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pmc-6076115-1 | A 35-year-old male presented to our out-patient department with complaints of recurrent abdominal pain and general weakness for 1 week. No history of prior surgery, trauma, or any other comorbidity existed. He had no alcohol abuse habit or familial history of pancreatic disease. The initial computed tomography (CT) scan demonstrated a large cystic lesion in the upper abdomen and the origin of the lesion could not be identified.
At physical examination, an immovable abdominal mass was detected in the upper quadrant was found and the tenderness and rebound-tenderness of the whole abdomen were obvious. Initial laboratory findings revealed mild leukocytosis (10.7 × 109/L), elevated neutrophil granulocytes (89% of the leukocytes) and elevated C reactive protein (CRP) (373.2 mg/L). The measured tumor markers were within the normal range. These results decreased the likelihood of a diagnosis of malignancy. His other laboratory investigations were within the normal reference ranges. Subsequently, he was admitted to the general surgery department. During his hospitalization, a CT scan and ultrasound (US) were performed again to assess the properties of abdominal lesions. The US showed that a large cystic lesion occupied the abdomen (Fig. ). The review result of the CT scan showed a large cystic lesion of the abdominal cavity, which was considered a lymphatic cyst combined with purulent inflammation (Fig. A). Under the direction of the B-ultrasonic scan, we obtained 20 mL liquid from the cystic lesion through fine needle aspiration. Cell morphological examination showed a large number of lymphocytes and fewer monocytes in the cystic fluid. Then we gathered the cast-off cells, and identified the cells by immunohistochemical (IHC) staining. We observed the positivity of CD31 and D2-40 in the cast-off cells.
Due to the volume of tumor occupied most of abdominal cavity interspace, laparoscopic exploration was hard to perform. Therefore, the patient underwent excision laparotomy of the cyst based on clinical and radiological findings. Abdominal exploration was performed and a cystic lesion measuring approximately 40.0 cm × 30.0 cm originated from the body of pancreas and extended into the mesocolon (Fig. ). The tumor invaded to the spleen and no invasion into vascular structures. In order to retain the pancreatic secretion function and achieve radical resection effect of the patient, distal pancreatectomy (including the cyst, the body, and tail of pancreas) and splenectomy were performed. The pathologic reports showed that, the cyst measured 22.0 cm × 15.0 cm × 5.0 cm (the out-flowing lymphatic fluid in the surgery caused decreased volume of the specimen), and the cyst had a thin membranous appearance. Histological analysis revealed the variable sizes of multiple cysts with thin walls and ectasia of the lymphatic vessels (Fig. ). IHC stains for SMA, CD31, and D2-40 showed positivity, but CD34 staining was negative. The final pathological diagnosis was pancreatic cystic lymphangioma. After 19 days, the patient was discharged without any complications. |
pmc-6076118-1 | A 36-year-old man who denied previous systemic disease had a history of drug abuse with ketamine for 6 to 7 years (at a frequency of 2–3 times per week, by nasal inhalation, and hence the dosage could not be measured), and had then ceased use for approximately 4 years.
He had suffered from dysuria, bladder pain, and a mild burning sensation during urination, especially over the urethral meatus and the perineal region, for approximately 1 month prior to admission. He ignored these symptoms initially, but the burning pain worsened, with concomitant urinary frequency and urgency. He visited another hospital for help, at which routine urine analysis revealed pyuria. Under the impression of acute prostatitis, oral antibiotic treatment with ciprofloxacin was initiated during an outpatient visit; however, his symptoms remained, with no improvement. Two days before admission to our hospital, the symptoms worsened, with a newly-developed decreased voiding amount (approximately 50 mL per void) and urgency with urge incontinence, accompanied by painful hematuria and blood clot formation, especially at the first urine void of the morning. The patient then presented to our Emergency Department. Urine analysis showed pyuria, over 100 white blood cells (WBCs)/high-power field (HPF), and significant tenderness and swelling of the prostate was noted upon digital rectal examination, but no pus-like urethral discharge was seen. Under the impression of acute prostatitis, for which oral antibiotic treatment had failed, the patient was then advised to undergo hospital admission for advanced antibiotic treatment.
After admission, we consulted an infectious disease specialist for evaluation, and antibiotic treatment with ceftriaxone was started immediately. A blood test revealed WBC 4870/μL without predominance of neutrophils or eosinophils. No marked elevation of serum C-reactive protein (CRP) (0.25 mg/dL) was noted. The patient's temperature after admission had remained within the normal range, and there were no accompanying signs or symptoms of toxicity. Several blood and urine cultures were performed, including tuberculosis, but all results were negative. The symptoms of pyuria (which remained over 100 WBCs/HPF), urgency, and painful hematuria persisted with no improvement after one week of intravenous antibiotic treatment. However, a sonogram performed upon admission revealed suspected bladder wall thickening. Due to the persistent symptoms that failed to respond to advanced intravenous antibiotic treatment for 1 week, abdominal computed tomography (CT) with contrast was then arranged. The CT scan showed asymmetrical wall thickening (thickness of up to 1.2 cm) of the anterior aspect of the urinary bladder with a mural nodule, and mucosal enhancement with perivesical fatty stranding (Fig. ). According to the above findings and the clinical symptoms, bladder cancer was highly suspected, and we discussed cystoscopy with bladder biopsy with the patient and his wife, which was then performed the next day. Prior to hydrodistension, the bladder mucosa presented with hypervascularity, but there was no tumor over the anterior wall of the bladder as seen on the CT scan. The bladder mucosa of the anterior wall was erythematous, with multiple hump-like changes, and several biopsies were performed. After hydrodistension at a pressure of 90 cm H2O for 8 minutes, the bladder capacity was approximately 150 mL, and bleeding over multiple aspects of the bladder was seen, with glomerulation and ulcerative changes (Fig. A and B). Pathologic analysis of the bladder biopsies showed erosive cystitis, characterized by denuded urothelial cells, with prominent infiltration by eosinophils, lymphocytes, neutrophils, and plasma cells over the mucosa and submucosal layer. In addition, hypervascularity and submucosal granulation formation with fibrosis were observed (Fig. A and B).
After the operation, the bladder capacity increased a little, to approximately 70 to 80 mL per urination, but the urgency, frequency, nocturia, and hematuria still persisted. We also prescribed an anticholinergic agent, a beta-3 agonist and a nonsteroidal anti-inflammatory drug (NSAID), and the patient was then discharged. During 2 weeks of outpatient treatment, his symptoms did not improve with medication. Thus, we discontinued medical treatment and performed hyaluronic acid (HA) instillation, once a week for a total of 10 times. After the treatment, the symptoms of urgency, frequency and nocturia improved, and the bladder capacity increased to 350 mL per urination according to the patient's own voiding diary; in addition, no morning hematuria or hematuria after holding back urine occurred. After the patient's symptoms had improved, we arranged follow-up MRI of the bladder and cystoscopy; on the images, no thickening of the bladder wall nor nodules were observed (Fig. ). Cystoscopy showed marked improvement of the previously-noted erythematous bladder mucosa, and there was neither active bleeding nor glomerulation seen during the whole procedure. Bladder biopsy near the previous biopsy site was performed, and the final pathologic analysis showed decreased inflammatory cell infiltration, regeneration of the urothelium, and less vascularity (Fig. A and B).
Written informed consent to publish this case report was provided by the patient, and the consent procedure was approved by the Ethics Committee of Tri-Service General Hospital. |
pmc-6076147-1 | We present the case of a female Caucasian patient, aged 22 years, which has fixed dental appliance for one year, who was admitted into the Infectious Diseases department for a feverish syndrome associated with migratory joint pain for the last 2 months, gait abnormality, weight loss. She was neurologically, rheumatologically, and imagistically (a lumber magnetic resonance imaging—MRI scan was performed and revealed a normal lumbar spine) investigated. At the time of admission, on physical examination, the following changes were noticed: altered general status, cachexia (BMI of 15.82 kg/m2), oxygen saturation 98%, heart rate of 100 beats per minute, systolic murmur in the mitral area grade IV of VI, blood pressure 95 over 60 mm Hg, and a hepatomegaly of 1 cm. Repetitive hemocultures were positive for S viridans, while transthoracic echography revealed a severe mitral failure through the anteromedial (A3) segment of the anterior mitral valve leaf (AMVL) prolapse with eccentric jet to the posterior wall. To complete the investigations a transesophageal echocardiography was also performed and certified the diagnosis of mitral valve infective endocarditis (a vegetation of 8 mm was attached to the anteromedial segment of the anterior mitral valve leaf with irregular edges and hypoechogenic aspect). The most important laboratory studies are presented in Table .
Treatment with Vancomycin and Gentamicin was initiated over the first 2 weeks, in parallel with the extraction of the dental braces, with a slow favourable evolution, the patient becoming afebrile. Subsequently, Ceftriaxone and Vancomycin treatment was continued, under which fever recurred, accompanied by a generalized, intense pruritic erythematous rash (considered as red man syndrome), which led to the cessation of the whole therapy. Antibiotic treatment with ampicillin was initiated, under which the patient became afebrile, allowing the administration of the antibiotic therapy for up to 4 weeks.
After obtaining negative hemocultures, the patient was referred to the Cardiovascular and Thoracic Surgery Department of the European Hospital Polisano for the surgical intervention.
The minimally invasive approach of the mitral valve was made through the video-assisted minithoracotomy. Upon mitral valve inspection, the presence of small vegetation on the free edge of the anteromedial (A3) segment of the anterior mitral valve leaf and the prolapse of this segment through chordal rupture, has been revealed. The vegetation was excised and 4 GORE-TEX Suture were inserted on the anteromedial (A3) and anteromiddle (A2) segments of the anterior mitral valve leaf. The mitral plasty was completed by the suture of a fully mitral ring Memo 3D ReChord Annuloplasty Ring no.26, with 10 Ethybond Excel 2-0 sutures, fixed with a COR-KNOT device.
Intraoperative and subsequently post-discharge transesophageal echography, highlighted normofunctional mitral plasty with a remaining regurgitation of grade I-II, good openness, minor tricuspid regurgitation, and mild pulmonary hypertension. |
pmc-6076191-1 | The patient was a 36-year-old woman. A dry cough had persisted for approximately 2 months, and her physician had treated her with oral antibiotics, but her condition did not improve. She then visited our hospital. The results of acid-fast bacterium smear and PCR for M tuberculosis were both positive. Lesions with a stenosis rate of 25% to 50%, based on the airway stenosis classification described by Freitag et al,[ were noted primarily in the left main bronchus. A local spray with fluticasone propionate nasal drops was administered for 1 week, 4 times in total and follow-up time period was 24months. Cicatricial stenosis was successfully prevented (Figs. and ). |
pmc-6076191-2 | The patient was an 81-year-old woman. She visited a physician for complaints of cough and fever persisting for several days. On radiography, pneumonia was suspected and treated; however, her symptoms did not improve. The smear and polymerase chain reaction (PCR) results for M tuberculosis were both positive; therefore, she was transferred to our hospital. Lesions with a stenosis rate of approximately 50% were primarily in the left main bronchus, and local steroid spray was administered for 1 week, 6 times in total and follow-up time period was19 months. This protocol was similar to that in Case 1. There was improving a narrowing of the bronchial lumen by the ulceration of the protruding granulation covered with a white coat, but she could not tolerate bronchoscopy and the lesion extended. Thus, treatment was switched to systemic steroid administration; however, cicatricial stenosis eventually remained (Figs. and ). |
pmc-6076191-3 | The patient was an 82-year-old woman. A dry cough had persisted for approximately 3 months and bloody sputum appeared. Therefore, she visited a physician. The results of acid-fast bacterium smear and PCR for M tuberculosis were both positive; therefore, she was transferred to our hospital. A lesion with a stenosis rate of 90% was at the entrance of the middle lobar bronchus. Local spray with triamcinolone acetonide was administered for 1 week, 2 times in total and follow-up time period was 2 months. There was improving a narrowing of the bronchial lumen by the ulceration of the protruding granulation covered with a white coat, but she could not tolerate bronchoscopy. The treatment was completed. She transferred to another hospital because her home was distant (Figs. and ). |
pmc-6076191-4 | The patient was a 52-two-year-old woman. A wet cough had persisted for approximately 1 month and a physician treated her with antibiotics. However, her condition did not improve. Results of the acid-fast bacterium smear and PCR for M tuberculosis were both positive. She was referred to our hospital. Lesions with a stenosis rate of 25% to 50% were primarily at the entrance of the left B6. Local steroid spray was administered for 1 to 2 weeks, 12 times in total and follow-up time period was 14months. This protocol was similar to that in Case 3. Stenosis improved. Cicatricial stenosis was prevented (Figs. and ). |
pmc-6076200-1 | A 70-year-old man was referred to our hospital by his primary doctor because of warmness, pain, and swelling in his left leg and a feeling of gait disturbance 2 days previously. The patient had a history of bronchial asthma, which was diagnosed at 50 years of age. Oral steroids had been prescribed from 61 years of age, and he receives insulin treatment due to steroid-induced diabetes mellitus. He also has a medical history of eosinophilic sinusitis, eosinophilic pneumonia, and cerebral infarction.
On physical examination, he was 1.76 m tall and weighed 68.0 kg (body mass index = 22.0 kg/m2). There was swelling and tenderness in his left leg and the left thigh circumference was greater than the right (46.7 cm vs 43.0 cm). His blood pressure, pulse rate, respiratory rate, and arterial oxygen saturation were 151/83 mm Hg, 110 beats/min, 16/min, and 98% (room air), respectively. The main laboratory findings were as follows: D-dimer, 44.1 μg/mL (normal range, <1.0 μg/mL); C reactive protein, 7.17 mg/dL (0.00–0.47 mg/dL); HbA1c, 9.6% (4.6–6.2%); protein C, 35% (64–146%); and antithrombin III, 85% (97–111%). The patient's other laboratory data are shown in Table .
Although the level of carcinoembryonic antigen as a tumor marker was increased (7.8 U/mL; 0.0–5.0 U/mL), malignancy was not observed on further examinations including computed tomography (CT). Electrocardiography exhibited sinus tachycardia and findings on the chest radiograph were normal. Venous ultrasonography showed extensive thrombosis in the left iliofemoral vein, left popliteal vein, and left posterior tibial vein. In addition to these thrombi, contrast-enhanced CT detected spreading of the thrombus in the IVC (Fig. ), with several thrombi in the left upper lobe branch and the bilateral lower lobe branch (Fig. ). According to these findings, he was diagnosed with PE and massive DVT.
On the day of admission, subcutaneous fondaparinux (7.5 mg once daily) was started and a Günther Tulip Filter (Cook Medical Inc., Bloomington, Ind) was deployed in the suprarenal IVC via the right internal jugular vein to prevent a fatal PE. Because the DVT did not improve despite the reduction of the PE after 7 days of treatment with fondaparinux, CDT was initiated on the eighth day of hospitalization. Hydrophilic guidewire was inserted across the thrombus using Merit MAK mini access kits (Merit Medical Systems Inc., South Jordan, UT), via the left popliteal vein. A 50 cm-long Fountain infusion catheter (SHEEN MAN Co. Ltd, Osaka, Japan) was then passed through the entire extent of the thrombus in the venous segment (Fig. ). Then, urokinase (240,000 IU/day) was repeatedly splashed out through the catheter in approximately 30 minutes, once a day for 6 days. In addition, continuous intravenous administration of unfractionated heparin was started to keep the activated partial thromboplastin time (APTT) 1.5- to 2.0-fold longer than the control value. After 6 days of CDT treatment, the pain and swelling in the patient's left leg improved, and the left thigh circumference reduced to 44.0 cm. However, because CT demonstrated residual thrombi from the IVC to the left posterior tibial vein, the thrombolytic treatment for DVT was switched from CDT to direct oral anticoagulant (edoxaban; 60 mg/day). The patient was discharged without bleeding and infection on the 17th day after hospitalization. The clinical course of this patient is shown in Fig. .
Approximately 2 months after the start of oral medical treatment, there are few thrombi other than in part of the left iliac vein (Fig. ), and D-dimer has decreased to 0.5 μg/mL. The thrombolytic therapy with edoxaban has been continued in this patient. |
pmc-6076878-1 | A 42-year-old male patient presented at the clinic with a debonded restoration in tooth #22 (). A clinical examination revealed acceptable periodontal condition and no carious lesions. After analyzing the size of the restoration and the desire of the patient in solving the problem, rehabilitation with PCRV (Componeer - Brilliant NG) was proposed.
The color matching was performed with a color shade guide of the PCRV system, and the A2/B2 dentin shade associated with the veneer (transparent) was selected. The color matching of the Componeer relies on the concept of natural layering, in which two layers of the incremental technique is able to mimic the natural aspect of the teeth. Moisture was controlled with a rubber dam and a new restoration was placed on #tooth 22 to reestablish the original anatomy (). The treatment proceeded with the selection of the PCRV size (medium), using the contour guide specific to the Componeer (). This contour guide presented different sizes of PCRV (small, medium, large and extra-large) for the antero-superior and inferior tooth. The dentist can always select the correct size for each patient, respecting the fundamentals of the aesthetic smile.
A minimal preparation was performed on the tooth buccal surface with a diamond bur #2068 (KG Sorensen, Cotia, Brazil) to facilitate the setting of the PCRV (). The dental wear did not involve dentin. It is important to highlight that there is no specific amount of dental wear for luting of a PCRV, and the dentist should evaluate minimal wear to facilitate the luting procedure. The dental substrate was etched with 37% phosphoric acid (Magic Acid, Coltene) for 30 seconds, followed by abundant water rinse and air drying. The adhesive system One Coat Bond (Coltene) was applied with a Technobrush (Coltene) on the tooth and on the internal surface of PCRV. The Brilliant NG composite resin (A2/B2 dentin) was used as the luting agent. Clinical steps of tooth wear, adhesive procedure and cementation were executed without the use of a rubber dam. However, it is important to note that the control of moisture was ensured through the insertion of the retraction cord (Pro Retract 0000 FGM, Joinville, Brazil). Such technique allows a satisfactory control of the gingival fluid and facilitates the correct positioning of PCRV.
The veneer was fixed on the tooth with the instrument “Placer” included in the Componeer system (). Excess resin was removed after a slight compression of the PCRV. The light-curing was carried out with an LED (Radii cal, SDI, Bayswater, Victoria, Australia) with a irradiance of 1.200 mW/cm2 for 40 seconds. The excess resin was removed and no final polishing was required due to manufacturers’ pre-polishing of the PCRV (). The patient was extremely pleased with the result. |
pmc-6076899-1 | A previously healthy 56-year-old man suffering from abdominal pain and jaundice was admitted with an initial clinical diagnosis of acute cholecystitis. The patient underwent an endoscopic retrograde cholangiopancreatogram (ERCP) and cholecystectomy. His symptoms did not improve and repeat imaging study indicated common bile duct narrowing. A 20 x 3.5 cm perihepatic abscess was found that required drainage and he underwent percutaneous transhepatic cholangiography (PTC) and biliary drainage. The cytologic examination was not performed on the drained material. Laboratory studies at that time revealed the following: WBC: 51.4 x 109/L (N: 4.5-11.0 x 109/L), Hb: 9.9 g/dL (N: 13.5-17.5 g/dL), serum Na+ 129 mEq/L (N: 135-145 mEq/L), serum K+ 3.4 mEq/L (3.5-5.0 mEq/L), serum albumin: 2.1 g/dL (N: 3.5-5.0 g/dL), lipase 303 U/L (N: 0-50 U/L), and AST/ALT 93/97 U/L (N: AST/ALT: 8-20/8-20 U/L). The patient was discharged on antibiotics after three weeks of treatment. One week later, he developed a fever, chills, and leukocytosis. He was readmitted into hospital. Abdominal CT showed multiple fluid collections within the liver parenchyma with the largest one being 2.2 x 2.0 cm in size. A CT guided liver biopsy of the presumed abscess was performed.
The biopsy showed an epithelioid to spindle cell neoplasm infiltrating between hepatocytes with markedly atypical nuclei and prominent necrosis (Figures , , and ). The tumor exhibited a pleomorphic pattern. Extensive immunostaining was performed, including hepatocellular carcinoma markers (AFP, HepPar1, Glypican-3, polyclonal CEA, and ARG1), other epithelial antigens (CK7, CK20, AE1/AE3, CAM5.2, EpCAM, and EMA) (), Inhibin, CD117, CD30, and CD3, and ALK-monoclonal, germ cell markers (AFP, OCT3/4, and HCG), melanoma markers (Melan-A, S-100, and SOX10), and endothelial (CD31) and muscle (smooth muscle actin) markers () were all negative. The tissue was exhausted.
Based on the inconclusive findings, a second liver biopsy was performed. The morphology was similar to the prior biopsy. Further staining was performed. The tumor cells were also negative for HMB-45, CD15, CD20, CD21, CD23, CD43, CD45, desmin, myogenin, calretinin, myeloperoxidase, D2-40, CD68, and clusterin (). Based on the radiographic features in combination with the morphology and immunophenotype, it was likely a primary hepatic lesion without epithelial, melanocytic, or hematolymphoid differentiation. As such, a primary liver sarcoma was favored.
Following the biopsies, the physician in charge ordered a PET/CT after reviewing the biopsy results in order to evaluate tumor size and potential metastasis (). A large markedly hypermetabolic central hepatic mass (14.0 x 8.5 x 8.5 cm) with likely central necrosis was identified, consistent with primary malignancy. Additionally, there were multifocal hypermetabolic liver lesions and hypermetabolic peritoneal implants suggesting peritoneal dissemination.
The patient was treated with one cycle of chemotherapy (adriamycin and ifosfamide) which caused severe confusion and further treatment was refused. The patient expired within 19 days of diagnosis. |
pmc-6076903-1 | A 34-year-old mother of two children presented in 29th week of her third pregnancy with hypertension. She is a diagnosed patient with diabetes mellitus for 6 years. However her previous two pregnancies, which were two and seven years back, were not complicated with hypertension or diabetes mellitus. She was not detected to have hypertension until the early third trimester of this pregnancy despite frequent clinic visits where blood pressure measurement was a routine practice. She described episodic palpitation, headache, and sweating suggestive of hyperadrenergic spells associated with episodes of high blood pressure. At the same time, her blood pressure and blood sugar levels were fluctuating and difficult to control with her usual medications. On examination, she was an averagely built lady with no features suggestive of syndromic associations (such as mucosal neuromas, café au lait spots, axillary or inguinal freckling, and iris hamartomas). Cardiovascular system examination was unremarkable except for a blood pressure of 170/110 mmHg. Ophthalmoscopic evaluation did not revealed retinal angiomas. Her 24 hour urinary vanillin mandelic acid level was 22.2mg/24hrs (1-13.6 mg/24hrs), 24 hour urinary metanephrine was 188.2µ g/24hrs (<350 µ g/24hrs), and 24 hour urinary normetanephrine was 653µ g/24hrs (<600 µ g/24hrs).
Ultrasound scan of the abdomen done at 29 weeks of POA by consultant radiologist showed a hyperechoic hypervascular well-defined right supra renal mass which is in contact with but not invading the right kidney. The ultrasonic appearance was suggestive of a pheochromocytoma and there were no lesions suggestive of metastasis elsewhere in the abdomen. Patients' management decisions were taken by a multidisciplinary team meeting held with the participation of the surgeon, radiologist, obstetrician, neonatologist, anesthetist, and the endocrinologist where they decided to proceed with an interval adrenalectomy to avoid the detrimental complications of handling hypervascular tumor which was closer to the liver at the time of the caesarean delivery. Her blood pressure control was achieved by an alfa adrenergic blocker (initially prazosin and later phenoxybenzamine) followed by a beta adrenergic blocker which was started after adequate alfa blockade. Blood sugar control was optimized with basal bolus regimen of insulin. Her delivery was done at the 36 weeks of gestation by an elective LSCS under close supervision for adrenergic crisis. Peripartum period was uneventful and there was no adrenal crisis. She gave birth to a healthy baby of a birth weight of 3.6kg. CECT abdomen with adrenal protocol was planned after one month postpartum. She was discharged on the 12th postpartum day after close maternal and neonatal surveillance. The patient and her family were well educated regarding the disease and the need for close monitoring of blood pressure. Her blood pressure was monitored weekly at the hospital. She was well compliant on twice a day premixed insulin and self-monitoring of blood sugar.
20 days after the delivery she presented with a sudden onset severe headache with vomiting. Blood pressure was normal and fundi did not revealed papilloedema. Noncontrast CT brain () showed early hydrocephalus with a suspicion of posterior fossa space occupying lesion.
She was transferred to neurosurgical unit and contrast MRI brain () showed two multiloculated cystic lesions one in relation to the fourth ventricle and the vermis and the other lesion in the periphery of the left cerebellar hemisphere which were suspicious of cerebellar hemangioblastomas. She underwent urgent ventriculoperitoneal shunting as an emergency procedure to relieve intracranial pressure while awaiting definitive management.
CECT abdomen with adrenal protocol () showed a right-sided adrenal tumor of 66×59mm size which is suggestive of a pheochromocytoma without any evidence of metastatic deposits in the abdomen.
She was started on Phenoxybenzamine 14 days before surgery for the optimum control of blood pressure and adequate alfa blockade to prevent adrenergic crisis.
Laparoscopic right adrenalectomy was performed without any major intraoperative or postoperative complications. This caused the reversal of all the clinical features of hyperadrenalism. Her antihypertensives were stopped after the surgery and her diabetic control improved. Her postoperative investigations are shown in .
Histology confirmed the diagnosis of pheochromocytoma.
She was evaluated for the syndromic associations particularly for von Hippel Lindau disease because of the presence of cerebellar lesions suggestive of hemangioblastomas.
Ophthalmology assessment revealed a lesion suggestive of a retinal hemangioblastoma (retinal angioma) on the right eye (). It was treated with laser therapy and currently under ophthalmology followup.
VHL genetic testing was found to be negative. Her two brothers and three children are currently under evaluation.
She is under close surveillance at neurosurgical clinic with regular MRI. The resection of deeply seated cerebellar haemangioma without any symptoms or enlargement over time may have adverse consequences neurosurgical team decided to closely follow up the patient and go for surgery when indicated. At 6 months, MRI did not show significant increase in tumor size and she is asymptomatic.
Following the surgery we were able to stop all the antihypertensive medications and her blood glucose is under control with only one oral antidiabetic medication (metformin). |
pmc-6076907-1 | A 17-year-old man was involved in a road accident in which he suffered the open fractures of the right femur and tibia. At the arrival to the Emergency Dept (ED), he was alert and hemodynamically stable and the Glasgow Coma Scale (GCS) was 15; the initial alignment of the fractured ends was performed in the ED with a gentle traction performed under sedation with iv. ketamine; a total body CT did not demonstrate other injuries. Approximately two hours after the admission the patient was taken to the surgical theatre for the external fixation of the fractured bones; at entering the operating room, the GCS was 8, the arterial pressure was 115/80 mm Hg, the heart rate was 115 bpm, and the arterial oxygen saturation (SPO2) was 85 at room air; the procedure was performed under general iv anesthesia with propofol and remifentanyl; the standard monitoring included the ECG, the noninvasive arterial pressure, the SPO2, and the end-tidal CO2 (ETCO2); during the intervention, the SPO2 rose to 100% at a FIO2=40% and all the other variables remained stable throughout the procedure after the 3-hour-long intervention in which the complete alignment of the bony ends was achieved; the patient was transferred to the Intensive Care Unit (ICU) still intubated and mechanically ventilated; the iv anaesthetics were gradually tapered until the complete suspension. Two hours later, the SpaO2 and the ETCO2 slightly decreased and anisocoria was observed; and an urgent CT scan of the head demonstrated a diffuse cerebral edema and the herniation of the cerebellar tonsils (Figures and , respectively). At this time, the pupils became bilaterally mydriatic and the EEG was almost isoelectric; due to the severity of the conditions, a MR scan was considered unnecessary. On the basis of the clinical and radiologic findings repeated boluses of iv. mannitol and steroids were given in the following hours aiming to reduce the intracranial pressure. An echocardiogram demonstrated a severe right ventricular depression with an ejection fraction of 20%. On the following day, the patient was declared brain dead according to the current Italian law.
At the autopsy, the cerebral microvascular network appeared diffusely plugged with BME (Figures –) and ischemia-related microcalcifications were scattered throughout the brain (); other organs were less extensively involved; no PFO was demonstrated. |
pmc-6076910-1 | This patient was a generally healthy 62-year-old male with a left lobe complex nodule within a nontoxic multinodular goiter that had been enlarging for approximately 3 years. In 2015, the patient had a FNAB reported as benign, BC II. Because of continued growth, he had a second FNA biopsy approximately six months later reported as a Hürthle cell neoplasm or suspicious for a Hürthle cell neoplasm, BC IV with Oncocytic / Hürthle cells dispersed mostly singly and in small fragments in a background of lysed blood. CKAE1/AE3, TTF-1, and thyroglobulin immunostains were positive (). Molecular testing with ThyroSeq® v2 revealed an absence of gene mutations or fusions but overexpression of the MET gene with an uncertain increased risk of malignancy. After repeat ultrasound imaging, the nodule had grown from 4.9 to 6.0 cm over the course of 1 year. He was euthyroid with negative anti-thyroid antibodies. There was no family history of thyroid cancer or known radiation exposures in his youth. He had no obstructive symptoms despite the size of the mass and denied shortness of breath, dysphagia, neck pain, neck pressure, or recent voice changes. His weight had been stable and appetite good. His past medical history was significant for a retinal detachment, hypertension, and inguinal hernia with a surgical history limited to eye surgery and hernia repair. He denied tobacco or alcohol use. On exam, the patient had an enlarged, firm thyroid gland with the left thyroid lobe causing significant tracheal deviation to the right. A neck CT scan demonstrated a markedly enlarged left thyroid lobe (7.2 cm in sagittal height) causing significant rightward tracheal deviation, minimal tracheal compression, and slight early substernal extension (). He had multiple opinions from both endocrinologists and surgeons with various recommendations from left thyroid lobectomy to total thyroidectomy. The patient had initially contemplated a hemithyroidectomy due to concerns for voice impairment that could impact his occupation as an attorney.
After a second surgical consultation, he elected to have another, more advanced molecular test performed on the same FNAB specimen. The ThyroSeq® v3 test has been designed to improve the performance of its previous version, ThyroSeq v2, specifically with respect to Hürthle cell tumors. This has been achieved by expanding the number of gene markers analyzed for mutations and gene fusions and particularly by incorporating the analysis of copy number alterations (CNAs), which are common in Hürthle cell cancers. ThyroSeq® v3 test results in this case showed CNAs involving multiple chromosomes with the pattern of genome haploidization which predicted a much greater probability that the left lobe nodule represented a Hürthle cell malignancy rather than Hürthle cell metaplasia or an adenoma. Based on the additional information provided by ThyroSeq® v3, in July, 2017, the patient elected a total thyroidectomy. At surgery, the overlying strap muscles were superficially adherent to the thyroid capsule on the left with a suspicion of minimal extrathyroidal extension of the tumor and a layer of muscle was left attached to the specimen. There were no paratracheal lymph nodes. He did require single gland parathyroid autotransplantation. Postoperatively, his parathyroid hormone and calcium levels were within normal limits. On final surgical pathology, an encapsulated 7 cm Hürthle cell carcinoma with 5 foci of angioinvasion was found along with foci of capsular invasion, without extrathyroidal extension (). A second opinion was sought and the reviewing pathologist reported 4 foci of capsular invasion and 3 foci of vascular invasion. The number of foci of vascular invasion was prognostically important and prompted more aggressive treatment and follow-up.
One month postoperatively, thyroglobulin was 308 ng/mL. A small thyroid remnant with 2.4% uptake in the surgical bed was found on I-131 whole body scan. An FDG-PET scan was negative for any activity in the thyroidectomy bed or for distant metastatic disease; therefore he was given 30 mCi of radioactive iodine to ablate the remnant. At this time, his thyroglobulin had decreased from 308 to 8.71 ng/mL. His postablation, I-131 whole body scan showed ablation of the thyroid remnant and no evidence of metastatic disease. By 10/27/2017, the thyroglobulin had decreased further to 0.2 ng/mL with no detectable thyroglobulin antibodies and a TSH of 0.09 uIU/ml indicating a favorable early response to initial treatment. |
pmc-6076919-1 | A 56-year-old Caucasian male with nonalcoholic steatohepatitis (NASH) experienced progression to cirrhosis and its complications including portal hypertension, esophageal varices, and ascites. He had no other significant past medical history. At the time of transplant in July 2015, the patient weighed 228 lbs (BMI 34). He received a liver transplant and was placed on a maintenance immunosuppressive regimen of tacrolimus 9 mg PO BID with a trough goal of 8-10 ng/mL, mycophenolic acid (Myfortic) 720 mg PO BID, and a prednisone taper. The patient remained stable on this regimen and had the following normal laboratory results at the beginning of September (): ALT 32 IU/L (normal=0-40), AST 23 IU/L (normal= 5-40), alkaline phosphatase (ALP) 83 IU/L (normal= 40-100), gamma-glutamyl transpeptidase (GGT) 36 IU/L (normal=10-64), total bilirubin 0.3 mg/dL (normal=0.3-1.9 mg/dL), BUN 26 (normal 6-20 mg/dL), and Scr 1.18 (normal 0.67-1.17 mg/dL) and INR 1.1.
On September 3, 2015, the patient was switched from mycophenolic acid to everolimus as part of a clinical research study investigating the renal sparing effects of everolimus to target a lower tacrolimus trough concentration of 3-5 ng/mL. At the time of everolimus introduction, the patient's weight was down to 210 lbs (BMI 31.9) and laboratory values that would impact the pharmacokinetics of everolimus were within a normal range: Hgb 12 mg/dL and albumin 3.7 g/dL. After the patient's first everolimus dose on a starting regimen of 1 mg PO BID, he reported new onset pain to the right flank area. At this time, there was an upward trend in his liver enzymes, ALT (69 IU/L), AST (35 IU/L), ALP (99 IU/L), and GGT (58 IU/L). The everolimus trough was subtherapeutic until late October when a trough of 3.8 ng/mL was achieved on a dose of 3 mg PO qam and 2.5 mg PO qpm ().
