clavicle, multiple lytic lesions among the cranium, humerus, clavicle, iliac, and pubic bone ( and ). Paranasal sinus CT showed a decreased bone density in the cranial, facial, and cervicothoracic bones; lytic lesion in the maxilla bone extended to the palatum durum and shifted the tongue inferiorly, with the measurement of 5.2 × 4.6 × 3.7 cm (). Similar lytic lesions were also found in the midline and right mandible, measured 3.8 × 3.2 × 2.8 and 2 × 2.3 × 2.1 in size, respectively. Workup laboratory examination showed the elevated intact parathyroid hormone level (PTH) (3.391 pg/mL), ureum (124 mg/dL), and creatinine (6.60 mg/dL) and normal range of calcium level (9.5 mg/dL) and phosphate (3.6 mg/dL). Histopathologic findings suggest ossifying fibroma with a differential diagnosis of fibrous dysplasia. No signs of malignancies were revealed in the histopathologic examination.
Based on the physical examination and laboratory findings, we concluded brown tumor as the diagnosis due to secondary hyperparathyroidism. The treatment for this patient was right parathyroidectomy and right isthmulobectomy with frozen section. The right isthmulobectomy was chosen because the patient has secondary diagnosis which was right nontoxic nodular enlargement of the thyroid gland. In addition, the technique was considered to be appropriate to access the parathyroid gland with the antecedent removal of the thyroid gland. Frozen section revealed hyperplasia of the right parathyroid. Three days following the surgery, PTH level was significantly decreased from the initial preoperative level (851.10 pg/mL). Result from definitive pathology anatomy was hyperplasia of the parathyroid gland.
One month following the surgery, the patient felt regression among her hard palate and mandible mass and ease in swallowing. During follow-up, we performed additional evaluative examination. Neck 4D CT was done 2 times at the 2nd and 5th month of follow-up. The first one showed multiple lymphadenopathy around the neck and left supraclavicle, no residual mass in the right thyroid, no abnormality in the left thyroid, and no ectopic parathyroid gland was found. While the second Neck 4D CT found a single solid lesion in the left thyroid and 2 solid lesions in aortopulmonary window, suggestive of parathyroid nodes. At the 6th month of follow-up, sestamibi parathyroid scan showed no abnormality and no residual mass in the right thyroid lobe. PTH level evaluation performed in the 4th and 7th month showed an elevation to 909.7 pg/mL and 1.137 pg/mL, respectively.
Patient complained pain in all her bones and started walking with a cane. Seven months following the first surgery, we decided to perform left parathyroidectomy and left lobectomy with frozen section and intraoperative PTH evaluation. Thirty minutes following parathyroidectomy, the PTH level decreased significantly to 76.95 pg/mL. Frozen section result revealed hyperplasia of the left parathyroid.
Two weeks following the second surgery, the mass regressed significantly, and symptoms were much reduced. |
pmc-6157168-2 | A 31-year-old female patient complained a mass in her maxilla and mandible. The mass was gradually increasing in size over the last 6 months. The mass caused talking disturbances, swallowing difficulty, dilating gap between upper teeth, and narrowing gap between lower teeth. She also complained hard masses in her fingers and the middle of chest. There was a decrease in height (15 cm in 8 years) and weight (19 kg in 8 years). The patient was on end stage of renal failure and has been treated with hemodialysis 2 times per week for eight years since 2008. The patient was planned to be performed renal transplantation. Comorbidities were reported among this patient, which are chronic heart failure, grade II hypertension, lung tuberculosis on therapy, and hepatitis C. The physical examination showed facial asymmetry with mass in the palatum durum, measured 4 × 3 cm in size (). Palpation revealed hard consistency originated from the bone, smooth surface, and no tenderness.
X-ray of the maxilla and mandible showed diffused decreasing bone density and multiple lytic lesion, which suggest metabolic bone disease or renal osteodystrophy or hyperparathyroidism (). BNO showed compression fracture in thoracolumbar vertebrae and multiple calcification in soft tissue abdominal and pelvic region compatible with renal osteodystrophy. Bone survey also showed the decreased bone density. Facial CT with contrast showed diffuse hyperostosis, lytic lesion in the mandible bone, maxillary bone, cranium, and multiple calcification in the soft tissue which is compatible with osteorenal dystrophy (). Thyroid ultrasonography showed bilateral multiple cysts in the thyroid, a firm lesion with calcification in the left parathyroid fossa, and oval lesion in the left pericarotid. Parathyroid scan showed an increased activity in the inferior aspect of left thyroid which was suggestive of parathyroid adenoma.
Laboratory examination showed the elevated ureum (202 mg/dL), creatinine (11.1 mg/dL), and normal calcium level (9.7 mg/dL). In November 2016, PTH level was >5000 pg/mL and phosphate level was 6.1 mg/dL.
Physical examination and laboratory examination lead us to brown tumor as the diagnosis due to secondary hyperparathyroidism. The treatment for this patient was subtotal parathyroidectomy with frozen section and intraoperative PTH evaluation. Intraoperative parathyroid hormone level was evaluated following subtotal parathyroidectomy. The result decreased to normal range 10.37 pg/mL (normal range: 10–65 pg/mL). Frozen section revealed hyperplasia of the parathyroid. Calcium level was evaluated every day for 7 days, and the result ranged from 5.0 mg/dL to 7.4 mg/dL. The parathyroid hormone on the 3rd month following the surgery was 4.31 pg/mL. Definitive pathology anatomy result revealed hyperplasia of the parathyroid with water clear cell parathyroid adenoma.
The masses in the maxilla, mandible, and sternum were observed to be significantly regressed. This patient then underwent renal transplantation approximately 4 months following parathyroidectomy. One month following the renal transplantation, PTH level was 6 pg/mL. |
pmc-6157170-1 | A 55-year-old Caucasian obese woman (body mass index = 35) was admitted to Surgical Department of our institution for acute abdominal pain. Her past medical history was negative for previous gastrointestinal disease or surgery. She was on medical therapy for hypertension, type II diabetes, and minor depression. Glasgow coma scale was 15. She referred an increasing acute abdominal pain risen 5 hours ago after a fish-based dinner. She has showed an acute diffuse peritonitis. White blood cell count was 32.000 U/μL, with neutrophilia (90%); other blood tests were in normal range. Body temperature was 39.2°C. Electrocardiogram showed sinus rhythm with 92 heart rate. Chest X-ray was normal. Abdominal X-ray showed free subdiaphragmatic air. CT scan confirmed the suspicion of small bowel perforation because of the finding of free fluid in the abdomen and an inhomogeneous mass in the small bowel. A nasogastric tube was placed, and it drained 50 mL of biliogastric material. Because of her status, she was immediately ran to the theater for exploratory laparotomy under general anesthesia and oral intubation. Although each clinical finding suggested a colonic or caecum perforation, during the systematic exploration of the bowel loops, surgeons found free intestinal fluid in the abdomen, fecal peritonitis, and (at 60–70 from ileocaecal valve) a 3 cm linear tear of the ileum which was caused by the curve edge of a shell mussel (). The foreign body was completely extracted from the lumen through the hole (), and the breach was sutured with simple double-strand stitches of polyglactin 3/0 parallel to the bowel tearing. Abdominal cavity washing was carried out with 2 liters of saline. Two drains were placed on suction for 24 hours. Antibiotic therapy (ciprofloxacin, meropenem, and metronidazole) and nil by mouth regimen were started. Patient was admitted in Intensive Care Unit for 12 hours, the weaning from the ventilator, and she was discharged at home in healthy status from the ward on the sixth postoperative day. At the 30-day follow-up, the patient was in good clinical condition, surgical wounds were completely sealed, blood tests were normal, and bowel function was recovered. |
pmc-6157181-1 | Case 1 was a 30-year-old, gravida 2, para 1 woman. At week 22 of pregnancy, she was admitted to our hospital because of a monochorionic twin pregnancy with cervical dilatation, frequent UCs on cardiotocogram, and polyhydramnios. As intervention, tocolysis was performed. Although UCs were reduced, ultrasonography revealed a maximum vertical pocket (MVP) of 12 cm (although the MVP of the co-twin was 4 cm) and the patient had dyspnea; therefore, emergency amnioreduction was performed, and 2,000 mL of amniotic fluid was drained during tocolysis. Intra-amniotic pressure was measured during the procedure. A saline-filled line was attached at one end to the hub of the needle and at the other end to a silicone stain-gauge transducer (DX-300; Nihon Kohden Corporation, Tokyo, Japan). Readings were recorded at the needle tip and were recorded if they were stable for 10 s. A zero pressure level line setting was performed at the estimated vertical line using the ultrasound-guided needle tip level (). Prior to this case, we did not perform this procedure for pressure measurement alone.
