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A 60-year-old male patient visited the hospital with severe posterior neck pain and a tingling sensation in both hands, which began two months earlier. His neurological exam was without deficit. Cervical spine magnetic resonance imaging demonstrated an 8-cm enhanced mass with internal necrotic changes involving the posterior neck muscle at the C2-3 level (). Additionally, abdominal chest enhanced CT and positron emission tomography-computed tomography (PET-CT) were performed to determine the tumor origin. He was diagnosed with urothelial carcinoma with multiple metastasis.\nOn cervical spine CT and CT angiography, which was conducted to determine the VA pathway and check the range of the mass, a metastatic tumor compressed the spinal cord and encased the left VA. An osteolytic lesion was found at the C2 and C3 levels (). The left VA was shown to be dominant and deviated toward the lateral side rather than taking the normal VA pathway. Therefore, palliative surgery was decided to reduce pain related to instability of the osteolytic lesion, reduce cancer-related pain and prevent neurologic deficits by decompression of the spinal cord. Surgery was planned for subtotal resection of the tumor and stabilization through occipital-cervical fusion.\nThe operation was performed with the posterior approach. The tumor was found to be approximately 7.91 × 5.24 cm in size and pinkish, located at the C2-3 level, and involved the muscle, C2 left lamina and pedicle (). The dissection plane was relatively well maintained, tumor decompression was performed, and the tumor around the VA was also removed. Immediately, the remnant mass around the VA was removed using pituitary forceps, and arterial bleeding occurred in the cranial direction extending approximately 1 cm beyond the mass removal location. We tried to control bleeding from the injured vessel with bipolar coagulation, a compressive packed absorbable gelatin sponge, and hemostatic agents, such as oxidized regenerated cellulose. However, we were not able to achieve successful control of bleeding. We used hemoclips around the site where bleeding occurred, and we exposed the proximal and distal parts of the injured VA by dissection. A direct primary repair was attempted. Afterward, the blood flow was checked using Doppler sonography, but the blood flow of the injury site was not detected. After cutting off some of the distal and proximal parts of the injured VA, anastomosis was performed using an artificial vessel graft (Impra_Carboflo short tapered 40A64C, 4–6 mm × 40 cm, CR BARD Inc., Murray Hill, NJ, USA). Constant blood flow around the injured VA was confirmed using Doppler sonography. As previously planned, the patient underwent occipito-C1-4-5-6 fusion for stabilization (). We completed the operation 2 hours after the VAI had occurred. The systolic blood pressure was maintained above 120 mmHg during surgery.\nAfter surgery, the patient was transferred to the angiography room for further evaluation, and transfemoral cerebral angiography was performed (). Angiogram showed occlusion of the left VA at the proximal part of the anastomosis. After mechanical thrombectomy, the left VA was recanalized. The right VA was nondominant, but some of the blood flow with the contralateral side and the right side was maintained. After 30 minutes, angiogram showed a gradual narrowing of the left VA, and a stent (LVIS & LVIS JR cerebral stent, 3.5 × 28 mm, MicroVention Inc., Aliso Viejo, CA, USA) was inserted into the anastomosis vessel to maintain the vessel's internal diameter. The patient was transferred to the intensive care unit after demonstrating that there was no narrowing of the left VA on the final angiogram (). CT angiography was performed to evaluate the VA on the seventh day after surgery (). No vascular abnormalities were identified in the posterior circulation with the left VA. The patient recovered without neurologic symptoms.
A 25-year-old female presented for routine prenatal care. She was a G3P1011 and had had one elective abortion as a teenager and one full-term delivery 3 years prior. She had preeclampsia with her previous pregnancy and was induced around 39 weeks’ gestational age. More details of this prior pregnancy were unavailable. During the third pregnancy, her blood pressure and urine protein were monitored closely. At 22 weeks and 2 days gestational age, she had early 24-h urine that was mildly elevated at 340 mg but had normal blood pressures and was asymptomatic. She was started on a baby aspirin daily, which she continued throughout her pregnancy. By 32 weeks and 2 days gestational age, she had another 24-h urine that was severely elevated at 770 mg. She still had normal blood pressures and was asymptomatic. At 37 weeks and 1 day gestational age, she presented with worsening lower extremity edema, headaches, intermittent spots in her vision, nausea, and vomiting. Her blood pressure was still normal and her deep tendon reflexes were normal. She had an elevated protein/creatinine ratio in her urine of 1.69 mg/dL (normal is <0.3 mg/dL). Her pregnancy-induced hypertension panel was normal. As she did not meet the criteria for preeclampsia, the decision was made to expectantly manage with close follow-up. The next morning, she was still symptomatic with headaches and nausea despite medications; however, her blood pressure remained normal. At this point, the decision was made to admit her in the hospital for induction of labor secondary to atypical preeclampsia with severe features. She was given intravenous (IV) magnesium during her induction and in her immediate postpartum period. She delivered a baby girl vaginally at 37 weeks and 3 days gestational age. The neonate was admitted to the Neonatal Intensive Care Unit for respiratory distress and was diagnosed with hyaline membrane disease and right-sided pneumothorax. She received two doses of surfactant and was discharged home on day 8 of life. At the mother's 6-week postpartum visit, she still had proteinuria with 3+ protein on her dipstick urine. She will be worked up for other causes of proteinuria.
A 74-year-old man under anticoagulation and antiplatelet therapy after mitral valve replacement surgery had mild head injury in May 2017. Computed tomography (CT) taken 2 months later revealed the presence of supratentorial CSH on the left. He had been observed without surgical exploration because he had no neurological symptom and there was prolonged coagulation due to the anticoagulation and antiplatelet therapy. The patient was followed up regularly, and the CSH did not show any change in size. In July 2018, he presented with aggravating headache and nausea which lasted for 3 days. He showed slight consciousness disturbance (Glasgow Coma Scale: 14) at the time of admission, which deteriorated within a few hours thereafter. No other neurological signs and symptoms were present. A CT scan taken on his admission demonstrated an appearance of bilateral CSH in the posterior fossa associated with hydrocephalus (Fig. ). Magnetic resonance imaging showed niveau formation in the CSH and the continuity of the right and left hematomas through the supracerebellar area (Fig. ). As his laboratory examination revealed prolonged coagulation (prothrombin time international normalization ratio: 3.6), vitamin K was administered to reverse the coagulation status. As his consciousness deteriorated rapidly, urgent burr hole irrigation for the posterior fossa CSH was performed under local anesthesia in the supine position (Fig. ). The CSH in the posterior fossa had the same characteristics as the common supratentorial CSH, which had an outer membrane and hematoma fluid inside. The hematoma was evacuated, then the cavity was washed thoroughly with saline. The supratentorial CSH was evacuated through another burr hole in the usual manner. His consciousness improved within a few hours after the surgery. The postoperative CT scans showed the disappearance of the CSH in the posterior fossa and improvement of the hydrocephalus. Follow-up CT scans taken 3 months after surgery demonstrated no recurrence of the CSH in the posterior fossa (Fig. ). The patient provided written informed consent for publication of this report.
We report the case of a 31-year-old Caucasian woman, gravida 3, para 1, who was referred after a second trimester fetal anatomy screening at 20 weeks gestational for a suspicion of a complex fetal cardiac malformation, for which several specialized opinions tried to reach consensus.\nThe obstetrical history of the patient includes a previous Caesarian section with a normal course of parturition and a spontaneous miscarriage. The current pregnancy presented a low risk for aneuploidy according to the performed cell-free fetal DNA test. The classical karyotype performed after the abortion did not reveal any chromosomal abnormalities.\nPrevious ultrasound evaluations were incongruent and reported the following findings:an isolated aortic arch anomaly (supposedly aneurysmal dilation from which the left common carotid artery emerges) and coarctation of the aorta with the anterograde flow; ventricular septal defect, coarctation of the aorta, and a vascular formation located superior from the aortic arch with the appearance of an arteriovenous fistula; aneurysmal dilation located above the pulmonary trunk bifurcation and a dilated left common carotid artery with a retrograde flow; minor ventricular septal defect with a normal ductus venosus triphasic flow.\nWe performed fetal echocardiography, which demonstrated a mild cardiomegaly with a left deviated 72-degree heart axis, normal aspect of the four-chamber view, a small membranous ventricular septal defect, and ductal aortic coarctation; the ductus venosus flow was normal (, and ). In addition, we identified an aneurysmal structure measuring 1.63/1.25/1.16 cm with turbulent Doppler flow, situated above the emergence of the pulmonary trunk and continued by a dilated vascular structure that bifurcates in the cervical region; the aneurysm seemed connected to the left pulmonary artery as well. A dilated left subclavian artery was also suspected (, and ).\nIn the context of complex cardio-vascular malformations, the patient requested the termination of the pregnancy by drug-induced abortion.\nThe hands-on dissection of the fetus revealed a set of abnormalities that could stand as an anatomical basis for what has been found during the ultrasound examination.\nThe first and the most pronounced aspect was the distention of the whole venous system of the neck and mediastinum. The specimen presented a linguo-facial vein that described a rather sinuous pathway alongside the inferior margin of the mandible (). Both the linguo-facial vein and the external jugular vein appeared with a markedly increased caliber, around 4–5 times larger than expected for this gestational age. Both left and right jugular veins and the right subclavian vein were assessed as three times larger than usual, respecting the normal relations to the neighboring structures ( and ).\nThe confluence between the left jugular and subclavian vein into the left brachiocephalic vein was observed to be very dilated to superior and inferior, extending above the superior margin of the omohyoid muscle as well as below the inferior concavity of the aortic arch. Moreover, on the inferior side of the enlarged brachiocephalic vein, a vessel could be observed descending lateral to the left vagus nerve and communicating with the left pulmonary artery. The left pulmonary artery was observed to be dilated as well, around twice as normal ().\nRegarding the great vessels of the heart, there are some anomalies to be discussed. A narrowing of the aortic arch was identified distally to the emergence of the left subclavian artery ( and ). A large, patent ductus arteriosus was found, ending right at the narrowing level observed in the aortic arch (ending right at the coarctation level) (). The left subclavian artery was dilated as well, sizing as much as the ascending aorta and the aortic arch, creating the illusion of a terminal branch rather than a lateral one ( and ).\nHeart analysis concluded no distinct changes in heart architectural formation for this gestational age. Atrioventricular and ventriculoarterial concordance was noted. Atria and ventricles were increased in relation to the mediastinum. Surprisingly for an aortic coarctation, the right atrium was not found to be enlarged.
A 65-year-old P3L3 postmenopausal woman had been ailing with low backache, vague abdominal pain, dysuria, and vaginal discharge for 1½ years. She had visited the orthopedic outpatient department (OPD) where she denied any history of trauma for her backache. There were no aggravating or relieving factors, and the pain was not eased even on changing position. Her lumbar spine and hip examinations were found to be normal. She was then referred to the gynecological OPD to rule out any genitourinary cause for her symptoms. On examination, she was moderately built, her pulse was 80/min, and blood pressure was 140/90 mmHg. Her per abdominal examination revealed diffuse tenderness in the lower abdomen while per speculum examination showed atrophic vagina with copious white discharge. On per vaginal examination, the uterus was found to be atrophied with free fornices and mild tenderness. Her investigations showed normal blood sugar and renal function test, urine culture had Escherichia coli infection and ultrasound abdomen which was performed in some peripheral centre reported bilateral hydroureteronephrosis. She was advised oral antibiotics and vaginal pessaries for her urinary and genital infection and analgesics for her backache along with referral to the urologist. The patient was relieved of dysuria and vaginal discharge, but she was noncompliant and did not follow up with the urologist as advised. Three weeks later, she presented to the emergency department with severe pain abdomen. The emergency ultrasound suspected an AAA which was also confirmed on CT scan to be 6 cm × 6 cm in size []. The patient was admitted under the vascular surgery unit and underwent successful aortic aneurysm repair after 3 days []. The patient stood the procedure well and was transfused 2 units of blood intraoperatively. Her postoperative recovery period was uneventful, and she was discharged on the 7th day after surgery. In the follow-up period, the patient was healthy at 1 month of her visit, following which she did not comply with further follow-up schedule.
A 7-year-old female presented with bilateral profound sensorineural hearing loss. She had a history of low birth weight (2190 grams), operation for congenital diaphragmatic hernia, and no automatic auditory brainstem response while in the neonatal intensive care unit.\nAfter being discharged from hospital, thorough examinations were performed by otolaryngologists. Physical examinations revealed normal auricles, external auditory canals, and tympanic membranes. Her neck motion was normal and no systemic malformations were detected. Audiologic tests including auditory brainstem response, auditory steady state response, and conditioned orientation reflex audiometry with and without hearing aid revealed bilateral profound sensorineural hearing loss. Computed tomography (CT) and magnetic resonance imaging of the temporal bones showed no inner or middle ear malformation. The patient did not have Down syndrome and had no apparent mental and growth retardation. Given the diagnosis of congenital deafness, hearing aids were provided to the patient when she was 6 months old. The patient was reevaluated 10 months after; however, any efficacy of the hearing aids could not be demonstrated.\nWe explained to the family that CI was recommended before the patient reached the age of 2 years; however, approval of the operation could not be obtained. CI in the right ear was eventually performed when the patient was 7 years old. There were no complications, and the intraoperative change in neck position was minimized. The length of anesthesia and operation was 255 min and 170 min, respectively. After surgery, the patient had no neurological deficits.\nThe day after surgery, the patient complained of dull neck pain in her right side. Physical examination showed no mass but weak, bilateral neck tenderness. Neck movement was restricted and her head was tilted to the right side, rotating to the other. Conservative therapy with oral acetaminophens was applied, but clinical features remained.\nOn the 5th day after surgery, the patient consulted an orthopedic specialist. Three-dimensional CT (3DCT) revealed asymmetry in atlantoaxial joint and rotation of the atlas with the odontoid process acting as pivot (). These findings lead to the diagnosis of AARF, and the patient was treated with indirect cervical traction at bedside for 1 week, and she was eventually discharged with a soft cervical collar for another week. Follow-up 3DCT showed normal appearance of the atlantoaxial joint (). Her neck moves normally with no pain.
The patient is a 54-year-old white male with a past medical history significant for bipolar I disorder with psychotic features and posttraumatic stress disorder admitted from the emergency department to the acute psychiatric floor with symptoms of mania and psychosis. Prior to admission, the patient had been taking variable doses of lithium for more than 10 years with poor adherence noted by the patient and prescription fill history. Per hospital records, the maximum daily dose of lithium the patient received was 900 mg. However, during this period, the patient received care at hospitals outside of the health care system, so it is unknown if the dose was adjusted.\nIn the emergency department, hospital day (HD) 1, serum lithium concentration was negligible at <0.2 mEq/L (reference range 0.6-1.2 mEq/L). Due to past therapeutic success, lithium was reinitiated with 450 mg lithium carbonate sustained action in the emergency department. Once admitted, an additional 900 mg of lithium carbonate sustained action was administered on HD 1 for a total dose of 1350 mg with plans to reinitiate a maintenance dose of 900 mg daily. Labs were not drawn until approximately 12 hours after receiving the 900-mg dose of lithium, so a baseline serum calcium was not available. Also on this day, the patient was continued on his home regimen of quetiapine 300 mg at bedtime. Serum calcium was measured to be at least 11 mg/dL through HD 4. On HD 5, serum lithium was therapeutic at 0.9 mEq/L with an elevated serum calcium of 11.1 mg/dL and normal PTH of 62 pg/mL. On HD 6 the patient remained asymptomatic of hypercalcemia but requested a different medication be started due to fear of thyroid complications he read about in an informational handout. Lithium was discontinued, valproic acid 750 mg twice daily was initiated, and quetiapine was increased to a total daily dose of 400 mg due to continued psychotic symptoms. Serum calcium taken on HD 10 and HD 13 were within normal limits at 10.1 mg/dL (). The psychiatric symptoms began to subside, and he was discharged on HD 15 on valproic acid 750 mg twice daily and quetiapine 400 mg daily. No other medications were administered during admission other than haloperidol intramuscularly for agitation on HD 2.\nOther potential causes of hypercalcemia were assessed. The patient did not exhibit any other signs of malignancy and had a thyroid-stimulating hormone that was within normal limits at 1.02 μU/mL (reference range 0.36-4.50 μU/mL). Additionally, the patient did not report taking any medications, vitamins, or supplements prior to admission, nor was he taking any medications inpatient that were suspected to increase serum calcium.\nDuring past trials of lithium therapy, including a time frame of 3 to 6 months prior to admission, the patient experienced a similar rise in serum calcium with a normal PTH (). The serum calcium collected in the absence of lithium treatment nearest this admission was 6 months prior. This value (10.1 mg/dL) appears to be the patient's baseline serum calcium in the absence of lithium, chronically near the upper limit of normal but <10.2 mg/dL. In all cases, serum albumin was within normal limits (3.4-5.5 g/dL), suggesting that serum calcium values were accurate. Using this information, lithium was considered the probable cause of hypercalcemia based on the Naranjo Algorithm Adverse Drug Reaction Probability Scale.