In early October, the patient experienced increasing liver enzymes (ALT=84 IU/L; AST= 42 IU/L; ALP=102 IU/L; GGT=53 IU/L; total bilirubin 0.23 mg/dL) with a tacrolimus trough concentration of 8.2, so a liver biopsy was performed to rule out rejection. The results showed mild portal inflammation with lymphocytes, pericentral sinusoidal dilatation with no hepatic plate atrophy, and inflammation adjacent to the central vein (RAI score = 1 out of 9). The trichrome stain did not have any perisinusoidal staining to indicate chronicity, nor was there duct injury, duct loss, cholestasis, endothelitis, or steatosis. Immunostains for hepatitis B surface antigen (HBsAg) and hepatitis B core antigen (HBcAg) were also negative. There was no report of fever, chills, dark-colored urine, or jaundice or any evidence of an acute hypersensitivity reaction (fever/rash). The immune cell function (ImmuKnow) assay was 338 ng/mL in late October close to the time of the biopsy (10/26/15). It was confirmed that there was no evidence of acute rejection.
By the end of November, two and a half months from the start of everolimus, the patient's serum liver enzymes reached their highest values, ALT (149 IU/L), AST (81 IU/L), and ALP, (215 IU/L) and the ImmuKnow assay done at this time resulted in a level of 412 ng/mL. Tacrolimus doses had not been changed and troughs ranged from 6.9 to 9.8 ng/mL. A second liver biopsy was done on 11/30/15 that showed mild patchy sinusoidal dilatation and focal mild inflammatory infiltrate with lymphocytes, eosinophils, and rare acidophil bodies (). There was no evidence of acute cellular rejection (RAI score= 1 out of 9), but the presence of eosinophils and focal mild portal inflammation was consistent with the possibility of drug injury. Everolimus was discontinued on December 1, 2015, and the patient went back to a regimen of tacrolimus 5 mg BID and mycophenolic acid 720 mg BID. After one week, the liver enzymes returned to normal: ALT (22 IU/L), AST (20 IU/L), ALP (105 IU/L), and total bilirubin (0.6 mg/dL). Since discontinuation, the patient denied pain and dizziness and reported improved energy. |
pmc-6076924-1 | A 69-year-old male with past medical history of type 2 diabetes and hypertension presented to the emergency department in New York City in August complaining of headache and diplopia. His headache abruptly began one week ago, was localized to the right occipital region, and gradually moved to his right orbit. Five days later he developed diplopia. One month prior to symptom onset, he hiked in a rural area of New York State, but he denied any tick bites or rash development. On presentation, our patient was hemodynamically stable, did not have any signs of acute infection, and denied any fevers or chills. He stated he had double vision when opening both eyes; however if he covered his right eye his vision normalized. Physical exam was significant for left sided cranial nerve 3 palsy. The rest of his physical and neurological examinations were normal. MRI and MRA were both negative. Syphilis serology was negative. Lumbar showed glucose of 101, protein of 77, and 74 white blood cells (84% lymphocytes and atypical lymphocytes). CSF was negative for VDRL, cryptococcal antigen, varicella zoster, HSV 1 and 2, and West Nile virus. He had positive Lyme titers by ELISA at 6.04 (negative < 0.90) and western blot showed five IgG and two IgM bands. He was started on acyclovir and ceftriaxone and experienced resolution of headache but continued to complain of diplopia. Lyme antibody in CSF was checked by ELISA and was reactive at 0.532 (reactive cutoff 0.144). Although the CSF-to-serum ratio of IgG by Eliza was only 0.0880, patient was treated for oculomotor nerve palsy secondary to Lyme meningitis. Acyclovir was discontinued. He was treated with ceftriaxone for four weeks as per The Sanford Guide to Antimicrobial Therapy guidelines. His diplopia resolved and he was asymptomatic two months after initiation of therapy. |
pmc-6076933-1 | A 45-year-old woman with severe right flank pain came to our outpatient clinic. Her past medical history included right ureteral calculi secondary to her right calyceal diverticulum. In addition to the past ureteral stone, there had also been a stone in the calyceal diverticulum. She had undergone URSL for a ureteral stone six months before this visit. Her past surgical history included a caesarian section. She was not taking any medication. The physical examination was within normal limits. Based on her past history, we suspected a recurrent ureteral stone. We performed computed tomography and diagnosed a right renal stone in her ureteropelvic junction (). Her colic was very strong and she was very obese (her height was 162.8 cm, body weight was 97.6 kg, and body mass index was 36.8 kg/m2). Therefore, we decided that URSL was preferable, which was the same treatment as that used for the previous right ureteral stone.
Her preoperative urinalysis did not demonstrate bacteriuria, and her hematological exam and laboratory exam were within normal limits.
Ten days after the visit to our outpatient clinic, we performed URSL. Intravenous administration of ceftriaxone 1 g was started 30 min before ureteroscopy. The patient was placed in the lithotomy position and draped in a sterile fashion, under general anesthesia. First, the urethra and the bladder were observed, and the bilateral ureteral orifices were identified using a 22.5 Fr rigid urethrocystoscope (Cystoscopes, Olympus, Tokyo, Japan). Second, a semirigid 6/7.5 Fr ureteroscope (Ultrathin, Richard Wolf, Knittlingen, Germany) was inserted into the right ureter without a guidewire. Observation of the ureter was performed, but there were no ureteral stones in the ureteropelvic junction. We inserted a guidewire, and 12 Fr digital flexible ureteroscope (URF-V, Olympus) was moved into the right renal pelvis. Intermittent irrigation was controlled manually at the lowest pressure with a 50-ml syringe. We observed all calyces systematically in order to confirm the targeted stone. However, we were unable to identify the ureteral stone or the calyceal diverticulum. Finally, we stopped the operative procedure. The operative duration was 38 minutes, and the ureteroscopic duration was 25 minutes. Because of the short duration of the operation, we did not place a ureteral stent.
Fifteen hours later, the patient felt severe right flank pain and became febrile (38.9°C). Computed tomography showed perinephric extravasation of urine and bleeding inside the diverticulum (). We diagnosed a rupture of the calyceal diverticulum secondary to ureteroscopy. We examined the urine culture and started ceftriaxone 2 g per day. On postoperative day 4, she became afebrile, and on postoperative day 7, we stopped administration of ceftriaxone and started oral antibiotics (tebipenem pivoxil 300 mg per day) guided by the reported urine culture (E.coli with extended spectrum beta lactamase). On postoperative day 14, she was asymptomatic; therefore, she stopped taking oral antibiotics. In postoperative month 3, we performed computed tomography and confirmed complete resolution of extravasation. |
pmc-6076934-1 | An 81-year-old lady presented to the ENT department with increasing dysphagia, shortness of breath, and stridor. Her past medical history included radiotherapy to the mediastinum for Hodgkin's lymphoma 14 years prior to presentation and radiation induced interstitial pulmonary fibrosis. She was on long-term Azathioprin and Prednisolone immunosuppressive therapy for this. One year prior to presentation she was treated for a fungating moderately differentiated squamous cell carcinoma of the tip of the nose with surgical excision. Histologically this measured 12mm x 11mm x 3mm with involvement of the deep margin, with no perineural or lymphovascular invasion. There was no lymph node metastasis, and the patient underwent 5 sessions of radiotherapy at 35Gray to treat the deep margin.
The patient was also investigated for symptoms of a lower respiratory tract infection 5 months prior to the latest admission with stridor. A sputum culture grew Haemophilus influenzae and Mycobacterium kansasii and the patient was advised 2 years of rifampicin, ethambutol, and clarithromycin by the respiratory team as per the British Thoracic Society recommendations []. Due to the duration of work-up of the diagnosis and need for 3 confirmatory sputum samples, the patient only had 6 weeks of triple therapy treatment prior to presenting with stridor.
Examination of the patient during the acute admission using nasoendoscopy revealed a left anterior vocal cord granulation and an exophytic pedunculated lesion from the right vocal cord causing ball-valving of the glottic inlet and diminished right vocal cord mobility. With the patient's history in mind, the initial working diagnosis was squamous cell carcinoma and a differential diagnosis of mycobacterial disease.
The patient was initially commenced on broad-spectrum antibiotics and steroids. As there was no response to medical treatment, the patient was taken to theatre for debulking to reestablish an airway and tissue samples were sent for both histological and microbiological examination. Figures and reveal pictures of the larynx both before and immediately after debulking. The patient was discussed in the joint head and neck multidisciplinary meeting pending the results of histological analysis.
Histology showed a spindle cell proliferation with intersecting fascicles and easily identifiable mitotic figures (). Initially the differential diagnosis included a low-grade sarcoma, especially as there was strong expression of desmin () and weaker expression of smooth muscle actin. However, the lesion demonstrated bland uniform nuclei and there was an inflammatory cell infiltrate of neutrophils and plasma cells. Immunohistochemistry also showed expression of CD68, suggesting a significant histiocytic component to the lesion, but no true granulomata were identified. Focally the surface had a slightly palisaded appearance with necrotic debris on the surface, possibly representing an element of caseous necrosis. Staining with Ziehl-Neelsen () and Wade-Fite () showed numerous acid-fast bacilli.
The bland nuclear morphology, inflammatory cell infiltrate, and mycobacteria indicate an inflammatory pseudotumour secondary to mycobacteria infection. The lack of epithelioid granulomata may be related to the underlying immunosuppression. The strong expression of desmin and the weaker smooth muscle actin, although slightly unusual, would be acceptable for a myofibroblastic proliferation.
After confirmation of the histological diagnosis, the patient continued to show respiratory deterioration despite the surgical debulking and being started on broad-spectrum antibiotics and steroids. The long-term antimycobacterial medications were not stopped during the admission. Indirect laryngoscopy of the vocal cords was performed at the bedside one week after debulking showing reproliferation of the granulation tissue () with compromise of the airway. Given the overall poor prognosis and resistance to medical and surgical interventions, and taking the patient's wishes into consideration, treatment was withdrawn. The patient's condition unfortunately deteriorated following this and she died shortly afterwards. |
pmc-6076939-1 | A 30-year-old gravida 1 para 1 female presented to our Emergency Department complaining of a vaginal mass present since the birth of her child 4 years earlier. At that time, she underwent an uncomplicated vacuum-assisted vaginal delivery and was unaware of significant lacerations or repairs. She felt that the mass had not changed significantly in size since the postpartum period, but she had never been evaluated by a physician. She complained of acutely worsening discharge over the previous month, described as watery yellow to pink and occasionally blood tinged. She denied changes in bowel movements, dysuria, hematuria, fevers, chills, night sweats, changes in appetite, or weight loss. She complained of both entry and deep dyspareunia.
Physical exam was notable for copious serosanguinous fluid within the vaginal vault. A well-circumscribed, smooth cystic structure approximately 4 cm in diameter was noted along the posterior vaginal wall. There was also a 0.5x1.0 cm exophytic lesion overlying the mass with serosanguinous drainage (). On rectal exam, the mass was noted to be separate from the cervix and within the rectovaginal septum. Rectal involvement was not appreciated.
Tissue biopsies were taken; however, they were of limited diagnostic value, showing fibrous tissue with acute and chronic inflammation and squamous debris, consistent with cyst wall and contents.
A pelvic ultrasound (US) was performed and showed a complex vaginal mass, inseparable from the cervix, measuring 5.1 x 3.8 x 5.4 cm (). Color Doppler demonstrated minimal peripheral vascularity (). Magnetic resonance imaging (MRI) was subsequently performed for further characterization (). The mass measured 4.7 x4.8 x 4.9 cm and appeared to arise from the posterior wall of the vagina, separate from the cervix. The mass was heterogeneously hyperintense on T2-weighted (T2W) images and hypointense on T1-weighted (T1W) images. Postcontrast sequences demonstrated enhancement of the wall, absent internal enhancement superiorly, and bulky, nodular, hyperenhancement inferiorly, consistent with a complex cystic mass.
The patient was taken to the operating room for an uncomplicated surgical excision. Histology was notable for hypocellular edematous myxoid stromal tissue alternating with hypercellular areas of stromal cells clustered around thin walled small to medium-sized vessels, consistent with angiomyofibroblastoma. Stromal cells were noted to be spindled with eosinophilic cytoplasm. Nuclei were round or ovular with fine chromatin (). Rare mitotic figures were noted. Immunohistochemical stains were negative for desmin, alpha-smooth muscle actin (α-SMA), and progesterone receptor but demonstrated focal estrogen receptor positivity. |
pmc-6076956-1 | Patient 1 is a 31-year-old Jordanian female with a history of a recurrent and itchy eruption involving the mid- to lower back, lateral chest wall, and the nape of the neck. This resolved with net-like pigmentation (Figures and ). The occurrence of the eruption was linked with fasting in Ramadan, in addition to travels to North America. No other medical problems were identified. |
pmc-6076956-2 | Patient 2 is a 16-year-old Jordanian female who presented with an itchy eruption of new onset. This appeared 3 weeks earlier and affected the upper to mid-back and the “V” of the neck (Figures and ). The occurrence of the eruption followed a 1-month period of strict dieting. |
pmc-6076956-3 | Patient 3 is a 45-year-old Jordanian female with an itchy eruption of 3 months' duration. This affected the nape of the neck and the upper back. No triggers were identified and the patient was otherwise healthy.
The patients' demographics and their clinical features are outlined in . Clinically, all patients were noted to have erythematous papules that coalesced to form plaques. These were arranged in a reticular pattern that was more prominent peripherally. In addition, patient 1 had associated vesicles and minimal erosions (Figures and ). In all patients, the lesions were symmetrically distributed and had a predilection for the trunk. Other involved areas included the lateral and posterior aspects of the neck (patients 1 and 3), the lateral chest wall (patient 1), and the lumbosacral area (patient 1). Different types of lesions coexisted in all patients including papules, patches, and plaques, in addition to vesicles and erosions in patient 1. A clinical diagnosis of PP was suspected clinically in patients 1 and 2.
The main histological findings are summarized in and . The histopathological features were similar in all cases, showing features consistent with early lesions according to Boer's criteria []. The major histological differential diagnoses were impetiginized spongiotic dermatitis, pityriasis lichenoides, and viral exanthem. Periodic acid-Schiff stain was negative in all specimens. Direct immunofluorescence was performed for patients 1 and 2 only and was negative.
The clinical course varied, but all three patients had eventual complete resolution of all lesions. Patient 1 was treated with superpotent topical corticosteroids prior to presentation to our department. However, there was no improvement and new lesions continued to emerge. The patient subsequently reported gradual spontaneous resolution 10 weeks after onset of the eruption, leaving postinflammatory hyperpigmentation. Patient 2 was previously treated with moderately potent topical corticosteroids and antihistamines without any improvement. New lesions continued to emerge. On initiation of doxycycline, the lesions cleared within 1 week. No recurrence was reported during a 10-month follow-up period throughout which the patient avoided strict dieting. Patient 3 reported spontaneous resolution of some lesions before presentation to our department. Doxycycline was subsequently initiated with complete resolution. |
pmc-6076969-1 | A 53-year-old man of Puerto-Rican origin presented to the endocrinology clinic after undergoing bilateral adrenalectomy for multifocal pheochromocytomas. He had a prior history of morbid obesity, obstructive sleep apnea, diabetes, and hypertension. He was followed by his primary care physician for persistent hematuria ranging from 3 to 35 red blood cells per high power field on urinalysis, as well as urinary frequency, weak stream, and nocturia three times per night for the previous three years. He had been unable to tolerate an empiric trial of tamsulosin for benign prostate hypertrophy due to orthostatic dizziness. Negative symptoms pertinent to this case include flushing, headaches, sweating, palpitations, anxiety, blurry vision, or dizziness. His family history was notable for death from a myocardial infarction in his father at the age of 57 and an unknown genitourinary cancer in his sister. There was no family history of adrenal tumors, hyperparathyroidism, medullary thyroid cancer, renal cancer, or pituitary tumors. The patient was a smoker with several past attempts at quitting.
Due to the persistent hematuria, smoking, and the family history of cancer, a CT urogram was performed to screen for bladder cancer. While no abnormalities were seen within the urogenital tract, bilateral, irregular, heterogeneous large adrenal masses () measuring 4.7 cm (R) and 1.6 cm (L) were noted. In addition, a prominent and suspicious lymph node was identified. Biochemical characterization of the adrenal masses revealed significantly elevated 24-hour urine normetanephrine (1090 micrograms/gram of creatinine; normal range, 0–400 micrograms/gram of creatinine), leading to the diagnosis of pheochromocytoma. Urine metanephrine level was within normal range. Cushing's syndrome was ruled out with an undetectable late-night salivary cortisol level. Electrolyte levels, kidney function, and complete blood count were within normal limits. In search for additional, extra-adrenal foci, a metaiodobenzylguanidine (MIBG) scan was performed but was nondiagnostic due to lack of cardiac activity. Nevertheless, given the available imaging and biochemical findings, there was concern for malignant pheochromocytoma, and the patient ultimately underwent an open bilateral adrenalectomy and paracaval lymph node excision. Intraoperatively, the patient required vasopressor support and a large amount of crystalloid resuscitation (13 liters) to maintain hemodynamic stability. Intraoperative ultrasound was used to identify one mesenteric lymph node of mildly suspicious appearance which was resected, in addition to a large retroperitoneal paracaval lymph node.
Surgical pathology confirmed pheochromocytomas in the bilateral adrenal glands (right, 5.0 x 3.5 x 2.5 cm, and left, 1.5 x 1.3 x 1.0 cm) which were both confined to the adrenal glands. The paracaval lymph node was described as paraganglioma versus metastatic pheochromocytoma measuring 1.6 cm in the greatest dimension with no lymphoid tissue identified. The immediate postoperative course was unremarkable. The patient was started on life-long glucocorticoid and mineralocorticoid replacement. His diabetes and hypertension resolved.
Due to the multifocal nature of the pheochromocytomas and the presence of first-degree relatives likely to be affected, the patient was offered genetic screening for familial paraganglioma syndromes. With the patient's informed written consent, genomic DNA was isolated from a peripheral blood sample and targeted gene sequencing was performed using PGLNext. Coding exons and adjacent intron nucleotides of the 12 targeted genes associated with hereditary pheochromocytoma syndromes were amplified and then sequenced using PCR and next-generation sequencing. Gross deletion and duplication analysis was also performed. The patient was found to have a heterozygous germline mutation, c.524A>G in the VHL gene, corresponding to the Y175C substitution in the protein. This was identified as a likely pathogenic variant and confirmed by Sanger sequencing. In one study, this alteration was described in a patient with a personal and family history of pheochromocytoma and no other VHL-associated tumors and segregated with disease in this family [].
In order to better define the risk of RCC in this patient and others with this mutation, we assessed the ability of Y175C VHL to degrade HIFα in vitro. Stable wild-type (WT) or Y175C VHL-expressing cells lines were generated by transfection and clonal selection of VHL-null 786-O cells derived from a human RCC as previously described [, ]. Control cells were transfected with GFP. We detected HIF2α expression in the control VHL-null cell line, while stable overexpression of either WT or Y175C VHL resulted in the disappearance of HIF2ɑ (, left panel). To further characterize the function of Y175C VHL under hypoxic conditions, the cells were placed into a hypoxia incubator at 1% O2 for 24 hours. As expected, the WT VHL lost the ability to induce HIF2α degradation in hypoxia (, right panel). The Y175C VHL similarly did not reduce HIF2α abundance in hypoxia. HIF2α abundance was also similar in WT and Y175C VHL-expressing cells after 6 hours and 12 hours of hypoxia (data not shown). Thus, under both normoxic and hypoxic conditions, Y175C VHL functions similarly to the WT with regard to HIFα degradation. |
pmc-6076973-1 | A 24-year-old male patient was admitted to the emergency room due to injuries to the left hemithorax as well as a transfixing laceration in the left arm caused by a shotgun of initially unknown calibre.
On examination, the patient was found to be alert and fully orientated. He was hemodynamically stable. His physical examination yielded a small entrance wound from the bullet in his midaxillary line on the left hemithorax at the 4th intercostal space. No exit or other gunshot could be found.
Computed Tomography (CT) of chest and abdomen showed two rib fractures, a transfixing wound at the lower left lobe, minimal hemothorax, 4 mm pericardial effusion, and foreign metallic body (bullet) in the near left ventricle apex; it was difficult to determine if the metal parts were inside the pericardium or within the musculature of the left ventricle ().
Given the risk of cardiac tamponade or cardiac injury, we decided to perform emergency surgery, despite the hemodynamic stability. Surgical access to the thoracic cavity was obtained by left anterolateral thoracotomy; this approach allows handling both pleural cavities in case of other lesions, extending to the other hemithorax.
Following the opening of the cavity, we observed the transfixing left lower lobe lesion with bone fragments, as well as a moderate amount of blood and clots in the pleural cavity (300cc approx.). Also, a hematoma could be spotted in the pericardial fat.
After pericardiotomy, we found a small amount of blood and noticed a small hole in the anterior wall of the left ventricle, without bleeding. Since we could not find the bullet, we decided to perform a radioscopy to determine its location, but we were unable to find it inside the thorax.
The cardiac lesion was repaired by separate sutures “U” with polyester suture line 2-0 and the lung segment resected with a mechanical suture. After repair of the injuries and review of the hemostasis, one drain was inserted.
Once the patient was at the Intensive Care Unit in stable clinical condition, we performed a Transoesophageal Echocardiography that showed a normal cardiac function, but the bullet was not seen.
We decided to perform a body CT that identified the bullet at the division of the femoris artery (); this was confirmed by a Doppler ultrasound that revealed a lumen obstruction of the profundal femoris artery (). The vascular team decided to perform surgery and removed a 0.38 calibre bullet, with no complications. The patient was discharged 15 days after the operation. |
pmc-6077322-1 | A 48-year-old man presented at the ophthalmologic out-patient department with a 3-day mild horizontal diplopia in the left direction followed by the onset of headache 17 days later. He denied nasal obstruction, epistaxis, nasal discharge, pain, hyposmia, and nasal swelling. There was no history of fever, weight loss, or nocturnal sweating. He had no history of diabetes, hypertension, or any neurological disease. On physical examination, cardiopulmonary examination was normal and neither lymphadenopathy nor hepatosplenomegaly was observed. Neuroophthalmologic examination revealed normal visual acuity, fields, and fundi. The pupils were equal and reactive to light and near stimuli. There was no ptosis, but there was limitation of movement of the left eye when he gazed to the left side. Function of the remaining cranial nerves was normal. There were no sensory or motor deficits in the upper and lower extremities; all tendon reflexes were normal. He was found to have isolated left abducens nerve palsy. Computed tomography (CT) scanning revealed soft-tissue density neoplasms filling the sphenoidal sinus (). Magnetic resonance imaging (MRI) scanning with gadolinium injection was performed and revealed a homogeneous mass lesion (2.8cm x 2.3cm x 2.9cm) occupying the sphenoidal sinus and invading and destroying the clivus (). Rhinoendoscopy revealed a mass at the sphenoidal sinus which was biopsied and histological examination revealed a malignant lymphoma. The immunohistochemical staining of tumor tissues showed CD3+, CD56+, Ki67>80%, LCA+, CD38+, and CD20− (). The lymphoma cells were positive for EBER in situ hybridization. The pathological diagnosis was ENKL. Plasma EBV PCR yielded 1.18 x 106 copies/ml. Ten days later the patient had the B symptom (fever, night sweats). The enlarged lymph nodes were checked in the neck, bilateral subclavian, alar, and inguinal. Contrast enhanced CT showed renal metastases. Bone marrow smear and biopsy showed active hyperplasia, immature lymphocytes accounting for 3%, and heterotypic large cells having a scattered distribution (). Flow cytometry analysis showed lymphocytes accounting for 6.8% and suggested phenotypic abnormal NK cells in the bone marrow. Cerebrospinal fluid analysis showed glucose (2.87mmol/L) and protein content (0.22g/L) with normal cell count and no malignant cells. Blood analysis showed complete blood cell reduction. The second bone marrow biopsy suggested hemophagocytic syndrome []. The clinical diagnosis was stage IV of ENKL. The patient asked to be transferred to the community hospital. |
pmc-6077324-1 | A 56-year-old female was diagnosed with primary biliary cirrhosis after presenting with pruritus and fatigue. Prior to this diagnosis, she worked as a business executive functioning at a high cognitive baseline. Jaundice and refractory ascites developed in the month prior to admission. Cognitive decline evolving over one month mandated her to take leave from work. She was referred to a tertiary centre specializing with hepatobiliary expertise. Her past medical history included arterial hypertension and gastroesophageal reflux, as well as cervicogenic headaches. She did not have psychiatric, legal, or relevant family history. Her baseline included diuretics, lactulose 10 ml TID, sodium benzoate 3g OD, metronidazole 250 mg BID, calcium carbonate 500 mg BID, and ursodiol 500 mg BID. Risperidone 1 mg OD and quetiapine 50 mg HS were prescribed at the time of referral but introduced after the neuropsychiatric presentation. No correlation could be established with her behavioural change and the pharmacotherapy after a meticulous review.
A general work-up was completed at the time of admission including albumin 34 g/L (N= 37-48 g/L), ammonia 20 mcg/dl (N= 15-45 mcg/dl), GGT 331 UI/L (N= 7-55 UI/L), ALT 74 U/L (N=9-30 U/L), AST 74 U/L (N=13-39 U/L), alkaline phosphatase 565 UI/L (N=36-110 UI/L), and INR 1.1. Psychiatry and neurology consultants reached the same conclusion: the patient's neuropsychiatric symptoms were atypical for hepatic encephalopathy []. She presented with personality alteration, psychomotor agitation, elevated mood, incongruous affect, ideoaffective discordance, and tangential, noninformative, and logorrheic speech as well as slightly decreased judgement. However, her orientation and insight were surprisingly intact. She scored 17/30 on the MOCA signifying a global cortical impairment. Disorganization and perseveration, along with auditory and somesthetic hallucinations, improved with neuroleptics. To provide an example, when this patient was informed of an upcoming neurology assessment, she completed her “homework” consisting of drawings and symbols. The neurological examination was unremarkable, mentioning absence of ophthalmoplegia, nystagmus, areflexia, gait instability, or primitive reflexes. The possibility of a diffuse cortical cognitive alteration with repercussion on expression, speech, memory, and personality was considered. Latent schizophrenia was clearly excluded, which would have been a contraindication to liver transplantation. However, a manic episode due to an organic disease was investigated.
Over the following weeks, she remained significantly disorganized, despite the fact that PRN doses of olanzapine and haloperidol managed her agitation. Lithium and regular doses of olanzapine were introduced, without success. An extensive work-up was performed, confirming Child-Pugh class C liver cirrhosis through biopsy. A brain MRI featured bilateral basal ganglia hyperintensities (). Several EEGs were performed within her two-month hospitalization. The initial two EEG studies were unremarkable, without triphasic waves, slowing of the baseline rhythm, or epileptiform features. This would be unusual for hepatic encephalopathy, considering the extent of the neuropsychiatric symptoms; hepatic encephalopathy would at least have been classified as grade 2 []. However pathognomonic signs encountered at earlier stages were absent: shortened attention span, lethargy, dyspraxia, altered sleep rhythm, irritability, dysarthria, and asterixis. Repeated EEGs performed two and three months after onset of neuropsychiatric symptoms, respectively, showed a discrete intermittent global slow cerebral dysfunction (). Cerebral SPECT and PET scans were essentially normal for patient age, showing no signs of significant atrophy. CSF analysis was essentially unremarkable. A thorough search for an underlying neoplasm included a thoracic CT scan, which demonstrated a calcified nodule at the right apex (PPD negative). It also included an abdo-pelvic CT scan, ascites analysis/culture, and alpha-fetoprotein level, all of which were negative. CSF, urine, ascites, and blood cultures were consistently negative. The inflammatory and autoimmune work-up was positive for ANA (1/640) and antimitochondrial antibodies (1/320). Otherwise, negative results were obtained for ENA, parietal cells antibodies, smooth muscle antibodies, TSH, thyroglobulin antibodies, transglutaminase antibodies, and protein electrophoresis. Copper intoxication was ruled out, as well as several indolent infections: HIV serology, anti-HCV, anti-HVA, anti-HBs, HBs-Ag, anti-HBc, Cryptococcus, VZV, and HSV.
She was discharged five months after her initial admission, preparing for a liver transplantation two months later. The surgery was uneventful. Interestingly, her husband noted a new tremor involving the left lower extremity. An asymmetrical parkinsonian akineto-rigid syndrome appeared three weeks prior to the transplantation. This extrapyramidal syndrome occurred before the introduction of immunosuppressive agents (tacrolimus, azathioprine, and methylprednisolone). Neuroleptics were not prescribed during this admission, so the only possible impact of medication would have been the chronic use of neuroleptics. Carbidopa/levodopa was added three weeks after transplantation; within two weeks, her improvement was remarkable. Normalization of her muscle tone, pull test, postural reflexes, gait, and cognitive status was observed. The psychomotor agitation evolved towards a picture of bradykinesia and postural tremor involving the upper limbs, head, and tongue. The transplantation is certainly the leading factor accounting for her clinical improvement; manganese-induced parkinsonism is renown to be refractory to levodopa administration, regardless of the posology or dosing. This hallmark of manganese-induced parkinsonism correlates with the nigrostriatal pathway preservation. We opted for a short trial of levodopa/carbidopa, monitoring closely the possibility of psychotic exacerbation [].
She remained amnesic of the entire pretransplantation period, since the behavioural alteration. A follow-up brain MRI demonstrated a complete resolution of the pallidal hyperintensities. Carbidopa/levodopa was discontinued five months postoperatively, and the extrapyramidal features never recurred. A thorough neuropsychologic evaluation revealed that she performed at her baseline; she eventually returned to work and was eligible to drive.
Although manganese levels were not measured during the symptomatic period, manganese accumulation might have caused the symptomatology. In a state of chronic hepatopathy, hypoalbuminemia favours the passage of manganese through the blood-brain barrier. Manganese can stimulate the release of dopamine from presynaptic storage sites. Chronic manganese overload stimulates monoamine oxidase activity, increasing dopaminergic degradation and accumulation of its metabolite, homovanillic acid. This metabolite has been found in excessive quantities at autopsy in patients deceased with chronic hepatopathies [–]. Dichotomic observations characterize the impact of manganese deposition on dopamine neurotransmission. Nonetheless, studies describing manganese serum level by spectroscopy demonstrate a positive correlation between the serum level and the pallidal index (comparison between cortex and pallidal intensity on MRI) [–]. Manganese accumulates selectively within the basal ganglia; a positive correlation was also established clinically with the severity of the extrapyramidal symptoms, linking serum manganese directly to dopaminergic depletion [, ]. Manganese acts as a blocker of the D2 postsynaptic dopaminergic receptors, sparing nigrostriatal neuronal integrity. Dedicated specific neuroimaging modalities corroborate this hypothesis []. Even more, in vitro manganese exposure was shown to impact dopamine by reducing its uptake as well as its subsequent amphetamine efflux [].
Our research and literature review highlighted this etiology retrospectively. We unfortunately did not document a manganese serum level at the time of the initial presentation. Despite that, we investigated our patient thoroughly, considering an extensive differential diagnosis as described above, mentioning hepatic encephalopathy and delirium. Her initial presentation, evolution throughout the transplantation process, and outcome are retrospectively strongly compatible with manganese overload. |
pmc-6077363-1 | A previously healthy 17-year-old male presented with the complaint of mild hemoptysis after sustaining a blunt trauma to the chest. He fell off a 3-foot cliff while hiking and landed on the right side of his chest. On presentation, the patient's pain was tolerable and he was breathing comfortably. His vital signs showed a pulse of 98 beats per minute, blood pressure of 110/60 mmHg, and an oxygen saturation of 98% on room air. His exam revealed minimal abrasions, ecchymosis, and tenderness over the right lower chest wall at the anterior axillary line. His lung exam revealed decreased breath sounds over the right lower lung field. A chest X-ray obtained within 2 hours of the trauma showed alveolar opacities in the right lower lobe with multiple cystic air spaces containing air-fluid levels (). There were no associated pleural effusions, pneumothorax, or rib fractures. A Computed Tomography (CT) scan of the chest showed thick-walled multicystic lesions with patchy air space opacities and consolidations in the right lower lobe (). No previous chest imaging was available for comparison. The described CT scan abnormalities, in the absence of extrapulmonary posttraumatic findings, were suggestive of CPAM with superimposed bleeding. The patient was admitted for observation and evaluation and placed on intravenous Amoxicillin/Clavulanate. Spirometry done the next day was normal. His complete blood count, basic metabolic panel and bleeding profile were normal. His C-reactive protein was elevated at 32.0 mg/L. Gram stain, acid fast stain, and sputum cultures for bacteria, fungi, and tuberculosis were all negative. Alpha-1 antitrypsin and immunoglobulin levels were within normal limits.
The patient was evaluated by a cardiothoracic surgeon and a right lower lobectomy was being considered. However, given the indolent course of his disease and his negative history for pulmonary infections thus far, the patient elected to defer further surgical evaluation and, instead, follow-up with clinical observation. He remained asymptomatic throughout the interval period and a chest X-ray repeated after one year was normal (). Finally, a CT scan of the chest obtained two years later showed complete resolution of the previously described abnormalities (). Due to the fact that his cysts resolved spontaneously with time after his trauma, the patient was finally diagnosed with TPP. |
pmc-6077457-1 | A 21-year-old female presented to the dermatology clinic with severe facial acne with some scars. Severity of acne was graded as 4 on IGA scale (investigator global assessment of acne) which is accepted by American FDA []. She has used topical treatments including topical retinoids (Tretinoin and Adapalene creams) for several months with no satisfactory results. On presentation, she did not have any other complaints and was not on any systemic treatments. Her weight was 45 kg.