The intra-amniotic pressure was 16 mmHg before reduction, increased to 29 mmHg with UCs during reduction, and then declined to 9 mmHg after reduction. At this point, the Doppler of the recipient and donor showed the following results: UA PI, 1.42 and 1.54; middle cerebral artery (MCA) PI, 1.56 and 2.79; umbilical venous flow volume (UVFV), 149 and 110 mL/kg/min; ductus venosus (DV) PI, 0.74 and 0.65; cardiothoracic area ratio (CTAR), 24 and 30%; inferior vena cava PLI, 0.26 and 0.34. These data were within the normal limit, but the recipient had moderate tricuspid regurgitation. After amnioreduction, the Doppler of the recipient and donor showed the following results: UA PI, 1.18 and 1.79; MCA PI, 1.59 and 2.79; UVFV, 225 and 114 mL/kg/min; DV PI, 0.81 and 0.70. These data were within the normal limit, but the recipient's UA PI decreased mildly and UVFV increased. At week 25 of pregnancy, the MVPs of the donor and recipient were 1 and 15 cm, respectively. Therefore, the patient was diagnosed with stage II twin-to-twin transfusion syndrome, and fetoscopic laser photocoagulation was performed. Thereafter, caesarean section was performed due to labor onset at week 28 of pregnancy. Male neonates were born weighing 1573 and 1709 g, with Apgar scores of 5 and 4 at 1 min and 6 and 4 at 5 min, and umbilical arterial cord blood pH of 7.312 and 7.264, respectively. They were admitted to the neonatal intensive care unit because of prematurity and low birth weight and respiratory distress syndrome. The neonates responded well to the treatment. |
pmc-6157181-2 | Case 2 was a 29-year-old, gravida 1, para 0 woman. Fetal ascites was observed at week 33 of pregnancy, and the patient was admitted to our hospital. Test results for maternal serum cytomegalovirus and parvovirus B19 IgM were negative. Abdominocentesis was performed for diagnosis, and 99 mL of ascitic fluid was drained, containing 92% lymphocytes; hence, diagnosis of chylous ascites was made. The subsequent pregnancy course was uneventful until term. However, abdominocentesis was performed again to reduce the risk of dystocia [] at week 37 of pregnancy, with drainage of 510 mL of ascitic fluid during labor preparation. Tocolysis was performed for preventing UC only during the procedure. The fetal intraperitoneal pressure was 18 mmHg before drainage, 14 mmHg after drainage, and 32 mmHg when confirming UC on palpation. At this point, the fetal Doppler showed the following results: UA PI, 0.98; MCA PI, 1.08; and DV PI, 0.87. These values were within the normal limit. After abdominocentesis, the fetal Doppler showed the following results: UA PI, 0.65; MCA PI, 1.04; and DV PI, 0.59. These data were within the normal limit, but UA PI decreased. Thereafter, ascites recurred, and the mother had spontaneous premature rupture of membranes at week 38 of pregnancy. Caesarean section was performed due to cephalopelvic disproportion. A male neonate was born weighing 3117 g, with Apgar scores of 8 at 1 min and 9 at 5 min and umbilical arterial cord blood pH of 7.221. He was admitted to the neonatal intensive care unit for chylous ascites. Ascitic fluids were drained twice. However, although there was ascitic fluid retention, the amount did not increase. He was transferred to another hospital. |
pmc-6157181-3 | Case 3 was a 30-year-old, gravida 1, para 1 woman. Fetal pleural effusion was observed at week 22 of pregnancy during a routine prenatal visit, and she was admitted to our hospital. Skin edema and ascites were not observed. Thoracentesis was performed, and 24 mL of the pleural fluid was drained, containing 95% lymphocytes; hence, a diagnosis of chylothorax was made. Although the amniotic fluid index was normal, the intra-amniotic pressure remained as high as 20–22 mmHg because of frequent UCs on palpation even if tocolysis was performed for preventing UC during the procedure. The fetal intrathoracic pressure was 30 mmHg before and 19 mmHg after drainage. At this point, the fetal Doppler showed the following results: UA PI, 1.39; MCA PI, 1.57; UVFV, 72 mL/kg/min; DV PI, 0.58; CTAR, 17.1%; inferior vena cava PLI, 0.4; and TEI index (left/right ventricle), 0.474/0.581.These values indicate that CTAR was low and the right ventricle TEI index was mildly high. After thoracentesis, the fetal Doppler showed the following results: UA PI, 1.26; MCA PI, 1.42; UVFV, 73 mL/kg/min; DV PI, 0.63. These data were within the normal limit and did not change. Because pleural effusion recurred within 7 days after the first procedure, a thoracoamniotic shunt was inserted into the left pleural space 4 days after the first procedure according to the Japanese protocol for thoracoamniotic shunt []. Thereafter, the pleural effusion reduced and did not recur. Consequently, she was transferred to another hospital at week 26 of pregnancy. |
pmc-6157191-1 | A 75 year old Caucasian male with a long history of retinal changes was seen in the clinic. He had initially presented 31 years earlier, in 1985, with pigmentary changes at the macula noted by his optician. At the time his best corrected visual acuity (BCVA) was 6/9 in the right eye and 6/6 in the left eye. Fundoscopy showed pigmentary changes and drusen which were more easily visible on intravenous fundus fluorescein angiography (IVFA) (Fig. ). There was no evidence of any choroidal neovascular membrane (CNVM) on any of the images. The patient underwent multiple further IVFA examinations, each time demonstrating no active leak. His retinal appearance was monitored, with no conclusive diagnosis made, nor treatment available.
Ten years later ongoing review identified that the patient had raised intraocular pressure along with optic disc changes and a diagnosis of glaucoma was made with appropriate treatment initiated. Subsequently, in 2001, the patient was diagnosed with hypertension (186/110 mmHg) which, at the time, was thought to be essential hypertension. Retinal examination demonstrated cotton wool spots and haemorrhages, consistent with hypertensive retinopathy (Fig. ). He was also noted to have elevated serum urea and creatinine levels which were assumed to be related to the diagnosis of hypertension.
Following bilateral cataract surgery in 2003, which was complicated by posterior capsule rupture in the left eye, the patient was only able to achieve a BCVA of 6/36 in each eye. Due to inadequate control of IOP left sided trabeculectomy was performed in 2005.
At the same time, aged 64 years, the patient’s urea and creatinine levels were recorded as 20.5 mmol/L (normal 2.8–7.2 mmol/L) and 474umol/L (normal 60-105umol/L) respectively and he started regular haemo-dialysis for end stage renal failure shortly afterwards. He then underwent dual renal transplant in 2009 and was initiated on systemic immunosuppression (Tacrolimus 3 mg/day) which he continues to this day. The biopsy results from the explanted kidneys are unfortunately not available. Subsequently both transplanted kidneys failed over the coming year, with no identified cause, requiring the patient to undergo bilateral nephrostomies.
In June 2010 the patient was referred back to the Ophthalmology services, aged 69, with a presenting vision of 52 ETDRS (Early Treatment Diabetic Retinopathy Study) letters in the right eye and 35 letters in the left eye. He was again noted to have bilateral changes at the maculae which were, at the time, attributed to possible age related macular degeneration, and a small area of possible sub-retinal fluid. Comparison was made between the IVFA in 1985 and the IVFA at re-referral showing a significant increase in the number and distribution of the drusen, but no vascular leakage (Fig. ). The poor vision in the left eye was found to be secondary to advanced glaucoma and significant changes at the macula. No treatment was appropriate and the patient was monitored for 5 years with repeated Optical Coherence Tomography (OCT) scans.
In 2015 the OCT demonstrated an increasing sub-foveal space, raising a suspicion of CNVM. A loading phase of intravitreal anti vascular endothelial growth factor (anti-VEGF) was initiated in the form of Ranibizumab 0.5 mg. As there was no response to six Ranibizumab injections, treatment was subsequently switched to Aflibercept 2 mg with three further monthly doses. Again there was no improvement and treatment was ceased (Fig. ).
Due to an unusual appearance and history when considering age related macular degeneration, a systematic review of his previous notes and images was performed as part of a retinal multi-disciplinary team.
At the most recent visit (October 2016) the patient’s BCVA was recorded in the right eye as 49 ETDRS letters and awareness of hand movements only in the left eye. The fundus examination demonstrated multiple subretinal drusenoid deposits, which were autofluorescent, mainly at the posterior pole. Careful review of the OCT images demonstrated that the deposits were in Bruch’s membrane, with an intact Retinal Pigment Epithelium (RPE) (Fig. ).
Autofluorescence imaging demonstrated large areas of increased and decreased autofluorescence involving the macula and spreading inferiorly (Fig. ). OCT continued to demonstrate subfoveal hyporeflective areas. Pale optic discs were noted bilaterally, consistent with the long standing diagnosis of advanced glaucoma.
Wide angle (102°) infra-red imaging demonstrates the limitation of the retinal disease to the posterior pole, involving the macula, crossing the vascular arcades and including the nasal peri-papillary region (Fig. ).
The young age of presentation with drusen, recurrent kidney failure and increasing subretinal deposits (Fig. ), led to the suspicion of this being a probable case of dense deposit disease. He is currently being tested for serum C3 levels and C3 nephritic factor as well as genetic testing for a variety of mutations in complement associated genes, with the results awaited. |
pmc-6157200-1 | A 71-year-old man underwent McKeown minimally invasive esophagectomy (MIE) for middle third esophageal cancer. The review of the operative report did not reveal any crus division or intentional hiatal widening. The operation lasted 5 hours and 20 minutes. The postoperative course was complicated by cervical esophagogastric anastomotic leak, dysphonia, and swallow dysfunction with subsequent aspiration pneumonia. The patient was successfully treated by long-term enteral feeding and intensive care and was discharged in stable condition on the 46th postoperative day.
The histological examination revealed a stage IIIA (pT2N2M0) poorly differentiated squamous cell carcinoma.