A 30-year-old averagely built gentleman was referred to our hospital with alleged history of ingestion of an organophosphorus compound three days prior. He was previously admitted to a private hospital, where he was intubated in view of low mentation, and need for respiratory support. On day 3 of admission there, a routine surgical tracheostomy was attempted but failed and the patient was referred to our hospital with a gauze bandage applied over the tracheostomy stoma wound and on ambu bag ventilation via a cuffed size 7, endotracheal tube.\nHe arrived in our emergency room within two hours and was found to be semiconscious, with a GCS of E2M4VT. He had small sized, sluggishly reacting pupils, was diaphoretic, with heart rate of 101 per minute, blood pressure of 160/100 mm of Hg, and a peripheral oxygen saturation of 100% on an FiO2 of 1. He had moderate subcutaneous emphysema extending to the face, neck, and upper arms. The tracheostomy stoma was roughly 2 × 2 cm wide with a tracheal rent of about 1.5 cm. There was an audible air leak from the site of tracheostomy stoma, despite the cuff of the endotracheal tube being well below the site of the stoma. An urgent chest radiograph was ordered and the patient was shifted to the intensive care unit for further management. By the time the patient had arrived in the ICU, the subcutaneous emphysema was found to have increased substantially and had by now involved the entire chest, abdomen, genetalia, and thighs, extending cranially up to the head and face. The patient was unconscious and unresponsive by then and his airway peak pressures were extremely high resulting in near impossible ventilation. A clinical diagnosis of tension pneumothorax was made and bilateral infraclavicular needles were placed to relieve air under tension. The chest radiograph confirmed the diagnosis of severe pneumothorax []. ENT surgeons attempted a tracheostomy through the previous stoma using a portex cuffed tracheostomy tube sized 7.5. However, as the skin to tracheal distance was very large, approximately 7-8 cm (due to the expanding subcutaneous emphysema), the tube kept slipping into the wrong track, anterior to the trachea. Repeated attempts at placing the tracheostomy tube proved unsuccessful. Then, an attempt was made to insert an endotracheal tube size 7.5 (cuffed) into the stoma which was immediately successful and ventilation was rapidly resumed []. Meanwhile, bilateral tube thoracostomies were performed. The endotracheal tube was fixed at a depth where air entry was equal in both lung fields. The lungs rapidly expanded and patient was subsequently ventilated successfully. By day 2, the emphysema had reduced substantially and the neck to tracheal tube distance had reduced. By the fifth ICU day, he was weaned off the ventilator and discharged from the ICU on the seventh day.
A 63-year-old man who had suffered from esophageal achalasia for more than 20 years complained of acute respiratory distress due to stenosis of the trachea resulting from advanced esophageal cancer with tracheal invasion. He was admitted as an emergency to the referral hospital and computed tomography revealed a huge esophageal tumor significantly compressing the trachea (Fig. ). The patient underwent placement of a Dumon Y-shaped tracheobronchial stent (T M Y stent:φcon) to avoid suffocation. He was diagnosed as having esophageal cancer with tracheal invasion, and then underwent chemoradiation therapy (CRT). Following 18 Gy of irradiation and a course of chemotherapy using docetaxel, cisplatin, and 5-fluorouracil, a large TEF developed despite significant tumor shrinkage (Fig. a, b). The physician decided to cease the CRT and treat him with the best supportive care. Oral intake was prevented due to aspiration through the TEF, and a gastrostomy was performed under endoscopy.\nThe patient was referred to our hospital as he was unable to eat. At the time of admission, he had a good performance status and no comorbidities. We initially considered the possibility of esophageal stenting to close the TEF. However, as CRT significantly decreased the size of the esophageal tumor, and there had been an esophageal dilatation due to pre-existing achalasia, an esophageal stent seemed unlikely to be able to close the TEF. Therefore, we decided to perform an esophageal bypass using a gastric conduit after obtaining informed consent.\nWe prepared a percutaneous cardiopulmonary support (PCPS) system that could be used whenever needed, and both arterial and venous catheter sheaths were inserted from the femoral vessels prior to an endotracheal intubation. We then intubated a 6.0 mm spiral tube under the guidance of both bronchoscopy and X-ray guidance. A cuff of the tracheal tube effectively sealed the space between the stent and tracheal tube and enabled positive pressure ventilation. We were then able to perform esophageal bypass without using PCPS. Esophageal bypass surgery was performed through upper abdominal and collar neck incisions. Bypass was made using a gastric conduit through the subcutaneous route, and esophagogastrostomy was performed using a circular stapler. A decompression tube was inserted into the esophagus through the distal stump covered with the greater omentum. After the surgery was completed, the tracheal tube was safely removed under bronchoscopy. The patient was able to start oral intake on postoperative day 9, and was discharged on postoperative day 23. Though he died of respiratory failure three months after the operation, he was able to take in sustenance orally and to live at home after the operation.
This patient is a 29-year-old white woman from the USA with a medical history significant for severe IBS-D (diagnosed at age 12) and anxiety disorder. In July 2015, she presented with severe bleeding hemorrhoids secondary to IBS, which required hemorrhoidectomy and anal sphincterotomy in August 2015. The week before the surgery she developed pharyngitis and was treated with azithromycin, which resulted in mucousy diarrhea and abdominal discomfort. She tested negative for C. difficile antigen and toxins at that time.\nA week after surgery, she developed a perirectal abscess that had formed at the site of the sphincterotomy and was prescribed orally administered ciprofloxacin. Despite moderate symptom improvement, in September 2015 she required an abscess incision and drainage procedure and Penrose drain insertion. Prior to the surgery she was given a single dose of clindamycin. An additional 2-week course of ciprofloxacin and metronidazole was then prescribed. In late September 2015 she was admitted to the hospital for two nights due to further complications related to the abscess and was then diagnosed as having a perianal fistula.\nIn November 2015, she was prescribed clindamycin for an episode of group C streptococcal-positive pharyngitis. In late November 2015, she was also diagnosed as having Ehlers–Danlos syndrome, which according to her medical record may partially explain the poor wound healing from the perirectal abscess. In December 2015, her fistula required an anus seton placement. She was treated with multiple courses of ciprofloxacin and metronidazole off and on from December 2015 to January 2016.\nIn January 2016, following up on her recurrent pharyngitis, she was diagnosed as having chronic tonsillitis which led to tonsillectomy. In February 2016, 2 weeks after the surgery she was prescribed clindamycin. At the beginning of March 2016, she was diagnosed as having bacterial vaginosis and was prescribed orally administered metronidazole. A week later she was diagnosed as having vaginal candidiasis and was prescribed orally administered fluconazole. In April 2016, she complained of dysuria and was prescribed ciprofloxacin. After 2 days, when urine analysis results came back negative, she was asked by her physician to stop the treatment.\nIn June 2016, she presented for follow-up with ongoing diarrhea and abdominal pain. She was diagnosed as having C. difficile diarrhea, her antigen and toxins laboratory results were indeterminate, and a toxigenic strain was confirmed by polymerase chain reaction (PCR). She was prescribed a 6-week course of orally administered vancomycin. After a week of treatment her symptoms worsened, and following discussion with her gastroenterologist her treatment was switched to a 2-week course of metronidazole. Hours later, she was admitted to the hospital for a 4-day period for colitis. Her C. difficile antigen and toxin test returned negative during her admission. She received intravenously administered metronidazole treatment during her hospitalization. Her symptoms improved during her hospital stay, with 1–2 soft bowel movements a day. At discharge her metronidazole course was stopped and she was again prescribed vancomycin, which she took for over a month. She continued to experience GI irregularity (3–5 bowel movements a day) beyond what she had experienced secondary to her IBS prior to her surgeries. In March 2017, she was prescribed rifaximin for 2 weeks to treat chronic diarrhea.\nIn November 2017, she was prescribed a series of clinical intestinal tests (SmartGut™, uBiome Inc., San Francisco, USA) with the instructions to administer the test at home whenever she was experiencing a noticeable change of GI symptoms, then follow-up with her health care provider to discuss the results. This sequencing-based test requires that patients use a sterile swab to transfer a small amount of fecal material from toilet paper into a vial containing a lysis and stabilization buffer that preserves the microbial DNA for transport by mail back to the laboratory for processing, which involves DNA extraction, 16S ribosomal RNA (rRNA) gene amplification, and sequencing []. She first used this test in November 2017, about a month after completing a 2-week course of rifaximin. The results revealed a number of microbial organisms that were outside the healthy reference ranges, but she was negative for all pathogenic organisms included in the test, including C. difficile (Fig. ).\nBetween November and December 2017, her GI symptoms worsened considerably; her daily bowel movements increased from 3–4 to 6–10, stool consistency became more mucous-like and gelatinous, and she was experiencing more pain with defecation. She re-tested with SmartGut™ test again in January 2018. Her results continued to reveal a number of microbial organisms outside the healthy range and, this time, her sample also indicated the presence of C. difficile (Fig. ). She immediately contacted her primary care provider, who re-tested her for C. difficile and confirmed indeterminate CDI by antigen and toxins A and B. Additional PCR testing at a regional laboratory confirmed the sample was positive for a toxigenic C. difficile strain. As a result of testing, her clinician started her on fidaxomicin; her symptoms improved rapidly. By April 2018, she had returned to her baseline in regard to her IBS-related GI symptoms with no blood in her stools.\nIn addition, the second SmartGut™ sample was tested for toxins A and B by sequencing at uBiome Inc. laboratory in San Francisco, USA, which resulted positive for both and confirmed the toxigenic nature of the C. difficile strain.
A 46-year-old Mongolian male, married, migrant worker presented to our department of neurology in the university hospital for the evaluation of tingling sensation and weakness of right upper and lower limbs.\nTwo months prior to his presentation to our hospital, the patient had nausea, hiccups, and epigastric pain. These symptoms were not accompanied by vomiting. Nausea and hiccups lasted for a week and they were not so severe as to require medical attention. A week later, he started to develop tingling sensation over his right upper limb. This persisted for nearly five weeks without the development of any other symptoms. Two weeks prior to the presentation, the tingling sensation progressed to involve the right lower limb. The tingling sensation was eventually followed by weakness in both right upper and lower limbs. The weakness in his right upper limb progressed to his right lower limb within a day and, thereafter, remained static. For these complaints, he was initially evaluated at a local hospital. He gave no history of weakness or sensory disturbances involving the left upper and lower limbs. There was no history of bowel or bladder incontinence. There was no history of fever, loss of consciousness, seizure, altered sensorium, or neck rigidity. However, he gave a history of sudden onset left eye pain followed by diminished vision which subsided on treatment two years back. There were no documentations available regarding the diagnosis and treatment for the same though. He did not have any history of alcohol intake or tobacco smoking. There was also no history of any chronic medical illness.\nClinically, his general condition was fair and stable. There was no pallor, icterus, cyanosis, oedema, palpable lymph nodes, or dehydration. On neurological evaluation, his higher mental functions and cranial nerves examination were intact. Eye evaluation with fundal examination was also normal at the time of presentation. Motor examination revealed normal muscle bulk but decreased muscle tone in both right upper and lower limbs. There was diminished power across all major muscle groups in the right upper and lower limbs. Power across the proximal muscle groups was 3/5 while it was 4/5 across the distal muscle groups in both upper and lower limbs. Deep tendon reflexes in both the limbs on the right side were moderately exaggerated (+++) whereas they were slightly exaggerated (++) on the left side. Plantar response was mute with diminished pin prick and fine touch sensation on the right upper and lower limbs while there was withdrawal plantar response with normal sensation on the left upper and lower limbs. Vibration and joint position senses were intact. Lhermitte's sign was absent in this patient. His gait, however, was unsteady.\nFull blood count showed haemoglobin 14.1 g/dl, white blood cells 11,900/mm3 (neutrophils 76%, lymphocytes 22%, monocytes 1%, and basophils 1%), and platelets 152,000/mm3. His erythrocyte sedimentation rate (ESR) was 30 millimetres per hour. Biochemical evaluation showed random blood sugar level 9.7 mmol/l, urea 27 mg/dl, and creatinine 1.1 mg/dl. Urine routine and microscopic examination was normal. His vitamin B12 level was 764 pg/ml, which was within the normal range. His blood tests for human immunodeficiency virus (HIV), hepatitis B surface antigen (HBsAg), and hepatitis C virus (HCV) were negative. Venereal disease research laboratory (VDRL) test was also negative. Peripheral blood film for malarial parasite was negative. Serological evaluation for antinuclear antibodies and anti-double stranded-deoxyribonucleic acid antibodies were also negative.\nNerve conduction study revealed normal amplitude, distal latency, and conduction velocity of right median nerve, right ulnar nerve, right common peroneal nerve, and right posterior tibial nerve. MRI of his cervical spine showed T1 iso- to hypointense signal and T2 hyperintense signal in central cervical spinal cord from first to sixth cervical level, showing subtle enhancement and causing expansion of involved cord (). This was probably suggestive of myelitis or demyelination. Based on the clinical presentation and MRI findings, a provisional diagnosis of NMO was made and the patient was started on intravenous methylprednisolone 1 gram per day for three days and oral pregabalin tablets 75 mg twice a day for three days. Cerebrospinal fluid (CSF) analysis and antibody assay were not performed yet and the patient wanted to get referred to higher centre.\nAt our centre, lumbar puncture was performed which showed clear and colourless CSF, total count 25 cells/mm3 with all monomorphs, protein 15 mg/dl, and glucose 3.6 mmol/l. CSF adenosine deaminase (ADA) was within normal limit. Visual evoked potential (VEP) was also performed which indicated bilateral conduction defect. The P100 latencies were delayed for the right eye at both frequencies and for the left eye at 1 degree. The amplitudes were within normal limits for both eyes at both frequencies. Farnsworth Dichotomous Test for colour blindness indicated bilateral normal colour vision. MRI study of the brain was normal (). NMO-IgG autoantibody test was still not available in our country at the time of presentation of the patient and, hence, the CSF sample was sent to India. Immunofluorescent assay revealed strongly positive NMO-IgG antibodies in the CSF sample. CSF immunoglobulin G (IgG) level was 52.7 mg/l which was also out of range (normal reference range: 0–34 mg/l).\nHence, the final diagnosis of NMO was made. Since he had already received a course of intravenous methylprednisolone for three days, he was given prednisolone 60 mg orally for a month and then in tapering dose. Oral pregabalin 75 mg twice a day was continued. His symptoms started to resolve and was discharged after a month of hospital stay. The patient and his family members were advised for physiotherapy for muscle strengthening exercises. He was able to walk with the help of a support during his discharge from the hospital. He was under regular follow-up for the next two months during which his muscle strength constantly improved. He was lost to follow-up after two months.
An 18-year-old healthy man presented to the emergency department (ED) with 1-day history of right testicular pain and vomiting with no fever nor abdominal pain. Physical examination showed mild tenderness in the right testis, no swelling, normal longitudinal position and cremasteric reflex. Scrotal Doppler ultrasound was performed and showed reduced vascularity in the right testis and the left testis was normal. Moreover, the size and echogenicity were maintained for both testes (). Two months prior to this presentation, the patient presented with the same attack with only mild tenderness in the right testis, and all investigations were within normal limits and he was discharged with oral analgesic drugs. The decision was made to undergo surgical exploration for possible torsion/detorsion of the right testis. The patient was transferred immediately to the operating room for bilateral orchiopexy. The right spermatic cord was engorged with no evidence of ischemia identified and bilateral orchiopexy was done successfully. After the surgery, the patient was kept for observation for 24 h and then discharged. After 2 days from the surgery, the patient came back to ED with testicular pain and post-surgical swelling. We decided to do computerized tomography (CT) scan of the abdominal and pelvis to rule out retroperitoneal pathologies or referred pain. The CT showed 4 × 3 cm lobulated mass noted at right paravertebral space invading the right psoas muscle at the level of L3 and L4 (). The CT scan also showed there is an associated fistula tract between the mass and infrarenal inferior vena cava with thrombosis associated with epidural component extending through the neural foramina compressing the spinal cord from the level of the L2–L4 (). The patient was referred to spinal surgery and CT-guided paraspinal lumbar biopsy was taken. The histopathology came with the diagnosis of Ewing sarcoma. The decision was made by the medical oncology to start on a systemic chemotherapy (vincristine + Adriamycin + cyclophosphamide alternating with ifosfamide + etoposide (VAC/IE)) regimen. After six cycles, the follow-up magnetic resonance imaging demonstrated significant resolution of the right psoas and posterior paraspinal metastatic disease. In addition, further resolution with residual intraspinal extradural metastatic disease noted at right L2–3 and L3–4 levels. The patient is still followed up as an outpatient clinic with medical oncology.