After initial laboratory works (lipid profile and liver enzymes) which were in the normal range, she was started on 20 mg isotretinoin. She was maintained on 20 mg (0.5 mg/kg) for 6 months. She had mild chelitis and skin dryness and complained of mild hair fall. Repeated liver enzymes and lipid profile after one month and 4 months were within normal range. Her acne has cleared completely.
She stopped the treatment because of inability to attend the clinic for few weeks.
After 2 months of stopping isotretinoin, she noticed a single whitish patch on her nose. She is fair-skinned, so the lesions were not apparent except on tanning after sun exposure. Antifungal treatment was used for few weeks topically with no improvement as it was thought to be pityriasis versicolor. Then the lesion began to expand, and new lesions appeared around mouth, cheeks, and right ankle area. Hand lesions appeared as well (). On Wood's light examination, the patches were revealed to be depigmented. The pattern of acrofacial vitiligo is noted [].
Thyroid function test initially showed low TSH, 0.177 uIU/L (normal range: 0.27-4.2), and normal levels of free T3, 6.11 pmol/L (2.8- 7), and free T4, 15.7 pmol/L (12-22). Three months later, TSH was high, 9.61 uIU, and normal free T3 (4.7 pmol/L) and free T4 (12.2 pmol/L) and thyroid antibodies were positive; thyroid peroxidase antibodies were 157.59 IU/mL (normal range: 0-5.6) and thyroid thyroglobulin antibodies were 66.09 IU/mL (normal range: 0-4.11). She was started on thyroxine and followed up at the medical clinic. Vitamin D3 was low, 47.11 nmol/L (normal range: 75-250 nmol/L), and she was started on vitamin D supplement as well. She had no family history of vitiligo. There was a family history of diabetes, hypertension, and systemic lupus erythematosus (SLE) and her auntie died from renal complication of SLE.
She was started on Tacrolimus 0.1% cream. Mild improvement was noted in some of lesions after 8 weeks. New lesions appeared again after another month. She stopped the topical treatment and opted to homeopathic treatment. |
pmc-6077506-1 | A 55-year-old woman (gravida 1, para 1) was referred to our hospital because of the progression of a lower abdominal tumor. At 45 years of age, she underwent a total abdominal hysterectomy (TAH) at another hospital for a leiomyoma, which persisted after the surgery. One year later, an attempt to reduce the progressing residual tumor was unsuccessful. Two years after the TAH, the tumor had extended into the IVC and right cardiac chamber; thus, she underwent tumor resection surgery at another hospital and was admitted to our care some years after her last surgery. Computerized tomography (CT) revealed a large tumor occupying the abdominal cavity and multiple bilateral pulmonary nodules (). The patient's course was complicated by renal failure due to ureteric stenosis, secondary to the expanding tumor. Her serum estradiol level was 11 pg/ml and FSH level was 103 mIU.
A transabdominal needle biopsy was performed to exclude a malignant tumor; there was no nuclear atypia and the mitotic index was low. Thus, the final histopathological diagnosis was leiomyoma (). On immunohistochemistry, the tumor was positive for estrogen and progesterone receptors. In addition, the tumor cells stained strongly positive for Alcian blue (pH = 2.5). Moreover, the staining disappeared after hyaluronidase digestion, suggesting that the tumor contained abundant hyaluronan (Figures and ). Thus, she was diagnosed with IVL and benign metastasizing leiomyoma.
The tumor temporarily responded to hormonal treatment (letrozole, medroxyprogesterone) and became smaller. However, the tumor eventually progressed. Among other conditions, she had a progressing lung metastasis, gastrointestinal obstruction, repeated cellulitis, and leg edema. The patient died of multiple organ failure due to tumor progression, 13 years after her initial surgery. |
pmc-6077506-2 | A 46-year-old woman (gravida 2, para 2) was referred to our hospital complaining of a lower abdominal mass and pain. Her medical history was unremarkable. She was initially diagnosed with a uterine leiomyoma by transcervical needle biopsy. CT revealed a large heterogeneous tumor occupying the pelvic cavity and an intravascular tumor within the dilated left internal iliac and ovarian veins (Figures and ). Her preoperative cervical cytology results were negative for intraepithelial lesions and malignancy. The endometrial cytology and needle biopsy results were also negative. Thus, the preoperative diagnosis was IVL, with extension of the tumor into the left internal iliac and ovarian veins.
Intraoperatively, multiple myomas were found within the uterine corpus and cervix, and the tumor extended to the parametrium and paracolpium. Detachment of the tumor from the left ureter and vaginal wall was very difficult. Intravenous tumors in the left internal iliac and ovarian veins could be palpated. The left internal iliac vein forming the common iliac vein was transected at the bifurcation region. In addition, TAH and bilateral salpingo-oophorectomy (BSO) were performed, resulting in the complete surgical resection of the tumor (operative time, 11 hours; blood loss, 8462 g). The resected uterus and adnexa weighed 897 g (Figures and ). There was no residual tumor detected in the venous resection stump.
The nodule resected from the uterus and the internal iliac and ovarian veins consisted of a proliferation of spindle cells. There was no nuclear atypia and the mitotic index was low. In addition, vessel endothelium cells and a vascular smooth muscle layer covered the IVL (Figures and ). The tumor cells stained positive for Alcian blue (pH = 2.5) and the staining disappeared after hyaluronidase digestion. However, compared to that in Case 1, the intensity of the staining was weaker and less diffuse (Figures and ). Similar findings for hyaluronan expression were obtained using the sample retrieved from the preoperative needle biopsy.
The histopathological diagnosis of the uterine and intravascular tumors was IVL. There has been no evidence of IVL recurrence, with the most recent follow-up at 38 months postoperatively. |
pmc-6077511-1 | A 35-year-old male presented to the emergency department after a tonic-clonic seizure. There was no significant past medical or surgical history. His physical examination was unremarkable, with no fever or focal neurological signs. In the previous 6 months, he reported anorexia and unintentional weight loss of 8 kg, with no other constitutional signs or symptoms. Brain MRI disclosed three ring-enhancing T1 and T2 hypointense cortical lesions, two located in the right frontal lobe and one in the left occipital lobe, associated with vasogenic oedema and absent leptomeningeal enhancement (). Based on the imagiological findings, infectious abscesses and metastatic deposits were considered the most probable etiologies. In subsequent diagnostic workup, abdominal CT revealed massive mesenteric infiltration and innumerous lymphadenopathies; therefore neoplastic peritoneal carcinomatosis was first considered. Chest CT and further radiological examination were unremarkable, excluding other organs involved.
Histological examination of the mesenteric lesions revealed multiple noncaseating perivascular granulomas (). The polymerase chain reaction (PCR) performed in the tissue specimen was positive for Mycobacterium tuberculosis, thus confirming the diagnosis of disseminated tuberculosis of CNS and peritoneum. Extensive laboratory workup for underlying acquired or hereditary immunosuppression was negative, including human immunodeficiency virus testing, immunoglobulin levels, and lymphocyte subset counts. Acid fast bacilli smear, cultures and PCR from sputum, CSF, and blood were negative. The patient was started on tuberculostatic treatment with adjunctive corticosteroids, in a four-drug regimen during the first two months, followed by additional two-drug regimen in the subsequent eight months. He had a favorable outcome, with complete regression of both cerebral and peritoneal lesions. |
pmc-6077527-1 | A 32-year-old male patient presented to our department due to gynecomastia and breast pain he had been suffering from for 2 years. The patient had already been seen by physicians from three different specialties before, including a urologist.
More than one year earlier, a gynecologist had performed breast ultrasound and described bilateral, mainly retromammillar gynecomastia. He classified his findings as grade 3 according to BIRADS (breast imaging reporting and data system) with a risk of malignancy not higher than 2% and suggested performing a biopsy and urological evaluation.
The patient went to see an endocrinologist next who diagnosed hypogonadotropic hypogonadism with elevated estradiol and prolactin levels (). On Magnetic Resonance Imaging (MRI) of the neurocranium, no abnormalities were found. The endocrinologist suggested controlling the hormone status and pointed out possible provocation tests to further specify the findings.
Lastly, the patient had been seen by a urologist in private practice. Physical exam, ultrasound of the abdomen, and MRI of the upper abdomen did not lead to diagnosis.
In our department, the patient reported a maldescensus testis in childhood which had resolved spontaneously. He had not undergone prior surgery and did not report any regular drug intake. Physical examination did not reveal abnormalities apart from bilateral gynecomastia. On ultrasound, a 1.6x1.6 cm hypoechogenic mass within the right apical testis without hypervascularisation was detected ().
Considering hormonal alterations, gynecomastia, and normal testicular tumor markers, we decided to perform testis-sparing surgery with frozen section using an inguinal approach. In the operating room, the tumor appeared to be capped and rather not malignant on frozen section and could be excised in sano. Final histology confirmed a Leydig cell tumor without histological signs of malignancy (). As chest and abdominal computed tomography did not show abnormalities, it could be classified as low risk.
On the first follow-up one month after surgery, the patient was in good general condition, yet gynecomastia had not regressed. Sexual hormones were within normal range. Half a year later, the patient had undergone a lifestyle change and lost 12 kg. Gynecomastia was still palpable but had significantly decreased. We recommended biannual follow-up for the first two years, and then yearly check-ups, including control of hormone levels, physical examination, and imaging of the chest and abdomen every 2 years. |
pmc-6077531-1 | We report the case of a 65-year-old man known for hypertension, cholelithiasis, and panic disorder with no personal or family history of pheochromocytoma, paraganglioma, Multiple Endocrine Neoplasia Type 2 syndrome, Von Hippel Lindau syndrome, Neurofibromatosis Type 1, or Succinyl Dehydrogenase mutations. He is a past smoker who quit 5 years prior to presentation and cumulated a 20-pack-year smoking history with no history of dyslipidemia or diabetes. The patient described a two-year history of frequent episodes of flushing, diaphoresis, systolic blood pressure surges up to 200 mmHg, loss of vision, headaches, palpitations, and tremors. He also complained of more frequent episodes of presyncope up to 6 times a day in the few weeks prior to seeking medical attention. The patient denied pallor, weight loss, weakness, or abdominal pain. His blood pressure was episodically elevated with only a moderately elevated baseline blood pressure. His only antihypertensive therapy at his first visit to our Endocrinology clinic was terazosin 1 mg once daily with only partial relief of his paroxysmal symptoms.
The patient was initially diagnosed with panic disorder and treated with cognitive-behavioural therapy. On physical exam, the patient's weight was 92 kg, his height was 1.77 m, and his BMI was 29.4 kg/m2. His blood pressure was 168/100 mmHg; his heart rate was regular between 90 and 100 bpm. His abdominal exam, however, revealed an obese nontender abdomen with a palpable right-sided suprarenal mass of 6-7 cm diameter, which was soft and mobile.
Two 24h urine collections for metanephrines and catecholamines were performed (c.f. ) and confirmed hypersecretion. No plasma aldosterone or renin levels were drawn, and no Cushing syndrome screening test was performed.
An MRI of the adrenals (c.f. ) reported a large right adrenal mass measuring 7.6 x 7.6 x 7.2 cm with T2 hyperintensity centrally and no loss of signal in T1. It was reported as highly suspicious for pheochromocytoma with a normal left adrenal gland, liver, and pancreas and no evidence of metastasis. Of note, the patient was not known for any underlying cardiac arrhythmia and had a normal baseline electrocardiogram in sinus rhythm at 95 bpm. No echocardiogram was ordered preoperatively.
The patient was referred to a urologist at an academic centre. He was then seen by Endocrinology one month prior to surgery. At his first visit to our clinic, he was counselled to have a high-salt diet and oral hydration was encouraged. The dose of terazosin was increased to 1 mg po bid x 1 week and then 2 mg po bid. Chromogranin A was elevated: 274 ng/mL (N < 82 ng/mL). An MIBG Scan was ordered, but the radiotracer was unavailable.
The patient was closely followed up by phone every week. Terazosin was increased to 2 mg po bid and metoprolol was added and increased to a maximal dose of 25 mg tid until he demonstrated orthostatic hypotension. The patient was admitted one day prior to surgery with a well-controlled blood pressure of 112/70 mmHg.
In the operating room, shortly after intubation and Foley catheter insertion and prior to surgical incision, the patient's blood pressure rose to 350/180 mmHg without improvement despite intravenous phentolamine boluses. Given the inability to control the patient's labile blood pressure, a decision was made to abort the operation and transfer the patient to the intensive care unit while remaining intubated.
In the intensive care unit, the patient required massive doses of intravenous phentolamine, nitroprusside, and nicardipine as well as intravenous hydration, as these are the main options for management of a pheochromocytoma hypertensive crisis as per current clinical practice guidelines []. Interestingly, his blood pressure would oscillate between 60/34 and 350/186 mmHg within a matter of 2-3 minutes in a cyclical pattern (c.f. ). A nasogastric tube was inserted, and the patient was started on phenoxybenzamine per tube. After 72 hours in ICU, he was weaned off intravenous antihypertensives and sedatives and extubated.
He was transferred back to the surgical ward, while gradually having his blood pressure medications uptitrated to phenoxybenzamine 120 mg po bid, metoprolol 100 mg po bid, and nifedipine XL 60 mg once daily. His blood pressure was then well controlled. The patient underwent successful open right adrenalectomy three weeks later. He was hypotensive intraoperatively, requiring vasopressors. He had an uncomplicated postoperative course.
Further investigations in hospital during his stay included free plasma normetanephrine 14.44 nmol/L (N<1.2 nmol/L) and metanephrine 6.09 nmol/L (N< 0.48 nmol/L), a negative cerebral CT scan, and an Octreoscan showing no site of extra-adrenal uptake. An Octreoscan was performed as a surrogate functional imaging modality given the unavailability of MIBG (recommended functional imaging modality) radiotracer at our centre around the time of this patient's admission. Pathology confirmed an 8 cm right adrenal pheochromocytoma without angioinvasion, extra-adrenal extension, or necrosis.
One month postoperatively, the patient was seen in the Endocrinology clinic and reported feeling overall well with no documented hypertension. He stopped all antihypertensive medications and had no palpitations, diaphoresis, flushing, headaches, or other symptoms of catecholamine excess. His 24h urine metanephrines and catecholamines normalized during follow-up (c.f. ). The patient has not undergone genetic analysis during follow-up.
Unfortunately, the patient sustained a ST-elevation myocardial infarction three months postoperatively, requiring urgent percutaneous coronary intervention and stent placement. Unfortunately, he was treated at another hospital and results from his coronary angiogram were not available to authors. He survived this acute cardiac event and continues to be followed closely by Cardiology. His longstanding elevated blood pressure secondary to his pheochromocytoma and his prior smoking history were significant risk factors for his myocardial infarction. |
pmc-6077533-1 | A 62-year-old male patient presented with progressive worsening of mental function, dysphasia, and ataxic gait in the last six months. Five years prior to presentation (in August 2012), he was diagnosed with communicating hydrocephalus possibly caused by tuberculous meningoencephalitis because of mental confusion and gait disturbance. He underwent a ventriculoperitoneal shunt surgery in one hospital. His mental confusion and gait disturbance immediately improved following the ventriculoperitoneal shunt. Results of CSF study were negative for tuberculosis. However, a provisional diagnosis of communicating hydrocephalus caused by tuberculous meningitis was made based on MRI findings of leptomeningeal enhancement in the basal cisterns (Figures and ). He had been treated with antituberculosis medication for the following six months after the shunting operation. After shunting and medical treatment, he returned to his work. He had been followed-up regularly every six months at that hospital. His physical and mental conditions were stable. He experienced no difficulty in work or daily activities.
Six months prior to the present presentation (December 2016), slurred speech and mental confusion with intermittent disorientation to time and place developed within several days. CSF analysis and MRI of the brain were performed. CSF analysis showed white blood cell (WBC) count of 9 cells/μL, red blood cell count of 33,000 cell/μL, protein level of 4228 mg/dL, lactic dehydrogenase (LDH) level of 224 mg/dL, and glucose level of 130 mg/dL. MRI of the brain showed multiple linear and nodular leptomeningeal enhancing lesions scattered in basal and left sylvian cisterns (). The extent of leptomeningeal enhancement in basal cisterns was markedly increased compared to that in MRI examination done in 2012. The size of the ventricle was small, indicating that shunt malfunction did not occur. There was no abnormal spike activity in his electroencephalography (EEG) except intermittent slow wave in his left frontocentral area. Under an impression of aggravation of tuberculosis meningitis, he was referred to our hospital (January 2017).
The patient's consultation in the Department of Infectious Medicine was carried out for aggravation of tuberculous meningitis/encephalitis. The doctor in neurology thought that tuberculous meningitis aggravated again. For possibility of drug-resistant tuberculosis, four-drug regimen (isoniazid 75 mg, rifampicin 150 mg, pyrazinamide 400 mg, and ethambutol 300 mg; tubes tab 4 times a day for 2 months followed by isoniazide and rifampicin for 7 months) against tuberculosis was used. Beside antituberculosis medications, steroid was prescribed. The patient's mental confusion, dysphasia, and irritability progressively improved over the course of one month at the outpatient clinic. He returned to his usual life again. He was able to work in his previous job without apparent complications.
His mental confusion and dysphasia accompanying gait disturbance gradually developed again within four months (June 2017), leading to reevaluation of the brain by MRI. There was no fever or signs of meningeal irritation in neurologic evaluation. MRI of the brain surface revealed extensive progression of diffuse leptomeningeal enhancement in the basal and left sylvian cisterns (). No intraparenchymal enhancing lesion was noted. Hydrocephalic change was not shown either. CSF examination showed WBC count of 110 cells/μL (lymphocyte 70%, macrophage 7%, and neutrophils 3%), red blood cell count of 7200 cell/μL, protein level of 4272 mg/dL, and glucose level of 102 mg/dL. Levels of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were 4 mm/hr and 0.05 mg/dl, respectively. Levels of adenosine deaminase (ADA) and immunoglobulin G were 8.0 IU/L and 901 mg/dl, respectively. Results of CSF culture for toxoplasmosis, fungus, cryptococcus, and herpes simplex virus were all negative. Gram-staining revealed many WBC without microorganism. Polymerase chain reactions (PCR) of the CSF against Mycobacterium tuberculosis, herpes simplex virus, varicella zoster, enterovirus, and Epstein-Barr virus were all negative. Culture for acid-fast bacilli (AFB) did not show any growth until eight weeks after incubation. For possibility of leptomeningeal metastasis, biopsy of the brain, and leptomeninges was requested.
Biopsy of the brain surface and leptomeninges was performed on the left frontal cortex and sylvian fissure proceeded by a small frontotemporal craniotomy. Postoperative course was uneventful. Histologic diagnosis revealed DLGNT without intraparenchymal brain lesion (). Monotonous oligodendrocyte-like or neurocyte-like tumor cells with round nuclei and clear cytoplasm were found (). Mitosis, microvascular proliferation, and necrosis were not evident. Immunohistochemical stainings for Olig-2 and synaptophysin were positive (Figures and ). Those for CD68, isocitrate dehydrogenase- (IDH-) 1, glial fibrillary acidic protein (GFAP), and neurofilament were negative. Ki67 proliferative index was low (5%). PCR for O6-methylguanine-DNA-methyltransferase (MGMT) methylation was positive. However, 1p19q codeletion was not detected by interphase fluorescent in situ hybridization (FISH). Methenamine-silver and PAS staining for fungal organism, Ziehl-Neelsen staining, and PCR for Mycobacterium tuberculosis were all negative. After histologic diagnosis of DLGNT, MRI of the whole spine was subsequently performed in order to detect further leptomeningeal spread. MRI showed multiple leptomeningeal enhancing nodules displaying high signal intensity on T2-weighted images (), disseminating along the whole spinal cord without intramedullary lesion. With a final diagnosis of DLGNT by invasive brain biopsy, medical records and imaging results were thoroughly reviewed again. PCV (Procarbazine, CCNU, and Vincristine) chemotherapy and radiation therapy of the craniospinal axis were planned. The patient's condition gradually deteriorated with apparent worsening of severe memory impairment, disorientation, and gait ataxia. |
pmc-6077536-1 | Miss A. L., a 17-year-old girl, with no pathological history and no notion of contact with dogs, reported since 3 months right thoracic pain, stage III of mMRC dyspnea, chest tightness, and some episodes of hemoptysis of low abundance evolving in a context of apyrexia, and conservation of the general state. The clinical examination revealed a right fluid effusion syndrome. The posteroanterior chest roentgenogram showed a homogeneous right basal opacity that effaced the diaphragmatic cupola and merged with mediastinum; its upper limit is convex ().
Thoracic ultrasonography revealed an intrapleural cyst with a duplication of its wall suggesting a proliferative membrane without associated pleurisy ().
Thoracic CT showed a right basal-thoracic cystic formation, measuring 126 ∗ 93 ∗ 93 mm, with a discreet slope with the adjacent parenchyma; its wall was thickened and enhanced after injection of contrast product. The lung parenchyma was without anomaly with the exception of passive atelectasis adjacent to the cyst, confirming the diagnosis of a right pleural cyst type II of Gharbi classification ().
The blood count was normal and the ELISA and Indirect Agglutination serologies were negative. In a second stage, the research for other localizations of the hydatid cyst was negative (abdominal ultrasound, echocardiography, and cerebral CT), hence the primitive character of pleural hydatidosis in our observation. During surgery, the presence of a cystic formation in the parietal pleural was noted. The delicate dissection had objectified thickened visceral pleura. The cystectomy was successfully performed without rupture and the piece was sent to the parasitology laboratory with evidence of proliferative membrane (macroscopically) and alive scolex in the intracystic fluid (microscopically) (). |
pmc-6077536-2 | Mr. SF, a 26-year-old man, without any notable pathological history, have a notion of contact with dogs in childhood, asymptomatic on the respiratory plane. The posteroanterior chest roentgenogram was performed for him as a preemployment checkup. It objectified a homogeneous oval opacity, well limited, left hilar, and having the internal edge in intimate contact with the left edge of the heart ().
In this context, a chest CT scan revealed a left anterolateral mediastinal mass with a total parietal calcification measuring 70 mm in height and 55 mm in lateral diameter ().
Echocardiography confirmed the presence of left-ventricular extracardiac structure without intracavitary lesion or associated pericardial effusion. Likewise, magnetic resonance imaging (MRI) showed a mediastinal cyst next to the anterolateral wall of the left cardiac ventricle, in close contact with the pericardium but with a cleavage plane and no mass effect on the cardiac cavities, measuring 72mm ∗ 53mm. Its tonality was hypointense on T1 and hyperintense on T2 ().
The blood count was normal and the ELISA and Indirect Agglutination serologies were negative. In a second stage, the research for other localizations of the hydatid cyst was negative (abdominal ultrasound and cerebral CT), hence the primitive character of pleural hydatidosis in this second observation. In operation, the heart was of normal volume with no intrapericardial mass. At the opening of the left pleura, the exploration found a solid mass contiguous to the mediastinal pleura and in contact with the left phrenic nerve. The careful dissection and excision of the mass were successfully performed without complications. |
pmc-6077542-1 | A 50-year-old lady presented to the Pondicherry Institute of Medical Sciences Hospital at Puducherry, India, with a complaint of acute abdominal pain. Contrast enhanced computed tomography (CECT) demonstrated the presence of bilateral ovarian mature cystic teratomas. Contrast enhancement within the right ovarian cyst suggested the possibility of malignant transformation (). Tumor marker carbohydrate antigen- (CA-) 125 was 27 IU/mL (normal <35 IU/mL). She underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy.
Gross examination showed the right ovary to be cystic and measured 12cms in diameter and is predominantly smooth except for an area of 3cm2 which had blunt pale soft projections (). The left ovary was grossly normal measuring 3cms in greatest diameter.
Microscopically the left and right ovary showed various mature tissues including bronchial mucosa, apocrine glands, cartilage, and skin with adnexal structures. The microscopy of the soft pale projections of the right ovary had papillary structures with fibrovascular cores which were lined by transitional epithelium exhibiting nuclear pleomorphism, hyperchromatism, and increased mitotic activity (). There was evidence of invasion of the ovarian stroma by nests of malignant epithelial cells (). The inked ovarian capsular surface was free of tumor. Immunohistochemistry (IHC) of the urothelial carcinoma showed cytoplasmic and membrane positivity for Uroplakin II (). A diagnosis of ovarian cystic teratoma with primary invasive urothelial carcinoma (TNM stage pT1aNxMx) was made based on the Pathological Stage Classification by the American Joint Committee on Cancer (AJCC) 8th edition [].
The patient was reviewed till 3 months following surgery and follow-up CECT revealed no evidence of recurrent tumor in the abdomen and pelvis. |
pmc-6077569-1 | An 8-year-old East Indian boy with Fitzpatrick skin type IV phototype complexion presented with numerous blue-gray macules and patches over the back, anterior trunk, arms, and legs of 8 months' duration. The lesions first appeared on the back and then spread to the anterior trunk, arms, and legs. Some of the lesions were mildly pruritic and some with preceding erythematous borders. The lesions were progressive and increased in size and number with time. There were no identifiable triggers. His past medical history was significant for Berry syndrome (a complex aortopulmonary malformation). The aortopulmonary malformation was repaired surgically at 10 days of life. The surgical repair was successful and the postoperative course was uneventful. Otherwise, his health was unremarkable and he was not on any medications. There was no history of previous skin eruption. He had no known family history of autoimmune disorder or similar skin disease.
On physical examination, there were numerous well-demarcated, oval, ash-brown macules and patches symmetrically distributed over the back, anterior trunk, arms, and legs (Figures –). The lesions measured 0.5 to 6 cm and some lesions were confluent. There were no erythematous borders and no desquamation. Darier's sign was negative. The mucous membranes, face, scalp, palms, soles, and nails were spared. A well-healed scar from previous sternotomy was noted on the chest. The rest of the physical examination was unremarkable.
Dermoscopy of a lesion showed faint gray-blue to bluish small dots over a bluish background, corresponding to melanin-laden melanophages in deeper dermis (Tyndall effect) (). The patient was diagnosed to have erythema dyschromicum perstans based on the clinical and dermoscopic findings.
Parents were reassured of the benign nature of the disorder and that the lesions would resolve with time. A skin biopsy was declined by the parents. |
pmc-6077573-1 | A 48-year-old male with past medical history of hyperlipidemia, HIV, and latent secondary syphilis presented for evaluation of loss of libido and erectile dysfunction for 2 months' duration. He had no other complaints. On examination, the patient was hemodynamically stable and did not show any signs of adrenal insufficiency. The only remarkable physical finding was a decrease in bilateral testicular size. On lab work, total testosterone level was 21.47 ng/dL (N: 300-1080 ng/dL) and morning cortisol was <1.0 μg/dl. (N: 6.7-22.0 μg/dL). Luteinizing hormone (LH), follicular stimulating hormone (FSH), and thyroid-stimulating hormone (TSH) as well as serum electrolytes were within normal limits. Upon reviewing his medication list, we found that the patient was taking MA (Megace) 800 mg daily as an appetite stimulant.
About 1 month prior to starting this medication, his total testosterone was normal at 548 ng/dl (N: 262-1593 ng/dl) along with his FSH, LH, prolactin, prostate specific antigen, and sex hormone binding globulin. Brain MRI showed only a partial empty sella and no other abnormalities. After excluding all other potential causes, MA was deemed to be responsible for his secondary hypogonadism. The patient was advised to taper down his MA slowly over a period of 6 weeks. Upon tapering down MA, the patient immediately showed improvement of his symptoms. His repeat lab work 4 weeks after discontinuation of MA revealed total testosterone, 798 ng/dl (N: 300-1080 ng/dl), and random cortisol, 6.0 μg/d. (N: 2.0-14.0 μg/dL). His libido returned, testicular size showed improvement, and he started to experience normal erections. He was started on an alternative appetite stimulant and is currently doing well. |
pmc-6077576-1 | A 21-year-old gravida 1 para 0 patient presented at 20 weeks for a routine anatomy scan that revealed normal anatomy survey including two umbilical arteries (). She had an unremarkable past medical history. The estimated fetal weight was 874 grams (27th percentile) at 26 weeks and 1306 grams (26th percentile) at 29 weeks. Ultrasound at 29 weeks revealed a single umbilical artery () raising the suspicion for a pathological process. Fetal echocardiogram was normal. At 31 weeks, the estimated fetal weight was 1349 grams (less than 5th percentile) with normal Doppler evaluation and biophysical profile. At 32 weeks, the amniotic fluid index was 10.1 cm, the biophysical profile was 10/10, and the cerebroplacental ratio decreased to 1.083. At 33 weeks, the amniotic fluid index decreased to 2.3 cm, maternal and fetal Doppler evaluations were normal except for abnormal cerebroplacental ratio of 1.08, and fetal heart tracing showed spontaneous prolonged decelerations. Secondary to the recurrent prolonged decelerations, a primary cesarean delivery was performed, and a live male infant was delivered weighing 1395 g with APGAR scores of 8 and 9 at 1 and 5 minutes, respectively. Placental pathology showed thrombosis of one of the umbilical arteries with necrosis of the medial myocytes (Figures and ). It also showed subendothelial fibrin deposition in stem villous blood vessels, chorionic villous hypervascularity, and a small subchorionic placental infarct. At birth, the baby had no signs related to thrombosis. Thrombophilia profiles showed a severe protein S deficiency (activity 13%) at birth which resolved at two months of age (activity 66%). The neonate has an uneventful clinical course since birth. |
pmc-6077586-1 | A 54-year-old Caucasian male with past medical history of profound intellectual disability, schizophrenia, posttraumatic stress disorder, Parkinson's disease, gastroesophageal reflux disease, and seizure disorder initially presented to the emergency department (ED) from an assisted living facility with fever, tachycardia, nausea, and vomiting of three days duration. The patient was diagnosed with sepsis of unknown origin and admitted to the hospital for further workup and treatment. On admission, vitals showed the following: temperature of 102.5F, pulse 123 beats per minute, respiratory rate 40 breaths per minute, O2 saturation of 96% on room air, and blood pressure 154/84 mm/Hg. On imaging, chest X-ray showed no evidence of acute cardiopulmonary disease. Electrocardiogram showed sinus tachycardia with possible right atrium enlargement and ventricle hypertrophy. Laboratory studies showed the patient had no leukocytosis; however, segmented neutrophil was 92.8% and absolute neutrophil count was 9.19 k/cmm. Hemoglobin and hematocrit were decreased at 12.8 and 38.0%, respectively. Atrial blood gas was unremarkable. Lactic acid sepsis was initially 1.96 mmol/L and later increased to 2.63 mmol/L. Procalcitonin was 0.32 ng/ml. Metabolic panel showed sodium was 149, chloride 114, BUN 37 mg/dL, AST 136, ALT 43. Urinalysis showed trace blood likely secondary to catheterization and urine ketones of 40 mg/dL. Urine culture was negative after 48 hours. Blood cultures from admission were negative after five days. Influenza screen was negative. Viral PCR was negative. Physical examination at admission showed the patient was in moderate distress, diaphoretic, agitated, tachycardic, and tachypneic. The patient was started on antibiotic therapy with vancomycin, levofloxacin, and piperacillin/tazobactam for sepsis of unknown origin and given fluid resuscitation. Home medications included the following: paliperidone 3mg daily, haloperidol 2.5mg twice daily as needed, citalopram 40mg daily, carbidopa/levodopa 25-100mg three times daily, and carbamazepine ER 200mg twice daily.
On hospital day 2, the patient developed increased muscle rigidity, tremors, hypoxemia with O2 saturation in the 70s on room air, increasing temperature (102.9F), tachypnea (max respiratory rate 45), and worsening tachycardia (max heart rate 130). Repeat labs revealed leukocytosis with a white cell count of 11.9 k/cmm and neutrophil count of 89.6 k/cmm. Patient also developed acute renal insufficiency with BUN and creatinine of 52 mg/dL and 1.53 mg/dL, respectively. Lactic acid further increased to 4.3 mmol/L. Total creatine kinase was 2249 u/L.
The patient was transferred to intensive care unit (ICU), intubated, and ventilated for airway protection. Close review of medical records from his assisted living facility indicated that the patient had received intramuscular injections of haloperidol 2.5mg seven times over the previous three weeks to help with behavioral agitation. The last dose was administered on the day of admission. Over the same period of time, it also appeared that the patient had been refusing many of his scheduled medications to include carbidopa/levodopa. The patient's history, examination, and laboratory findings strongly suggested a diagnosis of NMS and treatment was initiated accordingly. A one-time dose of dantrolene 125mg IV was given. Additionally, the patient received supportive care measures including application of a cooling blanket and infusion of cooled normal saline provided for hyperpyrexia. Intravenous vecuronium was started for severe muscle rigidity. All psychotropic agents were held.
Neurology recommended video electroencephalography (VEEG) due to the patient's history of seizures. VEEG showed no epileptiform activity and mild nonspecific encephalopathy. Neurology recommended the resumption of carbidopa/levodopa 37.5-150mg three times daily and carbamazepine 200mg twice daily with titration to a final dose of 500mg twice daily with a target carbamazepine level of 10-12. Throughout the ICU stay, the patient's total CK level trended downward, decreasing from 2249 u/L to 535 u/L. BUN and creatinine trended down to 23 mg/dL and 0.85 mg/dL.