At 3-month follow-up, multiple recurrences to right paratracheal lymph node, anterior chest wall, and right adrenal gland had been detected at positron emission tomography (PET) and computed tomography (CT) scan, and thus the patient was referred to the Oncological Unit to start adjuvant chemotherapy. However, a few days after admission, he complained of acute onset of severe upper abdominal pain, nausea, and dyspnea, which occurred immediately after a prolonged effort at defecation. On clinical examination, he was pale, bradycardic (35 beats per minute), hypotensive (blood pressure: 60/40 mmHg), and tachypneic (26 breaths per minute). The abdomen appeared excavated with diffuse tenderness and impaired bowel sound. Vesicular breath sounds were considerably reduced over the entire left hemithorax. After achieving satisfactory haemodynamic stability with high flow oxygen and iv fluid therapy, a CT scan with contrast was performed which documented the near-complete herniation of the small bowel, transverse colon, and greater omentum through a large defect (8.5 × 5 cm) of the left hemidiaphragm, resulting in ipsilateral massive lung collapse (). The patient was immediately transferred to our surgical unit to undergo emergency relaparoscopy for a giant diaphragmatic hernia. Due to the coexistence of several medical illnesses (alcoholic liver disease, chronic renal failure, and arterial hypertension), the patient was considered at high anesthesiological risk (class III, according to the American Society of Anaesthesiology Physical Status Classification System). After general anaesthesia, a double-lumen endotracheal tube was inserted in order to selectively ventilate the right lung in case of conversion to open surgery. The Hasson technique was used to create pneumoperitoneum 2 cm above the umbilicus, and three operative trocars were placed in the same sites of the previous operation (one 12 mm trocar in the left hypochondrium and two 5 mm trocars, respectively, under the xiphoid and in the right hypochondrium). After CO2 insufflation to a pressure of 12 mmHg, the abdominal cavity was explored with a 30° laparoscope. The entire hiatal anatomy was initially hidden by the herniated small bowel and transverse colon, which were gradually reduced into the abdominal cavity with progressive gentle traction (). This allowed for the identification of a large defect of the left hemidiaphragm, with its long axis oriented transversely from the gastric conduit to the superior border of the spleen. The left diaphragmatic pillar was not recognizable, and there was no evidence of hernia sac and diaphragmatic pleura (). Due to the direct communication between the peritoneal cavity and left pleural space, a hypertensive left pneumothorax was gradually developed, which mandated high fractions of inspired O2 and several vital capacity recruitment manoeuvres until the repair had been completed. The hernia orifice was primarily closed by approximating its anterior and posterior borders through a series of interrupted nonabsorbable stitches (0-Ethibond ™, Ethicon, Somerville, NJ, USA) (). In this way, a neo-hiatus was created, paying attention not to overtight the gastric conduit and preserve its vascular supply. At the end of the procedure, no drain tube was placed in the left chest, because the hemodynamic instability and the hypoxemia were definitively resolved. Before extubation, a chest X-ray showed a completely reexpanded left lung.
The postoperative course was uneventful, and the patient was discharged 7 days after surgery. No signs of hernia recurrence were identified on CT scan at 3-month follow up. The patient died 2 months later due to neoplastic cachexia. |
pmc-6157201-1 | A 27-year-old nulliparous woman presented to the emergency department complaining of abdominal pain on the background of chronic pelvic pain.
On admission, an enlarged right ovary 150cc in volume with a cyst measuring 6.5cm and low internal echoes was demonstrated on pelvic ultrasound. Abdominopelvic computed tomography (CT) scan also demonstrated a 6.5cm dense right ovarian cyst with a moderate volume of free fluid and no evidence of appendicitis. Tumour markers taken at the time of acute presentation demonstrated a serum CA 125 level of 8142 U/ml (reference range: <35 U/ml) which had significantly increased from 115 U/ml when performed 12 months prior. Serum alpha fetoprotein (AFP) and human chorionic gonadotropin (hCG) levels were both <2 U/ml.
She was referred to the gynaecology clinic at Westmead hospital for further urgent review and management. An ultrasound scan for deep infiltrating endometriosis (DIE) verified the presence of a right ovarian cyst (6.3 x 5.0 x 4.4cm) with bowel adherent to the posterior aspect of the uterus. A gynaecological oncological opinion was sought at this time in light of the significantly raised CA 125 recommending a repeat level in 2 weeks on the provisional diagnosis of endometriosis after reviewing the ultrasound images and patients history of initial presentation. Repeat measurement of serum CA 125 level taken two weeks from her initial presentation demonstrated a lower but still significantly elevated level of 2038 U/ml (day 12). Serum carcinoembryonic antigen (CEA) and CA 19.9 were <2 U/ml and 430 U/ml (reference range: <37 U/ml), respectively.
A multidisciplinary discussion with a gynaecologist oncologist was conducted to determine further management. Based on the images and decreasing serum CA 125 level an endometriotic leak from an ovarian endometrioma was considered most likely, with ovarian malignancy being the main differential and unlikely diagnosis.
At laparoscopy on day 58, stage four endometriosis and bilateral unruptured ovarian endometriomas, with features suggestive of leakage unilaterally, were revealed. Widespread endometriotic deposits were found at the upper and anterior abdominal wall, omentum, and bilateral uterosacral ligaments, likely secondary to leaking endometrioma (Figures and ). Laparoscopic excision of endometriosis, bilateral ureterolysis, bilateral excision of endometrioma, and insertion of a Mirena© intrauterine device were performed.
Histopathological examination confirmed the diagnosis of endometrioma. The patient recovered uneventfully and was discharged on the third postoperative day. At the third postoperative week the patient remained in a stable condition and routine follow-up with the general practitioner was recommended. |
pmc-6157206-1 | A 75-year-old woman was admitted in ICU after a cardiac arrest with return of spontaneous circulation, caused by tension pneumothorax. Now-flow and low-flow were 1 minute and 17 minutes, respectively. She was admitted 1 hour after the event, without sedation. Lungs were mechanically ventilated, the pleural drain was in place, pulse oximetry was 100% /FiO2 0.5, arterial pressure and heart rate were 160/70 mmHg and 110bpm, respectively, with norepinephrine at an infusion rate of 1mg/h, and temperature and glycaemia were 36.5°C and 8 mmol/L, respectively. There was no any sign of pneumothorax. Fever was actively treated without inducing hypothermia.
She was unconscious (Glasgow Coma Scale: 3/15), with a conserved bilateral photomotor reflexes. She had intermittent bilateral ocular revulsion and bilateral shoulders tremor. Propofol was initiated by a bolus followed by a continuous infusion and the movements of the eyes and shoulders ceased immediately. One hour later a 13 channels EEG ( in supplementary figures) diagnosed a NCSE as a pattern of generalized periodic spike-waves evolving in generalized rhythmic spike-waves at 1 Hz with high amplitude (> 200 µV), without response to stimulation. Midazolam was initiated by a bolus followed by a continuous infusion which permitted to achieve a burst suppression pattern ( in supplementary figures). A 4-channel sensor connected to BIS VISTA® monitor was placed, in order to monitor the two processed parameters and showed an isoelectric signal ( in supplementary figures).
On day 2 Clobazam and levetiracetam were added to ensure a bridging between IV and oral antiepileptic drugs. Conventional EEG recording showed a pattern of burst suppression and raw EEG from BIS monitor showed an isoelectric signal.
On day 3, the 4-channel sensor was replaced by a 2-channel sensor which was connected to patient's bedside monitoring. Propofol and midazolam were both decreased progressively. Few hours later (, , and ), while there were no any abnormal movements, raw EEG of BIS monitor connected to a Philips BIS module [panel A] and secondarily to a BIS VISTA module [panel B] showed a pattern of high voltage with irregular morphology, alternating with isoelectric signal, evoking spikes (white arrows), spike-waves (red arrows), and polyspikes (white star). BIS and SR values did not show significant variations.