A 52-year-old female presented to our polyclinic with increasing pain in the left upper quadrant in the previous month. She had been treated for hypertension and had a hysterectomy 7 years ago due to a myoma. The patient had quit smoking 3 years earlier and was taking alprazolam 0.5 mg/day for an anxiety disorder. A physical examination revealed minimal sensitivity of the left upper quadrant upon deep palpation and an incision scar from the gynaecologic surgery. Nothing else of note was detected. The patient's body mass index was 32.5 kg/m2, and laboratory measurements were within the normal range. Endoscopy and a colonoscopy conducted to determine the cause of pain revealed no pathological findings. An ultrasonic evaluation revealed no evidence of an aneurysm. However, magnetic resonance imaging (MRI) was conducted due to the presence of a hyperechoic lesion (53 × 39 mm in size) in the right lobe of the subcapsular area of the liver. The MRI study revealed a 35 mm diameter hemangioma in the liver segment 8 and a 40 mm aneurysm with a thrombus in the splenic artery of the pancreas tail region (). Tortuosity of the splenic artery, which had a proximal diameter of 1 cm, was detected. In angiographic multislice computed tomography (CT), minimal aneurysmatic dilatation was detected in the celiac truncus in the midsegment of the splenic artery, in addition to saccular aneurysmal dilatation, 50% thrombosed and in the size of 45 × 40 × 39 mm adjacent to the pancreas. A long segment of the splenic artery transection extended from the distal aneurysm to the splenic hilus ().\nFollowing a consultation with the interventional cardiovascular department, an endovascular intervention was planned for the treatment of the aneurysm. Endovascular interventions made from the femoral region were inconclusive because of extreme angulation of the truncus celiacus, aneurysmal dilatation of the trunk, and tortuosity of the splenic artery. The patient was informed about the surgical procedure, and written consent was obtained. H. influenza and pneumococcal vaccinations were performed. The gastrocolic ligament was opened by median laparotomy, and the aneurysm was revealed. In the middle segment of the splenic artery, approximately 5 cm of aneurysm, which was adherent to the upper pancreas and retroperitoneal area, was opened by controlling the proximal and distal flow of the splenic artery. Collateral flow was not monitored. The proximal and distal ends of the splenic artery were anastomosed to provide flow continuity (). The posterior and inferior wall of the aneurysm could not be resected because of excessive adhesions. The duration of the surgery was 185 min, and blood loss measured intraoperatively was 200 ml. No complications were observed in the postoperative follow-up period, and the patient was discharged without any problems on the fourth day. The patient was asymptomatic at the sixth month of postoperative follow-up. In angiographic CT, splenic artery flow was normal, and the residual aneurysm had regressed ().
A 31-year-old, otherwise healthy, young male patient presented with incidentally detected bilateral small solid renal and adrenal masses, a cystic mass at the head of the pancreas, and a paracaval mass. The patient defined no relevant family history and had no obvious symptoms. Physical examination findings (including serial blood pressure measurements) were within normal limits. Ultrasound (US) and abdominal magnetic resonance imaging (MRI) were performed to understand the nature of a recent, blunt, nonspecific upper abdominal pain and revealed 3 small, solid masses in the right kidney measuring between 1 and 4 cm and 5 masses in the left kidney measuring between 1 and 3 cm in size. All were suggestive of renal cell carcinomas (RCCs) (). An 8 cm solid mass completely replaced the right adrenal gland, while there were 5 small masses on the left side, all consistent with pheochromocytomas (). The paracaval mass, measuring 4 cm in size, could be not only an enlarged lymph node but also a paraganglioma. The imaging findings of the cystic mass at the uncinate process of pancreas were suggestive of a neuroendocrine tumor (). All other radiologic studies, including spinal and cerebral MRI, were normal. Serum and urine biochemical workup (including cortisol and vanilmandelic acid measurements) revealed normal findings regarding the functional status of the adrenal masses.\nThe patient was diagnosed as VHL disease and a staged surgical approach was recommended based on the radiologic findings. In the first stage, through a subcostal anterior transperitoneal approach, right adrenalectomy, resection of the paracaval mass, and enucleation for the 9 small solid renal masses were performed. Intraoperative frozen section findings were inconclusive about the actual biologic behaviour of the mass located in the uncinate process of the pancreas. Additionally, two other smaller pancreatic masses were palpated during the operation. Given the young age of the patient, we decided to perform a Whipple procedure to remove these pancreatic lesions. Pathologic diagnoses were Fuhrman grade 1, cystic clear-cell renal cell carcinoma (RCC) for only 1 of the renal masses (the rest were reported to be benign nodules), a pheochromocytoma (8 cm in maximal diameter) in the right adrenal completely destroying the gland architecture, a paraganglioma (2 cm in maximal diameter), and benign, well-differentiated neuroendocrine tumors of the pancreas.\nThe postoperative period was uneventful so the second stage was performed within 6 weeks. This time, through an intercostal extraperitoneal approach, 5 pheochromocytomas (largest of which was measuring 5 cm in maximal diameter) were removed in cortex-sparing fashion and 4 Fuhrman grade 2, cystic clear-cell RCCs were enucleated. Postoperative course was uneventful without any complications. After a followup of 1,5 years, the patient developed a left 1 cm renal mass, which was histologically proven by ultrasound-guided percutaneous biopsy to be a RCC and was treated by percutaneous, ultrasound-guided radiofrequency ablation. Pancreatic and adrenal functions were normal throughout the follow-up period and never necessitated any replacement therapy. Blood cortisol level (blood sample taken at 8 : 00 in the morning) was 21 μg/dL upon his last control visit. There was no adrenal or extra-adrenal pheochromocytoma recurrence being detected to date ().
A 10-year-old girl underwent application of a humeral lengthening frame of her right humerus to correct severe humeral shortening gradually. The shortening was secondary to osteomyelitis of the right proximal humerus aged 10 weeks. This had left her with a 10-cm discrepancy between the lengths of the right and left humeri, resulting in significant functional difficulties. The lengthening frame was applied with the elbow extended without any intra-operative complications. In recovery, her right hand was warm and well perfused with full sensation and movement of the wrist and small hand joints. On the first post-operative day, however, she complained of severe burning pain in her right arm and hand requiring significant opiates for relief and a pain team review. Examination revealed evidence of a partial right radial nerve palsy; weakness of right wrist extension, an inability to extend the fingers and diminished sensation on the dorsum of the first webspace. She displayed evidence of hyperalgesia with an exaggerated response to pin-prick on the dorsum of the first webspace. There was no mechanical allodynia. All other hand and wrist movements and sensation were intact.\nAn ultrasound scan of the right arm was performed. This revealed no haematoma or collection. The radial nerve was not visualised.\nOn the second post-operative day, her symptoms decreased slightly. However, on the third post-operative day, her symptoms did not settle further and the patient was taken back to theatre for exploration of the radial nerve due to ongoing severe pain. The radial nerve was found to be in continuity without any bruising or contusion. Distally, it was found to pass tightly against the most distal external fixator pin such that, with the elbow flexed the nerve became tightly pressed against the pin. The distal pin was removed and re-sited slightly proximal.\nPost-operatively, there was complete resolution of the right arm pain and hyperalgesia. Sensation to the first webspace recovered on the second post-operative day with motor function returning more gradually. There was full recovery of radial nerve motor function at 4 months post-operatively.
The first case is about a 53-year-old female patient who was admitted with the complaints of severe back pain and pelvic pain. The patient was diagnosed with type 2 diabetes mellitus 5 years back and was put on oral hypoglycemic medications, however, the patient was not taking drugs regularly and no proper blood sugar maintenance was done. She gave no history of any vertebral injury or surgeries. On admission, she was having severe muscle spasm over the lower lumbar spine with tenderness. No sensory or motor deficit could be elicited. She was febrile with body temperature of 38.2°C.\nLaboratory investigations showed random blood sugar of 450 mg/dl, glycated hemoglobin (HbA1c) of 9%, which indicated uncontrolled diabetes. Total and differential white blood cell count showed neutrophilic leukocytosis. Lumbar spine radiographs did not show any significant abnormality. CT scan of the lumbar spine showed presence of air pockets/gas within L2 and L3 vertebral bodies with extension of gas into prevertebral and bilateral paravertebral soft tissues at L2 and L3 level and also into bilateral upper psoas muscles [ and ]. There was no intradiscal gas. Hence, infectious osteomyelitis by gas forming organism was suspected and patient was advised a magnetic resonance imaging (MRI) examination of spine. MRI showed presence of altered signal intensities involving L2 and L3 vertebral bodies on inversion recovery images with associated prevertebral and bilateral paravertebral small collections extending to involve bilateral upper psoas muscles [ and ]. Multiple gas pockets were also noted within L2 and L3 vertebral bodies extending to prevertebral, bilateral paravertebral regions and into upper psoas muscles. The intervening intervertebral disc appeared normal. Hence, possibility of emphysematous osteomyelitis involving L2, L3 vertebral bodies was suggested and the patient was subjected to CT guided fine-needle aspiration cytology. The material was also sent for culture, which grew Klebsiella pneumoniae sensitive to third generation cephalosporins. The patient was started on insulin initially to control blood glucose and later switched over to oral hypoglycemic agents. Intravenous ceftriaxone 2 g/day for 4 weeks was started as antibiotic treatment, followed by oral levofloxacin 400 mg/day for 2 weeks. Patient showed marked symptomatic improvement and was discharged from the hospital after 4 weeks. Repeat CT scan of spine showed clearance of gas from L2 and L3 vertebral bodies.
A 28-year-old woman (1.65 m, 85 kg, BMI 31.2) was referred to our department five days postpartum with a bilateral femoral neck fracture.\nDuring the last two months before the delivery of her first child (begin with the sixth month of gestation), she had complained about increasing weakness and pain in both hips and thighs. In the last two weeks of her pregnancy (begin of the 35th week of gestation), she had to walk on crutches, as she was unable to bear her full weight due to the severe pain in her hips. During this time she took paracetamol daily. Her treating gynaecologist did not initiate any clarification of the symptoms by radiologic imaging methods in this period. Considering her severe pain, it was decided to deliver the child per elective caesarean section (37th +4 week of gestation). Three days postpartum, after being released from the hospital, she had an epileptic seizure and fell. She was admitted to a neurological department of another hospital. During her two-day stay in this department, the woman complained of severe pains in both hips. X-rays and a computer tomography (CT) of the pelvis were performed, revealing bilateral femoral neck fractures. Following this diagnosis, the patient was referred to our department for further treatment.\nRegarding her past medical history, she had childhood migraine and epilepsy. She suffered from her first epileptic seizure at the age of 15. She was treated with valproate over 11 years and remained seizure-free during that time. Her treating neurologist stopped the medication two years prior to her pregnancy. The patient did not have any other comorbidities. Particularly, she did not report on any past history of fractures, irregular menstruation, or family history of osteoporosis.\nAt presentation in our department, the clinical examination showed massively reduced hip movement on both sides because of severe pain. Serum laboratory examination was normal.\nBased on the age of the patient, the limited bone quality, the age of the fractures, and their displacement (Figures and ), it was decided to perform a bilateral hip joint replacement with the use of short-stemmed prostheses (). There were no intra- or postoperative complications. Both femoral heads were sent for further histopathological examination. The results of both femoral heads revealed fracture areas with hemorrhagia, proliferation of fibroblasts in the marrow cavities, and formation of focal woven bone as a sign of the bone remodelling, being indicative for a TOH. Postoperatively, the patient was mobilized on crutches under full weight bearing of the operated extremities. After consultation with our Department of Neurology, the patient restarted her valproate medication. Due to this medication, the patient was advised not to breastfeed her child. Since the TOH is regarded to be a self-limiting disorder, no specific osteoporotic treatment was applied. The further course was uneventful, and the patient was dismissed after two weeks.\nAt 1-year follow-up, the patient is free of any complaints and has no limitation in the range of motion of both hips (extension/flexion 0°-0°-100°, abduction 50°).
Our patient was a two-month-old male referred for evaluation of a left postauricular mass, present since birth. Workup by the patient's pediatrician including an ultrasound suggested a cystic mass prompting referral for surgical excision. The parents endorsed noticing the lesion at birth and that it had been painless and slowly progressive. Physical exam demonstrated a firm 2 × 2 cm subcutaneous lesion of the postauricular region. An MRI was obtained demonstrating a 2.3 × 1.4 × 2.2 cm well-defined solid mass involving the outer table of the right temporal bone and temporoparietal suture with intense peripheral enhancement and without restricted diffusion (). Initial resection in the operating room was undertaken, and a deep plane between the mass and skull was identified and followed reflecting the lesion off of the skull. Unfortunately, pathology demonstrated focal presence of tumor cells at the peripheral margin. The patient underwent a repeat resection, with a canal wall up mastoidectomy. The lesion was again resected en bloc, and the underlying cortical bone was drilled down to the inner table of the temporal bone with healthy appearing bone stock. Despite clinically normal-appearing bone, the pathology again demonstrated presence of tumor cells at the soft tissue margins, and clinically the patient demonstrated significant regrowth of the lesion. The patient returned to the operating room once more, with a fairly impressive progression of gross tumor, nearly 2.5 × 2.0 cm (). A revision mastoidectomy was performed, and neurosurgical consultation was obtained. The mass was excised en bloc resulting in a full-thickness craniectomy. The dura appeared healthy and unaffected by the tumor (). The wound was closed primarily, and the patient was observed overnight in the PICU before being discharged home postoperative day one in stable condition. The patient developed purulence at his incision site one month postoperatively requiring intra-washout with neurosurgery. The infection resolved without further complication or treatment requirement. He was seen at six months postoperatively with no evidence of disease in good condition.
We present a case of a 46-year-old African American female with a past medical history of hypertension who presented to the emergency department with concerns for pain in her eyes, hands and feet, rash, and sore throat. She reported that her symptoms started almost a week ago and were gradually getting worse. She worked at a nursing home and reported that half of the rooms were quarantined due to influenza. One week prior to presentation, she noticed she began having pain in her eyes and thought maybe she had gotten sick from one of the residents or her allergies were being triggered. She went home and took allergy medication, and went to bed. She then woke up the next morning and felt significantly worse. Her eyes were very red and were draining along with some blurring in her vision; she also reported her lips were swollen, cracked, red, and had a sore throat.\nShe reported having had two prior emergency department (ED) visits, the first one being for upper respiratory infection (URI) three weeks ago, for which she was treated with amoxycillin. The second visit three days ago was for similar complaints, during which she was found to be negative for strep throat and influenza and was discharged with an antibiotic ointment for her bilateral conjunctivitis. Her symptoms worsened over the next few days, and she began having swelling and pain in both her palms and soles of her feet. The patient's symptoms continued to worsen for the next few days, so she returned to our emergency room. In the ED, she was in significant pain and wrapped in blankets due to chills. She reported associated rhinorrhea, diarrhea, and an "itchy rash" on her back. Initial lab work was significant for an erythrocyte sedimentation rate (ESR) of 106 and a C-reactive protein (CRP) of 207.9. Her white blood cell count was elevated at 15.0, and her kidney function and liver enzymes were normal. She was given a 1 L intravenous (IV) fluid bolus and tramadol in the ED. She was also found to be negative for influenza and was admitted by our teaching service for further workup and management of her symptoms. During her hospital course, her rash appeared to worsen.\nThe rheumatology team was consulted initially and did an extensive workup, which consisted of human immunodeficiency virus (HIV) screening, antinuclear antibody (ANA), rapid plasma reagin (RPR), flu swab, anti-smith antibodies, anti-cardiolipin antibodies, monospot, SS-A and SS-B antibodies, and antineutrophil cytoplasmic antibodies (ANCA) antibodies, all of which was found to be negative. The dermatology team was also then consulted to evaluate the overwhelming skin findings. The patient had developed diffusely eroded lips and brightly erythematous oral mucosa (Figure ).\nHer eyelids were swollen and had irregular purpuric thin papules of varying sizes scattered over her legs, back, chest, and a few on the arms were noted. Some of the papules had peeled to reveal an erythematous base. Erythematous macules were scattered nearly confluently over the palms and soles (Figure ).\nThe palms and soles had multiple large deep intact bullae and near confluent erythema covering much of the plantar surface of the toes and distal sole of the foot (Figures -).\nA skin biopsy was performed, which showed full-thickness epidermal necrosis and subepidermal bullae formation (Figure ).\nShe also began to spike fevers, and her lips began bleeding and had a purulent discharge. The infectious disease team was consulted, and aztreonam was started, and clindamycin was subsequently added. She continued to spike fevers. Ophthalmology was also consulted for blurry vision and increased discharge. They recommended warm compresses and tobramycin eye drops. Due to continued fevers, an increase in lip bleeding, and concern for infection, the patient was started on vancomycin and acyclovir by the infectious disease team for possible viral infection in the setting of multiple oral mucosal lesions. Viral cultures, acid-fast bacilli culture, fungal culture were all negative, and urine was positive for Staphylococcus saprophyticus.\nIn the interim, the skin biopsy results were available, and they were found to be consistent with Steven-Johnsons syndrome vs. toxic epidermal necrolysis. The infectious disease team deescalated antibiotic therapy, and her IV antibiotics were stopped.\nThis case shows the importance of pattern recognition of Steven Johnson's syndrome, as this is a rare but serious condition that must be recognized and treated appropriately.