As the patient's condition improved, he was extubated and transferred to the general medicine unit. However, with resumption of carbidopa/levodopa, the patient's untreated psychotic symptoms exacerbated and included audio-visual hallucinations and delusional paranoia. He was also intermittently agitated and not sleeping well. Psychiatry was consulted for recommendations regarding treatment of psychosis in the setting of resolving NMS. Carbamazepine and lorazepam were recommended for management of agitation, and trazodone 100mg at bedtime was started for sleep. Psychotic symptoms and agitation persisted despite being on lorazepam 2mg three times daily as well as being on a therapeutic dosage of carbamazepine. The decision was made to start low dose quetiapine at 12.5mg at bedtime and titrated up to 12.5mg twice daily after four days, at which point the patient's psychosis diminished. The patient was monitored closely throughout for recurrence of NMS symptoms. Quetiapine was well-tolerated and the patient demonstrated improvement in psychosis, agitation, sleep, and appetite. After X days, the patient returned to his baseline mentation and was successfully discharged back to his assisted living facility. |
pmc-6077595-1 | A 46-year-old, brown-skinned woman with regular menstrual cycles and one child presented at the gynecology department of a philanthropic hospital in Vitória, Espírito Santo, Brazil, reporting a 6-month history of intense vaginal bleeding associated with abdominal pain. She had suffered vaginal discomfort over the previous week. She had no prior history of allergy, comorbidities, use of medication, or surgery. There was no family history of gynecological cancer. At physical examination, she was found to be in good general health, alert, pale, with a flaccid abdomen, and no signs of peritoneal irritation. A hypogastric mass was detected. There were no vulvar lesions. Speculum examination showed no lesions in the vagina but revealed the presence of a bleeding mass extruding from the external cervical os and associated with intense bleeding at manipulation. At bimanual pelvic examination, it was possible to palpate the pedicle of the lesion through the cervical os.
In view of the initial diagnostic hypothesis of a prolapsed fibroid, vaginal myomectomy was performed. There were no complications following surgery and the patient was discharged from hospital the following day in good clinical conditions.
Macroscopically, the pinkish-colored nodule measured 3.5 x 3 x 4 cm (). Microscopically, it consisted of a proliferative spindle cell nodule with gland-like, epithelioid, trabecular, and glomeruloid elements, without atypia. In some parts, the cells formed clear cell cords resembling ovarian sex cords. The core was rounded and normochromatic, and the cytoplasm was clear, resembling Sertoli cells. The stroma was partially hyalinized, resembling smooth muscle strips. There was no sign of necrosis and the mitotic index was low (2 mitoses/20 high-power fields) (Figures and ).
Immunohistochemistry confirmed the diagnostic hypothesis of a UTROSCT, with positive expression for CD56, smooth muscle actin, CD10, desmin, and pan-cytokeratin. The immunohistochemical markers for calretinin and inhibin were negative ().
The patient was readmitted to the department and metastatic screening was performed using computed tomography (CT) of the abdomen and chest. No abnormalities were found in the chest CT results. However, abdominal imaging showed the uterus to be greatly increased in size, with heterogeneous enhancement, an image resembling a cyst, probably of ovarian origin, in the left parauterine region, and a small amount of free fluid in the pelvis. No other abnormalities were found.
Serum levels of carcinoembryonic antigen (CEA) and CA 125 were <50 ng/ml and 15.50 U/ml, respectively.
Once the possibility of implantation metastasis had been ruled out, it was decided to submit the patient to a total abdominal hysterectomy with bilateral salpingooophorectomy. When accessing the abdominal cavity, a small amount of ascites was found and the left ovary was increased in volume, with a cystic appearance. The cecal appendix was normal. There were no complications following surgery and the patient was discharged from hospital the following day.
Histopathology performed on the surgical specimen obtained during the latest surgical procedure revealed uterine fibroids and focal adenomyosis, an endometrium with simple hyperplasia and no atypia, and a cervix with chronic cervicitis and squamous metaplasia. The right ovary was normal, while a hemorrhagic corpus luteum cyst was found on the left ovary. There was no sign of any residual UTROSCT.
The patient has been followed up regularly at the gynecological oncology outpatient department and remains asymptomatic one year after the second surgery. She will continue to be followed up as an outpatient for five years, with clinical examination and tomography of the chest, abdomen, and pelvis performed annually. At the end of the follow-up period, she will continue to undergo annual gynecological and clinical examination. |
pmc-6077596-1 | A 69-year-old man presented with dyspnoea, relapsing dizziness, falls, and systemic inflammatory response syndrome in our institution. Notable lab values were a white blood count of 17.7 /μl (normal range 4.0-10.0 /μl), an elevated C-reactive protein of 9.6 ng/ml (normal < 0.5 ng/ml), a haemoglobin level of 13.5 g/dl (normal range 13.5-17.5 g/dl), and an elevated international normalized ratio (INR) of 1.41 without anticoagulant medication.
Initial workup included computed tomography (CT) of the chest to rule out pulmonary embolism, which revealed right lower lobe pneumonia. In doing so, scans of the upper parts of the abdomen demonstrated liver cirrhosis without ascites or additional pathologies and with the greater omentum positioned almost entirely in the upper abdomen (). Intravenous antibiotic therapy was started for diagnosis of pneumogenic sepsis.
After six days of hospitalization the patient developed mild abdominal symptoms and his haemoglobin level decreased from 13.5 to 9.9 g/dl while INR increased from 1.41 to 1.51. An abdominal CT showed a moderate sized haemoperitoneum, particularly in the upper abdomen, left anterior perihepatic space, and surrounding a significantly enlarged segment of an omental artery in the left upper abdomen. Spots of enhanced tiny vessels also were visible in the right upper abdomen ventral to the liver (). SAM of the left omental artery (LOA) was suspected. As the patient remained haemodynamically stable with a borderline coagulopathic status, a noninterventional therapeutic approach was initially agreed upon. Fresh frozen plasma and erythrocyte concentrates were administered. Despite this therapy the haemoglobin levels further decreased to 7.8 g/dl during the next three days, so that an abdominal control CT was performed. This demonstrated slight progression of the haemoperitoneum. After multidisciplinary discussion the radiological department was asked to perform catheter angiography, if possible with transcatheter embolization.
Digital subtraction angiography (DSA) with selective superior mesenteric, common hepatic and splenic arteriogram was performed. These revealed an anatomic variant with absence of the left gastroepiploic artery (left gastroomental artery), the gastroepiploic arch being exclusively formed by the right gastroepiploic artery (right gastroomental artery), and consecutively a separate origin of the LOA (left omental branch) from an inferior pole splenic artery (Figures and ). Additionally, two tiny omental branches of the right gastroepiploic artery with small multisegmental dilatation and a typical “wind-sock” formed aneurysm of the LOA were visible (Figures and ). Although no active bleeding was detected, the aneurysm of the LOA was considered to be the obvious cause for the haemoperitoneum.
Superselective catheterization of the LOA through splenic artery and lower pole splenic artery with a microcatheter (2.7-F Progreat, Terumo, Tokyo, Japan) was technically challenging due to vessel tortuosity but succeeded, however, only just a few centimeters proximal to the aneurysm. The decision was made to embolize the LOA with N-butyl-2-cyanoacrylate (NBCA). Approximately 1 ml of a 1:3 mixture of NBCA (Histoacryl; B. Braun, Melsungen, Germany) with iodized oil (Lipiodol ultrafluid; Guerbet, Villepinte, France) was injected in the usual manner. The final DSA control confirmed the complete embolization of the aneurysm of the LOA with preservation of the splenic vessels ().
Postinterventional course was uneventful with no signs of omental infarction and with increase of haemoglobin levels up to normal levels.
One month after embolization and after therapy of his protracted pneumonia the patient was transferred to another hospital for early rehabilitation in a satisfactory general condition. |
pmc-6077600-1 | A 59-year-old male was scheduled for elective open retropubic prostatectomy for a benign enlarged prostate weighing approximately 65 grams. The patient's weight was 89 kg, ASA physical status II, diagnosed with essential hypertension two years ago, and controlled with ACE-I, Ramipril 10 mg once daily. No other morbidities were associated and no other medications were taken by the patient. The preoperative assessment did not reveal any other abnormality related to anaesthesia with normal vital signs, omitting Ramipril for 48 hours before the operation and normal baseline laboratory results including renal profile (creatinine 87 micromole/L, urea 7.9 mmol/L, Na 140 mmol/L, and K 4.1 mmol/L).
Following discussion with the patient and the surgical team, the anaesthetic plan was general anesthesia (GA) with postoperative patient-controlled analgesia (PCA) with morphine. Relatively uneventful induction of GA by propofol (2mg/kg), fentanyl (100 micrograms), and rocuronium (0.6 mg/kg) with endotracheal intubation, radial arterial cannulation for IBP monitoring, and two wide-bore peripheral cannulas (18G) were inserted. Induction was accompanied by hypotension (BP dropped from 112/68 to 73/46) and bradycardia (HR dropped from 78/min. to 38/min.) that required two successive doses of ephedrine each 6 mg were followed by restoration of BP and HR. Baseline arterial blood gas (ABG) after positioning was normal (). At 2 hours after the start of surgery, the estimated blood was about 350 ml and the urinary output (UOP) was 120 ml (over 2 hours) with mean arterial pressure (MAP) being maintained above 70 mmHg without further vasopressors required other than the initial 12 mg of ephedrine required immediately after induction. An arterial blood gas (2 hours after start of surgery) was initially performed for monitoring haemoglobin level showed hyperkalaemia (6.1 mmol/L) with acidosis (pH 7.33 and PCo2 6.2 kPa). The initial explanation was respiratory acidosis, and ventilation parameters were increased. Twenty-five minutes later ABG showed a decrease of PCo2 to normal with normal anion gap acidosis and increasing potassium to 6.5 mmol/L. Hyperkalaemia was treated with glucose-insulin (10 units of insulin added to 1 litre of glucose 10%) and mild hyperventilation and furosemide (20 mg bolus) with a change of the maintenance fluid from compound lactate solution to normal saline with the same rate (225ml/h). Forty minutes later, these measures had reduced potassium from 6.5 mmol/L to 4.1 mmol/L.
Despite normalization of potassium level (k 4.1 mmol/L) following the measures mentioned above, the acidosis persisted with maintained normal bicarbonate level and normal PCo2 (). From the time of normalized potassium, the acidosis required three hours to normalize which was two hours after recovery from GA. The presence of acidosis did not affect emergence from anaesthesia or recovery of the patient.
Postoperative follow-up of the renal function tests and electrolytes () revealed normalization over a period of two days postoperatively with the patient restored intake of ACE-I on day one postoperatively with no effect on potassium level.
At the end of surgery, the estimated blood loss was about 635 ml, UOP was 700 ml (over an operative time of 4 hours), and the infused fluids included 450 ml of Hartman's solution (over the first 2 hours), 950 ml of 0.9% normal saline, and 500 ml of Gelofusine 4% (over the second 2 hours) in addition to 1 litre of glucose 10% with insulin. No blood transfusion was required and no MAP <70 mmHg was recorded. |
pmc-6077606-1 | We present a case involving a 40-year-old female who was found to have a McSwain Type 5 inverted appendix on a computed tomography (CT) urogram for hematuria and flank pain. A review of her past imaging included a computed tomography (CT) aorta and abdomen/pelvis which also revealed this anatomic variant and appeared unchanged over the span of approximately nine months (Figures and ). Laboratory values to include white blood cell, red blood cell count, and lactate levels revealed no significant abnormality. The only laboratory derangement involved elevated transaminases, which were felt to be unrelated to her acute presenting symptoms. The patient did not report any history of a prior appendectomy. Her only relevant surgical history involved thrombolysis of the right common iliac artery and subsequent stenting of the left internal iliac vein due to compressive symptoms associated with May-Thurner syndrome. The patient did not undergo surgery and her initial presenting symptoms were felt to be unrelated to this imaging finding. |
pmc-6077606-2 | A 35-year-old male presented to the emergency department with nonspecific abdominal pain. The patient was afebrile with normal laboratory values to include normal lactate. The patient did not have any history of malignancy or other chronic medical conditions. Contrast enhanced computed tomography was performed, which demonstrated a fluid-filled polypoid mass within the base of the cecum (). No other concerning imaging findings were reported. A colonoscopy was performed which revealed a bulge in the cecum. No abnormal mass or inflammatory signs were observed. Findings were favored to represent an invaginated appendix, or cecoappendiceal intussusception, rather than a colonic mass and a biopsy was deferred. The patient was treated with bowel rest and antibiotics. She was discharged from the hospital after a few days with followup scheduled with gastroenterology. |
pmc-6077649-1 | A 50-years-old African female with medical history of hypertension, Diabetes Mellitus Type-2, and Major Depression Disorders presented with intractable vomiting and altered sensorium. About eight–ten hours prior to presentation, patient started to experience multiple episodes of nonbloody & nonbilious vomiting along with nausea leading to fatigue and altered sensorium requiring to be transported to hospital. Prior to initiation of the symptoms, she had suppressed appetite and skipped her dosage of Metformin 500 mg because of decreased oral intake and emesis. On presentation, patient was obtunded, responsive to pain, and poorly receptive to verbal stimuli. She had blood pressure of 123/81 mm Hg, respiratory rate of 25 breaths per minute, heart rate of 124 beats/minute, pulse oximetry of 97% on ambient air, and temperature of 97.6 Fahrenheit. On physical exam, she had mild distress, tachycardia, tenderness around epigastric area on deep palpation, and dehydration with poor skin turgor.
Due to state of presentation, computed tomography (CT) scan of the head showed no intracranial pathologies or cerebral edema presence. Venous blood gas showed pH 7.39, pCO2 31 mm Hg, pO2 52 mm Hg, HCO3 18.8, sodium 148 mmol/L, potassium 3.5 mmol/L, glucose 750 mg/dl, and lactate 2.9 mmol/L. Initial biochemistry analysis showed serum sodium 144 meq/L, potassium 4.8 meq/L, chloride 98 meq/L, bicarbonate 14 meq/L, albumin 4.2 g/L, and serum glucose 979 mg/dl. Corrected sodium was calculated to be 158 meq/L, anion gap 32, delta gap: 2, and serum osmolality 363 mOsm/kg. Ketone bodies were strongly positive in the blood and urine. shows additional biochemical values appropriate to the time interval.
Patient had received initial fluid resuscitation and, later, she was admitted to ICU requiring administration of normal saline, initiation of intravenous insulin infusion, and electrolytes repletion. Serum glucose levels were appropriately improving with goal of 50-70 mg/dl per hours, though serum sodium continued initially to peak before the values started to decrease. Patient started to be alert, awake, and responsive to commands with tolerating oral diet and improvement from admission assessment. Serum sodium levels were gradually controlled within normal range over 72 hours within admission. Patient was eventually transferred to medical floor for optimization of diabetic medication and education prior to discharge without any further events. |
pmc-6077655-1 | A 12-year-old male with a history of chronic myeloid leukemia presented to our dermatology clinic with new-onset hypopigmented patches that are slowly progressive and of varying sizes of six months' duration on his upper limbs, upper chest, and both knees (). Also, two depigmented macules were noted on his upper chest and lower abdomen. The patient denied any rashes or other skin changes and also denied any changes in hair, nail, and mucous membranes. Furthermore, Wood's light examination was negative. The patient was switched to dasatinib, at a dose of 70 mg once per day since two years, due to intolerance to imatinib. There was no personal or family history of autoimmune diseases or pigmentary disorders like vitiligo. The patient denied any use of topical medications or bleaching agents. A 3 mm punch biopsy from active hypopigmented lesion on the abdomen was performed. Histopathologically, it showed decrease melanocytes and basal layer melanin pigmentation. In immunohistochemistry, Melan A stain revealed decreased melanocyte. All positive and negative controls are examined and show appropriate reactivity. The patient was treated with close observation and reassurance. Through it all, the above clinical clues led to a diagnosis of skin depigmentation during dasatinib treatment. |
pmc-6077660-1 | A 44-year-old female was found to have an incidental FDG-avid right thyroid lesion following staging PET for colorectal carcinoma. She was asymptomatic from the thyroid lesion and biochemically euthyroid. There was no personal or family history of thyroid disease and no prior history of radiation exposure to the head and neck region. CT scan of the neck confirmed a 40mm thyroid nodule, and ultrasound guided fine needle aspiration of this nodule was suggestive of a follicular neoplasm.
A right hemithyroidectomy was performed. The gross specimen weighed 67 grams and the cut surface revealed a round solid well-circumscribe tan nodule, with scant compressed residual thyroid parenchyma at the superior pole. The entire specimen was submitted for examination. Sections showed the nodule to be entirely encapsulated by a thick fibrous band without capsular or vascular invasion. The tumour showed predominantly areas in keeping with usual follicular adenoma formed by microfollicles with scant colloid and lined by cells with bland round to ovoid nuclei ().
Present centrally and entirely within the encapsulated and conventional adenoma was an 11 mm focus showing distinct insular growth pattern with atypical cell morphology. The cells contained round to ovoid nuclei with irregular nuclear membranes, small nucleoli, and high nuclear to cytoplasmic ratio. The mitotic count was very high (8/10 high power fields), although tumour necrosis was absent, meeting criteria for PDTC (). In addition there were several regions, one of which was adjacent to the PDTC-like area, showing formation of trabeculae and high nuclear cytoplasmic ratio, without sufficient nuclear morphology or mitotic count for PDTC criteria ().
The PDTC-like focus and adjacent trabeculae region did show noticeably higher proliferation rate by MIB1 immunohistochemical (IHC) staining (). IHC staining showed retained expression of TTF1 (), with loss of thyroglobulin (which was retained in background follicular adenoma component and reduced expression in the adjacent trabeculae area) (). There was no expression of calcitonin, synaptophysin, chromogranin, or BRAFVE1 IHC staining. No nuclear features to suggest papillary thyroid carcinoma were present. Three lymph nodes excised with the thyroidectomy were negative for malignancy.
The background thyroid parenchyma showed patchy lymphocytic aggregates suggestive of lymphocytic thyroiditis. After multidisciplinary discussion, the patient proceeded to have a left completion thyroidectomy which was negative for adenoma or malignancy and also showed features of lymphocytic thyroiditis. Follow-up 12 months after resection shows patient is alive and well. |
pmc-6077661-1 | A 12.3-year-old male patient was referred to our pediatric endocrinology clinic for evaluation of short stature. He has been using levothyroxine (LT4) for hypothyroidism for more than 2 years. In medical history, he was born at term weighing 3500 g with uneventful gestation and delivery. His parents were first degree cousins. The height of the mother and the father was 165.5 and 172 cm, respectively. He had three sisters and one brother. His brother and one of the elder sisters were healthy and 175 cm and 165 cm tall, respectively. On physical examination, height was 129 cm (SDS: −3.2) and weight was 28 kg (body mass index, BMI: 16.8, −1.0 SDS). Target height was 175.2 cm (SDS: −0.2). Testicular volume was 2 ml bilaterally with a 3 cm penile length. Bone age was 9 years. Laboratory findings revealed that free thyroxine (FT4) is 1.2 ng/dl (N: 0.61–1.57), thyroid stimulating hormone (TSH) is 0.01 μIU/ml (N: 0.37–5), thyroid autoantibodies were negative, prolactin (PRL) is 4.5 ng/ml (N: 2.6–13.1), adrenocorticotropic hormone (ACTH) is 21.3 pg/ml (N: 4.7–48.8), cortisol is 6.8 μg/dl (N: 6.7–22.6), and insulin-like growth factor 1 (IGF-1) is 12.8 ng/ml (N: 85.2–248.8). Thyroid ultrasonography revealed a hypoplasic thyroid gland (1.7 ml) with normal parenchyma. On pituitary magnetic resonance (MRI), partial empty sella was detected with normal bright spot (pituitary height was 2.8 mm). Clonidine and L.DOPA stimulated peak serum growth hormone (GH) levels were 2.1 ng/ml and 1.9 ng/ml, respectively. With these results diagnosis of GH deficiency was confirmed, and recombinant growth hormone (rGH) was initiated. On follow-up, low dose (1 μg) ACTH stimulation test was performed, and adrenal deficiency was confirmed (peak cortisol: 12.1 μg/dl). Then, oral hydrocortisone replacement therapy was initiated (10 mg/m2/day).
At 14.3 years, he was still prepubertal with testicular volume of 3 ml bilaterally. Basal level of testosterone was <0.01 ng/ml. Then, LHRH stimulation test was performed, and central hypogonadism was confirmed (peak luteinizing hormone, LH; 0.62 mIU/ml, and peak follicle stimulating hormone, FSH; 0.85 mIU/ml). Intramuscular depot form of testosterone was initiated, 50 mg/monthly. |
pmc-6077878-1 | A 69-year-old African American male with a formidable medical history of paroxysmal atrial fibrillation (on amiodarone and warfarin), end-stage renal disease status post deceased-donor kidney transplant 2 months ago (on immunosuppressive therapy with mycophenolate, prednisone, and tacrolimus), hypertension, transient ischemic attack, right carotid artery stenosis status post carotid artery stent, and hyperlipidemia presented to our outpatient clinic for atypical left chest pain for 2 weeks. Pain was nonexertional, nonpositional, nonradiating, intermittent, and moderate in severity. In his last office visit after the kidney transplant, he was evaluated for light-headedness. He was found to be orthostatic hypotensive; therefore, blood pressure medications were adjusted that improved his dizziness. He endorsed good exercise tolerance. He self-medicated himself with antireflux medications, which helped his chest pain. The patient denied palpitations, shortness of breath, syncope, fever, chills, or headache. On admission, his vital signs indicated a regular pulse rate of 90 beats per minute and blood pressure of 110/70 mm Hg. The physical examination was unremarkable. |
pmc-6078126-1 | A 55-year-old woman reported a 24-hour history of unusual pain in her left carotid area irradiating to the ear. Colour Doppler ultrasound revealed an eccentric hypoechoic thickening (black arrows on Figure ) of the proximal bulbar internal carotid but also partially of the carotid bifurcation itself. A thin hyperechoic atheromatous fibrous plaque was also visible (small white arrows on Figure ) but no significant stenosis was found. Contrast-enhanced ultrasound showed normal capillary distribution of micro bubbles in the hypo echoic thickening, therefore excluding haematoma (Figure , black arrows). The avascular fibrous plaque was well demonstrated (small black arrows). Unenhanced Axial T1-weighted Magnetic Resonance (MR) imaging showed an hypo intense tissue (Figure , black arrows) around the proximal internal carotid. Intense enhancement of this tissue was shown on fat-saturated contrast enhanced T1-weighted images (Figure ). Carotidynia or TIPIC syndrome was diagnosed and the woman was immediately treated with non-steroid anti-inflammatory drugs. Doppler ultrasound performed after 14 days already showed rapid regression of both the symptoms and the perivascular inflammatory sheath (black arrows on Figure ). |
pmc-6078366-1 | A 26-year-old male, with a history of long-standing heart failure had multiple
hospital admissions in the past year despite optimal medical management. The
diagnosis of end-stage heart failure due to Chagas cardiomyopathy was confirmed by
serology a while ago, and an implantable cardioverter defibrillator was used for
sudden death secondary prevention. Echocardiography revealed a severely dilated left
ventricle (end-diastolic diameter of 72 millimeters), with severely depressed
function (ejection fraction of 18%) and 4+ mitral regurgitation. The right ventricle
also exhibit severe dysfunction with 3+ tricuspid regurgitation, tricuspid annular
plane systolic excursion of 15, and right ventricular systolic pressure of 65 mmHg.
The patient has been followed up in a different city of ours by another cardiology
team. At this point, he has never been considered for heart transplantation.
Nonetheless, the patient was admitted in the emergency room with cardiogenic shock,
in Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS)
level 2. He was initially managed with the use of two inotropes, intra-aortic
balloon pump and hemodialysis. No temporary or durable mechanical assist devices
were available at this hospital.
A right heart catheterization revealed low cardiac output (cardiac index of 0.9
L/min/m2, with systolic pulmonary pressure of 70 mmHg, transpulmonary
gradient of 16 mmHg and pulmonary vascular resistance of 6 Wood units. Filling
pressures were elevated (central venous pressure and pulmonary wedge pressure of 30
mmHg).
The patient was transferred to our hospital for heart transplantation assessment. At
admission, he had sudden hemodynamic instability that deteriorated into cardiac
arrest. Cardiopulmonary resuscitation measures were effective, but circulation was
maintained with escalating doses of vasopressors. A percutaneous venous arterial
extracorporeal life support (ECLS) (Maquet GetingeTM, Germany) through
the femoral vessels was inserted as a bridge to decision strategy. Hemodynamics
stabilized, vasopressors were discontinued, tissue perfusion indices normalized, and
the patient neurologic status was intact. He was extubated on the next day, renal
function normalized, an aggressive diuresis allowed a twelve-liter negative fluid
balance in the following five days ().
Eighteen days after ECLS initiation, the patient was submitted to an axial flow left
ventricular assist device (HeartMate II, Abbott LaboratoriesTM, Chicago,
IL) implantation with ECMO explant under median sternotomy with cardiopulmonary
bypass.
Postoperatively (), the patient had
mediastinal bleeding requiring surgical revision; coagulopathy and pericarditis. A
transient right ventricular dysfunction required a five-day administration of
intravenous inotropic support, aggressive diuresis and oral pulmonary vasodilators.
He was eventually discharged home on postoperative day 35 in fair condition,
requiring rehabilitation due to malnutrition and muscular weakness.
Sixteen months later, he is in functional class I with unremarkable recovery except
for a single episode of hemolysis that was treated with intravenous heparin. Pump
has functioned well with no evidence of failure or thrombosis. Late right heart
failure was not an issue, and his exercise performance is excellent.
Echocardiography revealed mild tricuspid regurgitation and right ventricular
systolic pressure of 30 mmHg. At this point, the patient does not manifest interest
in being transplanted. |
pmc-6078644-1 | A 65-year-old female came to our hospital in 2011 with history of intermittent pruritus in the perianal region for 1 year. The patient had neither genitourinary nor gastrointestinal symptoms, such as rectal bleeding, change in the bowel function, hematuria, dysuria, urinary frequency, or weight loss. Her family history was negative for skin, colorectal, or genitourinary cancer.
Local examination revealed a whitish gray skin lesion in the left perianal area with a 3 × 3 cm size. The surrounding skin was lichenification (Fig. ). Rectal examination was normal apart from mixed hemorrhoid. No enlarged inguinal lymph nodes were detected.
The perianal skin biopsy was consistent with EMPD. To exclude underlying malignancy, the patient was advised to undergo a screening colonoscopy, gynecological ultrasonography, and whole-body computed tomography (CT), which revealed unremarkable results. All laboratory examinations, including carcinoembrionic antigen, were normal.
Informed consent was obtained from the patient for publication of this case report and accompanying images. After signing the informed consent, the patient was placed in a jack-knife position on the operation table after anesthesia. The operation consisted of a wide excision with frozen section control of the margins and a flap reconstruction. The perianal diseased skin and anal mucosa up to dentate line were integrally excised, preserving the external and internal sphincters. The size of the defect was 7 × 6 cm (Fig. ). For the skin and the soft tissue defects, posterior thigh flap transposition was performed (Figs. and ). Then the dentate line was repaired and reinforced. A 28-Fr rectal tube was inserted into the anal canal. The total operation time was 296 minutes, and the estimated blood loss was 120 mL. The patient recovered without any complications and discharged home on the sixth postoperative day. After 6 years of follow-up, which included physical examination, ultrasonography, colonoscopy, CT scan, and random biopsies of the perianal skin, there was no evidence of recurrence and no influence in the anal bowel control function. |
pmc-6078649-1 | A 30-year-old, asymptomatic female presented to our hospital for a physical examination. The patient had a history of diabetes mellitus and no history of cigarette smoking, hepatitis, tuberculosis, hypertensive disease, or coronary disease. Written informed consent was obtained from the patient for the publication of the present study. Enhanced CT (Philips, Brilliance ICT CP 200063) of the chest revealed an anterior mediastinal oval tumor 2.3 × 1.7 × 1.3 cm in size with border regularity and without necrosis and calcification (Fig. ). A preoperative diagnosis of thymoma was considered due to the enhanced CT features. Routine blood, coagulation function, liver function, serum electrolyte, and electrocardiogram results were all within normal limits (Table ). For the purpose of providing a definitive diagnosis and treatment for an anterior mediastinal tumor such as thymoma, video-assisted thoracoscopic surgery (VATS) was performed under general anesthesia. Histopathological examinations using hematoxylin and eosin staining (Sinopharm Chemical Reagent Co., Ltd., Shanghai, China) revealed that the tumor exhibited the typical histological findings of a cavernous hemangioma, as it was comprised of a proliferation of sized vessels (Fig. ). The patient presented with chylothorax on the second postoperative day and was discharged on the 13th postoperative day. One-year post surgery, the patient was alive with no evidence of tumor recurrence. |
pmc-6078662-1 | A 53-year-old female is reported, having been admitted in our Department of Gynecology for postcoital vaginal bleeding and diagnosed with cervical adenocarcinoma based on the pathological biopsy, with an International Federation of Gynecology and Obstetrics stage of IIB. She also has had the diabetes and hypertension for more than 2 years. In addition, her blood glucose and pressure were within the normal limits under the treatment of insulin, metformin, and irbesartan. The patient had received the laparoscopic radical hysterectomy after 4 courses of chemotherapy and 25 courses of radiotherapy (GTV DT54Gy/25f/5w, CTV DT46Gy/25f/5w, PTV DT46Gy/25f/5w). After the surgery, 2 courses of chemotherapy had been given. Two years later (in 2016), the patient developed an abnormal vaginal bleeding, with the biopsy of vaginal cuff finding adenocarcinoma tissue. The magnetic resonance imaging and Positron Emission Tomography-Computed Tomography (PET-CT) showed a locoregional recurrence of 3 × 4 cm in vaginal cuff, which invaded the urinary bladder and the rectum. Unfortunately, the patient was so upset, making her last will in her room.
After a discussion of multidisciplinary treatment, a robotic-assisted laparoscopic total pelvic exenteration (RALTPE) was performed with the DaVinci SI system (Intuitive Surgical Inc, Sunnyvale, CA) on November 15, 2016, by a combined approach with gynecology, urology, and gastrointestinal surgery.
First and foremost, a carbon dioxide pneumoperitoneum of 12 mm Hg was established and the robotic system was docked. Five ports (3 for 12 mm and 2 for 8 mm) were applied for the camera and other instruments. After the first separation of the adhesive tissues in pelvic cavity and the isolation of the vessels and the ureters on both sides, the paravesical and pararectal spaces were developed in a retroperitoneal method. Next, the vessels were clipped from the roots, and rectal resection and radical cystectomy were performed. Then, the incision of all the organs and fascias along basin sidewall was performed. Thus, the en bloc dissection was completed.
Afterward, an anastomosis (by a stapler of Johnson No. 29) was made between residual rectum and sigmoid colon, which was 3 cm above the anal verge. A 20 cm part of ileum with vascular pedicle was mobilized at 25 cm away from the ileocecal region, and a side-to-side ileoileostomy was performed to recover the digestive continuity. The proximal part of the isolated ileum was sutured to form a neobladder. The 2 ureters were then implanted in the ileal neobladder with 4-0 Vicryl, and they were supported by 2 ureteral stents.
Finally, the distal part of neobladder was exteriorized as an output duct by 2-0 Vicryl using the ileostomy at right lower abdomen, with an F16 drainage tube indwelt in the neobladder. One abdominal drainage tube and one pelvic drainage tube were placed. In addition, an anal canal was indwelt.
The whole operation continued for 700 minutes, with an estimated blood loss (EBL) of 300 mL. Before returning to the department of gynecology, the patient stayed in Intensive Care Unit for 12 hours. The pathological results confirmed the recurrence with malignant cells that invaded the bladder and the rectum. In addition, the margins were negative. On the eighth day, a rectosigmoid anastomotic fistula was discovered, and a double-barrel Transversostomy was performed promptly. The patient was discharged on the 37th day. Three months later the 2 ureteral stents were removed without dysuria or other complications. After 17-month follow-up, the patient is alive and satisfied without any recurrence or distant metastasis. |
pmc-6078673-1 | A 29-year-old woman who had been bit by an insect on the left calf was admitted to our hospital with a chief complaint of continuous painful swelling of the bit area for 3 days. After scratched, the bite area became red and inflamed. The injury was not considered severe by the patient initially and the swelling of the calf was treated by self-medication with heat-clearing and detoxifying effects. The aggravating swelling and pain of the left calf impelled her to seek medical advice. After admission to our hospital, the patient developed septic shock symptoms characterized with diminished consciousness, pale skin, hypothermia, lack of urine output, and undetectable blood pressure. Laboratory studies revealed a white blood count of 13.8 × 109 cells/L, neutrophil count of 12.24 × 109 cells/L, and 88.7% polymorphonuclear neutrophils. She was admitted to the intensive care unit, receiving intravenous fluids and broad spectrum antibiotics treatments. Besides, she denied any history of diabetes mellitus, alcoholism, liver diseases, or trauma.
In the intensive care unit, the swelling increased and extended proximally to left knee and foot, complicated with blisters (Fig. A). Additionally, the patient developed cutaneous necrosis in the left ankle and popliteal space. Clinical examination showed her entire left calf was tensely swollen both medially and laterally, and the most obvious pain was localized to the bit area of the left calf. She had a loss of superficial touch sensation and 2-point discrimination over the entire sole of the left calf. She was unable to move her left leg actively, and any passive movements of the left calf, knee, and ankle joints caused severe pain. Palpation of the whole left leg revealed a mildly increased skin temperature and exquisite pain compared with her contralateral leg. The main differential diagnose was from deep vein thrombosis (DVT). Subsequent venous Doppler ultrasonography found no evidence of DVT, and only subcutaneous edema at the lower leg. On the basis of the postmedical history and clinical findings, in particular, increasing pain, loss of sensation, tense swelling, and severe pain to any stretch of the tissues, we diagnosed acute compartment syndrome affecting her left leg.
According to the diagnosis, the patient was then transferred to the operating room for surgical inspection of the tissues and decompression of the compartments by fasciotomy. A medial incision was made 2 cm posterior to the posteromedial border of the tibia. Extensive purulence was encountered in the subcutaneous fat and superficial fascia, which confirmed the diagnosis of infectious cellulitis. The purulence was collected for aerobic and anaerobic bacterial culture. After fascia was incised, the deep layer of gastrocnemius and partial soleus were found dark red, with widespread necrosis as well as drainage of thick and amber pus, which confirmed the diagnosis of infectious myositis. Next, the dissection proceeded to transverse intermuscular septum over flexor hallucis longus muscle, flexor digitorum longus muscle, and tibialis posterior muscle to release the deep posterior compartment. The necrotic muscle and the grossly infected soft tissue were thoroughly debrided without the injury of posterior tibial artery, veins, and tibial nerve. Then, the lateral incision was made 3 cm lateral to the crest of the tibia. Layer dissection through this incision to the periosteum revealed a little purulence but no necrotic muscle. The fascia over the anterior and lateral compartments was completely released. After inspection, decompression, and debridement, the areas were irrigated sequentially with 3% perhydrol, diluent iodophor, and normal saline. Eventually, the wounds were closed by vacuum sealing drainage to improve the wound circumstance and further reduce intracompartment pressures. She was treated with elevation of the left leg, empirical antibiotic therapy, and daily monitoring of peripheral blood circulation. Considering the severity of the left calf, we closely monitored the function of renal and prepared to amputate the infected limb in case of possible renal failure and death due to acute rhabdomyolysis.