Although it was not planned at that moment to request a conventional EEG recording, given the high suspicion of NCSE recurrence, the neurophysiology team was urgently contacted and the NCSE recurrence was confirmed and treated. The conventional EEG showed continuous generalized rhythmic spikes and spike-waves, sharply countered, of medium amplitude at 1-1.5 Hz ( in supplementary figures). Unfortunately the patient died on the fifth day. |
pmc-6157329-1 | A 54-years old male, weighing 66 kg, with tobacco use for 30 years, had undergone the primary partial laryngectomy 13 months before. He was scheduled for total laryngectomy and neck dissection. No abnormality was detected with preoperative physical examination and CT scan showed increased lung markings without metastasis. It also demonstrated moderate general condition with 3 cm of mouth opening, ECG with right bundle block, Malampatti grade II, without complaint of apnea or major depression signs. There was an 8 cm healed scar of a previous surgical incision along the cervical midline but with normal neck extension. Routine non-invasive blood pressure, ECG, and SPO2 were monitored. General anesthesia was induced with midazolam (1 mg), sufentanil (20μg), vecuronium (8 mg) and propofol (120 mg) followed by an attempted tracheal intubation using Macintosh laryngoscopy after preoxygenation. Nonetheless, the epiglottis and glottis were not viewed under Macintosh laryngoscopy, and then SPO2 dropped to 85% from 98%. Meanwhile, no effective ventilation was obtained by mask ventilation. The subsequent placement of neither an oropharyngeal airway nor a laryngeal mask airway (LMA) could resolve the difficult ventilation, which was accompanied by a quick drop of SPO2 to 25%, and an increased heart rate to 150 beats per minutes. In a short time, urgent tracheostomy was performed by a standby otolaryngologist, and then SPO2 and heart rate recovered after oxygen delivery through the placement of a coiled tracheal tube of 7.0 mm diameter. Anesthesia was maintained with continuous infusion of propofol, remifentanil, and inhalational sevoflurane. The patient emerged without sequelae from the short episode of hypoxia after the surgery was over. |
pmc-6157329-2 | A 57-year old male, weighing 66 kg, who had undergone semi-laryngectomy one year ago, was scheduled for total laryngectomy. The ECG was normal, and his blood pressure was 130/80 mmHg. The patient with Malampatti grade II had slight inspirational apnea which would be exacerbated by head lift. Therefore supine position was maintained. The lowest SPO2 of the patient was 90% under room air. Preoperative physical examination revealed that there was a fixed metastatic mass about 6 cm diameter upon the primary surgical scar of the previous incision along the cervical midline. CT scan of the neck reported malignant invasion of the tracheal wall. To prevent the slight apnea exacerbation, fibroscopy-guided conscious intubation was planned. The fibroscopy-guided tracheal intubation was performed successfully after administration of intravenous midazolam (1 mg), fentanyl (50 μg), and glottic topical anesthesia with 2% lidocaine (4 ml) spray through the suction channel of the fibroscope. After that, we proceeded with general anesthesia immediately and the surgery was uneventful. |
pmc-6157329-3 | A 63-years old male, diagnosed with recurrent laryngeal cancer after semi-laryngectomy 8 months ago, was scheduled to undergo total laryngectomy. He has 17 years' history of hypertension and his echocardiography revealed mild impairment of the left ventricular diastolic function. He has normal mouth opening and the airway was evaluated as Malampatti grade III. There were erythema and swelling under his right mandible with an erupted ulcer as well as neck immobility which were caused by tumor invasion. Non-invasive BP, ECG, and SPO2 were monitored. The preoperative blood pressure was 180/95 mmHg and was reduced to 130/80 mmHg following intravenous administration of urapidil (25 mg). After topical application of 2% lidocaine (4 ml) to the tracheal and oropharyngeal areas, intravenous injection of midazolam (1 mg) and fentanyl (50 μg) was followed by the fibroscope-guided nasal intubation. However, obvious structural deformation of the larynx was presented with impaired vocal cord mobility leaving only a“fissure” of space which thwarted the passage of the fibroscope even with 2.8 mm diameter. Subsequently, tracheostomy was performed under bilateral superficial cervical plexus block with assisted mask ventilation by 100% oxygen followed by 7.0 mm coiled tracheal tube placement. General anesthesia was maintained with intravenous propofol and inhalational sevoflurane. Dissection of the excised larynx verified the distorted structure of the glottis with an S-shaped stenosis. |
pmc-6157358-1 | A 42-year-old woman was referred to our center with severe gastroesophageal reflux disease (GERD) 5 years after having an open Nissen's procedure without mesh augmentation elsewhere. She had a giant recurrent hernia and pH-meter proven reflux; manometry showed moderate dysmotility. Surgery took 3 hours because of adhesions, and a configuration 2 graft deployment was performed. Recovery was uneventful until the 6th postoperative hour when orthostatic hypotension (systolic 85–90 mmHg) with an increased heart rate (120–125/min), unresponsive to fluid challenge, became evident. There was no chest pain, her enzyme levels and electrocardiography were normal. As hypotension in resting supine position and shortness of breath became evident, a bedside echocardiography and tomography were performed. Both revealed CT, and tomography also identified several “tacks” as the cause (). She was immediately transferred to cardiac intensive care for a possible intervention to release the tamponade. Heart rate and blood pressure slowly improved with titrated hydration under monitoring. She was followed up by serial echocardiograms, which showed slight decrease in tamponade. On the 4th day, she was transferred to the ward with a 90-minute heart rate and 115–120 mmHg systolic blood pressure. She was discharged from the hospital on the 10th postoperative day. A year after surgery, she was in perfect health, without any need for antiacid medications. Control imaging a month and a year after surgery confirmed the absence of any fluid collection.
The hyperdense nature of the element titanium in tomographic imaging allowed the anatomical analysis in this report. Contrast tomography was reconstructed in 3D with special intent to show the spatial distribution of all 16 titanium tacks, which were circumferentially deployed all around the hiatal opening (; Supplementary Data are available online at ). The video of the operation was reviewed, and starting from the first fixation, all tacks were numbered consecutively (). also shows the application of surgical numbering to the corresponding tacks in the 3D image. The axial, sagittal, and coronal sections were re-evaluated in-depth, with the guidance of the 3D restoration (). Targeted marking of any particular tack, by simultaneously identifying the same tack in all sections, allowed us to number all the tacks appearing in any particular tomography slice, according to the surgical numbering. A radiology expert, blinded to the numbering, was asked to assess the tomography slices to verify the positioning of the 16 tacks as probable causes of cardiac injury (coauthor B.A.). The closest distance from each tack to the pericardium was measured (). Tacks without any measurable distance from the pericardium were evaluated as “offensive” as the probable cause of tamponade, whereas tacks with a clear measurable distance were regarded as “nonoffensive” by the radiologist. The number of all tacks were then uncovered.
Furthermore, tack distribution with special reference to their proximity to the aorta and vena cava was measured. |
pmc-6157650-1 | A 61-year-old African American female with a history of chronic gastroesophageal reflux disease (GERD), dyspepsia, abdominal bloating, early satiety, alternating constipation, and diarrhea was referred to the gastroenterology clinic for diagnostic esophagogastroduodenoscopy (EGD) and colonoscopy. Her physical examination and laboratory workup were unremarkable. No radiographic examinations involving oral or rectal contrast had been performed. She reported compliance with split dose 4L polyethylene glycol 3350 solution (GoLYTELY, Braintree Laboratories, Inc., MA, US) bowel preparation. She was intubated for the procedures given her medical comorbidities. During her EGD, she was noted to have a scant amount of opaque, whitish, chalky residue in her stomach, along with a hiatal hernia, and was otherwise unremarkable. The digital rectal examination performed prior to colonoscopy revealed a similar substance with a more yellowish tinge. Colonoscopy revealed this substance coating significant portions of the colonic mucosa. It was first encountered in the distal sigmoid and was present throughout the colon proximally. The substance was initially suspected to be barium sulfate; however, the patient had not previously undergone any radiographic studies using enteric contrast agents. It was unable to be cleared and interfered with the visualization of the mucosa. On withdrawal, a large pedunculated polyp was found in the ascending colon (Figure ). The polyp was estimated to be 15 mm in size. A hot snare polypectomy was planned; however, the snare was unable to advance through the biopsy channel of the colonoscope despite several passes and the attempt was abandoned. On further withdrawal, several small polyps were seen in the sigmoid colon. Again polypectomy was attempted using cold biopsy forceps; however, was unsuccessful due to an inability to pass the forceps secondary to the clogged channel thought to be due to this substance. The colonoscopy was aborted and the patient was successfully extubated.
At this point, she was again questioned and denied any previous studies, investigations, or enteric contrast. She did, however, admit to taking one bottle of Tums almost every day for an unspecified amount of time. The patient was instructed to discontinue her Tums medication. Repeat colonoscopy one month later showed complete resolution of the foreign substance (Figure ), allowing for the successful polypectomy of the retained polyps. A histopathological examination revealed a 9 mm tubular adenoma in the ascending colon and several small hyperplastic polyps in the sigmoid colon. The clogged biopsy channel on the colonoscope used during the sentinel colonoscopy was easily cleared with standard cleaning practices and did not require refurbishment. |
pmc-6157651-1 | A 41-year-old woman who underwent open REYGB six years prior, presented with diffuse crampy abdominal pain, intermittent vomiting, and obstipation of few days. Physical examination revealed abdominal tenderness. Abdominal computed tomography (CT) scan showed dilatation of the biliopancreatic limb of the REYGB with SB intussusception through the jejunojejunostomy (Figures -).
Exploratory laparoscopy was performed with full reduction of a retrograde intussusception where involved bowel loops appeared viable. Extensive adhesions lysis was done and no bowel resection was deemed necessary. Bowel function returned two days after the operation and patient was discharged home. |
pmc-6157957-1 | The patient was an otherwise healthy 54-year-old male with stage 1, T1N0, squamous cell carcinoma who previously underwent partial right upper alveolectomy and obturator reconstruction 3 years prior. Two years postoperatively, he developed a local recurrence, requiring definitive upper alveolectomy including bone and hard palate resection from the midline to the right posterior molar. There was no history of radiation or plan for adjuvant therapy. Reconstructive options were reviewed including an osteocutaneous radial forearm or fibula flap, depending on the final intraoperative defect size. |
pmc-6158144-1 | A 68-year-old Japanese man presented at our hospital for evaluation and treatment of rectal cancer. He had no history of rectal cancer. He had been diagnosed with rectal cancer using colonoscopy for screening of rectal bleeding; following biopsy, the tumor was confirmed to be moderately differentiated adenocarcinoma. Barium enema showed a filling defect at the rectum below the peritoneal reflection (Fig. ). Contrast-enhanced CT (CECT) revealed swollen lateral lymph nodes (Fig. ). We diagnosed him as having rectal cancer cT4aN1M0 clinical stageIIIa (UICC 8th). We decided to perform neoadjuvant chemotherapy for locally advanced rectal cancer for the purpose of the prevention of postoperative local recurrence. We performed CapeOX therapy 1 course until the RAS results were out. Since the RAS gene was confirmed as the wild-type, we changed the regimen and performed FOLFOX + cetuximab therapy 1 course to reduce the tumor size. The reason why we chose cetuximab is we can perform chemotherapy with cetuximab until just before surgery. On day 14, he visited the emergency room due to perineal pain. Physical findings showed BT 38.0 °C, HR 117/min, BP 79/57 mmHg, tenderness, redness, and swelling of the perineum and scrotum (Fig. ). He had no tenderness of the abdomen. CECT revealed extensive emphysema in the scrotum and gluteus maximus muscle (Fig. ). We diagnosed as Fournier’s gangrene caused by penetration of a rectal cancer. We performed debridement, left orchiectomy, transverse colostomy with double orifices. Abscess cultures yielded Escherichia coli. Intravenous antimicrobial treatment was administered using cefmetazole (1 g every 6 h).