A 28-year-old woman (1.65 m, 85 kg, BMI 31.2) was referred to our department five days postpartum with a bilateral femoral neck fracture.\nDuring the last two months before the delivery of her first child (begin with the sixth month of gestation), she had complained about increasing weakness and pain in both hips and thighs. In the last two weeks of her pregnancy (begin of the 35th week of gestation), she had to walk on crutches, as she was unable to bear her full weight due to the severe pain in her hips. During this time she took paracetamol daily. Her treating gynaecologist did not initiate any clarification of the symptoms by radiologic imaging methods in this period. Considering her severe pain, it was decided to deliver the child per elective caesarean section (37th +4 week of gestation). Three days postpartum, after being released from the hospital, she had an epileptic seizure and fell. She was admitted to a neurological department of another hospital. During her two-day stay in this department, the woman complained of severe pains in both hips. X-rays and a computer tomography (CT) of the pelvis were performed, revealing bilateral femoral neck fractures. Following this diagnosis, the patient was referred to our department for further treatment.\nRegarding her past medical history, she had childhood migraine and epilepsy. She suffered from her first epileptic seizure at the age of 15. She was treated with valproate over 11 years and remained seizure-free during that time. Her treating neurologist stopped the medication two years prior to her pregnancy. The patient did not have any other comorbidities. Particularly, she did not report on any past history of fractures, irregular menstruation, or family history of osteoporosis.\nAt presentation in our department, the clinical examination showed massively reduced hip movement on both sides because of severe pain. Serum laboratory examination was normal.\nBased on the age of the patient, the limited bone quality, the age of the fractures, and their displacement (Figures and ), it was decided to perform a bilateral hip joint replacement with the use of short-stemmed prostheses (). There were no intra- or postoperative complications. Both femoral heads were sent for further histopathological examination. The results of both femoral heads revealed fracture areas with hemorrhagia, proliferation of fibroblasts in the marrow cavities, and formation of focal woven bone as a sign of the bone remodelling, being indicative for a TOH. Postoperatively, the patient was mobilized on crutches under full weight bearing of the operated extremities. After consultation with our Department of Neurology, the patient restarted her valproate medication. Due to this medication, the patient was advised not to breastfeed her child. Since the TOH is regarded to be a self-limiting disorder, no specific osteoporotic treatment was applied. The further course was uneventful, and the patient was dismissed after two weeks.\nAt 1-year follow-up, the patient is free of any complaints and has no limitation in the range of motion of both hips (extension/flexion 0°-0°-100°, abduction 50°).
In September 2014, a 12-year-old boy presented to the University of Minnesota Masonic Children’s Hospital emergency department with a recent history of neck pain and progressive weakness of his right arm. His symptoms began 1 week prior to admission when he woke from sleep with neck stiffness. The following day, he experienced nausea with emesis and was noted to have an oral temperature of 38.6°C. Neck stiffness worsened over the next few days with new-onset intermittent headaches. Upon initial presentation to the emergency department, weakness was noted and he had difficulty turning his head to the left, keeping it upright and raising his right arm. He did not have sensory changes. No other neurological deficits were noted. He had no rash, upper respiratory infection symptoms, joint swelling, sore throat, cough, abdominal pain, or dysuria. In the 6 months prior to the onset of illness, he had been camping in Central Minnesota and had traveled to the United States Virgin Islands and the Amazon rainforest in Ecuador. There was no history of a prior tick bite.\nAt the time of hospital admission, vital signs were normal but neurological examination was significant for absent muscle stretch reflexes in his right upper extremity. He had profound weakness in the proximal muscles and milder weakness in the distal muscles of the right upper extremity. Notably, he had less than antigravity strength in shoulder abduction, elbow flexion, and elbow extension and reduced strength ranging 3 to 4 on the Medical Research Counsel scale for muscle strength in the forearm and intrinsic hand muscles. He had severe weakness in his right sternocleidomastoid muscle resulting in the head drop and tilt to the right. Because of weakness, he used his stronger left hand to turn his head to the left. He had no sensory deficit, long corticospinal tracts signs, or sphincter dysfunction. A complete metabolic profile, an erythrocyte sedimentation rate, and a C-reactive protein were normal. A complete blood count and differential leukocyte count demonstrated that his white blood cell count was normal at 8.2 ×103/µL. His hemoglobin concentration was 16.1 g/dL, and the lactate dehydrogenase was slightly elevated at 205 U/L. Radiographic studies of the cervical spine were normal. Cerebrospinal fluid (CSF) examination demonstrated a lymphocytic pleocytosis with a white blood cell count of 71/µL. Magnetic resonance imaging (MRI) findings on the day of admission demonstrated abnormal cervical spinal cord signal, with hyperintensity extending from C1 level to the C7-T1 level, with associated cord expansion noted in sagittal T2-weighted images. Predominant involvement of the gray matter in the spinal cord was also noted in the axial T2-weighted image. These findings were diagnostic for transverse myelitis involving the C1-T1 segments with cord edema and preferential involvement of the gray matter ( and ).\nIn light of these findings, the patient was commenced on acyclovir for possible herpes simplex virus infection and ceftriaxone for possible Lyme disease. He was also treated with high-dose methylprednisolone, 10 mg/kg/d, for a 5-day course. A second MRI, 4 days after admission, showed significant improvement in the hyperintensity of T2 signal and edema ( and ). Additional MRI of thoracic spine revealed mild T2 hyperintense signal in the mid and lower thoracic cord, which was prominent in the central location extending from approximately T7 to L1. These findings had no clinical correlate. The patient’s motor deficit remained restricted to only his right upper extremity. He was discharged 5 days after admission with recommendations for intensive outpatient physical therapy.\nAfter discharge, he experienced progressive improvement in his right arm function. Upon evaluation 22 months after the onset of acute flaccid myelitis, he achieved good functional recovery but had significant residual weakness and atrophy, mainly limited to upper cervical segments C1-C5 on the right side evidenced by residual atrophy and weakness in the sternocleidomastoid, trapezius, and muscles innervated by the C5 segment including the deltoid, supraspinatus, infraspinatus, and to a lesser degree the biceps brachii. He recovered completely from deficits in elbow flexion and extension and regained normal strength in distal muscles.\nThe patient underwent an extensive laboratory investigation to identify an etiology for his acute flaccid myelitis, including HSV1/2 polymerase chain reaction (PCR) and serology, mycoplasma serologies, neuromyelitis optica IgG, dsDNA abs, arbovirus IgG/IgM serologies, varicella zoster virus PCR, a respiratory viral panel, antinuclear antibody screen, PPD, CMV, EBV, HHV6, human immunodeficiency virus, California encephalitis IgG, West Nile virus, Lyme IgG/IgM assays of cerebrospinal fluid and serum, and lymphocytic choriomeningitis virus serologies. These tests were all negative. A viral stool culture was, however, positive for enterovirus and preliminarily subtyped at the Minnesota Department of Health as echovirus 11. The sample was sent to the Centers for Disease Control and Prevention (CDC) for additional testing, which confirmed that the virus was echovirus 11.
A 78-year-old male was admitted to hospital for abdominal distension and pain. The patient had no history of epigastralgia or peptic ulcers and there was no significant relevant family history. A physical examination revealed a 20x12 cm palpable mass in the middle and lower abdomen, with minimal intrinsic mobility. Carcinoembryonic antigen (CEA), cancer antigen (CA) 724 and carbohydrate antigen (CA) 19-9 levels were normal. A computed tomography (CT) scan revealed a 21x14 cm heterogeneous mass extending from the fundus to the mid-abdomen, with a number of solid and cystic components within the tumor (). A gastroscopy revealed an intraluminal ulcer in the lower posterior wall of the gastric antrum; biopsy specimens were obtained. No metastatic lesions were identified in any other organs in either the abdominal ultrasonography or the CT scan. A biopsy confirmed the presence of an adenocarcinoma of the stomach with poor to moderate differentiation. A laparotomy revealed an extremely large tumor (22x15 cm) arising from the transverse mesocolon and adjacent to the greater curvature of the stomach. The mass was in close apposition to the pancreas and duodenum. There was no evidence of invasion or underlying peritoneal infiltration. The mass was resected en bloc with part of the transverse colon, and distal gastric resection was performed ().\nMacroscopic examination of the distal gastrectomy specimen revealed a Borrmann type II tumor in the stomach, measuring 3x3 cm. On histopathological examination, the tumor was identified to be an adenocarcinoma with poor to moderate differentiation that exhibited transmural infiltration (). No nerve or vascular invasion was evident. Two lymph node metastases were detected in 28 retrieved lymph nodes. According to the TNM classification, the gastric carcinoma was stage IIIA. Histopathological examination demonstrated that the extremely large mass was mainly comprised of epithelioid cells, and revealed focal necrosis, fibrosis and hemorrhagic areas (). Mitotic figures were recognized in 15 out of 50 high-power fields. Immunohistochemical analysis revealed that CD117 () and desmin () were positive in the neoplastic cells. However, tumor cells exhibited negative expression for CD34, S-100 and smooth muscle actin (SMA). Based on these results, the diagnosis was an EGIST of the transverse mesocolon that had not originated from the digestive tract. The postoperative period of the patient was uneventful; the patient was discharged two weeks after surgery. The patient was started on adjuvant chemotherapy with a FOLFOX regimen: oxaliplatin IV, 85 mg/m2 (on day 1); leucovorin IV, 200 mg/m2 (on days 1 and 2); 5-FU (fluorouracil) IV, 400 mg/m2 (on days 1 and 2) and 5-FU IV 22 hours in fusion, 600 mg/m2 (on days 1 and 2). This regimen was repeated every 2 weeks for 6 cycles. Additionally, the patient was simultaneously administered imatinib at a dose of 400 mg/day for 1 year. No evidence of tumor recurrence was identified after 24 months of follow-up.
A 38-year-old female presented to our hospital with complaints of pain in the upper abdomen for six months with increased intensity since last one month which was not related to food intake and was not radiating to the back. She had no symptoms of jaundice and fever. On abdominal examination, there was no palpable mass or other significant findings. There were no abnormal findings in the laboratory test. USG abdomen revealed cystic dilation of CBD with the normal gall bladder. Further evaluation with magnetic resonance cholangiopancreatography (MRCP) abdomen revealed dilated cystic structure seen in the region of CBD measuring about 55 mm with smooth distal tapering suggestive of CDC type 1a according to Todani classification (Figure ).\nIntraoperatively gall bladder was mildly distended with no calculi. Cystic dilatation of suprapancreatic part of CBD around 6 cm in largest dimension with dilated and tortuous cystic duct of around 2 cm opening into it (Figure ).\nWe performed a simple cholecystectomy with choledochal cyst excision with Roux en Y Hepaticojejunostomy. Post-operatively she was started orals on POD 2 and was discharged by POD 5. The post-operative pathology report showed features of choledochal cyst with chronic inflammatory pathology without evidence of malignancy. After a follow-up of six months, the patient is doing well without any morbidity.\nAnother case was a 23-year-old female with complaints of only pain in the right upper abdomen for four months, with no symptoms of jaundice and fever. Clinical examination revealed no significant findings. Laboratory investigations revealed no abnormality. On initial evaluation with ultrasonography of the abdomen showed a hypoechoic lesion in the region of GB neck with no gall stones. On further evaluation with MRCP was found to have a cystic lesion at the GB neck with a normal CBD. Preoperatively she was diagnosed as type 2 CDC. Intraoperatively, it was an isolated cyst of the cystic duct (type 6A) of size 2 × 2 cm2 with a narrow opening at its juncture with CBD, and CBD was normal (Figure ). Laparoscopic simple cholecystectomy was done. Post-operative histopathology report showed cyst wall lined by biliary epithelium and peribiliary glands with acute or chronic inflammatory infiltrate without any evidence of malignancy. The post-operative period was uneventful. She is doing well after five years of follow-up.
A 29-year-old male presented with pain in the left axillary area and ecchymosis preceded by a snap while lifting 200 kg in bench press three days earlier. He admitted a previous history of anabolic steroid use, with the last consumption being six months earlier.\nPhysical examination revealed an extensive hematoma and swelling in the medial side of the left upper arm and absence of the anterior axillary fold with pain in that area. Shoulder range of motion, both passive and active, was complete.\nPatient had recorded the training exercise so we could observe the injury. He was laying down on the bench press with an assistant by his head, and he was being recorded from his left side. He was lifting exactly 212.5 kg, and during the third repetition, at the beginning of the eccentric phase, the loss of the natural contour and immediate medial retraction of the PM muscle can be observed (video 1 in Supplementary Material available online at ).\nPlain radiographs did not show any abnormality. To determine the extension and localization of the rupture, ultrasound (US) and magnetic resonance imaging (MRI) studies were performed. US were interpreted as a probable partial tear of the left PM tendon, while MRI reported on a rupture of PM muscle at myotendinous junction with medial retraction of the inferior portion of the muscle belly.\nThe patient was treated surgically, thirteen days after the injury. Under general anesthesia on a beach chair position, we performed a modified deltopectoral approach. A rupture affecting the musculotendinous junction was confirmed intraoperatively. The medial stump was identified and controlled with a total of three threads in a Krackow fashion from three corresponding suture anchors. Three holes were drilled lateral to the bicipital groove just where the native footprint was located. The three threads were firmly tightened passing the sutures through the drilled holes with the arm adducted and tied with simple knots.\nThe patient was postoperatively immobilized in a sling. The second week after surgery, he was allowed to start a passive range of motion; on the third month, he started with resisted motion exercises. On the fourth month, he had returned back to his normal physical activity. In the last follow-up, one year after surgery, he did not mention any pain and was satisfied with the aesthetic. The range of motion regarding the affected shoulder was normal and he had started performing some weightlifting, nevertheless not lifting so much weight as before.
A 70-year old woman was admitted from a dermatologist to the Department of Plastic and Breast Surgery, Roskilde, Denmark. A biopsy verified well-differentiated full-thickness squamous cell cancer was found at the right side of the lower lip. The tumour had been present for three years. The patient had a history of ischemic heart disease with a percutanous coronary intervention and implantation of a stent in 2002. She was a heavy smoker. No alcohol overconsumption was reported. By clinical examination, a 15 × 15 mm sore, firm, central ulcerating tumour was seen located at the vermillion border (). At the mucosal side of the lip, leukoplakia was observed.\nNo enlarged lymph nodes in the cervical region was found at the clinical examination.\nSurgical intervention would remove approximately 50% of the lower lip. The patient was offered radiotherapy as an alternative to surgery and chose the former. She underwent a series of radiotherapy for a period of two months.\nFive weeks after the initial clinical examination at our clinic, the patient was readmitted to our department from the Department of Oncology at Copenhagen University Hospital due to recurrence of the squamous cell cancer located to the lower lip. At this point, the patient presented a monstrous, ulcerating tumour, involving most of the lower lip (). No clinical signs of systemic spread to lymph nodes in area was found by thorough clinical examination and palpation of the head and neck. Furthermore, a 18FDG-PET-CT was performed and ruled out further local or systemic spread.\nFirst, a total excision of the lower lip including the commisures during which free resection borders of 1 cm were confirmed by intraoperative frozen section diagnosis. 8 days after primary excision, histopathology confirmed free resection borders, and reconstruction of the lower lip by bilateral Fujimori technique was performed (, , ). Due to paucity of mucosal tissue between the orifice of the parotid duct and commisures of the mouth besides a relatively narrow labial sulcus, the remaining lack of mucosal lining was reconstructed by use of split skin harvested from the right thigh.\nAt a clinical follow-up three months after surgery, palpable enlarged, firm lymph node was found located to the lower jaw. Biopsy confirmed the histological diagnosis of metastasis from squamous cell carcinoma. Position Emission Tomography Scan (PET) confirmed the localisation of metastasis from a squamous cell carcinoma on the left side of the jaw, but as well on the right side. No distant metastases were found. Clinical presentation 5 months after surgery is seen in .\n5 ½ months after the initial admission to our department, bilateral neck dissection was performed. Several metastases with extensive perinodular growth was found on the right side, and infiltrating underlying musculature on the left side. The patient was referred to the Department of Oncology, Herlev Hospital, Denmark, where radiotherapy was given.\nOne months after completed radiotherapy, the patient presented in our clinic a very sore, ulcerated tumour at the jaw line on the left side. The tumour was adherent and dripping with what appeared to be saliva. Punch biopsies were not representative. A computer tomography (CT) scan confirmed local recurrence of aggressive squamous cell cancer. The patient was referred for more extensive surgery and further treatment, which is why eventual defatting of the lip was postponed.