On day 3 postoperation, the patient reported a remarkable pain relief in the area of the incisions. A reduction of the edema was also found. Cefoperazone–Sulbactam was treated intravenously twice a day on the basis of bacteria culture revealing heavy infection of Staphylococcus aureus and Escherichia coli. On day 10 postoperation, vacuum sealing drainage was removed, and the surgical site was still riddled with necrotic and purulent tissue (Fig. B). Additionally, the skin of former swelling popliteal fossa and foot were ulcerated and it was thought to arise from the spread of infection. To reduce the severity of deep wound infection, thorough debridement was performed on day 12. We explored and found purulence and necrosis bestrewed with segmental posterior muscular group of calf, especially the fascial spaces. Different from calf, wounds in foot and popliteal fossa just deepened to subcutaneous tissue. Then the necrotic, injected tissue, and inflammatory granulation were completely excised until healthy, and bleeding tissue was exposed. Eventually, the wounds, especially the deep muscle tissues, were irrigated repeatedly with 3% perhydrol, diluent iodophor, and normal saline, and were closed by vacuum sealing drainage. Scheduled redebridements were performed every 7 to 8 days until all necrotic and injection tissues were removed. When the wounds were well granulated, we applied an anterolateral thigh flap transplantation to close the medial and lateral calf wounds and a split-thickness skin gift to close the medial and lateral foot. After 6 weeks, medial and lateral calf wounds healed well (Fig. C).
When the wound healed completely, the patient underwent systematic rehabilitation for approximately 3 weeks, including rehabilitation assessment and treatment. The rehabilitation assessment was performed firstly. As shown in Table , the main problems were the ability barrier of daily life activities (standing, walking, transfer, lavatory, stairs, etc) and the movement dysfunction of the left knee and ankle (strength, mobility, etc). Besides, the sensory of the left lower extremity below the knee, the motor and sensory nerve conduction function of the left tibial nerve, and peroneal nerves were partly impaired. So the following rehabilitation programs were drew up: the low-power helium-neon laser to promote wound healing[; the ultrasound and audio pulse therapy to soften the scar and loosen the adhesion; the wax, arthrosis, and cold therapy to improve the motion of the joints. Also, the functional transcranial magnetic stimulation (10 Hz trains for 2 seconds; repeated 70 times with an inter-train interval of 4 seconds, a total of 1400 pulses and 7 minutes) was used to promote nerve function recovery. All the above treatments were performed daily. After 3 weeks, the patient underwent a second rehabilitation evaluation (Table ). All the scores of the evaluation index increased, albeit limited. Considering the high costs for the continued in-hospital rehabilitation, the patient chose to wear an orthopedic insole to achieve standing and then returned home. During the following 3 months after discharge, we conducted a telephone follow-up for the patient. After 1 month, she was able to walk independently, but slowly and instability. Moving up and down the stairs was limited. Three months later, the activities of daily living are markedly improved. She is able to walk, and moves up and down the stairs, independently. |
pmc-6078682-1 | The patient was a 39-year-old man and an illicit abuser of heroin and amphetamine. He injected 0.5 mL of 24% paraquat directly into his right cephalic vein due to hallucination. The patient was brought to our emergency department for management 4 hours after injection. He was fully conscious and had normal vital signs (pulse rate of 63 beats/min, respiratory rate of 16 breaths/min, and blood pressure of 112/69 mm Hg), except for mild hypothermia (body temperature of 35.8°C). Systemic review showed mild dyspnea, abdominal pain, and right wrist pain over the injection site. The only abnormal physical finding was the erythematous injection site and epigastric tenderness. Laboratory investigations, including a complete blood count, liver and renal function, and electrolytes initially yielded normal findings. Urinalysis yielded normal results, except the positive urine paraquat test (4+). The initial plasma paraquat concentration was 0.51 μg/mL. A chest radiograph was also showed normal findings. He was admitted to the intensive care unit and underwent one session of charcoal hemoperfusion therapy. A follow-up urine paraquat test performed 2 days later yielded negative results. He did not receive methylprednisolone or cyclophosphamide therapy. Acute kidney injury developed on the fourth day after intoxication, with the level of serum creatinine rising rapidly from 0.96 to 4.57 mg/dL and the daily urine output noticeably decreasing from > 2000 to 900 mL. We administered adequate fluid supplementation, keeping balance of urine output, and avoiding nephrotoxicity medication. The serum creatinine level improved gradually. Intermittent postprandial abdominal pain and constipation were found after paraquat poisoning. Otherwise, there was no dyspnea or other discomfort during hospitalization. The patient was discharged 13 days later in a stable condition. |
pmc-6078722-1 | A previously healthy 2 and a half years old girl was admitted to our hospital with a 3-day history of fever and vomiting, complicated by a sudden seizure of half a minute on the next day of admission. On admission, she had a temperature of 37.9°C, with neck resistance, but was negative of Kernig sign, Brudzinski sign, and Babinski sign. She was in a coma after seizure and had a Glasgow Coma Scale score of 5 (eyes 1, verbal 1, motor 3).
Laboratory blood testing showed leucocyte count 21,090 (4000–12,000) cells/μL, serum sodium 133 (135–145) mmol/L, and C-reactive protein 180 (0–8) mg/L. Serum interleukin (IL)-6 was 291.4 (1.7–16.6) pg/mL and IL-10 4.1 (2.6–4.9) pg/mL. Serum sodium fell to 118 (135–145) mmol/L on day 2 of hospitalization. Cerebrospinal fluid (CSF) examination revealed leukocytes 96 (0–10) cells/μL, with 60% mononuclear cells, protein 1.6 (<0.45) g/L, and glucose 6.27 (2.78–4.50) mmol/L. So, the primary diagnosis of this patient was bacteria meningitis and hyponatremia.
Cranial MRI was performed on day 3 of admission (6 days after her symptoms began) and showed right subdural effusion on T2-weighted image and a marked hyperintense lesion in the splenium of the corpus callosum (SCC) on T2-weight, fluid-attenuated inversion recovery (FLAIR) images, and diffusion-weighted images (DWIs) with a reduced apparent diffusion coefficient (ADC) mapping (Fig. ). According to the change of the cranial MRI, we made the supplementary diagnosis of bacteria meningitis with subdural effusion and MERS. On the fourth day of admission, listeria monocytogenes was detected in CSF cultures.
The patient received antibiotics treatment first with panipenem/vancomycin, which was then switched to ampicillin and vancomycin after listeria monocytogenes was detected. Also, intravenous mannitol and hypertonic fluid (3% sodium chloride) therapy were started. The patient clinical condition improved over the subsequent 7 days, with gradual resolution of her symptoms. She showed normal mental status after 7 days of hospitalization. Her Glasgow Coma Scale score reached 15 (eyes 4, verbal 5, motor 6). Serum sodium raised to 134 (135–145) mmol/L on day 4 of hospitalization. Re-examination of the cranial MRI on day 10 of hospitalization (13 days after his symptoms began) showed bilateral subdural effusion on T2-weighted image, but the splenial lesion had completely disappeared on T2 and FLAIR image (Fig. ). The patient performed well on 3 months postdischarge follow-up and the cranial MRI showed the absorption of subdural effusion (Fig. ). |
pmc-6078727-1 | In 2008, a 69-year-old man presented with increased white blood cell (WBC) (12.6 × 109 cells/L) during a physical examination, whereas the platelets (PLT) and hemoglobin (HB) values were normal. The patient had a fever, night sweats, and weight loss, without superficial lymphadenopathy and hepatosplenomegaly. The patient refused further diagnosis and treatment. In September 2011, the patient underwent a bone marrow puncture that showed 30.5% mature lymphocytes with 4.5% lymphoblasts. The flow cytometry suggested CLL with an abnormal B lymphocyte population accounting for 36.58% of non-erythroid cells and CD5++, CD19+, CD20+, CD23+, HLA-DR+, CD22-, CD38-, sIgMdim, and ZAP-70 expression for 87.6% of CLL cells. The patient declined treatment. In August 2012, routine laboratory results showed WBC 55.7 × 109 cells/L, lymphocyte (LY) 26.9 × 109 cells/L, PLT 69 × 109 cells/L, and HB 144 g/L. Another bone marrow puncture showed 60% mature lymphocytes with 7% lymphoblasts and with the same flow cytometry result; chromosomes: 46, XY; FISH: TP53 gene deletion. B-mode ultrasound examination found multiple enlarged lymph nodes (max 5.3 × 2.3 cm). We diagnosed the patient with CLL (Rai Staging IV). The patient was given chlorambucil (10 mg/m2 oral, twice daily from days 1 to 7), followed by 1 course of COP regimen consisting of cyclophosphamide (750 mg/m2 i.v. on day 1), vindesine (4 mg i.v. on day 1), and prednisone (60 mg/m2 i.v. daily from days 1 to 5), and 1 course of (fludarabine, mitoxantrone, dexaméthasone) FMD regimen consisting of fludarabine (25 mg/m2 i.v. daily from days 1 to 3), mitoxantrone (8 mg/m2 i.v. on day 1), and dexamethasone (20 mg/m2 i.v. daily from days 1 to 5). The WBC decreased to 22.1 × 109 cells/L, and LY decreased to 19.5 × 109 cells/L). Upon follow-up, the patient was in partial remission (PR). From July 2014 to July 2016, the patient underwent one course of FMD regimen consisting of fludarabine (25 mg/m2 i.v. daily from days 1 to 3), mitoxantrone (8 mg/m2 i.v. on day 1), and dexamethasone (20 mg/m2 i.v. daily from days 1 to 5); 2 courses of RFC regimen consisting of rituximab (375 mg/m2 i.v. on day 0), fludarabine (25 mg/m2 i.v. daily from days 1 to 3), and cyclophosphamide (250 mg/m2 i.v. daily from days 1 to 3); and 1 course of RFMD regimen consisting of rituximab (375 mg/m2 i.v. on day 0), fludarabine (25 mg/m2 i.v. daily from days 1 to 3), mitoxantrone (8 mg/m2 i.v. daily on day 1), and dexamethasone (20 mg/m2 i.v. daily from days 1 to 5).
In May 2017, a nasal endoscopy was performed and revealed a left nasal mass. The pathology suggested extranodal NK/T-cell lymphoma (nasal type, Fig. A; CD56-positive, Fig. B). The bone marrow biopsy and flow cytometry still confirmed the diagnosis of CLL (Fig. C; CD5-positive, Fig. D). Gene sequencing of the bone marrow specimen found the ASXL1 gene had a small segment of insertions/deletions, but no abnormalities were found in the nasal tissue specimen. On May 19th, the patient received 1 course of methotrexate, dexamethasone, ifosfamide, Mesna, etoposide, pegaspargase (SMILE) regimen consisting of methotrexate (2 g/m2 i.v. on day 1), dexamethasone (40 mg i.v.i.v. daily from days 1 to 4), ifosfamide (1500 mg/m2 i.v.i.v. daily from days 2 to 4), Mesna (300 mg/m2 i.v. three times a day from days 2 to 4), etoposide (70 mg/m2 i.v. three times a day from days 2 to 4), and pegaspargase (2500 U/m2 subcutaneously on day 2). Pneumonia and coagulopathy occurred after chemotherapy, and the patient died shortly thereafter. |
pmc-6078740-1 | A 66-year-old man was hospitalized in April 2017 for a weight loss of 7 kg and a nocturnal low-grade fever evolving since January 2017. The patient reported clinical tuberculosis in childhood. His medical history included arterial hypertension, dyslipidemia, coronary artery disease, and tobacco smoking. A bladder cancer diagnosed in 2015 had been treated with local resection and weekly intravesical instillation of BCG (BCG-MEDAC, strain RIVM 1173-P2, MEDAC, Lyon, France) for 6 weeks. In April 2017, a thoraco-abdomino-pelvic computerized tomography scan diagnosed pulmonary embolism, a sub-renal septic aneurysm and a collection in the right psoas muscle (Fig. A). A 2-deoxy-2-[fluorine-18]fluoro-D-glucose positron emission tomography combined with computed tomography (18FDG PET/CT) was subsequently performed and showed an intense hypermetabolism of the aortic aneurysm, with no other embolic foci (Fig. B). Physical examination found dyspnea and diffuse abdominal pain. Remarkable biological parameters included hemoglobin concentration of 10.8 g/dL (normal value, 13–16 g/dL), 0.7 G/L lymphocytes (normal value, 1–4 G/L), and a C-reactive protein of 60 mg/L (normal value, 0–5 mg/L). Surgical flattening of the aneurysm was immediately performed which showed rupture on the right flank of the aorta, explaining the psoas hematoma. Postoperative probabilistic therapy included 4 g tazocillin 3 times a day and 1 bolus of 320 mg gentamicin. Routine bacteriological investigations of an aneurysm specimen collected during surgery remained negative. Pathological examination yielded chronic granulomatous inflammation of the vascular wall leading to a differential diagnosis of BCG aneurysm. While routine microscopic examination after Ziehl-Neelsen staining (Kit Quick-TB, RAL DIAGNOSTICS, Martillac, France) remained inconclusive, the surgical specimen was examined by using fluorescence in situ hybridization specifically targeting the M tuberculosis complex rpoB gene. Briefly, the fresh specimen was cut and an imprint slide was prepared by dabbing the cut surface of the biopsy against a clean glass slide after 3 washes with Dulbecco's phosphate-buffered saline (DPBS) (Thermo Fisher Scientific, Illkirch, France), then heat-fixed at 90 °C for 5 minutes and flooded with 70% ethanol for 10 minutes. The slide was covered with 10 mg/mL lysozyme (37 °C for 30 minutes), then with 5 μg/mL proteinase K (37 °C for 5 minutes) and finally with 10 μL of solution containing the rpoBMTC probe. After hybridization and appropriate washings, the slide was stained with Ziehl-Neelsen staining in a dark room and observed under 100 times magnification using a red filter and a fluorescent microscope. The overall procedure took 20 hours. Combining rpoBMTC-FISH and Ziehl-Neelsen-staining yielded specific detection of tuberculous mycobacteria as red fluorescent Ziehl-Neelsen-positive bacteria (Fig. ). Identification at the species level was confirmed by multiplex polymerase chain reaction (PCR) amplification of regions of differences (RD) RD1, RD4, RD9, and RD12 as previously described[; and by PCR-sequencing of the rpoB gene which detected M bovis BCG with 99% sequence similarity with the reference species (GenBank AM408590.1).[ Culture for mycobacteria remained sterile. Tazocillin was discontinued and an anti-tuberculous treatment including ethambutol 500 mg 3 times a day plus an oral combination of isoniazid and rifampicin was continued for 2 months then relayed by an oral combination of rifampicin and isoniazid for 8 months. A CT scan performed at 6-month follow-up showed the absence of relapse of the aortic collection while the 18FDG PET/CT showed a reduction of the size and of the metabolism of the aortic aneurysm (Fig. C). A 9-month clinical follow-up indicated a favorable clinical and biological evolution. |
pmc-6078753-1 | A 43-year-old man was transferred to the emergency department from local community hospital because of accidental chlorine inhalation and rapidly progressive dyspnea. Six hours before, a severe chlorine gas leak occurred at a metal recycling facility. The patient tried to control the site and thus stayed in the workshop for nearly 30 minutes without effective protection. He complained of tearing eyes, throat burning, nausea, and especially dyspnea in the initial hours. His symptoms were significantly worse than before though he was ventilated via a mask with 100% oxygen. He was a heavy smoker and had no history of cardiac disease.
On examination he was mildly hypotensive, and had respiratory distress, and light yellowish, frothy nasal, and oral discharge. The arterial blood gas revealed: SaO2 60%, PaO2 36 mm Hg, PaCO2 43 mm Hg, pH 7.25, BE −8 mmol/L. The chest x-ray (CXR) showed bilateral infiltrative opacities (Fig. A), which were interpreted as interstitial and alveolar pulmonary edema. ARDS due to chlorine gas exposure was diagnosed.[ He was promptly intubated and ventilated with a lung-protective strategy. Other treatments included infusion of dopamine to increase mean arterial blood, intravenous methylprednisolone 1000 mg and ulinastatin to inhibit pulmonary inflammatory response. However, there was no significant improvement in the overall clinical condition. High doses of vasoactive drugs were required to maintain blood pressure at 100/50 mm Hg. A repeat chest x-ray revealed worsening interstitial infiltrates (Fig. B). His HR decreased to 30 bpm when a tracheotomy was performed on day 3.
Subsequently, four sessions of high-volume hemofiltration (HVHF) at 65 mL/kg/h was started. Vascular access was obtained by cannulation of the right femoral vein using a double-lumen catheter (Hemo-Access, Gambro, Hechingen, Germany). Blood was pumped from the outflow lumen by the roller pump of a blood flow/air trap module (AK 10; Gambro, Lund, Sweden). It was delivered to a polyacrylonitrile AN69 filter (Filtral; Hospal, Germany) 1.60 m2 in membrane surface. The replacement fluids were administered at 6000 mL/h by a predilution method. The circuit was anticoagulated by fraxiparine (Sanofi Synthelabo, Hangzhou, China) at an initial 3000 U loading dose and a 400 U/h maintenance dose. The daily fluid balance was kept at a range of 500 to 1000 mL negative.
After the first session of HVHF, the PaO2 improved from 53 to 71 mm Hg and the PaO2/FiO2 ratio increased from 59 to 102 mm Hg. His PaO2 continued to increase to 148 mm Hg, with gradually reducing support levels of mechanical ventilation and doses of vasopressors in the following session. CXR showed resolving pulmonary infiltrates (Fig. C). During the fourth session of HVHF (day 6 after admission), a PaO2 of 174 mm Hg, which corresponded to a PaO2/FiO2 ratio of 316 mm Hg, was observed. His renal function reports before and after HVHF were normal. On day 11, the patient developed ventilator-associated pneumonia caused by multidrug-resistant acinetobacter baumannii and was treated with cefoperazone and sulbactam. Weaning from ventilation was achieved on day 17. He was discharged on day 21 and 28 from the intensive care unit (ICU) and hospital respectively.
The patient complained of manifested persistent cough that lasted for 4.5 months after discharge, and the pulmonary function tests revealed severe mixed ventilatory defect. He was managed with inhaled steroids and bronchodilatadors. The patients were asymptomatic and the pulmonary function was improved two years later. |
pmc-6079180-1 | A 53-year-old man was referred to the Prosthodontics Department of Tehran University of Medical Sciences, one and a half years after surgical resection and radiotherapy of an adenoid cystic carcinoma (ACC) in the right side of the maxillary arch by a dose of 45 Grays (Gy). The patient was completely edentulous and dissatisfied with the retention and function (nasal reflux) of his existing maxillary obturator opposing a mandibular denture.
The patient requested implant-supported maxillary and mandibular prostheses. The most suitable sites for implant placement were determined with the aid of cone-beam computed tomography (CBCT), and the patient’s existing dentures were duplicated for fabricating radiographic stents. Three dental implants (Implantium®, Dentium, Seoul, South Korea) with the diameter of 3.5 mm and the length of 10 mm in the maxilla and 12 mm in the mandible were inserted in the jaws without any bone augmentation after converting radiographic templates into surgical ones (). The existing dentures were then relined by using a soft liner (Mollosil®, Detax Dental GmbH & Co. KG, Ettlingen, Germany) to relieve the pressure on the implants and to create a better fit with the underlying tissues during the osseointegration period. Six months later, during the second surgery, the most distal implant of the upper arch was removed due to the lack of osseointegration.
Two weeks later, another implant was placed instead of the failed implant but at a slightly more distal site. After another three months, the last implant was uncovered, and a healing abutment was secured. The presence of an acceptable osseointegration was confirmed clinically by torque test (OsstellTM, Mentor, Integration Diagnostics AB, Sävedalen, Sweden) and x-ray radiography. Two weeks later, primary impressions were made by using an irreversible hydrocolloid impression material (Alginoplast, Heraeus Kulzer GmbH & Co., Wehrheim, Germany) and prefabricated trays (Dandal, Taksan, Tehran, Iran). Final impressions were made by using splinted square impression copings with custom trays [] and regular body polyvinyl siloxane (PVS; Panasil® monophase Medium, Kettenbach GmbH & Co. KG, Schoenberg, Germany) for the maxilla and with a combination of zinc oxide eugenol (ZnOE, Cavex, Holland and IRM, Dentsply, USA) and regular body PVS for the mandible (). After determining the vertical dimension of the occlusion, the space analysis of the upper and lower arches indicated the necessity of choosing individual stud attachments with limited height requirements []. At this time, the patient declared pain during mastication; however, there were no other clinical or radiographic signs other than pain upon percussion.
After consulting with the surgeon, the treatment was continued by considering the possible failure of the suspicious implant. To compensate for the minimal implant divergence and to create a definite path of insertion for the obturator that would accommodate both attachments and the extension of the prosthesis into the defect (bulb), 15° angled abutments (Kerator, Daekwang IDM Co., Seoul, South Korea) were selected. Straight attachments (Positioner, Implantium®, Dentium, Seoul, South Korea) were used for the mandibular overdenture. While the wax-up was being prepared (two weeks after the initial symptom), the patient declared constant pain. Upon opening the healing abutment for further examination, the implant was removed. The patient was unwilling to receive any further surgical procedure, and implant replacement was not followed due to the failure risk []. Therefore, the treatment was continued with the two remaining implants in the maxillary arch, and the frameworks were fabricated.
Before processing the prostheses, the next visit was managed for another try-in with the frameworks placed in record bases. After confirming all the parameters in the try-in session (the vertical dimension of the occlusion, esthetics, phonetics, and the centric relation), the prostheses were processed (). At the delivery visit, the abutments were secured in the mouth with 30-newton centimeter (N/cm) torque according to the manufacturer’s recommendation (), and the prostheses were checked and adjusted. The patient received oral hygiene instructions and a recommendation to wear the obturator at night for managing mucosal and salivary secretions [,]. Subsequently, a panoramic radiograph was taken as a baseline for future evaluations. Some adjustments were needed during follow-up visits. After two years, regular six-month follow-ups showed acceptable conditions of the implants and the prostheses. Long-term observations will be used to ensure the patient’s oral health and the competence of the prostheses. |
pmc-6079185-1 | A 10-year-and-8-month-old male patient who presented emergently with a history of falling while playing soccer in a playground was admitted to the Department of Pediatric Dentistry, School of Dentistry, Isfahan University of Medical Sciences. His medical history was unremarkable.
Intraoral examination revealed a complicated crown-root fracture and an uncomplicated crown fracture of the maxillary left and right central incisors, respectively (). The teeth were slightly tender on percussion with no associated mobility and had normal response to vitality tests. Radiographic examination revealed an oblique fracture line in the maxillary left central incisor, ending at the cervical third of the root; the root was fully developed (), and had no periapical pathosis or displacement. After obtaining an informed consent, an emergency treatment was undertaken to stabilize the coronal fragment by splinting it to the adjacent teeth using acid-etch/resin and sealing the fracture line with flowable composite resin (Grandio Flow; Voco, Cuxhaven, Germany). At the second visit, pulpectomy with a working length of 27 mm was performed, and calcium hydroxide (Ultracal XS; Ultradent, South Jordan, UT) paste was placed as an intracanal medicament, with the access cavity being sealed until definite treatment. During the third visit, the root canal was filled, followed by temporary restoration of the tooth with glass-ionomer restorative material (Fuji IX; GC Corporation, Tokyo, Japan). After root canal therapy of the maxillary left permanent central incisor, the fractured part was separated to assess the fracture line, which revealed that it was extended subgingivally for about 2.5 mm distally (). To expose the fracture margins supragingivally, it was decided to extrude the fractured tooth via an orthodontic procedure. After oral prophylaxis, brackets were bonded to the upper teeth on a straight line from the primary right canine tooth to the left first permanent premolar tooth except for the tooth which had to be extruded. Subsequently, 0.014-inch Ni-Ti flexible wire was used for 2 weeks (). Leveling of the maxillary incisors of the patient continued by reactivation with 0.016-inch SS wire and the result of short-term fixed orthodontic treatment (4 mm extrusion in 5 weeks) is shown in .
To avoid relapse, a circumferential supracrestal fiberotomy, extending below the level of marginal bone, was performed prior to the retention period. After the retention period which lasted for 4 weeks, debonding was performed. The root canal was prepared for intracanal post placement by post drills; then, dual-cure self-adhesive resin cement (Clearfil™ SA Cement; Kuraray Noritake Dental, Tokyo, Japan) was placed in canal by a Lentulo drill. At that time, a fiber post (RelyX™ Fiber Post; 3M ESPE, St. Paul, MN, USA) was placed and excess cement was removed before light-curing. Then, the tooth was restored completely with composite resin () with both enamel- (Amaris® Enamel shades Translucent TN; Voco, Cuxhaven, Germany) and dentin-like materials (Amaris® Base shades Opaque O1; Voco, Cuxhaven, Germany) using the incremental technique. A custom-made mouth guard was fabricated for patient to prevent further trauma. In routine follow-up appointments after 3 and 12 months, clinical and radiographic examinations showed healthy tissues and teeth, and no evidence of apical periodontitis was seen (). |
pmc-6079216-1 | The patient is a 30 year-old right-handed male with bilateral malformations of cortical development (MCD) in right frontal and bilateral inferior temporal periventricular nodules causing medically refractory localization-related epilepsy. He also suffered psychiatric comorbidities of anxiety, panic disorder, and major depressive disorder (MDD). The semiology of his focal unaware seizures were not well lateralized on scalp EEG and are characterized by loss of contact, bizarre behavior, non-sensical speech, or strange vocalizations, lasting 30 s to few minutes, with few minutes of post-ictal lethargy. His seizures began at the age of 16 and he rarely has secondary generalization with his seizures. He occasionally experiences episodes of slowed thinking, dizziness, and heart-racing but this was not reliable for electrographic seizure during scalp EEG recordings and may or may not be a true aura.
Pre-operatively, he had daily seizures with a frequency of 3–6 seizures per day, often occurring in clusters. He was started on topiramate and switched to levetiracetam, lamotrigine, clobazam, clonazepam, lacosamide, and vigabatrin (enrolled in a clinical trial). He underwent treatment with and failed a total of six AEDs prior to surgical consideration. Pre-operative scalp electroencephalography showed bilateral high-frequency seizure discharges, right greater than left in the posterior and temporal regions (max T3, T4). Video-EEG showed 3–12 s high voltage high frequency inter-ictal discharges during sleep, every 10–15 s. He had several recorded seizures with blank stares, no automatisms, lasting 10–31 s with diffuse high-frequency high-voltage poly-sharp rhythmic discharges some appearing to start on the right and some on the left, all localizing posteriorly, suggesting occipital lobe. Functional MRI showed left language dominance. FDG-PET showed decreased uptake in the right medial temporal lobe cortical areas adjacent to the nodules. Neuropsychological testing showed bilateral impairment with prominent difficulties in visuospatial reasoning and integration. In total his work-up was not well lateralizing but suggested involvement of the occipital, parietal, and temporal regions.
The patient underwent a stereotactic depth electrode placement with the robotic-assistance (ROSA, MedTech; Montpellier, France). Multiple depth electrodes were placed in the bilateral parietal, temporal, occipital, and frontal lobes with several electrodes targeting the periventricular nodules and MCD. Stereotactic EEG showed broadly distributed inter-ictal discharges in bilateral posterior hemispheres, with all of the active interictal contacts located in the cortical structures near electrode entry rather than at the deeper tissue around the cortical malformations. A total of 19 seizures were captured with eight right sided onset, four left sided onset, and seven appearing bilateral. This included thirteen of his habitual seizures, seven originating from the right hemisphere and four originating from the left hemisphere as well as two in which laterality could not be determined. Similar to the inter-ictal findings, all seizure onsets were in the parietal-occipital-temporal junction on the cortical surface overlying the nodules. Interestingly, he had preserved awareness with right sided seizures and, however, had loss of contact in left sided seizures or broad propagation.
Surgical options including resection, laser ablation or RNS were considered. Surgical resection was not deemed a good option given the bilateral, broad posterior regions of onset with rapid spread. Similarly, laser ablation was not considered a good option. Ultimately there was consensus at the epilepsy surgery conference for centromedian (CM) thalamic RNS. An important factor in the decision was the broad connectivity to posterior association cortices as well as prior feasibility shown with centromedian DBS in patients suffering refractory epilepsy (discussed above). A depth electrode targeting the right CM thalamic nuclei was placed under MR image-guidance (ClearPoint, MRI Interventions; Irvine, CA, United States). The patient was then repositioned and using BrainLab stereotactic guidance (BrainLab; Munich, Germany), a craniectomy was performed near the right sided onset region. Two right parietal cortical strips were placed over the seizure onset zone for seizure detection as this was the most common site of seizure onset during the intracranial study. The RNS device (NeuroPace; Mountain View, CA, United States) (Figure ) was implanted into the craniectomy site and the electrodes were connected; using the CM depth electrode and the most active of the two parietal strips. To assess adequate localization of the depth electrode, a thin-cut CT was performed and co-registered with the pre-op MRI. This technique is commonly utilized for localization of electrode contacts, using the CT for contact localization and MRI for good resolution within the thalamus (as seen in Figure ).
As observed in prior studies (, ; ; ; ; ), we found that image-guided implantation resulted in accurate and safe implantation to the CM thalamus. Responsive stimulation in CM during seizures or false-triggers did not result in adverse side effects, changes in level of consciousness, or inadvertent perceptions (i.e., motor twitching, sensory changes, visual perceptions). This patient’s stimulation paradigm is two 40 Hz biphasic bursts lasting 200 ms (80 μs pulse width) of 1.5 mA current; this stimulation may be repeated up to five times based on persistence of the detection. Three of the four available detectors were used to trigger potential seizures: a line-length trigger in the parietal electrode; a bandpass filter 30–125 Hz in the parietal electrode with specified amplitude and duration thresholds; and an AUC measure in the centromedian electrode. Interestingly, as reported in prior CM DBS, stimulation of the deepest contact in our patient resulted in paresthesias, as expected based on the anatomically adjacent fibers of the medial lemniscus carrying sensory information. As there are no data reported from simultaneous cortical and CM thalamus recordings, the most pressing question for our group was whether intracranial recordings from the human CM thalamus could provide seizure related information or confirmation of network participation. In this initial report of ambulatory icEEG recordings from the human CM thalamus, the most notable observation is that many of the seizures as detected on the parietal strip are indeed present in the EEG from the CM electrode (Figure ). We were pleasantly surprised at the ability of the CM electrode to capture seizures. In fact, we are now using the thalamic electrode as one of the detection sources as well as the stimulation target. During obvious seizures noted on the parietal strip, increasing amplitude in the 8–14 Hz range is noted in the CM EEG. Large cortical slow waves are also reflected in the CM electrode. Representation of seizure patterns in CM supports our hypothesis that the expected connectivity between these two nodes in the network play a role in seizures and may provide for neuromodulation.
In addition, it has been noted that the EEG from the parietal strip meets criteria for seizure most of the time, while simultaneous scalp EEG does not show any electrographic seizure. This finding confirms what we have seen in other intracranial studies and animal models, that small areas of underlying cortex can experience seizure or even be constantly in sub-clinical status epilepticus without alteration of the background scalp EEG. With the use of microwires, some have reported seizures on microwires without any background changes on neighboring macro-contacts (). The CM thalamic electrode has episodes of electrographic seizures that appear to correlate more closely with the patient’s clinical seizures. We postulate that seizure propagation to and through the CM nucleus may play a role in clinical presentation of the seizure. With all detections, the CM nucleus receives a short burst of high-frequency stimulation (Figure ). No data is yet available on the long-term effects of chronic CM responsive stimulation to seizure frequency, nor can we make any statements regarding clinical efficacy with a single patient.
Ongoing analysis is quantitating the effect of CM stimulation on resultant CM EEG characteristics, parietal strip electrode EEG characteristics, and patient-reported seizure outcomes. Long term intracranial monitoring paired with responsive stimulation over the course of months to years is essential to begin to understand the dynamic nature of the seizure circuits in vivo. Targeted placement of electrodes in central nodes of SGN, such as in the thalamus, is a next step in characterizing and modulating this seizure network in real-time. |
pmc-6079308-1 | A 31 yr-old woman with complaints of massive abdominal distention and respiratory distress was referred to the gynecology and oncology department of an academic hospital, Mashhad University of Medical Sciences in Aug 2017. In past medical history, she mentioned a secondary infertility for four yrs and had one child aged eight yrs. The patient was candidate for In Vitro Fertilization (IVF) protocol due to tubal factors. In the first cycle of ovarian stimulation, metformin and Gonal-f 75 IU for six days were prescribed () and then continued for two days. The cycle was cancelled due to poor response after the second month from this protocol. She suffered from gradual abdominal distention.
Despite the failure of IVF, she was under the outpatient care and supportive treatment with possible diagnosis of hyperstimulation syndrome. Therefore, antagonist GnRH was prescribed for two days. At the next delayed month visit, because of persistent symptoms with the probability of hyperthyroidism, she received gonadotropin hormone agonist (Decapeptyl). She was re-evaluated due to unresponsive to treatment within this period.