After the first operation, we cleaned the wound every day. Because his right testis was in poor condition, we performed right orchiectomy under local anesthesia on postoperative day (POD) 7. Once the granulation tissue had formed (Fig. ), we performed abdominoperineal resection (APR) and dissected the left lateral lymph nodes. on POD30 (Fig. ). The amount of blood loss was 320 ml, and the operation time was 269 min. Pathological findings were type3, 35 × 30 mm, tub2, ly1, v2, ypT3N1a(263), and M0 Stage IIIa (UICC 8th). Histological grade was 1a (RECIST v1.1). The tumor left side wall was thin. Because the effect of chemotherapy was poor, we diagnosed as Fournier’s gangrene caused by penetration of rectal cancer.
After the second operation, we cleaned the wound every day. He left the hospital on POD59. On POD92, the wound was healed like that in the picture (Fig. ). Since he wanted to leave the hospital, we showed him the treatment procedure for scar healing. We performed CapeOX therapy eight courses as adjuvant chemotherapy. The patient had no recurrence for 1 year and 2 months after the operation. |
pmc-6158368-1 | A 5.5-year-old boy visited the Endocrinological section of the Pediatric Unit of the General Hospital of Terni, Italy, due to short stature. His mother's height was 170 cm, and his father's height was 172 cm (target height: 177.5 cm = >50° centile). In his family history, there was paternal familiarity for psychotic crises in his adolescence and mother with thrombophilia and history of five pregnancies with three spontaneous abortions and two parts. The mother took cardioaspirine during the pregnancy. No genetic disease was reported in his family. The child was born at term via spontaneous vaginal delivery. The birth weight was 3.050 gr (10°-25° centile), and the birth length was 45 cm (below the third centile). A villocentesis performed at week 12 of gestation due to an alteration in nuchal translucency on obstetric ultrasound did not reveal any abnormality (46, XY). After birth, a small ventricular septal defect and mild bilateral pyelectasis were detected, but within 6 months, both of these abnormalities spontaneously disappeared. Bilateral orchiopexy was performed at 19 months of age for undescended testicles. Regarding linear growth, auxological data recorded by the primary care pediatrician revealed short stature that was 2 standard deviation (SD) below the 3° centile on the Italian cross-sectional growth charts for height () since the first months of life. In the last 2 years, the child has exhibited an even more exacerbated decline in his growth curve. Before entering the hospital, first-level laboratory tests for the evaluation of short stature (i.e., full blood count, inflammatory markers, renal function, hepatic function, coeliac screening, and thyroid function tests) had been already performed. No abnormal results were evidenced. The bone age was delayed by 2 years.
In the hospital, his height was 99.2 cm (<3 SD), his weight was 15.5 kg (<3° centile), and his growth velocity was 4 cm/year (<3° centile). His arm span was of 94 cm, and his sitting height was of 55 cm: height ratio was <95%, his sitting height: height ratio was therefore >56% (slightly over the upper normal limit of 55.5%), and his body mass index was below the 50th centile (pathological Rappold index >8). Furthermore, he presented with unusual facial features that included hypertelorism, low-set ears, a low posterior hairline, down-slanting palpebral fissures, pectus excavatum, and ogival palate (Figure ).
However, Madelung deformity was not suggested by clinical examination and not detected by wrist X-ray findings. Radiological and laboratory tests to rule out related renal, cardiac, ophthalmologic, and audiological abnormalities did not find any significant alterations. At an evaluation conducted by an infant neuropsychiatrist, the patient exhibited a mild cognitive impairment compared with the standard for his age that included a prevalent verbal difficulty compatible with the diagnosis of NS. EEG and cerebral magnetic resonance imaging results were normal. The peak growth hormone (GH) value in a clonidine stimulation test was in the normal range (12.7 mg/L). The IGF-1 level was 47 ng/mL (normal values for age: 32–259 ng/mL).
Although short stature was disproportionate, diagnosis of NS was suspected, and genetic tests of the PTPN11 gene were requested. The entire coding regions and intron-exon boundaries of the PTPN11 gene were amplificated by polymerase chain reaction using genomic DNA and sequence specific primers. Both forward and reverse strands were directly sequenced using an automatic DNA sequencer. The results confirmed NS because a c.922A>G (rs28933386) heterozygous mutation in exon 8 of the PTPN11 gene leading to a p.Asn308Asp substitution was identified (reference transcript NCBI NM_002834.3 and NP002825.3). This mutation has already been described as pathogenic by Siegfried et al. ().
Due to the presence of a mesomelic short stature, the concurrent presence of two genetic mutations was considered and a genetic evaluation of the SHOX gene was requested. A test of chromosomal microarray analysis performed with the CGH array led to the identification of a micro-duplication at the level of the region PAR 1 (Xp22.33/Yp11.32) extended about 680 kb involving the SHOX gene. The microduplication in the pseudoautosomal region PAR1 has already been described in NS and its clinical significance therefore appears to be certain ().
GH treatment (0.2 mg/kg/wk) was prescribed. At a follow-up assessment 18 months after the initiation of GH treatment (weight-appropriate dosage), the following data were collected: height, 109.8 cm (<2 SD); weight, 18 kg (<3° centile); growth velocity, 7 cm/year (>90° centile); arm span:height ratio, 96%; sitting height:height ratio: 56%. Further evaluations have been scheduled for every 6 months.
Management of the case was approved by the Ethics Committee of General Hospital of Terni, Italy (2017-PED-03). The patients' parents provided their written informed consent for the publication of this case report, including the photo of their child. |
pmc-6158465-1 | A 15-year-old girl with ulcerative colitis who was in remission and administered 5-aminosalicylic acidand intermittent prednisolone treatment for the past 6 years was admitted to the pediatric emergency department with abdominal pain attacks for the past 2 weeks. Her parents were first-degree cousins. She had a healthy sibling. She was born via spontaneous vaginal delivery with a birth weight of 3.200 g. She had no family history of inflammatory bowel disease and chronic or autoimmune disease.
Her physical examination revealed a body weight of 49 kg (10-25th centile) and a height of 160 cm (25-50th centile). She had fever of 36 °C. She was pale and had mild abdominal tenderness. The cardiovascular and respiratory system examinations were unremarkable.
The laboratory examinations revealed the following results: hemoglobin 10.5 g/L, white blood cell count 8000/mm3, platelet count 216.000/mm3, total protein 5 g/L, albumin 2.7 g/L, iron 24 ng/L, iron-binding capacity 380 ng/dL, ferritin 13 ng/mL, C-reactive protein 0.6 mg/dL, and erythrocyte sedimentation rate 11 mL/h. Urinary tests and stool analysis were normal.
Abdominal ultrasound and magnetic resonance enterography revealed mucosal thickening in the transverse and descending colon. Colonoscopy was repeated, which revealed small filiform polyps with 0.5×1.5×3 cm dimensions throughout the colon but most common in the rectosigmoid, descending, and transverse colon (). Histopathological examination revealed inflammatory polyps associated with ulcerative colitis (). Informed consent was obtained from the patient’s parents. |
pmc-6158467-1 | A 39-year-old man suffering from ankylosing spondylitis for the past 10 years presented with fatigue. He denied having fever, nausea, vomiting, diarrhea, confusion, or headache. He had been on certolizumab pegol treatment for 6 months, starting with 400 and 200 mg every 2 weeks. His initial complete blood count showed the following results: hemoglobin 14.4 g/dL, hematocrit 52%, leucocyte count 8900/mm3, and platelet count 415000/mm3. His monthly complete blood count evaluation was totally normal till 5 months after the start of the treatment, and 1 month before our evaluation, the hemoglobin level was 11.4 g/dL; however, the patient was asymptomatic and this result was ignored. He denied any recent travel or consuming illicit drugs or quinine-containing drugs or water.