Our patient was a two-month-old male referred for evaluation of a left postauricular mass, present since birth. Workup by the patient's pediatrician including an ultrasound suggested a cystic mass prompting referral for surgical excision. The parents endorsed noticing the lesion at birth and that it had been painless and slowly progressive. Physical exam demonstrated a firm 2 × 2 cm subcutaneous lesion of the postauricular region. An MRI was obtained demonstrating a 2.3 × 1.4 × 2.2 cm well-defined solid mass involving the outer table of the right temporal bone and temporoparietal suture with intense peripheral enhancement and without restricted diffusion (). Initial resection in the operating room was undertaken, and a deep plane between the mass and skull was identified and followed reflecting the lesion off of the skull. Unfortunately, pathology demonstrated focal presence of tumor cells at the peripheral margin. The patient underwent a repeat resection, with a canal wall up mastoidectomy. The lesion was again resected en bloc, and the underlying cortical bone was drilled down to the inner table of the temporal bone with healthy appearing bone stock. Despite clinically normal-appearing bone, the pathology again demonstrated presence of tumor cells at the soft tissue margins, and clinically the patient demonstrated significant regrowth of the lesion. The patient returned to the operating room once more, with a fairly impressive progression of gross tumor, nearly 2.5 × 2.0 cm (). A revision mastoidectomy was performed, and neurosurgical consultation was obtained. The mass was excised en bloc resulting in a full-thickness craniectomy. The dura appeared healthy and unaffected by the tumor (). The wound was closed primarily, and the patient was observed overnight in the PICU before being discharged home postoperative day one in stable condition. The patient developed purulence at his incision site one month postoperatively requiring intra-washout with neurosurgery. The infection resolved without further complication or treatment requirement. He was seen at six months postoperatively with no evidence of disease in good condition.
A 45-year-old woman presented to medical attention for abdominal pain, episodic diarrhea, and a mild weight loss (<10% body weight). Informed consent was obtained from patients included in the study. All procedures performed were in accordance with the ethical standards of our institutional research committee. She underwent a colonoscopy that showed a 4 cm stenosis of the sigmoid colon, covered with pale mucosa that could not be passed by a conventional colonscope (13.2 mm) but could be passed with an enteroscope (9.2 mm) with which the cecum could be reached; the mucosa of the other colonic tract, as well as the mucosa of the terminal ileum, were unremarkable. Random ileal and colonic biopsies were obtained, which were unremarkable as well. Histology of the stenosis showed mild inflammation, mild stromal fibrosis, and architectural disruption. Subsequently, she underwent a computed tomography scan, which showed a mild thickening of the terminal ileum and sigmoid colon. The finding of thickened terminal ileum was further confirmed by a subsequent magnetic resonance imaging enterography, suggestive for Crohn disease (CD) localized at the terminal ileum. Due to the discrepancy of the radiological findings with the initial endoscopic examination, a further colonscopy with retrograde ileoscopy was performed, showing no visible endoscopic signs of inflammation and confirming the sigmoid stenosis. The repeated ileal and colonic biopsy sampling was again not diagnostic, showing a nonspecific pattern of inflammation affecting the sigmoid colonic specimens.\nThe physician in charge of the patient, in view of her clinical history and the radiologic finding of intestinal thickening, reckoned that the patient was affected by CD with terminal ileum and sigmoid colon localization and proposed a therapy immunomodulators: corticosteroids 1 mg/kg for 12 weeks together with antitumor necrosis factor (TNF) infliximab 5 mg at weeks 0, 2, and 6, followed by infliximab alone. After 6 months of treatment with infliximab (5 mg/kg every 8 weeks as maintenance), an endoscopic reevaluation was scheduled, showing a resolution of the stenosis in the sigmoid colon and a normal-appearing terminal ileum. The marked improvement of the sigmoid inflammation together with the finding of fibrosis on the corresponding histology further convinced the physician in charge of the patient in favor of a diagnosis of CD, and the anti-TNF treatment with infliximab 5 mg/kg was continued. After 3 months (9 months after the beginning of anti-TNF), the patient was admitted for obstructive symptoms. A computed tomography suspected neoplasia of ileocecal region. The patient underwent an uneventful ileocecal surgical resection. The surgical specimen consisted of a portion of 8 cm of terminal ileum with the cecum and the vermiform appendix. The ileal wall showed an increased consistency and a diffuse thickening, with cystic-hemorrhagic foci in the perivisceral fat. The ileal mucosa was corrugated and showed a polypoid structure with a wide base (1.5 cm maximum diameter) originating from the submucosa and determining ulceration of the mucosal layer. The ileocecal valve, the cecum, and the appendix were normal, except for a mild lipomatous filling of the submucosa.
We describe a healthy and active 58-year-old man, who four months prior to presentation noticed that he was gaining weight, in spite of participating in aerobic exercise daily and having no change in diet. Three months prior to presentation the patient began noticing an increase in abdominal girth, stating that it, ‘felt like a large ball in my belly. The patient began to develop occasional abdominal discomfort, which slowly increased in pain and frequency. The patient also developed symptoms of constipation and severe gastroesophageal reflux, for control of which, he began taking laxatives and a proton pump inhibitor. He eventually saw his primary care physician and had a computer tomography (CT) scan done, showing a large spherical cystic mass in the abdomen measuring 32 cm by 25 cm (Figure ). The CT scan findings in short were of a massive uniloculated retroperitoneal cyst seen non- communicating to the pleura, or any other abdominal viscera and occupying nearly the entire abdominal space, displacing his entire bowel to the patient’s left, with the cecum and ascending colon located anterior and left of the midline. The mass spanned from the pelvis inferiorly to the liver hilum superiorly. The liver and stomach were compressed. The patient was referred to a surgeon and the recommendation was made to proceed with surgery after complete evaluation. He has no risk factors related to the mesothelioma (exposure to asbestos or any virus) or any malignancy. We checked other risk factors including occupational (exposure to silica or cadmium), family history, social (alcohol intake and smoking) or history of trauma.\nIn the operating room, after a midline laparotomy, a massive retroperitoneal cyst was discovered to span nearly the entire abdominal width, displacing his entire bowel to the patient’s left, with the cecum and ascending colon located anterior and left of the midline. The mass spanned from the pelvis inferiorly to the liver hilum superiorly. The colon was dissected away from the mass and two layers of pseudomembrane were dissected off the anterior surface of the cyst. The mass was then dissected off the retroperitoneal organs using blunt dissection as there was only loose areolar tissue connecting the structures (Figure ). An initial attempt was made to remove the entire cystic mass intact, however the thin cyst wall ruptured and 15 liters of serous fluid was aspirated. There was no evidence of a tract or origin to the cystic structure. Grossly the mass appeared uniloculated with a smooth thin wall and straw-colored fluid. Microscopically, the sections showed a benign multicystic lesion lined by cuboidal cells which stained positive for pancytokeratin, calretinin, and D2-40 and negative for CD34. We are following our patient every 3 months and performed CT imaging before every visit for the first two visits, and then before every fourth visit. CT from the second clinical visit shows no recurrence and the patient has returned to his normal active state (Figure ). There is no recurrence on post-operative visits.
A 72-year-old male presented to emergency department with tender mass on lower back and posterior aspect of neck. Apparently, he had noticed it on his lower back 6 months ago and had grown in size with another similar swelling appearing on his neck, also they were increasingly painful and sore to touch. He had a history of ongoing sore throat for 3 years for which he was extensively investigated by ENT services. His CT neck and mediastinum did not show any abnormalities at that time. This was followed by laryngoscopy which showed a white patch on the right vocal cord, biopsy of which showed non-specific chronic inflammatory changes and cultures grew candida. Background history included COPD; he was heavy current smoker and smoked about 60 cigarettes per day. His medications included salmeterol/fluticasone propionate and ipratropium bromide inhalers. Vitals were within normal range. On examination, 4 cm nodular, firm, tender, immobile mass on lower back and 3 cm mass on posterior aspect of lower cervical spine were noted.\nHis laboratory investigation including renal and liver function, full blood count and coagulation profile were normal. A CT of thorax, abdomen and pelvis was arranged which showed 2 × 2 cm mass in right lung lower lobe likely malignant with no hilar or mediastinal lymphadenopathy, also 3 × 3 cm soft tissue mass at C6-C7 level posteriorly and 4 × 2 cm mass was noted on lumbar region with multiple prominent inguinal lymph nodes ().\nA referral for bronchoscopy was sent and biopsy of the nodule was planned. While awaiting bronchoscopy appointment, biopsy was taken from mass in lower back and send for histopathology. Histology showed poorly differentiated tumour cells in cohesive groups and sheets with moderate pleomorphism and eosinophilic cytoplasm; immunohistochemistry was positive for AE1/3, cytokeratin 7 and carcinoembryonic antigen antibody stain and periodic-acid schiff. Weak nuclear positivity for TTF-1 was also seen. Overall features were consistent with poorly differentiated adenocarcinoma likely of lung origin.\nHe was then referred to oncology services and unfortunately passed away within 5 months of diagnosis.
A 54 year old female Caucasian patient presented to the orthopaedic outpatient clinic complaining of left arm and left knee pain. She had been diagnosed with malignant melanoma of the palpebral and forniceal conjunctiva 2.5 years before and had received treatment at the regional ocular oncology centre in the form of surgical excision, cryotherapy and adjuvant Mitomycin C.\nDuring her oncological follow up 30 months following the original diagnosis, multiple bilateral pulmonary metastases all measuring <0.5 cms in diameter as well as a 4 × 2 cm right ischial lesion were identified. She was complaining of left arm and left knee pain and was therefore referred to the orthopaedic clinic.\nThe pattern of the pain was constant with occasional nocturnal exacerbations and was aggravated by weight bearing activities of both extremities. Clinical examination demonstrated localized areas of tenderness over the midshaft area of the left humerus as well as over the anteromedial aspect of the left proximal tibia. Furthermore a non-fluctuant swelling measuring 2 × 2 centimeters in diameter was palpable over the corresponding left proximal tibial metaphysis. All neighbouring joints examined normal. Her right ischial lesion was asymptomatic.\nRadiographic evaluation demonstrated lytic lesions of the left humeral diaphysis and proximal tibial metaphysis with associated cortical involvement and ill defined zones of transition, however no evidence of fracture (Figure ). A bone scan confirmed the presence of three active areas corresponding to the lucent radiographic areas of the left humerus, left tibia and right ischium. It did not identify any further skeletal lesions\nRegarding her osseous lesions she underwent intramedullary nailing procedures of both long bones on a semi-elective basis due to the danger of impending fracture. According to the Mirels classification for metastatic bone disease at risk of fracture, [] she scored 9 points for the humerus and 10 for the tibia.\nA standard reamed and locked nailing technique was applied for stabilization of both bones with no associated intra-operative complications (Figure ). Venting of both bones was performed during the procedure in an attempt to reduce intra-osseous pressure and prevent the risk of fat and tumour embolisation. Intramedullary reamings, as well as specimens obtained via open curettage of the radiologically suspect lesions were sent to the histopathology department.\nThe patient had an uneventful recovery and was discharged home within 4 days. Histological examination confirmed the presence of devitalized bone and extensively necrotic, heavily pigmented epithelioid tumour, consistent with a diagnosis of metastatic malignant melanoma. There was a low mitotic count (1/mm2) and no significant lymphocytic infiltrate (Figure ).\nAt her latest follow up visit in the orthopaedic clinic 6 months post-operatively, she remains pain-free with no radiographic evidence of further metastatic spread of the involved bones. Further oncological input consisted of palliative radiotherapy to her right ischial and left humeral lesions, as well as bisphosphonate administration. No conjunctival recurrence has been identified 3 years following the original diagnosis.
A 38-year-old male presented to the surgery outpatient department with complaints of odynophagia and chest pain for two weeks. The symptoms had started after a meal when the patient felt the sensation of a foreign body in the throat, which he reportedly swallowed with a bolus of food. As the initial evaluation with neck and chest X-rays was reported as normal, he did not receive any specific intervention. However, in the third week after ingestion, he developed chest pain and fever. On evaluation with a contrast-enhanced thorax, chest, and abdomen scan, he was diagnosed with a right-sided pleural effusion and a suspected foreign body in the periesophageal region of the lower thoracic esophagus close to the inferior vena cava (Figures , ).\nFrom the hiatus region, the foreign body was located at approximately 5 cm cephalad. An intercostal drain was placed under image guidance and approximately 750 mL of serosanguinous pleural effusion was drained. An upper gastrointestinal contrast study did not show any contrast extravasation from the esophageal lumen. Upper gastrointestinal endoscopy also did not show any signs of recent perforation or a foreign body in the esophagus. An after adequate chest optimization, he underwent laparoscopic foreign body removal.\nThe procedure was done under general anesthesia in the supine split leg position. After initial access and the creation of a pneumoperitoneum through the left paramedian 12 mm port using an open method, five additional 5 mm ports were placed, including two midclavicular line ports on either side, a left subcostal port for assistance, and an epigastric port for liver retraction. The gastrohepatic ligament was divided to reach the right crus. The phrenoesophageal membrane was then opened to enter the mediastinum. The esophagus was then looped with an umbilical tape at the level of the hiatus and used for subsequent retraction. The right side of the esophagus was dissected meticulously, preserving the vagus nerves. At about 6 cm from the gastro esophageal junction, there was a fibrotic tract along the lateral aspect, which was then cut with scissors exposing the 2.7 cm long metallic foreign body (Figure ).\nRight pleura were thickened and were not opened. The foreign body was then extracted and removed under vision (Figure ).\nThe tract, which was seen extending to the esophagus, was clipped on the esophageal side using a hemolock. A thorough mediastinal lavage was given and a 16 Fr suction drain was placed in the paraesophageal region. An upper gastrointestinal oral contrast study that was done on the second postoperative day did not reveal any contrast leak, and the patient was started on an oral diet. He had an uneventful postoperative course and was discharged on the fifth postoperative day.
A 35 years old male patient had undergone the operation of abdominal aorta artificial vascular replacement because of MFS and abdominal aortic dissection aneurysm in March 2005 (Fig. A). In December 2012, he had experienced sudden, severe chest pain and was readmitted to our hospital. The aortic contrast-enhanced computed tomography (CT) showed a sign of Stanford A type aortic dissection (Fig. B). The echocardiography revealed aortic sinus expansion with moderate or severe aortic valve insufficiency. The patient underwent second operation of aortic valve replacement, ascending aortic replacement and descending aorta-nasal stent implantation and was discharged on the seventh postoperative day. The patient came back to our hospital for a follow-up with aorta computed tomography angiography (CTA) on September 4th, 2018. The CTA revealed that the descending aorta has an aneurysmal dilatation for a 12.4 cm length. The cross-section diameter of the widest part was about 74 mm × 55 mm (Fig. C). The patient was hospitalised for the third time and was diagnosed with thoracic aortic aneurysm.\nOn September 11th, 2018, the patient underwent his third operation of TEVAR, we chose the transfemoral approach and inserted 2 consecutive Medtronic endovascular stent grafts (42 × 150 mm and 34 × 150 mm) (Fig. A, B). The proximal end of the stent was located in the lower of the left subclavian artery and the distal end lies above the celiac trunk artery. The position of the stents was normal by arteriography. The celiac trunk artery and the superior mesenteric artery were unobstructed; and there had no internal leakage between the connections of the stents (Fig. C). The patient was admitted to the ward of ICU after the operation and had no severe complications. The patient was discharged on the seventh day after surgery. Patient was regularly followed up in the outpatient department of our hospital, CT examination on January 21, 2019 found that the stents were in good position and no internal leakage was observed.