Trans-abdominal and transvaginal ultrasonography were performed that showed multiple multiloculated cystic masses with predominantly solid components in both adnexa. The results of cross-sectional CT-scan and magnetic resonance imaging suggested the ovarian neoplasm. Also, massive peritoneal and pleural effusion was detected (). In this time, 4 months after management of hyperstimulation syndrome, due to persistent large ovarian mass and increased tumor marker inhibin more than 3000 pg/mL, she was referred to our oncology department. Physical examination demonstrated enlarged masses extended up to hypogastric region which resembled 36 wks of pregnancy.
Exploratory laparotomy was performed that showed massive ascites fluid and multi solid cystic masses in both ovaries extended up to the Xiphoid. Complete resection of the tumor was done. Pathology report of frozen section was unable to confirm the malignancy. But, permanent histology indicated the tumor cells with round-to-ovoid nuclei and eosinophilic or vacuolated cytoplasm or microfollicular and trabecular. Moreover, Call-Exner bodies were observed in most areas which were compatible with juvenile granulosa cell tumors. A positive immunohistochemical staining for inhibin was the key point of this diagnostic feature ().
So, surgical staging surgery and optimal cytoreductive surgery without fertility preserving were done. At this stage, although pelvic and abdominal cavity appeared normal without any residual disease in the first surgery, one month later in the second surgery, we unpredictably encountered malignant tumor even in the omentum (stage IIIc of disease); so complete cytoreductive surgery was again performed. Then, three cycles of adjuvant chemotherapy with bleomycin, etoposide, and cisplatin (BEP) were prescribed. Unfortunately, she experienced metastatic diseases in pelvic and abdomen in less than six months, and currently is receiving the second line chemotherapy. Now the patient is under follow-up.
An informed consent was obtained from the patient for publication of this case report and accompanying images. |
pmc-6079310-1 | An 18 year old virgin girl was referred to clinic of gynecology in a university tertiary hospital with constant low grade lower abdominal pain from 2 weeks ago. Dysmenorrhea, gastrointestinal, or urinary tract symptoms was negative. She had regular menstrual cycles and her body mass index was 26.6. Because of abdominal obesity, we couldn't touch any masses in abdominal exam.
Since she was virgin, vaginal exam was refused and in rectal exam a large cystic mass with mild tenderness in the right side of pelvis was palpable. Abdominopelvic ultrasonography revealed 14×10 cm complex multiseptate cystic mass containing solid components in the right ovary and a little free fluid in the cul-de-sac. Right ovary and uterus were normal. These data were confirmed in abdominopelvic spiral CT scan with and without contrast.
Complete laboratory tests including tumor markers requested. All laboratory tests were normal except Lactate dehydrogenase that was 253 (normal upper limit: 280 u/l). In tumor marker panel, alpha fetoprotein, β-HCG, carcinoemberionic antigen (CEA) were in normal range. The serum concentrations of CA-125 and cancer antigen 19-9 were 6484 IU/Ml and 1309 IU/Ml (reference range 35 IU/Ml). Human Epididymis protein 4 (HE4) was 50.7 and Risk of ovarian malignancy algorithm (ROMA) was 11%. CA-125 and CA 19-9 were measured by using appropriate chemiluminescent immunoassay kits (ROSHE Company, ELECSYS 2010 devices).
Although HE4 and ROMA were in low risk for malignancy, because of very high levels of other tumor markers, ovarian malignancies were in the top of the differential diagnosis yet and after achieving written consent about cancer surgery, laparotomy with midline incision was done. A unilateral 14×10 cm cystic mass with very fine adhesions of cyst on the right side of corpus of uteri was detected. There was no free fluid or seeding in peritoneal cavity and cul-de-sac was not obliterated.
Abdominal organs had normal view but the omentum was covered with many diffuse small endometriotic foci (black puckered lesions). In spite of chocolate-like content of the cyst and almost certain diagnosis of endometriosis frozen section confirmed the diagnosis. Then ovarian cystectomy was done and the biopsy from peritoneum was taken. Histological examination approved the endometriosis of omentum and endometrioma. The serum level of the CA-125 and CA19-9 decreased rapidly post operation.
Ethical consideration
The informed consent for reporting of the case was obtained from the patient. |
pmc-6079425-1 | A 61-year-old female with a past medical history of hypertension, HIV, and hepatitis C presented to our hospital with a 1-week history of right groin tenderness and a palpable lump in her right groin. The patient stated that the lump had been growing in size since its sudden appearance approximately 1 week prior. The patient denied fever, nausea, vomiting, or changes in her bowel habits. Physical examination of the right groin, demonstrated a minimally tender irreducible hernia without overlying skin changes. Laboratory values were within normal limits aside from a mildly elevated white blood cell count of 7700/μL. A contrast-enhanced CT of the abdomen and pelvis was obtained for further evaluation which demonstrated a fat- and fluid-containing right femoral hernia (Figures and ). The appendix was located within the hernia sac with an associated fluid periappendiceal collection measuring 3.9 × 5.3 × 4.7 cm (AP × TV × CC). The margins of the appendix were thickened concerning for a possible acute on chronic appendicitis with periappendiceal abscess or mucocele. Subsequent surgical dissection of the hernia sac revealed a gelatinous coagulated fluid and an inflamed appendix with a mucocele at the tip. The appendix was resected and the femoral hernia was repaired. Given the large size of the defect, a large Proloop plug® was placed with an antibiotic vancomycin soak and stitched to the pubic tubercle. A histopathological examination of the appendix showed inflammatory changes including serosal congestion, edema, and fibrosis, consistent with chronic appendicitis with superimposed reactive features of hernia sac adipose tissue due to hernia incarceration ().
The patient recovered well, without complications, and was discharged home one day postoperatively. |
pmc-6079430-1 | A 72-year-old male patient presented to the emergency department (ED) complaining of neck pain, retrosternal oppressive chest pain, and progressive dyspnea, reporting also a change of the voice with rhinolalia. The patient's past medical history was significant for coronary heart disease. The patient was diagnosed with ST-elevation myocardial infarction (STEMI) in 2001, and non-ST-elevation myocardial infarction (NSTEMI) in 2006. A permanent pacemaker was positioned in 2009 for sinus node dysfunction.
In order to investigate iron deficiency anemia and a positive immunochemical fecal occult blood, the patient had undergone an outpatient screening colonoscopy four hours earlier.
The colonoscopy revealed three potential neoplastic lesions. The first one was a sessile polyp of 10 mm in diameter sited in the cecum, close to the ileocecal valve. It was removed with the diathermic loop, after infiltration of the mucosa with adrenaline.
A further two polyps were found in the ascending colon, both of about 7 mm in diameter.
As the cecal polyp exeresis was complicated by bleeding, a hemostatic clip was placed near the ileocecal valve. No obvious perforations were seen during the procedure (), and no symptoms related to perforations, such as abdominal distension, abdominal and chest pain, or dyspnea were identified at the physical examination immediately after the procedure.
However, two hours after the completion of the procedure, the patient started complaining of abdominal, chest, and neck pain and shortness of breath.
Additional information was obtained from the endoscopist who performed the procedure. He mentioned extensive diverticular disease of the sigmoid colon and good mechanical preparation (Boston Bowel Preparation Scale: BBPS 2-3-3).
On ED arrival, the patient was apyretic. He had a blood pressure of 140/80 mmHg, a heart rate of 65 bpm, and an oxygen saturation on room air of 96%. The patient described the chest pain as a constriction, not radiated, and exacerbated by deep breaths.
The airway was intact and he was able to talk, although with rhinolalia. The abdomen was slightly distended and soft, although abdominal pain without signs of peritoneal irritation was located mainly on the right quadrants.
Subcutaneous emphysema, with a clear crepitus on palpation, was apparent on the neck, right anterior chest wall, and anterior and right lateral abdominal wall.
Due to the reported anamnesis of cardiovascular pathology, an electrocardiogram was performed. It showed a T wave inversion in the inferior and lateral leads, without any pacemaker activity (). However, cardiac enzymes, as well as blood tests, inflammatory markers, and hemogas analysis were all unremarkable. Neck, chest, and abdominal X-rays were then requested to rule out the clinical suspicion of CP.
Right chest wall and cervical subcutaneous emphysema, pneumomediastinum, pneumoretroperitoneum, and bilateral subdiaphragmatic free air were reported on the chest and abdominal X-rays ().
The abdominal and lower thorax contrast-enhanced computed tomography (CT) scan revealed pneumoperitoneum and pneumoretroperitoneum, mainly located at the epimesogastrium, at the right anterior and posterior pararenal and perihepatic spaces, as well as diverticulosis of the sigmoid colon (). No questionable findings, such as an obvious intestinal perforation, peritoneal fluid, or radiological signs of peritonitis, were noted.
In view of the clinical and radiological findings, the patient's good general condition and hemodynamic stability, and the absence of peritoneal irritation and signs of inflammatory syndrome, the patient was admitted to the surgical department and treated conservatively, with absolute bowel rest, total parental nutrition, broad-spectrum intravenous antibiotics (ciprofloxacin 500 mg × 2 and metronidazole 500 mg × 3), and symptomatic care.
Vital signs on the day after the procedure included a blood pressure of 125/80 mmHg, a pulse rate of 75 bpm, a respiratory rate of 16 breaths/min, and a body temperature of 36.7°C. Follow-up chest and abdominal X-rays exhibited a resolving pneumomediastinum and pneumoretroperitoneum 48 hours after the admission. C-reactive protein was slightly raised to 0.86 mg/dl, without any other laboratory sign of inflammation. The patient's subcutaneous emphysema markedly resolved on the third postprocedure day.
Diet was started from water intake at the 5th day after the procedure, and oral antibiotics were administered instead of intravenous antibiotics. The patient recovered uneventfully and was discharged on the 12th day after admission.
The condition of the patient was observed in the outpatient clinic one week after his leaving the hospital, and was confirmed to be fully recovered without any further complications. |
pmc-6079435-1 | A 12-year-old boy, S. R., came to an evaluation in October 2015 in the Orthodontic Department of Sapienza University of Rome. His weight was 24 kg (<3 centile) and height was 1.20 m (<3 centile), born from cesarean delivery at 39 weeks to a mother affected by CCD. He presented open fontanels and patent sutures at birth. Closure of anterior fontanel occurred at 3 years of age. In 2016, he underwent a complete clinical and auxological evaluation at the Department of Rare diseases of Sapienza University of Rome. His weight was 21.5 kg (25 centile) and height was 118.5 cm (25 centile), and the hand-wrist X-ray showed skeletal age of 6 years. Clinical examination showed narrow clavicles and accentuated joint mobility. The patient can oppose the shoulders on the midline. Orthopedic assessment showed left lumbar scoliosis and right dorsal scoliosis.
Audiological examination: normal audiometry and impedentiometry with tympanogram type A on the right and type C on the left. The cocleostapedial reflex was present bilaterally for both ipsilateral and contralateral stimulations.
(1) Laboratory findings: deficit of vitamin D 25-OH (17.5 ng/ml), Beta-Cross Laps levels of 0.81 ng/ml. DEXA z-score -2.8.
X-Ray of the spine (2 projections) and long bones (2 projections) shows pseudarthrosis of the medial third of both clavicles, that appears hypoplastic. Iliac wings appear squared and narrow. Hypoplastic pubic bones. Valgus femoral necks. Widened cephalic nuclei of the femoral bone. Proximal pseudoepiphysis of the second and fifth metacarpi in both hands. Brachy-telefalangy with hypoplastic nails. Retarded skeletal age between 6 and 7 years. TC of the head: mastoid appear thickened and ivory. Narrow antral cavities with thickened walls. Tympanic cavities with thickened walls. Cochlea surrounded by compact dense bone. Hyperemia of presphenoidal adenolymphoid tissues. Deviation of nasal septum.
The orthodontic diagnosis is summarized in (Figures –). |
pmc-6079439-1 | An 83-year-old female with a past medical history of rheumatoid arthritis (on DMARD's), asthma, depression, gastroesophageal reflux disease (GERD), and lumbar spondylosis, as well as a past surgical history of right posterior total hip arthroplasty (1999), bilateral total knee arthroplasties (2003, 2012), and right shoulder hemiarthroplasty (2010), presented with five days of right hip pain and inability to ambulate after bending down. In the emergency department, initial radiographs revealed a right posterior hip dislocation, as well as chronic appearing fractures of the right greater trochanter and left inferior public rami (). Her right lower extremity was shortened, internally rotated, and adducted. A propofol-induced conscious sedation was performed by the emergency physician and closed reduction was attempted by an experienced orthopaedic resident. The reduction maneuver involved hip flexion, traction, adduction, and internal rotation followed by external rotation and abduction. After three attempts, post reduction radiographs were significant for a right inferior obturator hip dislocation (). The patient tolerated the procedure and was neurovascularly intact distal to her hip. Computed tomography (CT) was performed, which confirmed a persistently dislocated femoral head with intrapelvic migration through the right obturator foramen (Figures and ). Having failed three attempts at closed reduction, the patient was taken to the operating room for open reduction and revision arthroplasty.
Using a posterolateral approach, the femoral head was found to be locked inferior and posterior to the acetabulum. Manual traction was utilized to successfully extricate the femoral component from within the obturator ring. Both the femoral and acetabular components were stable; however, a large amount of posterior wear was noted on the liner, which was exchanged for a constrained component. A greater trochanteric hook plate with cerclage cables was then utilized for the fixation of the greater trochanteric fragment (). Excellent stability with a full range of motion was noted.
Postoperatively, the patient was weight bearing as tolerated, with standard posterior hip precautions including an abduction pillow. Aspirin 325 mg BID was used for deep vein thrombosis (DVT) prophylaxis. Although the patient initially did very well, she developed urosepsis six months after the index procedure, leading to an acute right periprosthetic septic hip with Proteus mirabilis. Radiographs showed greater trochanteric escape from the hook plate (). She then underwent irrigation and debridement with greater trochanter excision and hook plate removal (). The patient was discharged with 6 weeks of ceftriaxone antibiotics via a peripherally inserted central catheter and has since been doing well with no further dislocations. |
pmc-6079448-1 | An 11-year-old male Rwandan, a known patient of multiple hereditary exostoses (MHE), presented to our hospital with a 6-month history of unrelenting bone pain despite treatment with NSAIDs; he later developed (two weeks prior to admission) a dull persistent aching pain and swelling of his left lower thigh that was worse at night and unresponsive to morphine. His parents noticed that he had also lost weight and appetite. There was no history of trauma or a fall. He was diagnosed with MHE at age 3 and had been living a relatively normal life and attending school. None of his siblings or any other member of his family had MHE. His past surgical history was unremarkable. On general examination, he was well nourished, had mild pallor of the mucus membranes, no jaundice, lymphadenopathy, or skin rash. All the other parameters were normal. Examination of the musculoskeletal system revealed normal stature except for the curving deformity of the left leg. There were multiple palpable bony swellings bilaterally on the upper humeri and lower femurs. The lesion on the left distal femur was markedly enlarged and tender, with induration, reddening, and limited range of motion of his knee joint (). Repeat X-rays confirmed the presence of bony outgrowths (exostoses) on the medial and lateral aspects of the distal femurs bilaterally and left metaphyseal widening common in this condition as had been previously identified when he was diagnosed at age 3, and further investigations of the left distal femur swelling () with magnetic resonance imaging (MRI) revealed a distinct enhancing lesion in the distal aspect of the left thigh at the site of intense swelling and pain. MRI showed a lesion with hypointense signals on T1WI sequences and has heterogeneous signal intensities with moderate and heterogeneous enhancement on T1WI postcontrast study and on T2WI sequences; the lesion had heterogeneous signal intensities (Figures –). At surgery, there was a necrotic lesion, and excision biopsy at the site of the left distal femur exostosis was taken (Figures and ). Hematological evaluation was normal except for a mild leukocytosis. The results of the serum biochemical tests were also normal. We did not do genotyping for EXT-1 and EXT-2 due to lack of facilities.
Histopathological examination revealed a characteristic fibrous cartilaginous cap with a broad base (1.293 mm thick), covering a layer of normal appearing marrow and bone below which was a tumor-forming osteoid, an osteoblastic tumor as evidenced by presence of numerous bone spicules of varying maturity. There was also marked cellular atypia, grossly pleomorphic osteoblasts in the marrow with frequent mitoses. These features were those of a high-grade osteoblastic osteosarcoma. In conclusion, histopathological revealed an osteochondroma with an underlying high-grade osteoblastic osteosarcoma involving the marrow cavity (Figures –).
For staging purposes, CT scans of the chest, abdomen, and pelvis were done to investigate any presence of metastatic lesions (Figures and ). There were no metastatic deposits in the lungs, abdomen, and pelvis, and this was confirmed with PET scan in India where the patient was referred for specialized bone tumor treatment including limb salvage therapy. He was reevaluated, and the diagnosis of MHE and osteoblastic osteosarcoma confirmed. Whole body PET scan showed metabolically active disease in the distal left femur 8.6 × 8.1 × 16 cms in dimension with features consistent with osteosarcoma, multiple hereditary exostoses with evidence of skip lesions, loco-regional lymph node involvement, and no distant metastases. Histopathological review confirmed the earlier diagnosis of osteochondroma with osteoblastic osteosarcoma. The conclusion was that the patient had clinically localized disease. The following treatment plan was proposed and instituted: initiation with neoadjuvant chemotherapy followed by limp salvaging surgery and finally adjuvant chemotherapy. He received doxorubicin 35.5 mgs/m2 per day (day 1 and day 2), cisplatin 60 mgs/m2 for 7 days (days 1 and 2), methotrexate 12 gms/m2 per day, etoposide 100 mgs/m2/day for 5 days, and ifosfamide 2.8 gms/m2/day for 5 days followed by Ifosfamide 3 gms/m2/day for 3 days and subsequently pegylated Interferon-α2b 0.5 mcg/kg - 1mcg/kg. He completed a total of six cycles of chemoimmunotherapy.
Our patient is still alive and back at school with no evidence of disease after 11 months of treatment and follow-up and continues to be followed up by the oncologist. |
pmc-6079489-1 | A 65-year-old woman was admitted to our hospital because of low back pain and left posterior thigh and calf pain. When symptoms of sciatica began 2 months previously, she underwent radiography and magnetic resonance imaging (MRI) of the lumbar spine at another hospital. These showed a vertebral tumor in the lumbar spine. Both the patellar tendon and the Achilles tendon reflex were normal. The sensory exam was also normal. Although the left tibialis anterior (TA) muscle and extensor hallucis longus (EHL) muscle were manual muscle testing (MMT) grade 3, muscles other than the TA and EHL were MMT grade 5. Laboratory blood tests revealed hypoalbuminemia, anemia, and increased alkaline phosphatase and C-reactive protein. She had undergone resection of retroperitoneal DDL 5 years previously () and repeated resection for recurrence 3 years previously. Recurrence occurred again 1 year previously, and spinal metastasis of WDL component occurred in the L2 vertebrae 8 months previously () and gradually increased () in computed tomography (CT), but she did not undergo additional treatment (Figures and ). MRI showed a mass with high signal intensity on both T1-weighted images and T2-weighted images and no enhancement on gadolinium-enhanced T1-weighted images (Figures –). The revised Tokuhashi score [] was 11/15, and the Spinal Instability Neoplastic Score (SINP) was 10/18 []. Therefore, we diagnosed the vertebral tumor as the metastasis of WDL component in DDL and planned surgery for symptomatic improvement. Tumor curettage and L1 laminectomy followed by percutaneous pedicle screw fixation from the Th11 to L3 using intraoperative 3-D CT computer navigation were performed (Figures and ). Histological examination showed mixed well-differentiated and well-dedifferentiated liposarcoma in the primary lesion (Figures , , and ). Lipoblasts containing hyperchromatic nuclei were apparent in the well-differentiated area. Myxoid liposarcoma was ruled out in the dedifferentiated area. Positive staining for MDM2 (Figures , , and ) and CDK4 (data not shown) by immunohistochemistry and negativity of DDIT3 or FUS by FISH (data not shown) confirmed dedifferentiated liposarcoma. She could walk and had no pain in her back and no signs of palsy. However, the retroperitoneal mass subsequently increased, and she died 1.5 years after surgery. |
pmc-6079490-1 | We report the case of a 37-year-old gentleman who presented on a Sunday to the general surgical on-call with a four-day history of generalised abdominal pain postcolonoscopy. He had associated nausea and slightly reduced stoma output.
Past medical history includes asthma and Crohn's disease which had settled at the time leading up to the colonoscopy. There were no known drug allergies, and the patient takes azathioprine, salbutamol, and beclometasone. He is a nonsmoker and drinks minimal alcohol.
Past surgical history includes a complicated appendicectomy in 2007 resulting in a colostomy; a colonic perforation and retroperitoneal abscess secondary to Crohn's disease led to an ileostomy in 2010, and the ileostomy was reversed with an ileocolonic anastomosis formed in 2012. Anastomotic dehiscence occurred leading to major sepsis with abdominal wall breakdown and abdominal compartment syndrome. A debridement of the area was performed and left as a laparostomy, and an ileostomy was reformed. The area was later covered by a large skin graft in 2012. His colonoscopy was part of a preoperative workup for a procedure in a quaternary centre to assess his viability to repair his complex hernia.
On examination, his heart rate was 117 beats per minute (bpm), blood pressure 128/81 mmHg, respiratory rate 15, and oxygen saturation 98% on air. There was a large mass overlying the hernia to the left of the midline and on abdominal palpation; the mass was ballotable with crepitus, was slightly tender, and had a cough impulse (Figures –). In addition, a stoma was present. The chest was clear to auscultation, and GCS was 15/15.
Bloods on admission revealed a C-reactive protein of 219 mg/L (0–5 mg/L) and were otherwise unremarkable.
There is a huge amount of free air, which is most likely secondary to a recent colonoscopy that has probably blown off the ascending colon stump. The colon cannot be traced beyond the midtransverse colon in the current scan (). A large midline abdominal wall hernia containing several bowel loops with most of the gas seeping into the mesentery within the hernia can be seen. Part of the gas is also seen in the intrahepatic and right perinephric space.
The patient was managed with an ABCDE approach. Tazobactam with piperacillin (Tazocin) was administered as per local guidelines. Intravenous fluids, analgesia, and monitoring of output were commenced.
Given the result of the computed tomography scan, the surgical registrars had consented the patient for a laparotomy plus proceed as necessary. This was halted when the consultant on-call reviewed the patient an hour later.
A conservative approach was adopted, and the patient was discharged just days following admission. He did very well and is currently undergoing review at one of our quaternary centres for his abdominal wall reconstruction.
Following his admission and conservative management, contact was made with the quaternary centre and they reported that the colonoscopy had gone to plan and was carried out meticulously and there was no evidence of a perforation; it was noted that there was evidence of inflammation macroscopically and microscopically and this was determined to be nonspecific. |
pmc-6079493-1 | A 57-year-old Caucasian male presented with a ten-year history of nonprogressive ring scotomas OU (). He denied other ocular- or nonocular-associated symptoms or any prior ocular trauma. Family history was negative for any retinal disease, uncorrectable vision loss, hemeralopia, or nystagmus. Best-corrected visual acuities on presentation were 20/25-2 OU. Ishihara color vision testing was diminished to four out of eleven plates in each eye. Anterior segment examination was unremarkable except for mild nuclear sclerotic cataracts in both eyes. He exhibited typical funduscopic findings for pathologic myopia including staphylomatous changes, parapapillary atrophy, and inferotemporal lacquer cracks in the left eye and areas of chorioretinal atrophy in both the posterior pole and periphery OU. |
pmc-6079493-2 | A 52-year-old African American female was referred for evaluation after three years of hydroxychloroquine treatment for rheumatoid arthritis. The medication dosage was never supratherapeutic, and she denied visual changes on the medication. Interestingly, her visual complaints predated the use of the medication by two years, but no baseline visual field testing had been performed. She described her mother as having “macular degeneration and retinitis pigmentosa” that began as central vision loss in her forties and progressed to nyctalopia and peripheral vision loss. Visual acuities were 20/20 OU, and anterior segment examination was unremarkable. Ishihara color vision testing was diminished to ten out of fifteen plates in the right eye and eleven out of fifteen plates in the left eye. The right eye had received laser retinopexy after posterior vitreous detachment for symptomatic retinal holes associated with lattice degeneration. |
pmc-6079493-3 | A 42-year-old Caucasian female who originally presented 22 years prior with perimacular pigmentary changes had been diagnosed with bilateral choroidal osteomas, although neither eye had an orange choroidal lesion nor hyperreflective plaque by B-scan ultrasonography on any prior testing. She denied progression of vision loss, although visual acuities at original presentation were 20/20 OD and 20/30 OS and declined to 20/30 OU when diagnosed with PCD. Ishihara color vision testing was diminished to three out of fifteen plates in each eye. Her family history was negative for eye-related phenotypes. Anterior segment examination was normal, but fundus examination showed perimacular arcuate and circumferential nasal retinal lightening with central pigmentary clumping OU. |
pmc-6079500-1 | In May 2017, a 48-year-old male patient presented in our department with pain in his right knee. The complaints were progressive over the past years. The patient denied any history of trauma. The patient did not have any comorbidities.
The physical examination showed a diffuse pain over the medial and lateral joint space, respectively. The Zohlen sign was positive. The collateral and cruciate ligaments were stable. The range of motion was extension/flexion 0–5–100°. Anteroposterior and lateral radiographs of the knee demonstrated a mild osteoarthritis (grade II according to Kellgren and Lawrence) with a large cystic lesion of the lateral femoral condyle ().
For further differential diagnosis, magnetic resonance imaging (MRI) was performed. MRI demonstrated a cystic lesion of a size of 4.2 × 3.1 × 1.2 cm with hypointensity on T1-weighted and hyperintensity on T2-weighted images (). Diffuse cartilage lesions grade II-III according to Outerbridge of the medial compartment were evident. To exclude other pathologies, an open biopsy of the region was carried out. Histopathological examination showed the presence of a simple, solitary bone cyst without any signs of malignancy or rheumatic disease.
Based on the clinical, radiological, and MRI findings and the progressive complaints of the patient, the possible treatment modalities (sole filling of the cyst versus medial partial knee replacement and cyst filling versus total knee replacement and cyst filling) were discussed with the patient, and he was advised to undergo total knee replacement. Regarding the treatment of the bone cyst, the decision was made for complete curettage of the lesion and filling with a synthetic resorbable bone graft substitute (Cerament, Fa. Bonesupport, Lund, Sweden). Intraoperatively, 15 ml of Cerament were necessary to augment the cyst (). We decided to insert the bioabsorbable bone graft prior to the cutting preparation and not vice versa, because preoperative templating of the prosthesis could not predict the amount of the bone defect that would become evident after the cutting, and we were not sure whether technical difficulties might occur regarding the creation of a smooth surface for the anchorage of the prosthesis. No synovitis or fibrinoid degeneration were intraoperatively evident. During preparation for the total knee arthroplasty and the distal, dorsal cutting of the femur, the augmented bone area of the lateral condyle was evident (). Since only the dorsal part of the lateral condyle was evident and the remaining bone quality was good, we decided to perform a standard, cruciate-retaining total knee replacement (Triathlon® CR, Fa. Stryker, Duisburg, Germany) (). At the end of the surgery, the intraoperative range of motion was extension/flexion 0–0–140°.
Postoperatively, the patient was allowed to put full weight-bearing on crutches. The further postoperative course was uneventful, and the patient was dismissed after 9 days. Postoperative radiographs of the right knee confirmed the correct position of the prosthesis and a proper filling of the large bone cyst with Cerament ().
4 months later, the patient presented again in our department with clinical signs of an arthrofibrosis. He reported on a decrease of the range of motion after the 4th postoperative week. At presentation, the range of motion was limited to extension/flexion 0–30–85°. There were no clinical signs of an infection. Laboratory examination showed a C-reactive protein concentration of <2 mg/dL and a white cell blood count of 12,800 × 106/μL.
Revision surgery and an open arthrolysis were then performed. Intraoperatively, there were no signs of an infection or a third-body wear reaction due to Cerament. Multiple samples of soft tissues were taken and sent for further microbiological and histopathological examination. All microbiological findings were negative. The histopathological examination confirmed the presence of an arthrofibrosis grade 2 with >20 fibroblasts/high-power field with no evidence of a third-body wear or granulocytes. Postoperatively, continuous passive motion therapy was immediately started at the site of a femoral nerve block during the first 5 postoperative days and under full weight-bearing of the operated extremity. The further course was uneventful. Postoperative radiographs of the knee showed an excellent osseointegration of Cerament (). At dismissal, the range of motion was extension/flexion 0–0–100° and remained during the follow-up of 12 months.
Informed consent was obtained from the patient. |
pmc-6079505-1 | A 58-year-old woman with a history of current cigarette smoking, hypertension, and hyperlipidemia presented to the emergency room at our center reporting recurrent episodes of severe central chest pain over the preceding 24 hours. While her ECG showed no significant ST segment shifts, troponin I levels were slightly increased (0.025 ng/mL). Accordingly, she was referred for coronary angiography in the setting of a non-ST segment elevation MI.
Catheterization was performed via the right radial artery using the 6 French (F) Amplatz R1 and 6F Judkins L 3.5 diagnostic catheters (Medtronic Inc., Minneapolis, MN, USA). The only angiographic abnormality noted was a moderate stenosis of the mid left anterior descending coronary artery (LAD) (). To further assess the physiological significance of this stenosis, an iFFR PrimeWire (Volcano Corp, San Diego, CA, USA) was placed in the LAD, following exchange of the Judkins catheter for a 6F Extra Back-Up (EBU) 3.5 guiding catheter (Medtronic Inc., Minneapolis, MN, USA). Of note, initial angiography through the EBU guide catheter prior to advancing the wire showed good coronary flow. It was, however, not possible to advance the wire to the lesion. Accordingly, the PrimeWire was removed from the vessel and then an angiogram of the left coronary artery was taken. Angiography revealed only a stump of the left main coronary artery (LMCA) with occlusion of both the LAD and of the circumflex (LCx) coronary arteries (). Marked (3 mm) anterior ST segment elevation then developed, and the patient became progressively hypotensive with systolic pressure falling to a nadir of 58 mmHg. Inotropic and pressor infusions were commenced.
We, at this point, decided to establish mechanical circulatory support with the Impella CP device (Abiomed, Danvers, MA, USA) prior to attempting to reestablish patency of the left coronary system with stents. We first placed a 6F sheath in the left femoral artery. Just as we gained access, the patient developed ventricular fibrillation. This was immediately treated with one 150-Joule biphasic nonsynchronized shock. Following this, the 6F sheath was changed out over a 0.35″ Wholey wire (Medtronic Inc., Minneapolis, MN, USA) for a long 8F arterial sheath. Then, a 6F multipurpose diagnostic catheter was advanced through the sheath to the LV. Next, the Impella 0.18″ deployment wire was advanced through the multipurpose catheter to the LV and the multipurpose catheter then withdrawn. The 8F sheath was then changed out for a 14F sheath, and the Impella CP device advanced over the wire to the LV. The wire was withdrawn, and satisfactory positioning of the device was confirmed by fluoroscopy with the inflow within the LV and the outflow above the aortic valve. The Impella device was then activated and placed on “auto mode” which allows the device to ramp to P9 level resulting in a cardiac output of 3.5 L/min. The mean arterial pressure increased to 80 mmHg, and there was no further occurrence of ventricular dysrhythmias.
Having initiated mechanical circulatory support, we then set out to reestablish patency of the left coronary system. A 0.014″ Runthrough guidewire (Terumo, Somerset, NJ, USA) was advanced into the LCx artery, and a second 0.014″ Runthrough wire advanced into the LAD. The vessels were then dilated with 2.0 mm × 20 mm and 2.5 mm × 20 mm Trek (Abbott Vascular, Santa Clara, CA, USA) balloons with restoration of flow first in the LAD and then in the LCx.
We then placed a 3.0 mm × 18 mm Resolute stent (Medtronic Inc., Minneapolis, MN, USA) from the LM to the LAD. Next, we rewired the LCx through the LAD stent struts. We then stented the LCx with a 3.0 mm × 38 mm Resolute stent. After this, a 3.0 mm × 20 mm Trek balloon was placed within the LAD stent, and a 2.5 mm × 15 mm Trek balloon placed within the LCx stent. Final deployment of the stents was with simultaneous inflation of the 2 balloons (kissing balloon dilation) (). Finally, a 4.0 mm × 9 mm Resolute stent was deployed from the shaft to the ostium of the LM. Angiography demonstrated reestablishment of patency of the LM, LAD, and LCx (), and intravascular ultrasound of the LAD and LM showed good stent apposition in these vessels. Immediately on completion of the procedure, a 2D echocardiogram was performed in the lab. This showed normal LV systolic function (LVEF = 55%). By this time, all inotropic and pressor agents had been discontinued. The patient was then weaned from the Impella device, and the device was removed from the cardiac catheterization laboratory. At our center, the usual approach to device removal is with suture-mediated closure. Because of the emergent need for Impella support in our patient, a technique of crossover balloon tamponade was used () with completion angiography to confirm hemostasis and absence of femoral artery abnormality or complication (). The general approach at our center to cases of primary PCI in the setting of cardiogenic shock is to retain the Impella hemodynamic support for a minimum of 24 hours to allow for myocardial recovery. However, in this case, because of the rapid identification of shock and the prompt recovery of mean arterial pressure and the ability to discontinue all pressor support, we elected to remove the device while in the cardiac catheterization laboratory. An additional parameter that supported the latter decision was demonstration of a mixed venous oxygen saturation ≥ 65% on P3 setting for 30 minutes.
Following her procedure, the patient did well with no recurrence of symptoms, hemodynamic abnormalities, or dysrhythmias, and she was discharged home 2 days later. Follow-up coronary angiography 6 months after the initial procedure () showed continuing patency of the left coronary system without any significant residual stenosis. At 24-month clinical follow-up, the patient remains angina-free and with continuing normal LV systolic function. |
pmc-6079508-1 | A 38-year old male presented with a chief complaint of chronic and worsening left hip pain. The patient reported a lifelong history of pain and disability and described his current pain as sharp, aching, burning, severe, and constant, occurring daily. Relevant history included a diagnosis of hip dysplasia at age 1 year and confirmation of Legg-Calve-Perthes disease at age 18. The patient walked with a significant limp and reported difficulties with activities of daily living such as putting on socks and shoes and pain on getting out of bed. He also reported the use of a heel lift to equalize his leg lengths and expressed frustration at his daily pain. He had attempted to manage his pain with home exercise, activity modification, strengthening, anti-inflammatories, ice, and rest at length, all without success.