His current complete blood count showed the following results: hemoglobin 5.3 g/dL, Htc 16.1%, mean corpuscular volume 90 fL, leucocyte count 4460/mm3, and platelet count 28000/mm3. Coagulation profile was normal with a negative D-dimer result. Biochemical analysis showed a significant elevation of lactate dehydrogenase level of 5680 U/L (upper limit of normal: 220 U/L), with a total bilirubin of 2.2 mg/dL, direct bilirubin of 0.32 mg/dL, and haptoglobulin of 28 mg/dL (lower limit of normal: 30 mg/dL). Renal functions were normal, and there was no proteinuria. Peripheral blood smear showed schistocytes in almost 20% of every erythrocyte per field. The patient was considered as having thrombotic microangiopathy, and plasma exchange along with 1 mg/kg/day of methylprednisolone were started. Before plasmapheresis, samples were collected for ADAMTS13 evaluation. With a daily plasma exchange and corticosteroid treatment and cessation of certolizumab, there was a rapid and dramatic improvement in the clinical and laboratory results of the patient, and after 10 consecutive exchange sessions, the plasma exchange was stopped, and the patient was discharged on corticosteroid treatment alone. No relapses were observed, and the corticosteroid treatment was rapidly tapered and stopped. ADAMTS13 activity was found to be 86%, which was normal as expected. Written informed consent was obtained from our patient for publishing this case report. The follow-up results of our patient are summarized in . |
pmc-6158768-1 | A 91-year-old male patient was referred to Matsue City hospital from a clinic for treating a right incidental renal tumor in November 2016. The case background is summarized in . Because the patient requested eradication of the disease, a laparoscopic RN procedure was performed in December 2016. Although surgical approach was conventional intraperitoneal approach, insufflation pressure was restricted <8 mm Hg considering advanced age. He experienced postoperative night delirium and was treated by administration of haloperidol. The patient was discharged on postoperative day (POD) 10 (). The pathologic diagnosis identified a multilocular clear cell renal cell carcinoma (RCC), Fuhrman grade 2, pT1b. |
pmc-6158768-2 | A 92-year-old male patient was referred to Matsue City hospital for treating a right renal tumor in February 2017. The case background is summarized in . The patient was in poor general condition with multiple comorbidities, which were considered to be critical obstacles for surgical treatment. Nevertheless, laparoscopic RN was performed in March 2017 because of the presence of progressive symptoms of a massive hematuria. Although surgical approach was conventional intraperitoneal approach, insufflation pressure was restricted <8 mm Hg considering advanced age. Postoperative continuous administration of heparin occurred for 7 days to prevent pulmonary infarction because of deep vein thrombosis (DVT) in lower extremities. Because the patient experienced postoperative night delirium, he was treated by administration of haloperidol and risperidone. The patient was discharged on POD 19 (). Although CT diagnosis was of right T3a RCC (), the pathologic diagnosis was invasive urothelial carcinoma, grade 3, pT3. |
pmc-6158768-3 | A 90-year-old female patient was referred to the Department of Urology from the Department of Neurology in Matsue City hospital for treating a left incidental renal pelvic tumor in July 2017. The case background is summarized in . The patient presented with a poor general condition and with multiple comorbidities, which were considered critical obstacles for surgical treatment. However, a laparoscopic radical nephroureterectomy was performed in August 2017 because of the urgent request for surgical treatment by the patient's daughter. Although surgical approach was a conventional retroperitoneal approach, insufflation pressure was restricted <8 mm Hg considering advanced age. Postoperative continuous administration of heparin was performed for 7 days to prevent pulmonary infarction because of DVT in the lower extremities. The patient experienced postoperative night delirium occurrence, and she was treated by administration of haloperidol. A postoperative ultrasonography of the lower extremities was employed to determine the increase of DVT. However, she complained of no symptoms caused by DVT in the lower extremities. She was discharged on POD 15 (). The pathologic diagnosis was invasive urothelial carcinoma, grade 2 > 3, pT3. |
pmc-6158818-1 | A 20-year-old Danish woman was referred with constant pain in the right proximal thigh that had progressed for 8 years. She had no other known medical conditions. Menarche was at age 13. There was no family history of sclerosing bone diseases. The limb pain was aggravated at night and was moderately relieved during physical activity and by cold baths. She was on daily analgesic medication with nonsteroidal anti-inflammatory drugs, paracetamol, and weak opioid agonists, and she was on sick leave from her university studies at the time of referral due to her symptoms. She was using shoe inserts because of leg length discrepancy.
At age 11 she had consulted a rheumatologist because of hip joint pain. Guided by ultrasound a hip joint puncture was performed due to fluid gathering; serology was negative. After the puncture, the joint pain ceased.
Six months prior to referral to our clinic, she was involved in a traffic accident and hit by a car from the right at knee level. She was discharged from an emergency room (ER) with no suspected fracture and a radiological examination was not performed. She reported that there was significant worsening of the femoral pain after the traffic accident.
A clinical examination revealed no skin lesions, café au lait spots, or redness; there was no increased temperature or swelling in her right thigh. Leg length was unequal but within normality.
She was instructed to fill out a diary with daily registration of pain on an analog scale from zero to 10, where increasing values were equivalent to more intensive pain. Orally administered analgesics were required when the pain exceeded a score of 6 or more, and the score 10 was reserved for symptoms that kept our patient from falling asleep and that did not respond adequately to orally administered analgesics.
Magnetic resonance (MR) and positron emission tomography-computed tomography (PET-CT) scans revealed significant increased cortical thickness in the right femoral diaphysis that partially obliterated the medullary cavity (Fig. ). The surface of the sclerotic bone was uneven. A Tc99-bone scan showed a pathological increase in uptake in most of the right femoral shaft (Fig. ).
Biochemical bone markers were normal, including normal alkaline phosphatase.
Screening for the GNAS1 activating mutation R201 was negative in peripheral blood; as was mutation screening of the SQSTM1 gene, which has been reported to be mutated in Paget’s disease of bone.
A bone biopsy was performed and showed thickened bone springs with preserved lamellar structure and slightly accentuated cement lines. There was no fibrosis, inflammation, or increased alcian positivity, which would be suggestive of active osteomyelitis. There were no signs of malignancy or neoplasia.
A gynecological examination prior to referral had found a mild degree of polycystic ovaries (PCO) with normal androgen status. There were no endocrinopathies.
During the clinical investigation, four tentative diagnoses were raised: atypical fibrous dysplasia, Paget’s disease of bone, chronic non-infectious osteomyelitis, and melorheostosis. The young age of our patient, normal alkaline phosphatase, and sparing of the metaphysis of the affected bone made Paget’s disease of bone highly unlikely.
The cortical thickening is atypical for the diagnosis of fibrous dysplasia. The absence of café au lait spots, the normal age at menarche, the absence of other endocrine disorders, and the negative GNAS1 mutation examination weighed against a diagnosis of fibrous dysplasia. The long bones are a common location for chronic non-infectious osteomyelitis and this can present in childhood; however, the radiological appearance and bone histology did not provide support for this differential diagnosis. With special emphasis on the uneven surface of the affected bone on X-ray (Fig. ), the diagnosis of melorheostosis, which was fully compatible with the relatively unremarkable histology, was found to be the most plausible diagnosis. In addition, the normal bone chemistry is in keeping with melorheostosis [] although in polyostotic cases alkaline phosphatase can be increased [].
The timeline for intervention and clinical outcome is presented in Fig. . Initially, zoledronic acid 5 mg was administered intravenously three times with 10-month and 12-month intervals; our patient had pain relief after the first administration, but a poor effect of the second and third injection. Using an analog pain scale she reported average pain of 9 prior to treatment, 6 after treatment with zoledronic acid, and 4 from 2 to 10 months after initiation of treatment with denosumab. Looking at the usage of pain medication, it went from daily dosage before treatment to 23% of days after treatment with zoledronic acid, and to no orally administered analgesics for 8 months following treatment with denosumab.
In October 2015, denosumab was chosen as the second line of treatment based on the hypothesis that the pain was caused by increased bone turnover. A dose of 60 mg was injected subcutaneously with some remission of pain that lasted for 6 weeks. At the same time as the pain relapsed, there was an increase in alkaline phosphatase, PINP, CTx, and osteocalcin in blood tests. Biochemistry before and during treatment is listed in Table . Based on these findings it was decided to shorten the interval between administrations to further strengthen control of a hypermetabolic state. The second dose of 60 mg was given after 8 weeks and this interval has been kept up to the present.
Biochemistry after treatment with both zoledronic acid and denosumab showed suppression of bone-type basic phosphatase, osteocalcin, collagen I, and procollagen I, with a relative increase in parathyroid hormone, and plasma ionized calcium within the normal range. The injections with denosumab were well tolerated with no adverse effects reported. During the whole treatment period, the injections were administered from our out-patient clinic without cancellations.
After the first three doses given 8 weeks apart, there was pain remission for 8 months; in this period our patient did not take any orally administered analgesics. At the latest follow-up in February 2018, she complained of a slight increase in pain and occasional use of paracetamol and nonsteroidal anti-inflammatory drugs.
The treatment with denosumab was well tolerated with no side effects observed.
A Tc99-bone scan and computed tomography (CT) scan were performed in February 2017 and in February 2018, 5 and 17 months after the first injection with denosumab; the pathologically increased activity and cortical thickening were stationary. |
pmc-6158825-1 | A 66-year-old morbidly obese man (body mass index of 47 kg/m2) was medically treated for hypertension, dyslipidemia and coronary artery disease for years. The patient underwent embolization of an occipital epicranial pseudoaneurysm induced by a previous head injury. Immediately after the intervention, the patient experienced a non-ST elevation myocardial infarction for which a coronary angiogram demonstrated a severe triple-vessel disease. A preoperative echocardiogram also showed a severe mitral insufficiency on a mixed mechanism. The patient underwent an uneventful coronary artery bypass graft operation with left internal mammary graft and a trans-septal bioprosthetic mitral valve replacement.