A 48-year-old Greek female patient was referred to our institution for the management of biliary trauma after laparoscopic cholecystectomy. In her medical history, she had had two laparotomies: an ileoanal anastomosis with j pouch for ulcerative colitis 15 years ago, while 5 years ago she was driven to the operating room after a colonoscopy for peritonitis. Due to non-specific upper gastrointestinal symptoms she had had an upper abdominal ultrasound (US) that revealed cholelithiasis (at least two gallstones of diameter 0.8 and 0.7 cm) 2 months before surgery and elective laparoscopic cholecystectomy was performed 18 months before her referral to us.\nThe duration of laparoscopic cholecystectomy was 150 minutes while the procedure was completed laparoscopically and there is no record of intraoperatively identified biliary injury. From the 2nd postoperative day and up to her referral to our institution, recurrent episodes of cholangitis with severe pain, fever with chills and jaundice began. Magnetic resonance cholangiography (MRC) was performed in order to delineate the biliary anatomy and assess the level of injury. A triple bile duct injury, with right and left hepatic duct ligation and common bile duct cross-section, was revealed (Bismuth type V, Figure ). Attempts at permanent biliary decompression with repeated endoscopic retrograde cholangiopancreatographies (ERCP), combined with percutaneous transhepatic duct catheterization failed and for 1 year postoperatively bile drained from abdominal drains. On the 13th postoperative month, she was referred to another hospital for biliary draining (Figure ). Through the left drain, a guidewire was passed only to be found later during ERCP in the duodenum in a place other than the papilla of Vater via a false route. On the next episode of cholangitis, both left and right biliary trees were successfully decompressed and 18 months after LC, she was referred to our hospital for surgical reconstruction.\nThe intraoperative findings were as follows: the hepatic duct was cut and double ligated with clips, the right anterior hepatic duct was closed with an endo-clip, the right posterior hepatic duct was cut and ligated with one endo-clip, the left hepatic duct was cut and had a catheter passing through a partially open clip via a false route to the duodenum while the right hepatic artery was clip-ligated as well. After partial resection of segment IV of the liver, extensive dissection of the biliary tree was performed and then a plastic reconstruction of all major right hepatic ducts to a "common" right hepatic duct was performed with PDS 6-0. Finally, Roux en Y hepaticojejunostomy of both the newly formed common right hepatic duct and the left hepatic duct was performed at separate sites. The catheter in the left hepatic duct was left in place while the one in the right hepatic duct was removed. Three months after the operation, cholangiography showed patency of the left and right hepatic ducts (Figure ) and after removal of the remaining stent, the patient has had no complaints. The liver function tests have so far returned results within the normal limits.
An 8-year-old girl weighing 28 kg was scheduled for elective multiple dental abscess drainage and rehabilitation. She was diagnosed with Rett syndrome when she was 9 months old and currently suffered from refractory epilepsy and global developmental delay. Because of communication impairment associated with her developmental disability, it was imperative to involve her mother during the entire perioperative course.\nIn her preoperative examination, she appeared drowsy and spasticity was noted in her limbs. Mallampati score evaluation was not possible. She only responded to her parents and exhibited discomfort from the pain she was experiencing from her intraoral abscess. She was maintained on several antiepileptic medications: topiramate 25 mg, clobazam 5 mg, levetiracetam 500 mg, lacosamide 50 mg and oxcarbazepine 300, each of the above given twice a day. Her epilepsy had worsened with increased frequency and was thought to be aggravated by the pain that her intraoral abscess was causing. She exhibited epilepsy during chewing due to pain it elicited. Her laboratory tests including coagulation function and electrolytes were normal.\nAfter obtaining informed consent from her parents, the patient was kept nil by mouth for 6 hours after solid food before surgery and no premedication was administered. The antiepileptic medications were given at 6 am in the morning with sips of water and she was scheduled for the surgery at 8 am. We planned to perform an opioid- and muscle relaxant free-balanced general anaesthesia with nasotracheal intubation.\nIn the operating room, the standard American Society of Anesthesiologists monitors were connected to the patient. She was pre oxygenated and anaesthesia was induced with sevoflurane in air and oxygen via face mask. Intravenous access was secured after achieving inhalational induction, followed by administration of 700 mg of magnesium sulphate as an IV infusion over 30 min (1.5 mL of 50% MgSO4 added in 15 mL of 5% dextrose). Gentle nasotracheal intubation was performed under propofol 85 mg and lidocaine 25 mg 1.5 mL 2% lignocaine diluted to 10 mL in normal saline) IV without the use of any muscle relaxant. The vocal cords were anaesthetised using 0.5 mL of 2% lidocaine-adrenaline spray during direct laryngoscopy. A 5 mm ID cuffed endotracheal tube was used followed by throat pack insertion. anaesthesia was then maintained on 50% oxygen in the air and 2% sevoflurane. This was supplemented by buccal infiltration of 5 mL of 2% lidocaine with 1:200,000 adrenaline by the surgeon. She was also administered 500 mg of acetaminophen IV for analgesia.\nThe procedure took approximately 120 min. Vital signs were stable throughout the procedure. The estimated blood loss was less than 10 mL. After the return of spontaneous ventilation, the throat pack was removed, the trachea was extubated and the patient was transferred to the paediatric intensive care unit (PICU) for postoperative care. For postoperative analgesia, the patient was administered 60 mg of diluted IV diclofenac twice a day and 500 mg of IV acetaminophen four times a day.
A 36-year-old male patient visited our department with a complaint of replacement of missing teeth. The patient was otherwise in a normal health condition. On clinical examination, the right maxillary first molar was absent. In addition, a supernumerary premolar-like tooth, distal to the right maxillary second premolar was observed, as well as a second premolar in the left maxilla was missing. The upper third molars in both sides were erupted and well aligned in the arch, while the mandibular third molars in both sides were not found clinically. In the lower right quadrant, a total of four, well-aligned, premolar-like teeth were observed. The tooth distal to the canine appeared as the first premolar, the tooth distal to that appeared as a mini-sized second premolar, and the following two teeth had the same anatomy and size with the second premolar. Thus, it was difficult to distinguish them as rather permanent or supplementary. In the lower left quadrant, the first premolar had caries and the second premolar was retained root state, followed by two more teeth (). Due to the destruction of the crown, it was difficult to distinguish which was the permanent or the supplementary tooth. There was no relevant familial history of dental abnormalities. Panoramic radiograph confirmed these clinical findings and additionally revealed an impacted supernumerary tooth mesial to the first molar in the left mandible (). There was no impacted lower third molar in the mandible.\nIt was decided not to extract the erupted supernumerary teeth since the patient had no complaint and the occlusion was not impeded. However, before giving prosthesis in the upper right first molar region, alignment of the supernumerary had to be considered. The patient was referred for endodontic treatment of the caries lesion of the lower right premolar and to an oral surgeon for extraction of the retained root. Also, the opinion on the impacted supernumerary tooth was requested if it could affect the lower left first molar or the impacted supernumeraries would develop into cystic lesions.
A 26-year-old woman using soft contact lens for the correction of mild myopia presents to emergency department for evaluation of a foreign body sensation of her right eye. On history, she reports standing in close proximity to a construction worker breaking a tile wall, when she felt a sudden onset of foreign body sensation. On initial examination, the patient's visual acuity was 6/6-OD and 6/6 OS. The intraocular pressure was 13 on the right eye and 15 on the left. A slit-lamp examination of her left eye was within normal limits. Her right eye examination was notable for mild conjunctival injection, a thin self-sealing corneal wound. This wound was initially estimated as a linear erosion of 2.5 millimeters in length located in the upper nasal quadrant and was Seidel negative. The anterior chamber depth was normal with few cells and no glair. The iris showed no disruption. The lens was clear, and examination of the posterior segment was unremarkable. Due to a high degree of clinical suspicion, a gonioscopic examination was performed showing a small dark foreign body embedded in the inferior chamber angle at 7 o'clock. An entry point was visible on the soft contact lens used at the time of injury confirming that the entrance wound was at the cornea.\nA noncontrast CT scan (NC-CT) of head and orbits revealed a regular border radiopaque foreign body in the chamber angle, anterior to the lens. The patient was taken for surgical intervention. Removal of the foreign body via limbal incision entering on the superior aspect was complicated by disappearance of the object from the surgeons view. The procedure was stopped, and the patient was taken immediately for another NC-CT with concern for passage of the IOFB into the posterior segment. The CT demonstrated the IOFB resting in the zonules area adjacent to the lens (). 24 hours later using a slit lamp with gonioscopy, the location of the IOFB was marked by an intentional corneal abrasion followed by a fluorescein staining. A bilevel wide opening above the marking exposed the foreign body, allowing for its uncomplicated removal. The IOFB was identified by a forensic lab as an iron. Two months following surgery, an unintentional bleb was observed (). Anterior segment optical coherence tomography (ASOCT) showed a scleral flap covered by conjunctiva (). The patient reported that she was unable to wear her contact lens, likely secondary to this finding. Despite normal intraocular pressure, a surgery for resecting the bleb was scheduled. However, surgery was canceled due to acute conjunctivitis. Six months later, the patient reported improvement, and examination revealed a significant flattening of the bleb, which allows for contact lens use. Of note, the patient's visual acuity was 6/6 OU.
A 44-year-old Japanese male, an office worker, was transported to our emergency department with a complaint of temporary loss of consciousness. He had experienced fatigue for 3 years and had fallen easily in his daily life starting 2 months before admission. He was feverish and had a cough and phlegm for several days before admission. On the way to work, he felt drowsy and fell down the stairs of the train station, necessitating transport to our hospital by ambulance. The peripheral blood analysis noted mild elevation of white blood cells. The blood chemistry test disclosed mild elevation of C-reactive protein and mild liver dysfunction (). He was thin, i.e., his height was 174 cm and he weighed 52 kg (). Chest computed tomography (CT) showed severe infiltration in the upper posterior fields of both lungs as well as food debris in the esophagus. He was diagnosed as having aspiration pneumonia. His past medical history included diabetes mellitus that had been treated by a local doctor; glycated hemoglobin was approximately 7%. He had experienced ileus six times since age 30 years. He was not married. His father had died of dilated cardiomyopathy at the age of 70 and had also been thin. On the other hand, his mother was healthy but his maternal uncle was diabetic. His younger sister (Case 2) also had mild muscle weakness of the four extremities. Aspiration pneumonia was treated by intravenous administration of antibiotics under conditions of food-take restriction combined with temporary insulin infusion, and the respiratory symptoms subsided. On the neurological examinations after improvement of pneumonia, he presented with typical clinical manifestations of DM1 (), such as forehead balding, hatchet face with bilateral ptosis, nasal speech, mild muscle weakness of the four extremities, handgrip myotonia, and diffuse muscle atrophy. Electromyography performed to test the biceps brachii muscle and femoral quadriceps muscle on the left side revealed frequent myotonic discharges. Because DM1 was strongly suspected, we recommended that, after providing informed consent during genetic counseling, he undergo genetic testing for DM1 together with his younger sister. The number of CTG repeats in the DMPK gene was abnormally expanded to about 600 repeats in the elder brother and about 900 repeats in the younger sister, in contrast to those of healthy individuals who have 5 to 37 repeats (), thereby confirming the diagnosis of DM1 in these siblings ().
A 22-year-old, right-handed young man who had been practicing soccer at a competition level sustained a left shoulder injury in September 2015 when playing soccer. He described two successive traumatisms: First, he has been hit laterally, with his arm at his side, by another player and immediately experienced intense pain. Then, he fell onto this shoulder, which was injured again. He did not report any sensation of dislocation. This shoulder had never before been injured or painful. Initially, he was conservatively managed by his coach (i.e., he wore a sling and took some medication). However, after a few days, he felt that persistent pain and loss of function were unusual and sought a medical consultation. At that time, 10 days after the trauma, pain was located posteriorly, exacerbated by palpation and in an armed position. Anterior and posterior apprehension tests were both negative. Passive and active shoulder motions were normal. Active external rotation, with the arm at side and in abduction, was painful. There was a slight asymmetry of strength in external rotation compared to the contralateral side, but it was not obvious and could be explained by the pain. There were no hornblower or dropping signs. Passive internal rotation was difficult and painful as well. Radiographs were judged to be normal (). Ultrasound suggested a posterior cuff lesion without concluding in a precise diagnosis. MRI confirmed a very unusual isolated tear of the TM at the myotendinous junction without any other associated lesions (). There was no labral, capsular, or bony lesion in favor of a posterior instability. The other tendons of the rotator cuff all remained inserted. An arthro-computed tomography scan was consecutively performed and did not reveal any contrast dye moving throughout the joint, thus confirming the integrity of the articular capsule and comparable to what has been described in the case of IS myotendinous ruptures (). Knowing the natural evolution of this kind of lesion (based on what we know from IS lesions) [, ] and the potential functional consequences for the shoulder in this young patient, we hypothesized that it was preferable to repair this lesion early. We ultimately performed an open surgical repair after a 3-week delay following the initial trauma.
A 44-year-old Hispanic male with no known history was brought to the emergency department (ED) via Emergency Medical Services (EMS) after being shot in the chest, abdomen, and left shoulder. With EMS, the patient was conscious and had a stable pulse. In the trauma bay, the patient became unresponsive with a loss of pulse, and CPR was initiated. Upon physical examination, the patient was observed to have one high-caliber GSW to the left upper quadrant of the abdomen, one high-caliber GSW to the left periumbilical region of the abdomen, one high-caliber GSW to the fourth intercostal space of the left chest just medial to the midclavicular line with an expanding hematoma, and one high-caliber GSW to the left shoulder with a floating left shoulder. In the ED, a left thoracotomy, along with a central line and endotracheal intubation, was performed to release a pericardial tamponade that was likely caused by gunshot trauma to the heart. Cardiac massage and MTPs were initiated and an aortic cross-clamp was placed. Palpable pulses were achieved with the support of cardiac massage and MTPs after the release of a pericardial tamponade via pericardiotomy and multiple rounds of epinephrine. Following this ED course, the patient was immediately transported to the operating room (OR) for DCR surgery and MTP.\nUpon arrival in the OR, the patient was aggressively resuscitated with high doses of epinephrine for vascular support. The thoracic cavity was surgically explored first. The patient was unstable due to active bleeding from a left hilar and left upper lobe injury. A left lung hilar clamp was placed to control the active bleeding while a tractotomy repair of the left lung injury was completed. The left hilar lung clamp was then released with minimal active bleeding from the site of the lung injury. Once this bleeding was controlled, an inspection of the remainder of the thoracic cavity began. At this time, the ascending arch and the descending portions of the aorta appeared intact. Upon further inspection, there was a hematoma and injury to the left subclavian vein which was immediately packed with gauze, and pressure was applied. Inspection and irrigation of the remainder of the thoracic cavity revealed no additional hemorrhagic injuries. During this time, the patient also received massive transfusions of pRBCs, platelets, and plasma.\nImmediately after stabilizing the thoracic cavity of any hemorrhaging, exploration of the abdominal cavity commenced with a midline exploratory laparotomy incision. A liter of clotted blood was discovered upon opening the abdominal cavity. The four abdominal quadrants were initially inspected and significant injury to the omentum was found. Solid organs were inspected next, with no injuries observed to the liver, kidneys, spleen, or pancreas. Finally, the bowel was run from the distal esophagus to the colon with no evidence of injury along the alimentary tract. After careful inspection, the abdomen was packed to provide hemostasis for the omental injuries, and attention was focused back on the chest.\nSignificant oozing and bleeding from a tributary of the left subclavian vein were noted from the left shoulder upon reinspection of the chest. This tributary was clipped to control the active bleeding. Following this, the soft tissue bleeding surrounding the left chest and shoulder region was controlled via electrocautery and suture ligature. Further inspection of the left shoulder indicated an extensive left shoulder fracture with significant bone loss. Additionally, there was significant bleeding from the bone fracture itself. This was also packed with gauze for hemostasis, and a bullet fragment was removed from the left shoulder. After controlling the bleeding left chest and shoulder, a reinspection of the entire thoracic cavity indicated no further hemorrhage from the noted sites of injury or any additional sites.\nAfter confirming that the patient was hemodynamically improving with high doses of epinephrine and continuous MTPs via arterial blood gas (ABG) and vital sign monitoring, the decision was made to attempt to release the aortic cross-clamp. To begin, the intrathoracic aortic cross-clamp was only partially released with good toleration from the patient. As the patient continued to tolerate this partial release, the sequential decision was made to release the intrathoracic aortic cross-clamp completely. At this time, the patient continued to hemodynamically tolerate the complete release of the cross-clamp; however, he was observed to have decreased chest movement of his right chest. A right thoracostomy tube was placed with 900 cc of bloody output. Attention was focused back on the abdomen for a reinspection that indicated no bleeding in addition to the ligated omental bleed. The abdominal cavity was then left open, packed with a wound vac sponge, and sealed with a wound vac in accordance with damage control surgery protocol. A reinspection of the thoracic cavity indicated no additional obvious bleeding, and hemodynamic values via vitals and ABG monitoring were stable at this point. The decision was made to close the thoracic cavity with the consent of the cardiothoracic surgeon who had arrived for a consultation. Two left chest tubes and one right chest tube were placed upon closure of the thoracic cavity.\nNotably, throughout the DCR surgery, serial ABGs were monitoring the progress of the MTPs and trending the pH and coagulation values. In total, the patient was transfused with 18 units of pRBCs, 12 units of fresh frozen plasma, and three units of platelets with epinephrine and calcium for support of MTPs. CBC, coagulation, and ABG monitoring findings are listed in Table . During this operation, a continuous cardiac massage and warm saline were also administered to the heart. Additionally, an external convective warming blanket and additional cloth blankets were utilized, and the operating room temperature was raised to 80°F to prevent hypothermia and counteract the onset of this part of the lethal triad. The patient’s estimated blood loss was 5 L and 150 mL of urine was produced by the end of the surgery. The patient was also noted to be positive for coronavirus disease 2019. Radiological findings are demonstrated in Figures -. Surgical visualizations are demonstrated in Figures -.