On physical examination, the left leg was significantly shorter, with the leg length discrepancy measured grossly at approximately 2.5–3.0 cm. Range of motion (ROM) of left hip was decreased and measured as follows: 0–85° of flexion, 5° of internal rotation in flexion (IRF), 5° of external rotation in flexion (ERF), 30° of abduction, and 5° of adduction. Abductor strength was 4/5 bilaterally. Neurological examination was unremarkable.
Radiographs revealed left hip dysplasia with upsloping of the acetabular socket. LCPD affecting the femoral head was confirmed, with an incompletely formed femoral head noted and a leg length discrepancy measured at 2.5 cm.
The preoperative plan included left hip total hip arthroplasty. Imageless, computer-assisted navigation was used during surgery to assist with leg length equalization and component placement (off-label use).
Surgery was successful and at three weeks postprocedure, the patient was progressing well, had experienced significant pain relief, and was satisfied with his surgery. Left hip range of motion was improved, most notably in flexion and ERF. Postoperative ROM was measured at 0–95° flexion, 5° IRF, 30° ERF, 30° abduction, and 10° adduction. Radiographs revealed that the left hip was well aligned and concentrically reduced. The left leg was lengthened by 2.5 cm, and leg lengths were even (). |
pmc-6079508-2 | A 47-year old female presented with a chief complaint of severe right-sided hip pain that was chronic in nature. Relevant history included right hip surgery at 10 years of age to address symptoms of Legg-Calve-Perthes disease. She reported no relief from this procedure and in the interim, had sought relief through multiple conservative treatments without success. The patient also reported chronic low back pain, contralateral knee pain, and right-sided groin pain and thigh pain which she attributed to a significant LLD.
On physical examination, the right leg was significantly shorter, with a noticeable LLD present. Range of motion of right hip was decreased and measured as follows: 0–80° flexion with significant pain at end range, 5° IRF with significant pain, 5° ERF, 20° abduction, and 10° adduction. Abductor strength was 4/5 bilaterally. On orthopedic testing, anterior impingement test, Patrick-FABERE, and lateral impingement tests were all positive on the right. Neurological examination was unremarkable.
Radiographic examination revealed a 3.5 cm LLD with the right leg shortened, ovoid femoral head, joint space narrowing, sclerosis, osteophytes, acetabular dysplasia, shortened femoral neck, and trochanteric overgrowth. Diagnoses of Legg-Calve-Perthes disease and secondary osteoarthritis were confirmed.
The preoperative plan included a right hip total hip arthroplasty. During surgery, computer-assisted navigation was again used to assist with component placement and monitoring of changes in leg length (off-label use).
Surgery was successful and at three weeks postprocedure, the patient reported significant pain relief and was satisfied with the outcome of her surgery. She reported the use of a cane when walking long distances but was otherwise ambulating without the use of assistive devices and was progressing well in physical therapy. Range of motion had improved, most significantly in flexion, ERF, and abduction, and was measured at 0–95° flexion, 5° IRF, 30° ERF, 35° abduction, and 5° adduction. Radiographs revealed equalized leg lengths and implants that were well aligned and concentrically reduced (). |
pmc-6079514-1 | A 15-year-old female was returning for follow-up after a 1-month medroxyprogesterone acetate challenge test. The patient had been seen at the clinic prior to age 10 and returned at age 14, reporting menarche at age 14. The patient returned at age 15 and reported that menstruation had started and stopped twice. Free testosterone was high (6.8 pg/mL), and polycystic ovary syndrome was suspected. This patient history may have deterred clinicians from initially including a differential diagnosis of MRKH. The patient was given the medroxyprogesterone acetate challenge test for suspected secondary amenorrhea and returned for follow-up, after 1 month. The patient had not menstruated after the medroxyprogesterone challenge test.
In a detailed sexual history, the patient reported being sexually active, including vaginal penetration and excluding anal penetration. At this visit, the patient reported continued amenorrhea, lower abdominal pain, and frequent urinary tract infections (UTIs).
Upon attempted collection of a genital swab specimen for sexually transmitted disease (STD) testing, labia minora and majora were present, but no opening to the vagina could be identified, such that the genital swab could not penetrate beyond a wall of pale pink, thin tissue, immediately past labia minora. Further physical examination of the genital tract, or insertion of a speculum, was not possible due to this abnormality. There were no masses in the abdomen, and urethral and rectal openings were intact and fully developed. Ultrasound confirmed the lack of a vaginal canal, and magnetic resonance imaging (MRI) confirmed the presence of a remnant uterus, consistent with a diagnosis of MRKH. The MRI also screened for possible concomitant defects.
MRI results confirmed a suspected diagnosis of MRKH with uterine aplasia. In the presumed location of the uterus, there was a longitudinal soft tissue plate measuring 2.5 × 1.4 centimeters. There was also no direct communication to the vulvar region. Bilateral ovaries were identified and demonstrated developed follicles. The presumed location of the vaginal canal was visualized with fluid and debris inside, which may have resulted from a lack of an opening to the vulva. MRI also revealed a mildly asymmetric and dysmorphic sacrum and L5 vertebral body.
Bilateral kidneys were present in the expected location. The left renal collecting system was duplicated, with mild hydronephrosis and hydroureter, extending to the level of the left common iliac artery. Both ureters on the left side were mildly dilated, down to the crossing of the iliac artery. More distally, the caliber of duplicate ureters was within normal limits. The right kidney was unremarkable but with a slightly prominent ureter.
The patient had noted hearing loss. A pure tone audiometry test demonstrated conductive hearing loss at low frequencies, in the right ear. However, auditory brainstem response testing of the inner ear (cochlea) and brain pathways for hearing were within normal limits.
The patient had a history of psychiatric diagnoses and was receiving pharmacological treatment (clonidine and methylphenidate) and counseling. Socioeconomic and familial challenges likely contributed to development and exacerbation of psychiatric and behavioral issues. In addition to facing poverty, the patient's mother was deaf and partially blind, and the patient served as caretaker.
When the diagnosis of MRKH was delivered and explained to the patient and her mother, the patient reported regular bladder and bowel function and continued amenorrhea. The patient also reported suicidal ideation, but reported major improvements in quality of life after taking residence in a behavioral health rehabilitation facility and maintaining regular psychiatric appointments and medications.
After explanation of the diagnosis using charts and diagrams, the mother and patient confirmed that they understood the implications on reproduction. The patient continued to affirm that she briefly menstruated at ages 14 and 15. When asked to elaborate, the patient explicitly reported 4 months of menstruation, followed by amenorrhea, followed by 2 more months of menstruation, then amenorrhea until present, now approaching her 16th birthday. The patient was referred to an adolescent obstetrician-gynecologist for consultation for possible vaginoplasty/vaginal creation. |
pmc-6079551-1 | A 45-year-old male motorcyclist with a history of hypertension, hyperlipidemia, and coronary artery disease was brought to the emergency department after being struck by another car on the highway at speeds of at least 40 miles per hour. Upon presentation, the patient was evaluated using Advanced Trauma Life Support (ATLS) principles. He had a patent airway on arrival and was breathing spontaneously on room air. His initial heart rate was 87 beats per minute, and his blood pressure was 124/63 mmHg without signs of significant hemorrhage. He had an initial Glasgow coma score (GCS) of 15 with equal and reactive pupils. The patient admitted to consuming alcohol and had a serum alcohol of 243 mg/dL. A later CT of the head demonstrated a subcutaneous hematoma without any intracranial abnormalities. His remaining physical examination revealed left lower quadrant abdominal pain without signs of peritonitis, ankle deformities bilaterally, pain with hip range of motion, and blood at the urethral meatus. Given his physical examination findings, subsequent imaging confirmed an unstable pelvic fracture with diastasis of the symphysis pubis of 6 cm, widening of the left sacroiliac joint, a left ischial pubic ramus fracture, and a urethral injury (). He also had a left ankle dislocation and a right compound fracture of the distal tibia and fibula. No intraabdominal injuries were identified on CT imaging of the abdomen. The pelvis was stabilized with a binder by the orthopedic surgeons with subsequent emergency irrigation, debridement, and open reduction and internal fixation (ORIF) of the open ankle fracture as well as reduction of the left ankle dislocation. He was extubated after the procedure and monitored in the ICU while the remaining preoperative medical workup was completed including X-rays and CT scans with 3D reconstructions of the pelvis reconstructions. A hydromorphone patient-controlled analgesia (PCA) pump was utilized for pain control.
On hospital day 2, the patient was deemed fit for surgery and was taken to the operating theater for a combined operation by the orthopedic surgeons for ORIF of the pubic diastasis, sacral fracture, and sacroiliac joint followed by the trauma surgeons to reconstruct the abdominal wall and inguinal canal. The trauma team performed the exposure of the pubic symphyseal region and the pubic diastasis. A Pfannenstiel incision was made, and the planes were dissected exposing the left spermatic cord. The orthopedic team then performed a gentle open reduction of the pubic diastasis taking care to ensure that the bladder and urethra were not incarcerated. The Asnis III cannulated screw system and a Matta pubic symphyseal plate (Stryker GmbH, Switzerland) were utilized under C-arm fluoroscopic guidance with appropriate alignment of the AP and inlet and outlet pelvis views. Once the Mata plate was in place and the orthopedic reduction was completed, we proceeded to reconstruct the anterior abdominal wall. Since the Cooper ligament was destroyed, it was dissected to allow direct visualization of the pubic rami. The abdominal wall defect was measured to be 10 × 12 cm. We then used a modified Stoppa technique by placing the 6 × 6 in Prolene mesh under the damaged internal inguinal ring, making sure the spermatic cord on the left side was not injured or pinched, securing it in place using sutures, including direct suturing to the periosteum of the repaired pubic symphysis and the plate as needed. The medial borders of the mesh were tucked inside the opened rectus sheath on the right side and secured laterally with fires of a 5 mm Covidien Endotack (Medtronic, MN, USA) to the remnants of the conjoint ligament. The midline was then repaired with sutures, including the mesh as reinforcement. The patient did well postoperatively with postreduction films demonstrating appropriate alignment (). He was discharged to rehab on postoperative day 5. There were no recurrences during the follow-up period of 10 years. |
pmc-6079552-1 | A 75-year-old man was admitted to our hospital due to exertional dyspnea that had been manifesting for several months. The patient had coronary risk factors, including hypertension, dyslipidemia, family history of coronary disease, and past history of smoking, and was taking medication prescribed by his regular physician (nifedipine 20 mg/day for hypertension; bezafibrate 400 mg/day for hyperlipidemia). On admission, physical examination and laboratory data revealed no specific findings: white blood cell count, 3990 cells/μL; hemoglobin, 14.8 g/dL; platelets, 26.7 × 104 cells/μL; glucose, 95 mg/dL; blood urea nitrogen, 14 mg/dL; creatinine, 0.71 mg/dL; estimated glomerular filtration rate, 81.7 mL/min; uric acid, 6.0 mg/dL; aspartate transaminase, 21 U/L; alanine aminotransferase, 15 U/L; total bilirubin, 0.6 mg/dL; creatine kinase, 85 IU/L; creatine kinase-MB, 8 IU/L; C-reactive protein, 0.1 mg/dL; low-density lipoprotein cholesterol, 150 mg/dL; high-density lipoprotein cholesterol, 63 mg/dL; triglycerides, 113 mg/dL; glycated hemoglobin, 5.4%; brain-type natriuretic peptide, 24 pg/mL. However, the exercise stress test revealed slight ST depression in leads V4-6 on electrocardiography. Based on the clinical symptoms, the patient was suspected of coronary artery disease. Adenosine triphosphate-stress radionuclide myocardial perfusion imaging revealed inferolateral wall ischemia. Coronary computed tomography indicated that the RCA originated from the left coronary sinus and passed between the aorta and the pulmonary artery (). Total occlusion in the midportion of the abnormal RCA and 90% stenosis of the left anterior descending coronary artery (LAD) were suspected. The RCA lesion had atherosclerotic findings such as spotty calcification and mild positive remodeling (). The patient was diagnosed with effort angina pectoris and underwent coronary angiography, which revealed tight stenosis at the LAD-D1 bifurcation and a completely occluded RCA originating from the left coronary sinus. The abnormal RCA had multiple collaterals from the LAD and left circumflex branch (Figures and ). Because the patient had a coronary artery anomaly and multivessel stenosis, coronary artery bypass grafting was proposed for revascularization, but the patient refused any surgically invasive treatment. Therefore, percutaneous coronary intervention (PCI) was performed for revascularization. The following revascularization systems were used: right femoral artery approach; guiding catheter, 7-Fr Amplatz Left 2 Mach 1 (Boston Scientific, Natick, MA, USA); guide wire, Grand Slam, XT-R, and Sion blue (both from Asahi Intecc, Aichi, Japan); microcatheter, Mizuki (Kaneka Medical, Osaka, Japan) and Caravel (Asahi Intecc); balloon, Ikazuchi 1.0 × 10 mm (Kaneka Medical), Tazuna 2.0 × 15 mm (Terumo Corporation, Tokyo, Japan), and Raiden 3.5 × 10 mm (Kaneka Medical); stent, Ultimaster 3.0 × 18 mm (Terumo Corporation); and intravascular ultrasound (IVUS) catheter, 40 MHz rotational OptiCross (Boston Scientific). Cannulating the guiding catheter to the anomalous orifice of the RCA was difficult, and it was not possible to achieve adequate backup support (). Using the XT-R guide wire, the Mizuki microcatheter could not be passed though the chronic total occlusion lesion until it was replaced with a Caravel microcatheter. The wire was then changed to Sion blue (). IVUS revealed diffuse eccentric calcified plaque. The intramural course of the proximal ectopic artery was elliptical with some lateral compression. However, the stenosis of the proximal intramural course was not so severe that we did not deploy the stent in the proximal portion (). Multiple ballooning and angioplasty with a drug-eluting stent were performed (), and an optimal result was obtained (). At the same time, PCI was performed for LAD revascularization, and an optimal result was obtained (). No exertional dyspnea was noted following PCI. At approximately one year after intervention, exercise stress radionuclide myocardial perfusion imaging and coronary angiography revealed no in-stent restenosis or ischemia. The patient expressed satisfaction with the outcome of the intervention. |
pmc-6079558-1 | In February 2018, we treated the patient, a 58-year-old male who developed erythematous skin with severe itching and flaking presented on the entire body surface. Detailed history suggested that the patient consulted a private physician for a toothache for which he was prescribed with methampyrone 500 mg orally. After taking a single dose of the drug, he developed maculopapular and erythematous rash with itching that followed by bullous exfoliation of the skin. Past medical history included hypertension and postprimary coronary intervention in 2011. The patient has been taking aspirin 80 mg QD, amlodipine 10 mg QD, and atorvastatin 20 mg QD.
On examination, the patient was conscious and alert, but he looked weak. Hemodynamics was stable, with the respiration rate of 24x/minutes, body temperature of 37.8°C, and SpO2 of 97–99% while breathing suplementary oxygen with nasal cannula. There were conjunctivitis and turbid corneal in the bilateral eyes (not shown), ulceration of the mouth, and swollen lips (). He had generalized skin erythema and irregularly shaped itchy purpuric macules. Nikolsky's sign was clearly elicited with a detachment of the epidermis from lower layers when slightly rubbed, and extension of existing bullae to the clear skin indicated an active TEN. The epidermal detachment was observed over 30% of the body surface area (BSA).
Treatment for the patient was involving replacement of fluid loss and also maintaining electrolyte imbalance and antibiotic therapy. He started methylprednisolone 125 mg TID along with cyclosporine 50 mg BID. After 2 days of hospitalization, his skin lesions did not show improvement.In turn, skin change progressions rapidly extended from 32% at hospital admission to 62% of BSA involved with 16% in grade I and 46% in grade II hemorrhagic blisters (). The SCORTEN score [] used to prognosticate risk for death from TEN was three in this patient with the corresponding predictive mortality rate of 35.3%.
By clinical judgement of the lack of patient's response to initial therapy, we decided to treat him with TPE. The procedure was performed using the COBE Spectra Apheresis System (Terumo BCT, Inc., Lakewood, CO) and a double membrane filtration device via central vascular access. The exact filtered plasma volume was calculated with regard to patient's weight and hematocrit level. On each exchange, about 2 L of plasma was removed at blood flow of 50 mL/minute; the replacement of fluid consisted of about 1 L of 5% albumin and 1 L of normal saline. Anticoagulant citrate dextrose solution-A (ACD-A) was used as an anticoagulant during the procedure at a ratio of 1 : 12. In addition, two grams of IV calcium gluconate was administered as prophylaxis against citrate toxicity. TPE was started on day 3 and provided every 2 days for a total of three procedures.
The patient's condition rapidly improved after the completion of the first TPE session. Blistering with extensive epidermal necrosis halted after the second and third session of TPE, and then the epidermal sheet began to dry up and the skin erosions started to heal. Rapid reepithelization occur by 1 week of the introduction of TPE ().The patient made an uneventful recovery, and he was discharged home in 8 days of hospitalization with good condition. Proper instructions were given regarding a possible relapse and methampyrone avoidance. At the checkup, the lesions had completely disappeared. |
pmc-6079559-1 | The patient is a 71-year-old white male who was found to have a 3.5 cm right kidney mass and had been followed by the urology team closely at VA Pittsburgh Healthcare System. Urine cytology was suspicious for malignant cells. He underwent a radical right nephrectomy on February 3, 2014. Pathology showed clear cell RCC. The tumor was located at the lower pole with a size of 4.5 cm (pT1b) and Fuhrman nuclear grade 2. All margins were not involved by carcinoma, and there was no vascular invasion. He had been followed with a regular CT scan every year. He was found to have small bilateral lung metastasis and lymphadenopathy in 2016. The PET scan on April 26, 2016, revealed FDG activity in the lung and hilar and mediastinal lymph nodes. He underwent endobronchial ultrasound biopsy of the mediastinal lymph node which confirmed to be metastatic from clear cell RCC. Due to his comorbidities and mild thrombocytopenia, we started him on lower dose sunitinib at 37.5 mg per oral daily ×4 weeks every 6 weeks in May 2016. In total, he received 7 cycles of sunitinib. He had been followed every 6 weeks in the clinic. He only developed fatigue due to mild hypothyroidism for which he received levothyroxine. During the follow-up, he was found to have worsening thrombocytopenia with platelet counts in the range of 60,000 to 90,000. A follow-up CT scan and PET scan in October 2016 showed improvement of the lung metastasis and lymphadenopathy. He was last seen in the clinic on March 13, 2017.
He was admitted on March 29, 2017, due to muscle weakness, fatigue, poor oral intake, and difficulty swallowing for 2 weeks. During admission, his platelet count was found to be 13,000, serum creatinine 2.3, total bilirubin 4, AST/ALT > 2000, INR 2.9, calcium 7.5, creatine phosphokinase (CPK) > 5000, and uric acid 12 (see ). Sunitinib was discontinued on the first day of admission. CT head revealed no evidence of metastatic disease. Chest X-ray did not show evidence of infiltration or effusion. Echocardiogram showed severe global hypokinesia with LVEF of 30–35%. His LVEF was 55% prior to starting on sunitinib. He quickly developed lactic acidosis and acute respiratory failure. In the intensive care unit, he received bicarbonate, high-dose oxygen, furosemide, and treatment for hyperkalemia. Despite all treatment support, he continued to decline. His family chose to deescalate care, and he died on April 1, 2017. |
pmc-6079566-1 | A 62-year-old female patient who is heavy smoker presented with a burning sensation and discomfort in her left breast that has been recurring over a month prior to admission to the hospital. No fever, chills, or any other symptoms were described. She reported a past medical history of hypertension and a surgical history of hemorrhoidectomy, dilation and curettage surgery, colonoscopy, and gastroscopy.
Physical examination revealed a palpable left breast mass (measuring approximately 3 × 3 cm) in the upper quadrant with no overlying skin changes. The right breast exam was normal. No palpable locoregional lymphadenopathy (axilla and supraclavicular lymph nodes) was noticed. Routine blood tests (complete blood count with differential, electrolytes, prothrombin time, partial prothrombin time, and international normalized ratio), chest X-ray, and electrocardiogram (ECG) were all normal.
Magnetic resonance imaging (MRI) of the left breast showed an ill-defined deep retroareolar spiculate lesion extending over 3 × 1.5 cm revealing early enhancement peak with associated architectural distortion. There were no axillary lymph nodes or abnormal bone signal intensity. No cutaneous thickening or retraction was seen. Findings were suggestive of BIRADS type IV lesion ().
An excisional biopsy was performed and revealed breast tissue with extensive lymphocytic infiltrate intermixed with neoplastic epithelial cells (). Immunohistochemistry results were positive for CK AE1/E3 antibody in the neoplastic epithelial cells with no expression of estrogen or progesterone receptors, and HER2/neu was not overexpressed (). The lymphocytes in the background stained positive for both CD3 and CD20 (Figures and ).
The patient underwent a left modified radical mastectomy. Eleven lymph nodes were dissected and free of tumor. The mastectomy specimen showed a 3.5 × 3 × 3 cm cavity at the site of the previous excisional biopsy. On histological examination, apocrine metaplasia was identified but no residual tumor was detected. To note, apocrine metaplasia is a very common incidental benign finding that is considered part of or associated with fibrocystic changes, and hence, does not affect prognosis and management []. Accordingly, no adjuvant hormonal therapy, chemotherapy, or radiotherapy was given to the patient.
No evidence of recurrence was noted on a 2-year follow-up. |
pmc-6079567-1 | Written informed consent for publication was obtained by the patient's parents.
Our patient is an 8-year-old girl, with a positive family history for both skeletal malformations and bipolar disorders (BD). Her pre-perinatal history was uneventful. She was referred to our Unit because of learning difficulties and behavioural problems. The neurological examination did not show focal neurological deficits. Dysmorphic features were evident at the first observation. She showed several facial dimorphisms such as flat face, blepharophimosis, hypertelorism, broad nasal bridge, and high palate. Bones and joints defects were also evident: pectum excavatum, single transverse palmar crease, brachydactyly, flat foot, and stature below 25th percentile (). Because of these features, she previously underwent genetic consultation and performed array-CGH analysis revealing a chromosomic 8q22.1-q22.3 duplication (hg19/96.846.254-101.630.576x3, 101.726.279x2) encompassing the GDF6 and SDC2 genes, inherited from her father. Thus, our 8-year-old girl presented with clinical and genetic features of Leri's pleonosteosis, within a larger microduplication involving different genes not strictly related to our patient phenotype. In particular, the hypothesis of autosomal recessive optic atrophy (OPA6) was excluded by a general ophthalmologic examination and a fundus examination, since the contiguous region 8q21.13-q22.1 is responsible for recessive optic atrophy [].
She also met the DSM-5 criteria for attention-deficit/hyperactivity disorder (ADHD), specific learning disorder, speech sound disorder, and developmental coordination disorder. In particular, she showed a highly pressured pattern of speech, difficulty in sustaining attention, high levels of activity, and low frustration tolerance. Furthermore, she presented a pattern of bipolar-like phenomena that did not meet the criteria for bipolar I, bipolar II, or cyclothymic disorder. Nevertheless, according to DSM-5 category, she met the criteria for the diagnosis of “other specified bipolar and related disorder” owing to the occurrence of hypomania episode without prior major depressive episode or a manic episode.
The clinical features are shared with both her father and her grandfather that present an overlapping duplication in the 8q22.1-q22.3 region. They show facial dimorphism (flat face, blepharophimosis, hypertelorism, and broad nasal bridge) and brachydactyly and are affected, respectively, by cyclothymic disorder and bipolar II disorder. Also her grandfather's brother received a diagnosis of bipolar II disorder. Unfortunately, he has never performed an array-CGH analysis, but he shows skeletal malformations consistent with Leri's disorder. Along paternal line of our patient, more members are affected by mood disorders associated with skeletal deformations. Unfortunately, none of them agreed to perform the array-CGH analysis, thus the information is incomplete to build a family tree chart. Nevertheless, the chromosome 8q22.1 microduplications were documented in our patient and his father and grandfather.
Our 8-year-old girl's developmental milestones had been mildly delayed. In particular, she presented a delayed achievement of the expressive language. Thus she started a speech-language therapy when she was 4-year-old and continued it for two years. During infancy, she also presented a divergent strabismus surgically treated at the age of 3 years. She also had genu recurvatum and hip developmental dysplasia (type-II Graf) within the first year of life. Our first neurological examination failed to detect major focal signs, but gross and fine coordination impairments with orofacial dyspraxia and speech phonological deficits were observed.
The behavioural observation revealed high levels of impulsivity and a persistently elated mood with increased activity and energy for most of the day. She often displayed restlessness, hyperactivity, and difficulty remaining focused. She also was more talkative than usual and prone to engage conversations with strangers in public, with an indiscriminately friendly approach and a high level of enthusiasm. On the whole, her social behaviour towards adults was often inappropriate. Furthermore, the conversation content appeared inappropriate to the contest with flight of ideas and abrupt shifts from one topic to another. She also showed amusing irrelevancies and theatrical mannerisms and an inflated self-esteem with uncritical self-confidence. Moreover, rapid shifts in mood over brief periods of time might occur. During these episodes, her mood became irritable and she presented decreased need for sleep, diminished ability to concentrate, inconclusiveness, and angry bursts when her wishes were denied.
The neuropsychological assessment included the cognitive profile (WISC IV) [], visual perception and motor coordination (VMI) [], executive functions (TOL) [], and verbal and spatial memory (Corsi test) []. The IQ score was in the low average (total IQ score 88), with the lowest score in nonverbal and fluid reasoning (perceptual reasoning index = 80). A moderate impairment was revealed in visuospatial short-term working memory (Corsi backward span = 2; −1.89 SD) and in backward verbal span (digit backward span = 2; −1.29 SD). Planning ability was largely below the average (rule violations = T > 100, >−2 SD; number of additional moves = T > 100, >−2 SD, TOL), suggesting a poor mental planning and problem-solving skills. She also showed deficits in visual perception and motor coordination skills at the visual-motor integration test (VMI Beery, 1997; standard score = 78; 7th percentile). The reading and writing tests scored below the average (oral reading speed of a text: 0.88 syllables/seconds/−2 DS; number of errors = 14/5° percentile; writing errors: −2 DS). She lacked knowledge of spelling rules that made her enable to read and write complex words. In general, she showed difficulties in managing the aspect of phonological processing which underpins the acquisition of literacy. Working out meaning from a whole sentence was another of her weaknesses due to the working memory deficit. Instead, when dealing with words in isolation, she had less difficulty in comprehension. She also showed very low concentration and attention abilities. During the assessment, she had to be constantly refocused and prompted to continue. All in all, our patient's neurodevelopment and psychiatric symptoms caused a marked impairment, especially in social relationship and in academic performance. |
pmc-6079576-1 | A 70-year-old male with myelodysplastic syndrome treated with double cord allogeneic blood stem cell transplant 34 months ago complicated with chronic GVHD-related glomerular nephropathy, adrenal insufficiency, and end-stage renal disease on hemodialysis presented to clinic after 2 weeks of joint pain. Physical exam revealed normal strength, and he was treated with nonsteroidal anti-inflammatory therapy. Four days later, he developed worsening joint pain, lower extremity calf pain, and hoarse voice. Examination at this time was notable for 3/5 strength in his lower extremities. He was admitted to the hospital and treated with 50 mg intravenous hydrocortisone every 12 hours for three days and intravenous fluids for a suspected postviral myositis. His symptoms resolved, and he was discharged home.
Five days afterwards, he was readmitted to a different hospital for dysphagia and concern of aspiration pneumonia. A gastric tube was placed, he was treated with intravenous ceftriaxone and metronidazole, and he was sent to a rehabilitation facility. Of note, he was not treated with a quinolone or aminoglycoside antibiotic. Another four days later, he presented to our hospital with worsening pneumonia. At this time, physical exam revealed decreased proximal muscle and grip strength, weak palate elevation, diplopia upon prolonged upward gaze, and 3/5 strength in lower extremities. He did not exhibit muscle fatigue from repetitive use. Initial evaluation for this patient was unremarkable for brain or cranial nerve lesions, motor neuron disease, neuromuscular junction disorders (NMJ), or other myopathies (). He was treated with stress-dose steroids (50 mg methylprednisolone every six hours) for concern of myositis, antibiotics, and other supportive measures. Four days after this admission, the antiacetylcholine receptor (AChR) antibody (Ab) panel revealed elevated ACR binding and modulating antibodies correlating with diagnosis of myasthenia gravis. Titers for AChR binding Ab were elevated at 2.15 nmol/L, and AChR modulating Ab was elevated at 45%. He was started on pyridostigmine and plasma exchange (5 exchanges, 3500 ml per exchange). Three days into this therapy, he developed respiratory failure from MG crisis and worsening aspiration pneumonia. Due to worsening symptoms, the patient requested hospice care, and he patient passed away soon thereafter (). |
pmc-6079582-1 | A 15-year-old boy was brought to the emergency department, presenting pain in his left hip after a bike accident during a BMX race. Physical examination showed an external rotation of the lower limb and an irreducible hip flexum. The patient was not able to move the hip nor bear weight. Additional examination showed no neurovascular damage.
X-rays confirmed diagnosis of obturator hip dislocation (Figures and ). Closed hip dislocation reduction was immediately performed under general anaesthesia on an orthopaedic table. Like most hip dislocations in children, it resolved easily with gentle traction [, ]. The radiological assessment was completed with a CT scan, which showed a small impaction of the superolateral part of the femoral head (Figures and ), Pipkin classification type 1, and a small bone fragment in the obturator foramen. After the reduction, the patient was not allowed to bear weight for 6 weeks, as it is recommended for children older than 10 years [], and hip flexion over 60 degrees was forbidden.
Gadolinium contrast MRI was realised 2 months after the trauma, diagnosing an internal and middle femoral head's pillar avascular necrosis (), Steinberg classification type 1C.
We decided to perform a drilling of the femoral head followed by stem cell injection. Four boreholes were made from the greater trochanter up to the femoral head with a 3.2 mm drill (), in which we placed autologue stem cells from the iliaque crest. After the operation, the patient was allowed to bear weight a maximum of 5 kilograms for 6 weeks.
On the 6th-week postoperative X-ray, we noticed a radiolucent area on the femoral head without loss of sphericity. We therefore performed an MRI 10 weeks after the drilling, which showed a slight depression of the superolateral angle of the femoral head, with resorption of the necrotic zone.
One year after the surgery, the patient no longer complained of pain. He is able to walk without lameness and practice BMX at a high level again. Hip flexion is symmetric at 120 degrees. External and internal rotations were 30-0-20, versus 30-0-25 for the right hip. The X-rays () do not show any degenerative sign. Regarding social function and mobility, the patient scores were 1 on the assessment of Jensen (independent) and 9 on the assessment of Parker and Palmer (able to walk inside and outside the house and go shopping without any help). Both scores were the same compared to that before the trauma. |
pmc-6079597-1 | A 53-year-old female was admitted for evaluation of flank pain radiating to the left lower quadrant of her abdomen. Relevant past medical history includes previous left renal calculi requiring ureteral stenting and nonischemic cardiomyopathy with reduced ejection fraction. Laboratory studies were remarkable for leukocytosis and acute renal injury. Imaging studies revealed multiple adjacent obstructing calculi in the mid left ureter causing moderate left-sided hydronephrosis. Patient was boarded for emergent cystoscopy and underwent left ureteral stent placement with no intraoperative events.
Given the patient's history of cardiomyopathy, she underwent preoperative cardiac evaluation revealing a 10 × 10 mm mitral valve “vegetation” on transthoracic echocardiogram. Initial concern was for endocarditis, and the patient was started on antibiotic therapy. However, blood cultures obtained on admission remained free of microbial growth and the patient exhibited no symptoms consistent with overt endocarditis. A transesophageal echocardiogram done to better delineate the consistency of the lesion revealed a 10 × 7 mm noncalcified mass with uniform echodensity located on the atrial side of P2 (Figures and ). Differential diagnosis at this time included myxoma, papillary fibroelastoma, liposarcoma, and less likely, an infectious vegetation. Left heart catheterization revealed nonobstructive coronary artery disease and mild mitral regurgitation.
Given the increased risk of embolization with mitral valve masses greater than 1 cm, we decided to undergo minimally invasive mitral valve excision and valve repair with P2 resection. Histopathological findings confirmed a 9.0 × 8.0 × 6.0 mm myxoma () attached to the external valve leaflet. The tumor was composed of stellate cells with eosinophilic cytoplasm, indistinct boarders, oval nucleus with open chromatin, and indistinct nuclei in the background of a myxoid substance (Figures and ). The patient's postoperative course was complicated by respiratory insufficiency likely related to obstructive sleep apnea which resolved within a few days following the procedure. Patient was discharged home with multidisciplinary outpatient follow-up. |
pmc-6079598-1 | A 60-year-old woman presented with 1-year history of low back pain with lateral aspect of left leg pain and severe neurogenic claudication. There was no neurological deficit. Plain films showed narrowing of L4-L5 disc space and degenerative spondylolisthesis of L4-L5. MRI of L4-L5 showed a degenerative change of intervertebral disc, severe bilateral foraminal stenosis, and moderate central stenosis.
On the axial T1W image, the space between the left common iliac artery and the left psoas muscle was 18.98 mm at level of intervertebral disc space L4-L5 which almost obliterated prepsoas space at level of upper vertebral body of L5 (). Her symptoms did not improve after conservative treatments. She was scheduled to perform MIS-OLIF with decompressive laminectomy and fixation with cortical bone trajectory screws at L4-L5.