In the days following surgery, the patient developed severe delirium. On postoperative day 9, a sterile sternal dehiscence was documented for which a sternal reconstruction was done with titanium plates and partial bilateral pectoralis myocutaneous flaps (Titanium Sternal Fixation System, Johnson and Johnson®, Markham, ON). In the immediate postoperative period, the patient had atrial fibrillation witch required direct current cardioversion. De novo right hemidiaphragm elevation was seen on the chest X-ray with hemidiaphragmatic paralysis suspicion (Fig. ). The patient responded well to the clinical management and was extubated on postoperative day 19. Respiratory insufficiency was treated with non-invasive ventilation that was not well tolerated by the patient, which forced reintubation. A right hemidiaphragm elevation was present on the chest X-ray (Fig. ) and a transthoracic ultrasonography confirmed the diagnosis of diaphragmatic paralysis.
After three and a half weeks of conservative management, a decision was made to perform a right thoracoscopic hemidiaphragm plication following informed consent from the family. Plication was performed using resolvable sutures to lower the diaphragm and reinforced sutures were passed through the chest wall and tied at the skin level. The chest X-ray showed a lowered hemidiaphragm immediately after plication (Fig. ). The patient was kept intubated for another 5 days, to minimize stress on the sutures. The dynamic compliance increased progressively from 19 to 22 ml/cm measured at the preoperative period to 40–50 ml/cm postoperatively during pressure support ventilation. Subsequently, sedation and mechanical ventilation were discontinued and the patient was successfully extubated. During this period of time, the patient weight, volume status, delirium status, hemodynamic and hepatorenal values were all stable. Chest X-ray on the following days demonstrated better lung expansion on the right chest. The patient recovered and was discharged without any further respiratory distress and complication.
Five months following diaphragmatic plication, pulmonary function testing revealed a forced expiratory volume in the first second of 1.40 L, a forced vital capacity of 1.81 L, a total lung capacity of 3.53 L and a Tiffeneau index of 77%. A follow-up echocardiogram demonstrated a well-functioning mitral bioprosthesis and a normal left ventricular function. Three years later, similar pulmonary function testing was documented and the patient remain in a clinical functional class I (NYHA). |
pmc-6158831-1 | A 44-year-old married woman (gravida 2) was diagnosed with recurrent ovarian cancer and admitted to our hospital. Five years ago, she underwent concurrent right adnexectomy for the ovarian mass, subtotal hysterectomy, and left adnexectomy. The pathological diagnosis was (right) ovarian serous papillary carcinoma (stage IV). The uterine wall and cervix were affected by cancer tissue, and the left fallopian tube and left ovary were also invaded. She received 12 courses of taxinol plus carboplatin chemotherapy after surgery. Two years prior, a new pelvic mass was found by ultrasound, which was cystic and solid mixed and had a size of about 6 cm × 4.6 cm. Considering the diagnosis of ovarian cancer recurred after chemotherapy, the patient did not continue to receive treatment. Six months ago, CT images showed that multiple nodules and masses in the abdominal cavity and pelvis had increased (10.2 cm × 5.8 cm). Therefore, the patient received two courses of chemotherapy (paclitaxel liposome plus carboplatin) again.
Recently, the patient planned to continue her chemotherapy. The patient’s Karnofsky Performance Scale score was 90, and a hard pelvic mass with unclear boundary could be palpated. The patient’s white blood cell count and hemoglobin level decreased, and degree I chemotherapy-induced myelosuppression was considered.
Examination found the carbohydrate antigen 19–9 increased (89.16 kU/L), carcinoembryonic antigen increased (1.57 μg/L), human epididymal protein 4 increased (1477 pmol/L), carbohydrate antigen 153 increased (50.75 kU/L), carbohydrate antigen 125 increased (2210 kU/L). The patient’s premenopausal risk ovarian malignancy algorithm (ROMA) index was 99.72% and postmenopausal ROMA index was 99.46%.
CT showed multiple nodules and masses in the abdominal and pelvic cavity, and some lesions were accompanied by calcification (Fig. ). The larger mass in the pelvic cavity was 10.2 cm × 5.8 cm in the maximum section image and had an irregular shape and unclear boundaries with the surrounding bowel. Multiple lymph nodes were found on the right axilla, mediastinum, right chest wall, and retroperitoneal space. The right renal pelvis and ureter dilatation (not shown in the figures) was caused by the oppression of swollen lymph nodes. After multidisciplinary team consultation, we considered that the multiple metastatic lesions and lymph nodes could not be completely removed by reoperation. After communicating with the patient and her family, continuation of chemotherapy was agreed upon. |
pmc-6158831-2 | A 51-year-old woman (gravida 3) was admitted to our hospital due to abdominal distension and defecation that had increased for 6 months and had been aggravated for 3 days. Six months prior, the patient had abdominal distention, decreased diet, and increased abdominal circumference without obvious inducement. These symptoms were accompanied by lower abdominal discomfort and increased frequency of defecation. Ultrasound examination revealed peritoneal effusion and a pelvic mass that had a maximum cross-sectional area of 14.5 cm × 10.7 cm. Abdominal swelling and a palpable lower abdominal mass with irregular surface and tenderness were found by physical examination. The gynecologist considered that the poor mobility lesion was formed by the uterus and adhesive bilateral ovarian masses, but there were still some gaps between the mass and pelvis wall. The lower margin of the lesion went deeper into the posterior of the uterus and rectum, and no obvious metastatic nodules were found in the vaginal fornix. Premenopausal and postmenopausal ROMA index were increased (99.24% and 99.46%, respectively). Neuron-specific enolase was increased (21.16 μg/L) and sugar antigen-24, − 19, and − 153 were all increased. CT examination found cystic and solid masses (with cystic components) in the bilateral ovarian area (Fig. ). The peritoneum, greater omentum, and mesentery were thickened and accompanied by multiple nodules. All lesions were contrast enhanced in different degrees, with a large amount of fluid in the pelvic cavity.
During the operation, about 500 mL of red ascites was sucked out, a few miliary nodules on the top of the diaphragm were found, and the liver surface was smooth. The maximal omental mass of about 4 cm in diameter was attached to the spleen. A liver mass of about 6 cm × 5 cm × 3 cm was adhered and extensively infiltrated to the hepatic flexure and ascending colon. The axial diameter of the para-aortic lymph node was about 1.5 cm. There were multiple metastases on the mesentery of 0.5 to 1 cm in length, and the maximum diameter of the bilateral ovarian masses was about 10 cm. The ovarian masses were closely adhered to the sigmoid colon, uterus, and bladder. These masses and adhesions filled and closed the pelvic cavity (Fig. ). During the operation, the upper boundary of the lesions reached the pelvic inlet, the lower pole went deeper into the posterior part of the uterus and rectum, its two sides were close to and squeezed the iliac vessels, and the rectum could not be touched. The thickened and hardened posterior wall of the bladder was tightly adhered to the peritoneum, so the mass could not be moved. The appendix was about 8-cm long, which was invaded by the tumor and became thickened and hard. The upper part of the right ureter was thickened, and hydronephrosis was evident with a diameter of 2.5 cm.
The left ovary mass was taken for frozen pathological examination and diagnosed as a malignant ovarian tumor. Then, subtotal ovarian cancer resection and abdominal pelvic tumor cytoreduction were performed (Fig. ). The retroperitoneum was removed from the pelvic inlet, and the tumor was then resected from the posterior part of the rectum. The bilateral lower ureter gradually extended to the periphery of the uterus. The whole uterus and bilateral uterine appendages were removed. Resection of the greater omentum and appendix, the sigmoid colon, the rectum, and intestinal anastomosis was performed without visible residual lesions. The operation lasted for 3.5 h. Bleeding during the operation was 1600 mL, infusion was 4250 mL, and transfusion of red blood cells was 4.5 U and of plasma was 600 mL. |
pmc-6158874-1 | The patient was a 79-year-old man with chief complaints of exertional dyspnea, leg edema, and weight loss. On transthoracic echocardiography (TTE), a 25 × 40 mm mobile pedunculated mass continuous with the right ventricular heart muscle was detected in the right atrium and the patient was admitted to our department for close examination and treatment. At admission, his height was 162.0 cm, body weight was 61.1 kg, body temperature was 36.3 °C, pulse was 62 beats/min, blood pressure was 112/59 mmHg, and SpO2 was 100% (room air). Pulmonary sounds were clear with no crackles, and heart sounds were regular with no murmur. Leg edema was present.
Plain chest radiography revealed a cardiothoracic ratio of 49% with no cardiac dilation. Electrocardiography revealed a sinus rhythm with a heart rate of 71 beats/min with nonspecific ST-T segment changes. Blood chemistry revealed the following: white blood cell (WBC) count of 51.9 × 104/μL, hemoglobin (Hb) of 14.9 g/dL, platelet (Plt) count of 16.3 × 104/μL, creatine kinase (CK) of 81 U/L, creatine kinase-MB (CKMB) of 8 ng/mL, lactate dehydrogenase (LDH) of 161 U/L, C-reactive protein (CRP) of 0.10 mg/dL, carcinoembryonic antigen (CEA) of 0.7 ng/mL, prostate-specific antigen (PSA) of 1.2 ng/mL, squamous cell carcinoma (SCC) antigen of 1.2 ng/mL, and soluble IL-2 receptor: 633 U/mL. Inflammatory parameters were within the normal range and the soluble IL-2 receptor level was slightly elevated, but the levels of other tumor markers were within their normal ranges. A coronary computed tomography (CT) scan showed no significant stenosis. It was deemed very difficult to completely excise, so we decided on partial tumor resection with MICS to reduce the risk of tumor embolism and to obtain an accurate pathology diagnosis. Therefore, we did not perform CAG. If we had performed a CAG, we may have seen arteries feeding the tumor.