A 42-year-old El Salvadoran woman presented to the ED after a syncopal episode. The patient's symptoms began with left sided chest pain radiating to her left arm followed by dizziness. She had loss of consciousness for 5 minutes and had suffered multiple syncopal episodes with increasing frequency over the past year. She was advised to see a cardiologist for slow heart rate but did not follow up. She denied palpitations, vision or hearing changes, or seizure activity.\nThe patient grew up in rural El Salvador and immigrated to the United States 10 years ago. Of note, her sister of 40 years of age, who currently lives in El Salvador, also recently began suffering similar episodes over the past 2 years.\nOn examination, her blood pressure was 105/66 mmHg and was found to be bradycardic; her heart rate fluctuated between 30 and 42 beats per minute. The patient's lungs were clear. There was no lymphadenopathy or peripheral edema. Investigations showed negative serial troponin levels and normal hematologic and blood chemistry tests. The patient's electrocardiogram was remarkable for sinus bradycardia, with conserved axis and segment intervals and no other conduction abnormalities (). Chest X-ray was normal. Her cardiac ultrasound showed an ejection fraction of 62% (). The patient was diagnosed with symptomatic sinus bradycardia. A pacemaker was placed.\nDuring her initial hospital course, a first Chagas serology test was ordered given the fact that the patient had no other identifiable causes or risk factors for cardiomyopathy beyond the epidemiological context from which she came. This result came back positive after discharge, and the patient was followed up as an outpatient for a confirmatory second serology, which was also positive. Without any other clear etiology, it is suspected that Chagas was the cause of the cardiomyopathy.\nIt was agreed upon by the medical team that, given the seropositive status of the patient with only electrocardiographic evidence of disease and no appreciable cardiomegaly or congestive changes, antiparasitic treatment should be offered. This issue was discussed at length in follow-up visits with the patient. While she initially expressed interest in receiving pharmacological treatment, she has been persistently hesitant to commence therapy. This remains an active discussion with the patient.
A 5-year-old girl was referred to the Department of Pediatric Dentistry, Faculty of Dentistry, University of Medical Sciences, Kerman, Iran with the chief complaint of pain in tooth #55. Her mother had no history of diseases or consumption of any medications during her pregnancy. The child had been born by caesarean section and was the fourth and last child of the family. The mother and father of the child were, respectively, 34 and 41 years old at pregnancy. There was no history of genetic or dental disorders or anomalies in the familial history. The parents were cousins on the maternal side. The child exhibited normal height with below normal weight curve. The patient had normal skin, hair, and general health, with no history of hereditary or acquired medical conditions. Extraoral examinations did not reveal any asymmetry or facial edema. The results of complete blood count (CBC), Alkaline Phosphatase, Ca, P, ferritin and fasting blood sugar (FBS) tests were within normal range. Intraoral examinations did not reveal any oral lesions. Examination of the teeth revealed severe destruction of the deciduous teeth on the right maxillary quadrant () and the teeth #51 and #52 had fistulas.\nPeriapical and panoramic radiographs were prepared. In all the primary teeth on the maxillary right quadrant, the pulp chambers and root canals were so large and a very thin layer of hard tissue was seen around the root canals and pulp chambers. The permanent teeth in the same quadrant exhibited the same configuration ().\nBased on clinical and radiographic findings, the problem was diagnosed as regional odontodysplasia. With both patient’s and her parents’ consent, the primary teeth on the maxillary right quadrant were extracted due to the pain and infection during a period of 6 months. A temporary acrylic resin prosthetic appliance with a bite plane was fabricated to preserve the alveolar ridge during the period of skeletal growth ().\nPeriodic clinical examinations were scheduled due to the possibility of involvement of the permanent teeth on the affected side, as well as for monitoring the eruption of permanent teeth and development of the maxillary arch. Oral hygiene instructions and dietary counseling were performed. The patient was kept under follow-up visits until she was 10 years old. The panoramic radiograph, taken during the periodic examinations, showed the involvement of permanent teeth on the right maxillary side ().\nHaving obtained the patient’s and parents’ consent, the maxillary right lateral incisor was extracted due to severe pain and fistula. The surgically-removed tooth was histologically examined under an optical microscope in the ground and decalcified section. In most areas, dentin was atubular with many areas of amorphous material (). Based on the histopathological features, the provisional diagnosis of regional odontodysplasia was confirmed.
A man in his 60s had been diagnosed with coronary spastic angina by coronary angiogram with spasm provocation when he was in his 50s and was being treated with medications (nifedipine Controlled release (CR) 40 mg/day, nicorandil 15 mg/day). He had experienced no chest symptoms since starting the medications. No significant ST changes had been noted on previous periodic electrocardiographic examinations (). The patient was fitted with a Holter monitor as regular testing for sinus bradycardia (Rubenstein type I) in November 2020. The patient experienced no chest symptoms during the recording. Next day, after removing monitoring device, he was subsequently seen on an outpatient basis. Electrocardiography for regular check-up showed giant negative T waves in multiple leads (), and echocardiography showed decreased wall motion on the apex (, ). A blood sample showed mildly elevated troponin T (0.109 ng/dL). Based on the diagnostic assessment, acute coronary event was suspected, and the patient was immediately hospitalised. Emergent coronary angiography showed no significant coronary artery stenosis (). Left ventriculography of right anterior oblique) view showed a narrow neck and apical ballooning during systole (, ). Left ventriculography of left anterior oblique view showed that apical wall motion was severely decreased while basal portion of left ventricular septum and posterolateral wall normally contracted (). Takotsubo syndrome was considered a possible diagnosis, and other diseases were excluded according to the guideline. As the patient did not experience any physical or emotional stress and had no chest symptoms during the clinical course from before onset to hospital discharge, it was concluded that he had asymptomatic takotsubo syndrome.\nHolter monitoring performed before admission showed a consistent sinus rhythm; no significant arrhythmias were recorded. Holter monitoring showed repeated ST elevations for 20–60 min, followed by a return to the baseline and the gradual formation of negative terminal T waves. Every time ST changes repeated, T wave inversion becomes deeper and deeper and the giant negative T wave was gradually formed in a period of half a day ().
A previously healthy 42 years old male presented to our institute with history of gradually progressive and painless swelling over left calf since two months. He was a non-smoker, laborer by occupation. The patient noticed a firm swelling in calf region of left leg 5 years ago. No history of trauma or infection prior to the appearance of the mass was reported. No family history of any such swelling in the past. Patient was operated for swelling over calf region 20 years back but no records were available. There was a history of gradual increase in deformity of left foot since 3 years and the patient had started walking on toes on left side with no dorsiflexion at ankle joint.\nOn examination, there was a single, non-tender, hyperpigmented scar of size 8×3 cm over mid-calf region fixed to underlying structures. A large, well defined non-tender, firm, swelling was palpable in posterior aspect of left leg measuring about 28×8 cm extending from tendoachilles region up to 5 cm distal to popliteal fossa and medially and laterally up to border of tibia and fibula respectively. The overlying skin was normal with no discoloration and local raise of temperature. Movement of knee joint was normal. There was fixed equinus deformity of left foot (). No inflammatory signs, skin changes or adenopathies were present. No bruits were heard on auscultation. Neurovascular examination of left leg and foot was normal. Laboratory findings were within normal limits. Radiological examination revealed large soft tissue mass with linear and streak-like ossification around the left tibia. MR Angiography () showed arteriovenous malformation in left calf with multiple feeding arteries arising from popliteal, peroneal and anterior tibial artery and large draining veins draining deep into venous system of leg. The tibia and fibula marrow showed normal signal intensity.\nBecause of patient symptoms and with clinical diagnosis of a vascular malformation, a wide surgical excision of the lesion was done. Through a 25 cm longitudinal incision across the calf, posterior compartment muscles were exposed. The mass was found completely involving superficial group of posterior compartment muscles sparing the deep compartment with no attachment to periosteum or bone (). Peroneal artery and vein were found to be embedded in the lesion and thus sacrificed. Plane of dissection was between superficial and deep muscles.\nThe lesion was completely removed along with overlying cutaneous scar with wide surgical margins leaving posterior tibial artery in continuity. Intraoperative, complete dorsiflexion of foot was achieved with intact vascularity of leg. The excised specimen was very hard like bone and had to be cut longitudinally with saw (). Grossly the resected specimen showed ossified tissue covered with skin and soft tissues including muscle, tendons and adipose tissue measuring 15×7×5 cm. The cut surface of the ossified area was grey white, gritty and congested (). Microscopically, it revealed features of a vascular malformation with numerous blood vessels of variable size and shape composed of arteries and veins which were dissecting soft tissues and interstitial planes of skeletal muscle.\nMany of the vessels were thin walled with anastomosing and a sinusoidal appearance. Some of them showed fresh and organized thrombi within this vascular background, extensive osseous metaplasia characterized by mature lamellar bone formation was seen. (). The diagnosis was consistent with arterio-venous malformation with extensive osseous metaplasia. At the time of recent follow up after one year from the operation, no local recurrence of the tumor was demonstrated clinically and radiologically. No restriction of motion of ankle joint was found. Patient is presently walking with a normal gait.
A 16-year-old post pubertal female with Angle's class I malocclusion with bimaxillary protrusion presented to our department for orthodontic treatment. According to the protocol of our department, all the essential orthodontic diagnostic records including facial photographs [] were taken and analyzed. Treatment plan for this patient was fixed orthodontic mechanotherapy with extraction of all four premolars and retraction of anterior teeth. As with conventional orthodontic mechanotherapy retraction of canine takes around five to seven months. To reduce the treatment time, we have decided to retract the canine by distraction device. Patient was informed about the treatment procedure, and a written informed consent was obtained.\nThe individual canine distractor was a tooth-borne, semi-rigid device. After the bands were fabricated for the canine and first molar, an impression was obtained, the bands were transferred into the impression material, and the study cast was made. The device consisted of an anterior section, a posterior section, a screw, and a hex wrench to advance the screw []. A 360° activation of the screw produced 0.5 mm of distal movement in the canine tooth.\nAfter the first premolar extraction, vertical osteotomies were carried out at the buccal and lingual sites of the interseptal bone adjacent to the canine tooth. The vertical osteotomies were connected with an oblique osteotomy extending toward the base of the interseptal bone to weaken the resistance [Figures and \n]. The distractor was cemented in place after the surgery. Slight swelling was observed on the patient's face after the surgery, and it was resolved after three to five days, postoperatively.\nThe distraction was initiated just after the surgery. An advancement of 0.5 mm was performed per day, until each canine tooth was distracted into the desired position in two weeks. The patients were closely monitored during the distraction period, and at the time of the activation of the device to retract the canine, patient often felt pain, but pain was subsided after 5 to 10 minutes of the device activation. Intraoral photographs of a patient, before and after rapid canine distalization, are shown on right side [Figures and ] and on left side [], in the mandibular arch. Initially, we have planned the distraction of maxillary and mandibular canines simultaneously, but our canine distractor was bulky, as we have used custom made distractor which was inexpensive. So, we planned to distract the maxillary and mandibular canine separately.
A 43-year-old male patient presented with metastatic PTC. He had been diagnosed with PTC in February 2004 and underwent total thyroidectomy at another hospital. High-dose (150 mCi) RAI therapy was performed twice in May 2004 and July 2006. In April 2007, right neck lymph node dissection was performed due to the discovery of recurrent PTC in cervical lymph nodes. Despite the application of additional high-dose RAI therapy in August 2007, hematogenous lung metastasis was observed in April 2009, and a fourth course of RAI therapy was performed in July 2009.\nNevertheless, chest computed tomography (CT) in February 2010 showed interval increase in the size of multiple hematogeneous metastasis in both lungs. For the further treatment, he was transferred to our hospital. On the basis of chest CT scans () performed in August 2010, the disease was classified as progressive disease according to the Response Evaluation Criteria in Solid Tumors (RECIST) criteria. Additionally, two measurable lesions in the right gluteus muscle () were confirmed to be metastatic PTCs by ultrasound-guided needle biopsies (). Increase in serum thyroglobulin (Tg) level was also noted after he was transferred to our institution. Considering changes in both imaging studies and serum Tg level after four cycles of RAI therapy, the disease was considered as refractory to RAI therapy. Therefore, the patient was recruited for an investigational study of sorafenib, which had been approved by the institutional review board, and received his first medication in September 2010.\nAfter 2 months of sorafenib administration (800 mg per day), a follow-up chest CT scan showed slight decreases in metastatic lung and muscle lesions compared with previous examinations (). Because there was no evidence of disease progression or newly developed lesions revealed by imaging studies, which were repeated every 2 months, sorafenib was maintained at a full dose.\nUntil August 2011, after 11 months of sorafenib administration, multiple metastatic lesions in lung parenchyma and in the right gluteus muscle showed little interval change. However, chest CT scan revealed marked increase in the size of the subcarinal and right interlobar nodes, which had previously been stable, and a newly enlarged right paratracheal lymph node. In follow-up chest and abdominal CT in October 2011, though multiple metastatic nodules in both lungs showed stabilization in size, progression of mediastinal lymph nodes and multiple new hepatic metastatic masses were identified. In addition, a lumbar spine magnetic resonance imaging scan, obtained due to back pain, revealed extensive metastatic lesions of thoracic spine, lumbar spine, sacrum, and pelvis, which were not identified in previous chest or abdominal CT scans. Because the disease had progressed, palliative treatments, such as external beam radiation of the lumbar spine, were planned.\nOn October 8, 2011, radiotherapy of the metastatic lesions of the lumbar spine and the sacrum was initiated. In addition, sorafenib was discontinued on October 17, 2011. After the first fraction of radiotherapy, the patient visited an emergency room for dyspnea and fever on November 2011, after 17 days sorafenib discontinuation. A chest CT showed marked progression of metastatic lesions in the lungs, which had been stable under sorafenib, and the development of pleural metastasis with bilateral effusion (). To relieve the symptoms, the pleural effusions were drained using a pigtail catheter. On the 12th day of hospitalization, a liver biopsy was performed for further evaluation and management. The histopathology, which was supported by immunohistochemical staining (), was consistent with the anaplastic transformation of follicular cell-derived carcinoma []. In addition, BRAF V600E mutation was found in the tissue.\nCT pulmonary angiography performed on the 13th hospital day revealed marked progression of lung, pleural, and mediastinal metastases compared with a chest CT scan obtained 2 weeks prior. No pulmonary thromboembolism was identified. The patient died on the 14th day of hospitalization due to respiratory failure.