Intraoperatively, after general anesthesia, the patient was put in right lateral decubitus position. Fluoroscopy was used to confirm true AP and true lateral of L4-L5 intervertebral disc space. Lateral retroperitoneal approach to lumbar spine was performed. Guide wire and sequential dilator were placed and then retractor blades and L4 stability pin were placed as usual. Unfortunately, when the retractor blades were distracted, the left common iliac artery was found in the operating field. This could be explained because the left common iliac artery was close to the edge of left psoas muscle as .
The retractor blades and stability pin were then removed. The psoas muscle was retracted and guide wire was replaced more posteriorly. The operation was performed as usual and MIS-OLIF PEEK cage (a 6° lordotic-angled CLYDESDALE®) 10 mm × 50 mm was inserted into the intervertebral disc space under fluoroscopic assistance. The final position from fluoroscopy revealed the tantalum marker of MIS-OLIF PEEK cage was pushed more to the right side of the vertebral body. Reposition of MIS-OLIF PEEK cage was not performed at that time. Posterior decompressive laminectomy at L4-5 and cortical bone trajectory screw fixation was then performed in the prone position.
Postoperatively, the preoperative pain on the left leg disappeared. However, she developed pain and numbness on her right leg corresponding to L4 dermatome. Plain films showed the position of MIS-OLIF PEEK cage was placed too deep over the edge of the right lateral vertebral body (). She then was brought to the operating room to reposition the MIS-OLIF PEEK cage. CT and MRI were not performed before the second operation due to remarkable malposition of the MIS-OLIF PEEK cage with acquired pain and numbness of her right leg.
Intraoperatively, after general anesthesia, the patient was put in right lateral decubitus position. The MIS-OLIF PEEK cage was reached from left lateral approach. The removal tool and slap hammer were attached to MIS-OLIF PEEK cage. The slap hammer was impacted to remove MIS-OLIF PEEK cage. However, the MIS-OLIF PEEK cage could not be repositioned and was attached with vertebral bodies. The cause of this malposition might have been from the compression of posterior cortical bone trajectory screws fixation. The posterior approach was then performed to remove rods from the cortical bone trajectory screws. The removal tool and slap hammer were then attached to the MIS-OLIF PEEK cage. Unfortunately, the MIS-OLIF PEEK cage became stuck and was unmovable.
The MIS-OLIF PEEK cage teeth might have locked with the right lateral end plates of vertebral bodies (). The patient was then placed in reverse jack-knife position for opening of the right lateral intervertebral disc space. Retractor blade pins at L4 and L5 vertebral bodies were gradually distracted (). The MIS-OLIF PEEK cage then was gently pulled back and adjusted to a more anterior trajectory to achieve an acceptable position ().
Postoperatively, the pain on her right leg disappeared and the numbness was improved. She was able to walk without pain. At 3 months follow-up, her back and leg pain had significantly improved and her right leg numbness disappeared. The Oswestry Disability Index was 64.4 at preoperative time and was 26 and 20 at 2 weeks and 3 months postoperative, respectively. |
pmc-6079599-1 | A 74-year-old female with no past medical history presented to the emergency department following sudden loss of consciousness while sitting on the couch witnessed by her husband. The patient was unresponsive for one minute and regained consciousness spontaneously. Postsyncopal symptoms included weakness and dyspnea, but she denied any associated chest pain or palpitations. Her husband also denied witnessing any tonic-clonic movement and urinary or fecal incontinence during the episode. Upon arrival of the paramedics, the patient was alert and oriented. Her vitals at the time of evaluation were blood pressure 80/62 mmHg, heart rhythm regular, tachycardia with a rate of 123 beats/minutes, respiratory rate of 23 breaths/minute, and a room oxygen saturation of 89%. She was given an intravenous fluid bolus and transferred to the emergency department. Upon arrival, her blood pressure improved to 94/71 mmHg, but she remained tachycardic at a rate of 120 beats/minutes with a respiratory rate of 21 breaths/minute and an oxygen saturation of 92% on 2 L nasal cannula. Physical examination of her head and neck was normal. Chest wall examination was normal without any abnormal movement or tenderness. Patient's lungs were clear to auscultation bilaterally, and no wheezing or crackles were appreciated. Heart and abdominal examinations were unremarkable. Examination of extremities was normal without any edema or signs of a deep venous thrombosis (DVT).
Arterial blood gas investigation revealed hypoxemia (pH: 7.40, pCO2: 28, and pO2: 61). Her levels of serum electrolytes, glucose, blood urea and creatinine, and complete blood counts were normal. Computed tomographic (CT) scan of her head was negative for any bleeding, embolism, or aneurysm. Her chest X-ray was clear. An electrocardiogram showed a regular rhythm with sinus tachycardia and diffuse T-wave inversion in leads II, III, AVF, and V1 to V6.
CT angiography of the lung demonstrated a large saddle embolus bridging the main pulmonary arteries with extensive segmental and subsegmental clot burden bilaterally, greatest within the left lower lobe but seen within all segments of both lungs. Evidence of right heart strain with flattening and mild leftward bowing of the interventricular septum was also discovered. A transthoracic echocardiogram showed normal left ventricle function without patent foramen ovale, atrial septal defect, or ventricular septal defect. A Doppler scan of the legs showed a DVT involving the distal right femoral vein and right popliteal vein.
Thrombolysis was initiated via catheter-directed therapy. The patient was stable during and after the procedure. Follow-up pulmonary arteriography confirmed resolution of the saddle emboli. The patient was then started on standard anticoagulation treatment with unfractionated heparin and an oral anticoagulant. A blood sample was obtained to study the thrombophilia panel before treatment. An inferior vena cava (IVC) filter was placed without any complication.
Repeated lower extremity ultrasounds confirmed resolution of the DVT. Due to her old age and presence of unprovoked DVT in the leg, she was highly suspicious for an occult malignancy. Abdominopelvic CT scan demonstrated an abnormal appearance of the uterus. There was a rim enhancing mass seen centrally within the uterus with central low attenuation, and additional low attenuation was observed within the endometrial canal. Pelvic ultrasound showed a 3.5 cm uterine mass with increased vascularity. Biopsy of the mass showed early stage endometrial adenocarcinoma. Protein C and S testing of the patient was normal, and the only risk factor for VTE was the uterine malignancy. The patient was discharged five days later on dabigatran, and she reported no further complications or adverse effects. The patient was followed by gynecologic oncology and had laparoscopic removal of the uterine mass. Patient's repeated imaging following the procedures did not show any repeat growth. |
pmc-6079603-1 | A 64-year-old male patient, resident of Vadodara city, Gujarat state located in the western India, who is a known case of diabetes mellitus II, hypothyroidism, and ocular myasthenia gravis since 3 years presented to our tertiary care hospital named Sheth VS General hospital located in Ahmedabad city (Gujarat state) with 8 days history of acute fever, malaise, generalized rash, and multiple joint pains. He complained of an acute onset of sensorimotor quadriparesis and urinary retention since 7 days which was followed 1 day later by H/O altered sensorium. He had no history of headache, vomiting, seizure, dimness of vision, double vision, dysphagia, change in voice, and neck/back pain. The patient had already received methyl prednisolone injection pulse therapy 1 g each for 5 days before presenting to our institute to which he responded partially in form of improved level of consciousness. The patient had no recent history of travelling outside the state of Gujarat or India.
The patient was conscious oriented following verbal command and had no neck rigidity. Cranial nerves: left eye ptosis+; no facial/neck flexor weakness; and mixed dysarthria+.
Nutrition-no undue wasting or hypertrophy; tone: spastic both upper limbs (UL) with flaccid both lower limbs; power (according to the MRC scale): 4/5 in both UL and 1/5 in both LL; with B/L hand grip weakness and B/L dorsiflexor weakness;
Deep tendon reflexes (DTRs) were +3 in both UL with B/L pectoralis reflex and jaw jerk+; DTRs were absent in both lower limbs (LL), and planters were absent.
Impaired joint, position, and vibration sensations up to metatarsophalangeal joints in both the lower limbs and up to metacarpophalangeal joints in both the upper limbs were noted with normal cerebellar examination.
Including complete blood count, random blood sugar, renal and liver function tests, and serum electrolytes were within normal limits except for slightly raised WBC to 14500/µL, CRP 16, and CPK total 84. Serum CHIKV IgM detection by ELISA came positive. Serum chikungunya real-time PCR, which is a qualitative test of chikungunya RNA by a standard procedure, was positive. RNA was extracted using the QIAamp Viral RNA Mini kit (Qiagen). One-step RT-PCR was performed using the access quick RT-PCR kit (Promega), in accordance with the manufacturer's protocol, employing primer pairs targeting the E1 gene designed from the nucleotide sequence of the reference S27 strain (GenBank accession number AF490259; CK 13, TTA CAT CAC GTG CGA TA C; CK-14, CTT TC TCT CAG GG TGC GAC TTT). The amplification was performed in a 50 µL total reaction volume with the Promega Access Quick One-Step RT-PCR kit, 50 pmol of each forward and reverse primer, and 2 µL of extracted viral RNA, in accordance with the manufacturer's instructions.
Electroencephalogram (EEG) showed generalized bilaterally symmetrical diffuse theta-delta slowing suggestive of diffuse encephalopathy. His electrophysiology study showed distal symmetrical sensory motor axonal polyneuropathy more in the lower limbs than the upper limbs. CSF routine micro: protein, 96 mg/dL, glucose, 60 mg/dL, and total cells, 140 with lymphocytic pleocytosis with negative Gram and ZN stain. CSF viral panel done by PCR was negative for neurotropic viruses. This included pan-flavivirus RNA, pan-enterovirus RNA, pan-paramyxovirus RNA detection and pan-herpes DNA detection. Patient's sample was positive for ANA (antinuclear antibody) by immunofluorescence with a dilution of 1 : 100 and 1 : 200, a homogeneous pattern, and +1 intensity. On further workup, ANA profile by immunoDOT was negative. Anti-aquaporin-4 Ab became negative. Serum and CSF investigations are depicted in Tables and .
Multiple tiny altered signal intensity foci with restricted diffusion seen in subcortical and periventricular white matter of bilateral (B/L) cerebral hemispheres, corpus callosum, and b/L corona radiata with normal MR angiogram are shown in . Axial T2W sagittal section showing D7 intramedullary hyperintensity is shown in . Patchy areas of T2W hyperintensity in the cervicodorsal cord predominantly from the C7 to D9 level without significant postcontrast enhancement are shown in . These findings were consistent with features of encephalomyelitis.
Our patient was managed with IV immunoglobulin (IgG) in a total dose of 140 grams as well as supportive treatment for his comorbidities. He was discharged in a stable hemodynamic condition with power 5/5 in both the UL and 3/5 in both the LL. The patient was advised supportive treatment and neurorehabilitation in form of active physiotherapy.
The patient responded well; his power improved; he was able to walk with one stick support with a normal bladder function with a current modified Rankin scale (mRS) of 4. No radiological investigations were done in follow-up. |
pmc-6079606-1 | A 39-year-old male presented with a one-week history of a progressive, painful right paraspinal mass. He reported a history of subcutaneous abscesses which were typically treated with oral antibiotics. His current mass progressed in size and became exquisitely painful despite a recent trial of outpatient Bactrim (sulfamethoxazole and trimethoprim) DS. Examination revealed a firm, tender, nonfluctuant, and nonmobile right-sided paraspinal mass with mild erythema and without drainage (). Slight ptosis of his right eye and intermittent right arm numbness were also noted. His laboratory data demonstrated no evidence of infection with a white blood cell count of 5.9 k/μl without bandemia. Remaining complete blood count values included hemoglobin of 17.5 gm/dl and a platelet count of 441 k/μl. A chemistry panel was notable for a bicarbonate of 33 mmol/l and a creatinine of 1.31 mg/dl. Computed tomography described a 2.7 × 3.3 cm mass involving the right inferior trapezius muscle without gas or fluid collections as well as a 3.9 cm right apical lung lesion (). An MRI of the T-spine showed the initial mass with additional smaller masses in the paraspinous musculature (). Percutaneous biopsy was consistent with metastatic adenocarcinoma of unknown primary, likely from GI or pulmonary source. Staging PET revealed hypermetabolic right apical lung mass and paratracheal nodes, as well as hepatic, left adrenal, and paraspinous muscle masses. The patient received the first 5 of 10 fractions of radiation therapy during his initial admission and was discharged with outpatient oncology and radiation oncology follow-up. |
pmc-6079608-1 | An 88-year-old male presented to a large community teaching hospital with a primary complaint of an irritating, generalized skin rash. The patient was afebrile. He reported recently receiving vancomycin and piperacillin-tazobactam at another area hospital for lower extremity cellulitis. Due to the extensive nature of the skin rash, he was admitted for further clinical assessment.
The patient's past medical history was significant for hypertension, benign prostatic hyperplasia (BPH), stage 3 chronic kidney disease (CKD) (baseline serum creatinine, 1.8 mg/dL), class 3 obesity (BMI 43), cholecystectomy, and left knee replacement surgery. Due to BPH progression, the patient had been using a Foley catheter for the past year which was changed monthly.
Initial laboratory results were unremarkable except for a slightly decreased red blood cell (RBC) count of 3.97 × 106/µL, hemoglobin 11.9 g/dL (reference range, 13.5–8.0 g/dL), and hematocrit 36.5% (40.5–54.0%). His serum creatinine was 2.02 mg/dL with an estimated glomerular filtration rate of 31 mL/min/1.73 m2 and elevated blood urea nitrogen (BUN) of 37 mg/dL (7–18). Urinalysis revealed a clear, yellow appearance, trace leukocyte esterase, 2+ white blood cell (WBC) count, 2+ RBC, occasional bacteria, and <1 squamous epithelial cells. An initial urine culture produced no growth after 24 hours.
The patient's skin rash, which covered more than fifty percent of his body, was treated with intravenous methylprednisolone 60 mg every 8 hours along with diphenhydramine 25 mg every 8 hours as needed. As the rash improved, the methylprednisolone was changed to oral prednisone (40 mg/day). During treatment, the patient experienced an increase in serum creatinine to 2.49 mg/dL and a BUN of 100 mg/dL. Oral prednisone was tapered to 20 mg/day, and the patient's rash improved with treatment.
During hospitalization, the patient's WBC count became elevated to 12.6, but he remained afebrile. His Foley catheter was changed, and urinalysis from the catheter was performed. Urinalysis demonstrated a cloudy, yellow appearance, 3+ leukocyte esterase, 1+ RBC, 4+ WBC clumps, and 2+ bacteria. Urine Gram stain and culture results revealed catheter colonization by Gram-negative rods with a final result of >100,000 colony-forming units/mL of Cedecea neteri. No other microorganism was identified from the catheter. The patient received empirical therapy of intravenous aztreonam (500 mg/8 h) until antibiotic susceptibility evaluations performed using the MicroScan WalkAway 96 Plus (Beckman Coulter) enabled de-escalation of therapy. MIC determination revealed that the C. neteri isolate was sensitive to piperacillin-tazobactam, ceftazidime, ceftriaxone, cefepime, aztreonam, gentamicin, tobramycin, nitrofurantoin, ciprofloxacin, and sulfamethoxazole-trimethoprim, but resistant to ampicillin, ampicillin-sulbactam, cefazolin, and cefoxitin, and intermediate to cefuroxime. The patient's WBC count returned to normal range, and therapy was de-escalated to ciprofloxacin 250 mg twice daily for 5 days prior to the patient's discharge to a rehabilitation facility. |
pmc-6079609-1 | A 22-year-old Hispanic female with history of deep vein thrombosis (DVT) and pulmonary embolism (PE) at the age of 16, followed by diagnosis of SLE, acquired protein S deficiency and secondary APS, failed anticoagulation with Coumadin and enoxaparin due to noncompliance, status post inferior vena cava (IVC) filter placement, and currently on fondaparinux and chronic prednisone (20 mg) presented with generalized weakness, malaise, recurrent fevers, and elevated blood pressure. The patient had a road traffic accident and a viral upper respiratory tract infection diagnosed one week before this admission. She was not compliant with her medications including fondaparinux at this presentation. Clinical assessment revealed a fever of 101.3-degree Fahrenheit, blood pressure of 140/115 mmHg with tachycardia up to 130 s, anemia with hemoglobin of 6.5 gm/dl, and acute kidney injury with creatinine of 1.4 mg/dl and ESR of 95.
The patient was treated with broad-spectrum antibiotics for possible infection due to the presence of fever, tachycardia, and leukocytosis, concerning for sepsis. However, her symptoms did not subside with antibiotic treatment. Renal function continued to decline, and hemoglobin continued to drop along with worsening thrombocytopenia requiring multiple units of blood transfusion. She developed livedo reticularis, right upper extremity weakness, memory loss, cyanotic left toes with diminished bilateral dorsalis pedis pulses, and absent right radial pulse. The arterial Doppler study revealed absence of flow in the distal right radial artery. MRI brain was consistent with multifocal embolic stroke. Echocardiogram to evaluate for cardioembolic etiology revealed no thrombus but a new mitral regurgitation (MR). Incidentally, she was also found to have splenic infarcts. Her clinical scenario was consistent with widespread embolization or thromboses with end-organ damage. Blood cultures were negative and echocardiogram revealed no vegetation. Therefore, the etiology was unlikely to be infective endocarditis or sepsis. Disseminated intravascular coagulation (DIC) and thrombotic thrombocytopenic purpura (TTP) were excluded by the lack of significant schistocytosis on a peripheral blood review and normal ADAMTS13 activity. Immunology workup revealed low complement C3 and C4 with high CH50, high titer of anti-double stranded DNA (dsDNA) antibody, positive LA, positive aCL, and negative aB2GPI. Infection screen for bacterial and viral etiology including syphilis, HIV, and HCV was negative except for positive CMV PCR with a viral load of 2300 IU/ml. Renal biopsy showed class II lupus nephritis and thrombotic microangiopathy with glomerular capillary and arteriolar thromboses, consistent with APS (Figures and ).
Given her history of APS with the described workup profile, diagnosis of CAPS was made and concurrent CMV infection was identified as the precipitating culprit. Anticoagulation with intravenous heparin was initiated for widespread thromboses despite the presence of thrombocytopenia. The patient also received pulsed intravenous methylprednisone. Plasmapheresis was initiated followed by intravenous immunoglobulin (IVIG) for 5 days (400 mg/kg body weight daily) due to lack of significant clinical improvement. Mycophenolate and hydroxychloroquine were used for class II lupus nephritis. Due to immunosuppression, CMV infection was treated with valganciclovir for about 4 weeks till viral load became undetectable. With gradual recovery, anti-dsDNA antibody, aPL as well as aCL titers decreased, and complement levels normalized with resolution of skin lesions, mitral regurgitation, AKI, anemia, and thrombocytopenia. She was transitioned to enoxaparin (60 mg twice daily) on discharge and continued on prednisone (60 mg daily), mycophenolate (1000 mg twice daily), and hydroxychloroquine (200 mg twice daily). Fondaparinux was not continued because of acute kidney injury. Over the course of the following one year, her prednisone was tapered. She remained free of recurrent thrombotic events. |
pmc-6079612-1 | We present a clinical case of a 31-year-old man diagnosed with HIV-1 infection, with CD4 T cell count of 35 cells/mm3 (4%) and HIV RNA 305349 copies/mL (log10 5.48) having initiated ART with abacavir/lamivudine and nevirapine. Around two weeks after starting ART, the patient is admitted due to a sudden cognitive impairment (anhedonia and memory loss) with progression to gait change and imbalance. The cranial computerized tomography (CT) scan showed no lesions but the cranial MRI revealed ventriculoencephalitis ().
The cerebrospinal fluid (CSF) had 38 nucleated cells/mm3, 175 mg/dL proteins and 37 mg/dL glucose (glycaemia 82 mg/dL). The CSF CMV and EBV viral load were 189000 (log10 5.28) and 799 (log10 2.90) copies/mL with negative CSF neurotropic microorganism serologies and molecular identification (HSV 1/2, VZV, Cryptococcus, Brucella, Treponema pallidum, Borrelia burgdorferi, JC virus, Mycobacterium tuberculosis, and Toxoplasma gondii). The final considered diagnostic was mainly CMV-related ventriculoencephalitis and ganciclovir was started.
Nevertheless, the patient started left conjugate horizontal gaze palsy with abducting horizontal saccadic (or jerk-type) nystagmus of the right eye as well as a slight anisocoria with left eye miosis. These changes were enclosed in the one-and-a-half syndrome and left-sided Horner's syndrome. The patient also presented a grade II-III paresis of the right lower limb. The cranial CT scan (performed fifteen days later) revealed a dubious right linear protuberancial hypodensity without signs of intracranial hypertension.
Cranial MRI was repeated one month later revealing improvement of the ventriculitis signs but a larger hippocampus and left mesial temporal region involvement with a discrete increase of the lateral ventricles dimensions.
Due to these clinical and imagiological changes and because we could not exclude tuberculosis infection, classic first-line tuberculostatic therapy was empirically started (stopped after excluding this infection) and foscarnet was added to ganciclovir (until a negative CMV viral load was achieved). At this point, the hypotheses of limbic encephalitis, epileptic activity or paraneoplastic encephalitis could not be excluded. The lumbar puncture was repeated and CSF antineuronal antibodies, HHV-8, and other neurotropic microorganisms were negative.
The electroencephalogram (EEG) showed frontal and frontotemporal bilateral activity with occasional periodic discharges, independent of nonabrupt three-phase bifrontal lesions. Anticonvulsant therapy with levetiracetam and topiramate was started. One month later, the cranial MRI was repeated and a new frontal lesion was detected. The body CT scan was normal. The imagiological reevaluation showed a slight improvement of the frontal lesion with new cerebellum lesions. The suspicion of lymphoproliferative disease was meaningful. Cranial MRI with diffusion and perfusion study with spectroscopy was performed and revealed very suggestive images of CNS lymphoma (). For definitive diagnosis, a stereotactic brain biopsy was done. The histological study revealed a polymorphic cellular infiltrate with histiocytes, CD3+ T-lymphocytes, and CD 20+ B-lymphocytes (mixture of numerous small cells and large activated cells with positive hybridization results for EBV-encoded RNA (EBER)). The histology was suggestive of polymorphic lymphoproliferative EBV-positive disease. The molecular studies were not done because the sample was insufficient to perform the clonality analysis (CSF EBV viral load was 12405 copies/mL; CSF HIV and CMV viral load were both undetectable).
At this point, chemotherapy with rituximab was started and radiotherapy was scheduled. Nevertheless, the patient developed a sudden neurological worsening with a generalized tonic-clonic seizure, refractory to the instituted measures, culminating in death. |
pmc-6079617-1 | A 51-year-old Japanese man was referred to our hospital for abnormal ultrasound findings during a medical examination. He had no complaints or relevant family history. His past history included diabetes, hypertension, dyslipidemia, and fatty liver. Physical examination showed nothing of note. Laboratory findings, including serum concentrations of oncological markers such as alpha fetoprotein, cancer antigen 19–9, neuron-specific enolase, and carcinoembryonic antigen, were within the normal range. A contrast computed tomography (CT) scan revealed a 72 × 49 mm mass closely adjoining the left external iliac vein. Magnetic resonance imaging showed that the mass was isointense with muscle in the T1-weighted image (). A positron-emission computed tomography (PET-CT) scan showed abnormal integration at the tumor site (). These findings suggested that the tumor was a leiomyosarcoma probably derived from the left external iliac vein.
Before surgery, we discussed with the vascular surgeons how to deal with the left iliac vein. In general, blood vessel reconstruction and subsequent anticoagulation therapy are performed. However, the patient was engaged in physical labor and did not want to take an anticoagulant after surgery. So, we finally decided to perform combined resection of the left iliac vein with the tumor. Extirpation of the tumor was performed. We could easily peel the tumor from surrounding tissue except at the left external iliac vein where, as predicted, the tumor was strongly adhered. Therefore, complete tumor resection was achieved by combined resection of the external iliac vein.
Pathological examination revealed a gross, well-defined, firm tumor of 60-mm at the greatest diameter. The cut surface was gray-white with a whorled appearance. Microscopically, the tumor was mostly composed of interlacing fascicles of spindle cells with a mild to moderate degree of cellular pleomorphism and was considered to be a low-grade leiomyosarcoma. Furthermore, there were focal areas of high cellularity and bizarre nuclei (). Immunostaining for α-SMA () was positive, and that for S-100, c-kit, and DOG-1 were negative. The positive ratio of MIB-1 was low at 5–10% (). Contrary to expectation, the tumor was separated from external iliac vein, where only fibrous adhesions without infiltration were present (). There was a thick blood vessel in the tumor that was thought to be a branch vessel of the left external iliac vein (). Its vessel intima was preserved, and immunochemical staining for CD31 was positive (). However, the tunica media and tunica externa of this vascular wall were diminished and completely replaced by tumor cells (Figures and ). Therefore, we definitively diagnosed venous leiomyosarcoma originating from a branch vessel of the left external iliac vein.
Immediately after surgery, the patient developed left leg pain and swelling. However, the painful swelling of his left thigh improved one week later and ultimately disappeared by about 3 months after surgery. The patient has remained free from recurrence at 30 months after surgery. |
pmc-6079622-1 | Our patient is a 37-year-old male who was previously diagnosed as a case of juvenile nasopharyngeal angiofibroma. He was diagnosed with the condition at 30 years of age during which he underwent his first surgery by the lateral rhinotomy approach. Tumour was seen to involve the nasopharynx and sphenoid sinus, eroding the basisphenoid. Optic nerve and carotid artery were not involved. He underwent endoscopic excision 2 years later for tumour involving nasopharynx and sphenoid sinus. The patient remained symptom-free for 2 years, following which lateral rhinotomy excision was performed for a recurrent tumour involving nasopharynx, sphenoid sinus, eroding basisphenoid, and vidian canal. Postoperative histopathology confirmed the presence of angiofibroma and ruled out the presence of any sarcomatous element. In view of the frequently recurring nature of the tumour, the patient was given 45 Gy, 25 fractions of conformal radiotherapy. During the routine follow-up nasal endoscopy after 3 years, a fleshy vascular mass was seen in the nasopharynx. The patient underwent subtotal excision at another centre by lateral rhinotomy.
The specimen block was reviewed. Sections examined showed spindle-shaped tumour cells arranged in long intersecting fascicles, with moderate nuclear pleomorphism and increased mitosis (). Tumour cells were immunopositive for smooth muscle actin, while negative for CD34, cytokeratin, CD 56, S100, and HMB 45. MIB-1 labelling index was 25%. Overall features were suggestive of leiomyosarcoma.
Contrast-enhanced computed tomogram showed heterogeneously enhancing soft tissue mass lesion in the right posterolateral wall of nasopharynx measuring 6 × 3.7 × 4 cm with erosion of the adjacent bone with involvement of the pterygoid muscles ().
In view of the extensive involvement of skull base, surgery was not considered to be suitable for providing a negative margin. Patient has thus been planned for neoadjuvant chemotherapy (gemcitabine based) followed by chemoradiation. |
pmc-6079623-1 | A 71-year-old Qatari male patient was diagnosed with oral cavity HNSCC with stage cT4 N0 M0 in 1997 and underwent radiotherapy in London, UK. He developed post-radiation necrosis and neck fistula, which was treated with a skin flap. After initial chemo-radiation in 2016, a recurring HNSCC involving the supraglottic region and tongue base was identified. On the 12th of January 2017, a second-line treatment with nivolumab was started (3 mg/kg every 2 weeks for five cycles) after declining chemotherapy. However, due to non-compliance the patient refused further treatment. Two CT scans of the patient neck were taken before treatment and 10 days after the fifth cycle of the treatment. PET CT scan was carried out 239 days after the fifth cycle (7 months, 25 days) of treatment. The antibody response to the NY-ESO-1 antigen was measured in the plasma using enzyme-linked immunosorbent assay (ELISA) against a known immunogenic NY-ESO-1 peptide. The cellular response to the NY-ESO-1 antigen was investigated in patient’s peripheral blood mononuclear cells (PBMCs) using an enzyme-linked immunospot (ELISPOT) assay for interferon-gamma (IFN-γ) production by T cells against the NY-ESO-1 overlapping peptides. Flow cytometry was used to determine the expression of PD-1 in the patient CD3+ T cells before and after nivolumab treatment. A panel of 27 plasma biomarkers (cytokines and chemokines) was analyzed by multiplex analysis.
After the fifth cycle of nivolumab treatment, the patient’s bleeding from the tumor site at the neck stopped and CT scan follow-up showed stable disease, no progression, or distant metastasis (Figure A). It showed a mild increase in size, measuring about 5.1 cm × 4.6 cm, 10 days after the fifth cycle (Figures A–C) compared to 4.5 cm × 4.3 cm before nivolumab treatment (Figures A–A) which suggests of pseudo-progression. On the other hand, 163 days (5 months, 10 days) after the fifth cycle of nivolumab treatment, the patient was seen by an oncologist and found to be in a fair general condition. Because the patient declined to have any follow-up CT scans and blood tests, a mobile medical team visited him several times and evaluated him as feeling well with an on and off cough. The patient also complained of limited pain on the left sub-mandibular angle but physical examination showed no palpable mass in that area. The patient was again seen by the medical team 194 days (6 months, 10 days), 209 days (6 months, 25 days), and 226 days (7 months, 12 days) after the fifth cycle of nivolumab treatment. On all visits, the patient had some cough with blood, a small soft tissue mass was observed on the left side of the neck. However, no hard mass was observed. The patient was admitted to the National Center for Cancer Care and Research (NCCCR) 234 days (7 months, 20 days) after the fifth cycle of nivolumab treatment with left mandibular pain and swelling. He had productive cough of copious whitish sputum with no fever. PET CT scan was carried out at day 239 after the fifth cycle (7 months, 25 days) and the patient was found to have progressed (Figures B–F).
The humoral immune response to the NY-ESO-1 antigen was measured. ELISA results showed that out of the four different plasma dilutions tested (1:100, 1:400, 1:1,600, and 1:6,400), 1:100 and 1:400 were found to be the optimum dilutions to differentiate the anti-NY-ESO-1 antibody level before and after nivolumab treatment (Figure A). ELISA results showed that the NY-ESO-1 antibody levels at 1:400 plasma dilution were significantly higher before nivolumab treatment compared to samples taken 11 days after the third cycle (third cycle-11 days, ****p < 0.0001), 8 days after the fifth cycle (fifth cycle-8 days, ****p < 0.0001), and at progression stage (fifth cycle-226 days, ****p < 0.0001) (Figure B). We used pooled plasma from five healthy donors as a negative control. Interestingly, the patient plasma recognized the NY-ESO-1 (11–30 amino acids) peptide which represents one of the most known immunogenic epitope of the NY-ESO-1 antibody. No response was obtained with the non-immunogenic long peptide (amino acids 85–111) (data not shown). The ELISA result was confirmed using Western Blot analysis which showed the recognition of an 18 kDa band by the NY-ESO-1 antibody in the patient plasma (data not shown).
The cellular immune response to the NY-ESO-1 antigen was measured in the PBMCs of the patient before and after nivolumab treatment for IFN-γ secretion using ELISpot assay. Specific IFN-γ secretion was demonstrated against a pool of 43 peptides representing the whole length of the NY-ESO-1 protein (PepMix) (Figure C). IFN-γ secretion was slightly increased in T cells tested 11 days after the third cycle (third cycle-11 days) and was significantly higher 8 days after the fifth cycle (fifth cycle-8 days, **p = 0.002) of nivolumab treatment compared to control before treatment. Interestingly, there was a significant decrease in IFN-γ secretion by the patient T cells collected at progression (fifth cycle-226 days, **p = 0.0028) (Figure C). No IFN-γ secretion was obtained in the presence of the negative control, PSA PepMix (data not shown).
The PD-1 expression by T cells was investigated in the PBMCs of the patient before and after the fifth (fifth cycle-8 days) cycle of nivolumab treatment using flow cytometry analysis. Nivolumab treatment demonstrated a 15-fold decrease in the expression of PD-1 by the CD3+ T cells when compared to the value obtained before treatment (Figure B). Although both subsets of CD4+ and CD8+ T cells expressed the PD-1 molecule, its expression was dominant in the CD4+ T cells population before treatment (Figure D). The expression of PD-1 in the CD4+ and CD8+ T cells population was below detection limits after nivolumab treatment (data not shown).
The cytokine/lymphokine profile was investigated in the plasma of the patient before and after the third cycle (third cycle-11 days) and the fifth cycle (fifth cycle-8 days) of nivolumab treatment as well as at progression (fifth cycle-226 days) using multiplex analysis. We have classified the cytokine/lymphokine profile, based on its upregulation or downregulation status after nivolumab treatment, into two groups (Tables and ). Group 1 comprises 10 biomarkers that were significantly upregulated after the third cycle-11 days (Table ). These are IFN-γ, tumor necrosis factor-alpha (TNF-α), interleukin (IL)-6, IL-8, IL-10, granulocyte-macrophage colony-stimulating factor (GM-CSF), macrophage inflammatory protein-1β (MIP-1β), chemokine C-X3-C motif ligand 1 (CX3CL-1), CXCL-11, and soluble CD137 (sCD137). It is important to mention that four of the biomarkers (IL-10, GM-CSF, CX3CL-1, and sCD137) also continued to rise after the fifth cycle (fifth cycle-8 days) of nivolumab treatment (Table ). Group 2 comprises five biomarkers that were significantly downregulated after the third cycle-11 days and also continued to decline after the fifth cycle (fifth cycle-8 days) of nivolumab treatment (Table ). These are granzyme A, granzyme B, perforin, soluble first apoptosis signal (sFAS), and IL-17A. Two biomarkers (IL-10 and CX3CL-1 also known as Fractalkine), important for immune activation, were significantly reduced at progression (fifth cycle-226 days, Figures A,B). Moreover, two biomarkers (IL-6 and IL-8), important for immune inhibition, were significantly upregulated at progression (fifth cycle-226 days, Figures C,D). The remaining 12 biomarkers analyzed [IL-2, IL-4, IL-5, IL-7, IL-12 (p70), IL-13, IL-21, IL-23, MIP-1α, MIP-3α, MIP-1β, and sFASL] showed no significant change (data not shown). |
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