TTE showed a 40 × 30-mm mobile pedunculated tumor in the right atrium that was continuous with the right ventricular heart muscle (Fig. ). Transesophageal echocardiography (TEE) showed a solid septated tumor with an irregular surface invading the free wall of the right atrium and surrounding the annulus of the anterior cusp and right and left coronary cusps of the aortic valve (Fig. ). Contrast-enhanced CT showed invasion based on soft tissue intensity near the tricuspid valve above the anterior right ventricle in the region between the aorta and pulmonary artery and around the pulmonary artery (Fig. , ). Cardiac magnetic resonance imaging (MRI) showed a thickened anterior wall near the tricuspid valve and a mass protruding into the lumen and expanding into the region between the aorta and the pulmonary artery (Fig. , ); in addition to the patient’s advanced age, these features made it difficult to perform extended cardiac surgery. On fluorodeoxyglucose positron emission tomography (F-18 FDG-PET), there was abnormal accumulation in the right atrium surrounding the aortic root (Fig. ).
Surgery was performed to prevent tumor embolism-induced sudden death and to obtain a pathological diagnosis. Anticoagulation was not performed preoperatively because it was unlikely that the tumor was a thrombus. A double-lumen tube was inserted during surgery and a Swan-Ganz catheter and 14-Fr cannula were inserted through the right internal jugular vein after draping. In a supine position with 30° elevation of the right side, a 4-cm skin incision was made in the fourth intercostal region at the medial aspect of the nipple. Meanwhile, the femoral artery (FA) and femoral vein (FV) were exposed. A pericardiotomy was performed 2 cm anterior to the phrenic nerve and the pericardium was elevated. After systemic heparinization, an 18-Fr blood supply tube was inserted through the right FA, and a 25-Fr cannula was inserted through the right FV to establish a cardiopulmonary bypass (CPB). The superior vena cava was blocked with a bulldog clamp and the heart rate was controlled at 40–50 bpm with a β-blocker. An oblique incision was made in the right atrium with the heart beating, and the lumen was observed. The tumor adhered to the anterior surface of the right atrium but not to the annular region and had marked mobility. The tumor was grasped with an Endocatch and the pedicle of 1-cm width was transected using electric cautery (Fig. ). The lack of any residual right atrial tumor or shunt was confirmed and the right atrium was closed in a double suture pattern; the patient was then weaned from CPB. The pericardium was closed as far as possible, the wound was closed by the standard method, and surgery was completed. The operative time was 1 h 56 min, and the duration of CPB was 38 min (Additional file ).
Intraoperative macroscopic findings revealed a tumor with a smooth, greyish-white surface (Fig. ). A blackish-brown region suggestive of hemorrhage was present inside and on pathological examination (Fig. ), a diffuse proliferation of round cells with a high nuclear-to-cytoplasmic (N/C) ratio were observed on hematoxylin and eosin staining. The tumor cells were mainly medium- and small-sized cells that contained nuclei with a shallow cut that were the same size or slightly smaller than the nuclei of vascular endothelial cells; large cells were also present. Broken nuclear products and histiocytes phagocytosing these products were also observed. Upon immunohistological staining, the tumor cells were CD79α-positive and CD3-negative. B-cell-derived cells were overwhelmingly predominant, which suggested that the lesion was a B-cell-derived tumor. Epithelial membrane antigen (EMA) immunostaining was negative. Based on these findings, the patient was diagnosed with diffuse large B-cell lymphoma (DLBCL).
Extubation was performed 6 h after surgery and the patient was transferred to a general ward 2 days after surgery. The disappearance of the tumor from the annular region was confirmed on TTE 5 days after surgery and the patient was discharged when he was able to independently walk 6 days after surgery. After observation at an outpatient clinic, rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) therapy was initiated 37 days after surgery. After 8 cycles were administered in total, an FDG-PET scan performed 456 days after surgery showed no abnormal accumulation (Fig. ), indicating CR. |
pmc-6158900-1 | Patient MDX245, a 48-year-old female, presented with bilateral multiple pulmonary metastases from low grade endometrial stromal sarcoma. She was initially treated with laparoscopic surgery and a regimen including lobaplatin, doxorubicin and ifosfamide. After only 2 cycles of chemotherapy, her disease progressed in the lungs and severe myelosuppression developed. Clinical investigation indicated that the patient could be a candidate for apatinib therapy. The MiniPDX tests with 4 different targeted drugs in 5 regimens showed that the lung metastasis responded to single agent apatinib and apatinib in combination with olapanib, but not to metformin, pazopanib or pazopanib combined with olapanib (Fig. a). Indeed, 4 months post treatment, the patient achieved partial regression in her lung metastases that lasted for 8 months (Fig. b, c). The patient was currently being followed up. |
pmc-6158926-1 | A 72-year-old man presented to our hospital with a chief complaint of chest pain on exertion. He had multiple coronary risk factors including hypertension, dyslipidemia, and diabetes mellitus. Electrocardiography revealed no clear findings of ischemic ST-T changes or prior Q-wave myocardial infarction. Transthoracic echocardiography revealed an ejection fraction of 63.3% with mild left ventricular hypertrophy and no segmental wall motion abnormality. Coronary computed tomography angiography revealed a mixed plaque in the distal left main trifurcation with suspicion of significant stenosis. He was admitted to our hospital to undergo coronary angiography with a diagnosis of stable exertional angina pectoris.
Diagnostic coronary angiography revealed distal left main trifurcation disease including significant stenosis in the distal left main trunk (LMT), ostial left anterior descending artery (LAD), and ostial left circumflex artery (LCX) (modified Medina classification 1-1-1-0) () []. No significant stenosis was present in the right coronary artery. Because the SYNTAX score was calculated as 15 [] and the patient refused a surgical approach, PCI using a DES was planned. Because both the LCX and intermediate branch were large vessels, it was essential to preserve the blood flow of these side branches after main vessel stenting. Therefore, we decided to perform PCI using the simultaneous jailed balloon and jailed Corsair technique for this left main trifurcation lesion.
After obtaining informed consent, PCI for the left main trifurcation lesion was performed. An 8 Fr AL 1.0 guiding catheter with a side hole (Hyperion; Asahi Intecc, Aichi, Japan) was engaged into the left coronary artery via the right femoral artery. A SION guide wire (Asahi Intecc) was initially inserted into the LAD. To protect the two large side branches, a Balance Middleweight Universal II guide wire (Abbott Vascular Japan, Tokyo, Japan) and a SION Blue guide wire (Asahi Intecc) with a Corsair Pro microcatheter (Asahi Intecc) were introduced into the intermediate branch and LCX, respectively ().
According to the angiographic and intravascular ultrasound images, we decided to perform direct crossover stenting from the LMT to LAD using the simultaneous jailed balloon and jailed Corsair technique. A DES (SYNERGY 3.0 × 16 mm; Boston Scientific, Natick, MA, USA) was advanced into the LAD, and a semicompliant balloon (canPass 2.5 × 15 mm; Japan Lifeline, Tokyo, Japan) was advanced into the intermediate branch. Subsequently, a Corsair Pro microcatheter was introduced into the LCX. The simultaneous jailed balloon and jailed Corsair technique was then performed. The side branch balloon in the intermediate branch was initially inflated at low pressure (3 atm) (). The main branch stent balloon in the LMT to LAD was then inflated three times at nominal pressure (11 atm), which simultaneously jailed the side branch semi-inflated balloon in the intermediate branch and the Corsair Pro microcatheter in the LCX (). When the jailed Corsair Pro microcatheter was removed with rotation of the shaft, only small friction was felt. Blood flow was preserved in both the intermediate branch and the LCX after main vessel stenting, and there were no signs of plaque or carina shift into the side branches ().
After reinsertion of a SION Black guide wire (Asahi Intecc) into the jailed intermediate branch via stent struts using a double-lumen catheter (Crusade K; Kaneka Medix, Osaka, Japan), kissing balloon inflation was performed with a 3.0 × 16 mm stent balloon in the LAD and a 2.5 × 15 mm canPass balloon in the intermediate branch (). Final kissing balloon angioplasty was then performed with a 3.0 × 16 mm stent balloon in the LAD and a 2.5 × 15 mm canPass balloon in the LCX (). A final coronary angiogram showed excellent results without any complications, including side branch narrowing or occlusion (). The postprocedure course was uneventful, and no major complications including ischemic events, heart failure, or access-site complications were observed. The patient was discharged the day after the procedure, and his angina symptom completely disappeared after PCI.
Because left main trifurcation was anatomically crucial, an early follow-up coronary angiography was performed 2 weeks after the procedure. It showed excellent results and no findings of restenosis in both the main vessel and side branches. In addition, fractional flow reserve (FFR) was measured and there were no signs of significant ischemia in both distal LAD (FFR: 0.88) and LCX (FFR: 1.00). |
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