Presenting concerns This is a case of a 22-year-old white female who presented at 34 weeks with preterm premature rupture of membranes. She had a history of two prior cesarean deliveries. A left ovarian tumor, not noted during the course of her current pregnancy, was noted on cesarean delivery. Current pregnancy When she presented with preterm premature rupture of membranes, repeat cesarean section was performed again. At the time of surgery, another 8 cm mass on her left ovary was diagnosed intraoperatively. This mass was not diagnosed prior to surgery. A second trimester ultrasound was performed that did not show any ovarian cyst. During the surgery, the mass appeared tan with punctate focal hemorrhage. The mass was unable to be separated from her left fallopian tube, so the mass, the left fallopian tube and part of the left ovary were all removed. We were able to leave a small amount of residual ovarian tissue. Pathologic description noted a multiloculated and cystic mass with clear mucinous fluid consistent with mature cystic teratoma (Figure ). Her postoperative course was uncomplicated. Previous pregnancy Her antecedent pregnancy was complicated by right ovarian torsion at 13 weeks secondary to an ovarian tumor. A right salpingo-oophorectomy was performed. The right ovary was sent to pathology and noted to be infarcted with no viable tissue from the mass to make a definitive diagnosis (Figure ). During that same surgery, an 8 cm, irregularly shaped mass with a focally hemorrhagic surface was noted on the left ovary. A left ovarian cystectomy was performed, and final pathology showed a benign mucinous cystadenoma (Figure ). At the time of cesarean in the same pregnancy, a metachronous mucinous cystadenoma was noted on her left ovary; cystectomy and partial left oophorectomy were performed. Follow-up and outcomes Following her third cesarean delivery, this patient was surprised to learn that she had developed another ovarian cyst in pregnancy necessitating removal. She was concerned about the function of
On the 26th of April 1999, a 38-year-old Nigerian male presented at our dental clinic for the evaluation of a right mandibular swelling that had been progressively increasing in size for the preceding 18 months, when it was first noticed by the patient. The patient’s chief complaint was a swelling which was asymptomatic. On examination, there was a bony hard swelling extending from the lower right canine to the lower right first molar, with an overlying clinically normal oral mucosa. There was both lingual and buccal expansion of the mandible. Apart from the swelling, the patient appeared clinically healthy. The patient had a full complement of teeth with mobile lower right premolars and lower right first molar. The medical history was insignificant. The clinical differential diagnoses were ossifying fibroma and ameloblastoma. An incisional biopsy was performed, which was reported as basaloid squamous carcinoma.\nThe financial implication of surgical management made the patient delay treatment for 8 years. During this period, he lost his job as a factory worker, due to irreconcilable social embarrassment secondary to the facial mass.\nThe patient volunteered that the lower right premolar, lower right first and second molars had exfoliated and the tumor had increased in size. There was an associated history of occasional pain, pus and bloody fluid discharge. The patient had been treated at a private clinic with antibiotics.\nOn examination, the lesion now extended from the lateral incisor to last molar. It was covered by an erythematous oral mucosa that bled readily on palpation but was not tender on palpation. The swelling measured about 26×20 cm in its greatest diameter. Radiographical examination revealed a multilocular radiolucent lesion which extended from the lateral incisor to the angle of the mandible. Mandibular resection was done with iliac bone reconstruction in April 2008. The surgical procedure was well-tolerated, the post-surgical course was uneventful and no recurrence has been reported.\nMicroscopic examination of the surgical specimen showed features of plexiform ameloblastoma [] , displaying extensive squamous metaplasia with abundant keratin pearl formation []. In addition, some areas of lamellate parakeratin deposition within the connective tissue stroma displayed an appearance reminiscent of Pacinian corpuscles []. There was no papilliferous component to the neoplasm and no inflammatory reaction to the stromal deposition of parakeratin. There was focal cystic change within the epithelial islands and extensive areas of myxoid stroma change. A diagnosis of keratoameloblastoma was made. Microscopic review of the initial biopsy specimen showed features consistent with this final diagnosis [].
A 53-year-old man, who had undergone sternoturnover for pectus excavatum as a child, was diagnosed with acute myocardial infarction, and percutaneous coronary intervention was performed as the acute treatment of the culprit lesion. Two drug-eluting stents were implanted at the aneurysmal site of the left anterior descending artery and the high lateral branch. However, coronary angiography after coronary intervention demonstrated residual stenoses of the left anterior descending artery, high lateral branch, and left circumflex artery, and intravascular ultrasound revealed stent malappositions at the sites of the coronary aneurysm (Fig. ). Because the duration of patency of the stent implanted within the coronary artery aneurysm was unpredictable and a residual lesion was present, he was referred to our department for CABG. Echocardiogram showed left ventricular dysfunction with an ejection fraction of 19%. Enhanced computed tomography revealed bilateral occlusions of the internal thoracic arteries and a small fragile sternum after sternoturnover fixation (Fig. ). Considering postoperative respiratory dysfunction associated with instability of the thorax following median sternotomy, low left ventricular function, and left coronary artery as the only target vessel, we selected left thoracotomy for CABG without cardiopulmonary bypass.\nThe left radial artery and great saphenous vein were harvested with the patient lying supine under general anesthesia. He was then placed in a semi right lateral decubitus position (Fig. ). The thoracotomy was made by a left anterolateral incision at the fifth intercostal space. The pulmonary ligament was dissected to open a route for the grafts. The pericardium was opened posterior to the left phrenic nerve, and the target vessels were exposed. After systemic heparinization, the saphenous vein graft was anastomosed to the descending aorta using the PAS-Port System (Cardica Inc., Redwood City, CA, USA). The venous graft was sequentially anastomosed to the left circumflex artery and high lateral branch. The radial artery graft was anastomosed proximally to the venous graft and distally to the left anterior descending artery. The patient had an uneventful recovery and was discharged on postoperative day 12. Postoperative enhanced computed tomography proved all bypass grafts patent (Fig. ).
48-year-old, tall, and slim male had a fall at home following an attack of seizures, with resultant injury to the right hip region leading to pain and inability to bear weight on the same side. Prior to injury the man could walk normally. He did not suffer from any other comorbidities. His pedal pulses were palpable and showed no signs of ischemic injury to the limb. The plain radiograph of the right hip showed a displaced unstable intertrochanteric fracture right hip (). Surgical fixation of the fracture was deferred considering his neurological condition; hence, he was managed conservatively with boot and bar application with traction onto right foot. He declined the use of plaster boot due to claustrophobia and cut open the cast himself. He was then put on skeletal traction through an upper tibial skeletal pin.\nOn day 14 following the injury, he complained of severe excruciating pain in the anteromedial aspect of the right thigh, below the inguinal region with an associated globular swelling over the same location, of the size of a cricket ball, not associated with any appreciable thrill and bruit. He gives history of swelling showing diurnal variation, with an increased size in the morning. He was managed conservatively with pain killers and hot fomentation, considering it to be an organized hematoma. In the following 2 months, there was an improvement in the pain over size of swelling, with a more worrisome developing pallor. The hemoglobin levels were found to drop progressively, pondering to find the cause for it.\nTwo and a half months following the injury he was referred to a tertiary care centre. Color Doppler ultrasonography right thigh revealed a pseudoaneurysm arising from profunda femoris artery and its branches. Fresh radiograph revealed a right sided, nonunited intertrochanteric fracture with a greater tuberosity fracture missed initially.\nHe was managed in a staged fashion. First, to begin with, an endovascular repair of aneurysm arising from profunda femoris artery was done (). Conventional angiography () revealed a pseudoaneurysm located over the posteromedial aspect of the femur though a posterolateral location has also been reported in a case of proximal femoral nailing for intertrochanteric femoral neck fracture []. There were no symptoms of peripheral ischemia following the surgery. A week later he was treated for the hip fracture with a DHS fixation with TBW (tension band wiring) with added autologous bone grafting (). Postoperatively on day 2 the drain was removed and patient was mobilized out of the bed. Nonweight bearing walker aided ambulation was begun. He was refrained from weight bearing on operated limb and crossed leg maneuvers. The patient is under followup at present, pain free, and walking unaided.\nWe bother the readers to note the possible occurrence of such aneurysms in proximal femur fracture, which need not mandatorily be of early onset. An association of seizure disorder and its medications (tab. valproate 500 mg twice daily and tab. eptoin 100 mg thrice daily dosage) with such an osseovascular traumatic event is unlikely but does need further investigation.
The patient was a 64-year-old man with destructive injury of both lower extremities due to a machine accident. His left ankle and heel bone, along with the soft tissue, were torn off, and the peripheral blood supply and sensation to the toes of the left foot were lost. We amputated his left leg below the knee. The left fibular head was retained after the left foot operation. Two months later, the patient came to our hospital for additional treatment after debridement of necrotic tissue operations twice. There was still approximately a 20 ×10-cm area on the right foot and right external ankle exposing the distal fibula fractures. X-ray showed that the right external ankle bony defect and the right medial ankle mortise widened; fortunately, the left fibular head was retained after left foot amputation (Fig. ). We reconstructed the right ankle using vascular anastomosis of the fibular head and a flap taken from the left stump. The Ethics Committee of the Second Affiliated Hospital of Soochow University approved the study.\nWe used an ultrasonic Doppler instrument (Model: ES-1000SPM Hayashi Denki Co., Ltd, Hong Kong China) to find the perforating branch of the peroneal artery before surgery, and near that location we created a flap approximately 20 × 10 cm in size according to the right foot wound (Fig. ). Surgery was performed with the patient under general anesthesia. The patient was placed in the supine position on the operating table with the left knee joint slightly bent and the left lower limb stump in internal torsion. The incision began in the popliteal space, swept down to the fibular head, and continued along the lateral peroneus muscles to the stump extremity. We cut the skin and subcutaneous tissue, then separated the peroneus longus and soleus muscles. We found the nervus peroneus communis on the inner posterior margin of the biceps femoris tendon and dissociated it for protection. The peroneal perforator was located in the intermuscular space between the peroneus longus and triceps surae muscles, then we freed the peroneal artery and 2 accompanying veins. We cut off the biceps femoris tendon, fibular collateral ligament and other muscle tissue attached to the fibula, extracted the fibular head, and then divided the superior tibiofibular joint. On release of the tourniquet, bleeding spots on the fibula and residual muscle tissue showed that the blood supply was satisfied. Therefore, we could then cut off the vessels and take the fibular head (Fig. ). We sewed the biceps femoris tendon and fibular collateral ligament with deep fascia around the lateral tibial plateau to avoid instability of the knee joint. We completely debrided bone splinters and necrotic soft tissue from the lower right limb and smoothed the edges. The free fibular head was fixated to the distal fibula with a fibular plate. The reconstructed inferior tibiofibular joint was reinforced with 3 lag screws before anastomosis of the vessels in the transplanted fibular head with the recipient area. Lastly, we made a suture flap in the wound and inserted rubber drainages (Fig. ).\nDue to necrosis of the skin and soft tissue of the distal flap 7 days after the operation, part of the reconstructed external ankle bone was exposed, so we transferred the sural neurocutaneous vascular flap to repair the skin and soft tissue defects of the lateral malleolus. X-ray examination 3 months after the operation showed that the fracture had a bony union and the shape of the transplanted fibular head was similar to the normal external ankle. At 12-month follow-up, walking function had been recovered with the help of a left artificial limb (Fig. ). According to the Baird–Jackson scoring system, the curative effect was satisfied.
We report the case of a 28 years old female patient who underwent a right knee arthroscopy and partial meniscectomy at 26 years of age with short term relief of her mechanical symptoms. Seven months after the surgery she reported progressive medial knee pain, disabling and persistent, without meniscal symptoms. Her body mass index and limb alignment were normal and physical examination revealed no ligament laxity. The MRI showed post-surgical changes in the medial meniscus, without re-rupture, with intact anterior and posterior horns. The articular cartilage was reported as grade II/III disease in the medial compartment.\nOur team performed a collagen scaffold meniscal transplantation, a porous collagen-glycosaminoglycan matrix composed of 97% purified type I collagen isolated and derived from bovine Achilles tendon. Surgery was done with an all-inside technique and the fixation of the collagen was achieved with Sequent Meniscal Repair Device® [ConMed®, NY, USA].\nRehabilitation consisted on restricted ROM (0 – 90 degrees) in the first four weeks and protected weight bearing for eight weeks. The patient completed her rehabilitation protocol at five months post-surgery, with normal gait and range of motion, with residual pain after walking for long periods.\nSix months after allograft implantation, the patient suffered a new knee sprain with external foot rotation while going down a small flight of stairs. She complained of a new onset of medial knee pain and joint locking symptoms. MRI was performed immediately, but its results regarding scaffold integrity were inconclusive due to the high-intensity T1 and T2 signal changes.\nThe patient asked for an immediate surgical relief. Arthroscopic examination showed a fracture of the posterior third of the meniscal implant, with an unstable fragment of 10 x 6 mm. (, ) This fragment was yellow in color, similar to the remaining implanted meniscus, which was stable at inspection and hook probing. The stable implant represented 65% of the whole implanted tissue. The unstable scaffold fragment was removed and submitted for histopathological analysis, which revealed different processes occurring in the implant (). The recovery of the patient was good, with a knee severity score of 88, three months post-operatively.\nA reconstruction of the three microscopic photographs of the sagittal cut of the implant was done (). A 40X amplification of the articular tip of the meniscus (), and a 200X amplification of the periphery of the implant revealed distinctively identifiable vessels ().
A 34-year-old Chinese man presented to the rheumatology department of our hospital with a 1-week history of muscle weakness and pain. He was referred by the general practitioner in the company he worked in for further evaluation. The patient admitted a history of sudden onset weakness involving his upper and lower limbs 1 day prior to his presentation. The patient was well before he went to bed at 10 pm, but when he woke at about 6 am he was unable to move his upper and lower limbs; just his neck. Symptoms lasted about 1 hour, then resolved completely. The patient had physically demanding work the day before. One week earlier, the patient complained of pain and weakness in lower limbs when he woke up for urination at around 4:00 am, to the point he reached the toilet with extreme difficulty. This took about 30 minutes to resolve.\nThe patient attributed that to his long flight the proceeding day. Since then, he had been experiencing muscle pain mainly involving the thigh, gradually increasing in severity and later involving the arm which was also associated with stiffness and mild weakness.\nOn the top of musculoskeletal symptoms during the past 3 months he reported sweating, heat intolerance, palpitations, and unintentional weight loss of 6 kg despite good appetite, but did not report any changes in skin, and hair.\nThere was no history of preceding fever, vomiting, diarrhea, trauma or seizures. The patient denied having shortness of breath, urinary problem, abdominal pain, backache, involuntary movements, and sensory loss in any limb. There was no history of recent drug ingestion. He denied any intake of supplements, alcohol or drug abuse. He smoked 1 pack of cigarettes daily for 8 years. There was no significant past illness, and family history was noncontributory.\nOn examination, the patient was conscious and oriented, and looked slightly anxious. He was afebrile with a pulse of 90 beats per minute, respirations of 16 breaths per minute, and blood pressure 120/70 mm Hg. The thyroid gland was diffusely enlarged, firm, smooth, and not tender, with no audible bruit. There was no lid lag, lid retraction, or exophthalmos. Fine tremor was detected on outstretching of the hands. Cardiac, respiratory, and abdominal examination were normal. Musculoskeletal examination was unremarkable apart from quadriceps, deltoid, and biceps tenderness. Neurological examination of the upper and lower limbs revealed normal tone, proximal muscle weakness (4/5 power in the lower limbs, 4+/5 power in the upper limbs), normal distal muscle strength. Reflexes were brisk and plantar response was flexor bilaterally. There were no sensory abnormalities and the cranial nerves were intact.\nLaboratory studies revealed potassium of 3.2 mmol/L (3.5–5.1 mmol/L), TSH<0.005 μIU/L (0.25–4.55 μIU/L), FT4 71.1pmol/L (12–22 pmol/L), FT3 22.6 pmol/L (3.1–6.8 pmol/L), and total creatine kinase(CK) 587 U/L(39–308 U/L). All other lab tests including full blood count, erythrocyte sedimentation rate, kidney and liver function test, blood sugar, serum calcium, serum magnesium, serum phosphorus, hepatitis serology, brucella agglutination test, lactate dehydrogenase, rheumatoid factor, and antinuclear antibodies (ANA) were normal.\nHis clinical presentation and laboratory abnormalities were consistent with thyrotoxic periodic paralysis. Patient was referred to endocrinologist who prescribed him propranolol 40 mg BID and Carbimazole 45 mg/day. On follow up 2 months later, the patient was subjectively well, and free from TTP attacks. His thyroid function test and serum potassium were normal.
We report the case of a 31-year-old Caucasian woman, gravida 3, para 1, who was referred after a second trimester fetal anatomy screening at 20 weeks gestational for a suspicion of a complex fetal cardiac malformation, for which several specialized opinions tried to reach consensus.\nThe obstetrical history of the patient includes a previous Caesarian section with a normal course of parturition and a spontaneous miscarriage. The current pregnancy presented a low risk for aneuploidy according to the performed cell-free fetal DNA test. The classical karyotype performed after the abortion did not reveal any chromosomal abnormalities.\nPrevious ultrasound evaluations were incongruent and reported the following findings:an isolated aortic arch anomaly (supposedly aneurysmal dilation from which the left common carotid artery emerges) and coarctation of the aorta with the anterograde flow; ventricular septal defect, coarctation of the aorta, and a vascular formation located superior from the aortic arch with the appearance of an arteriovenous fistula; aneurysmal dilation located above the pulmonary trunk bifurcation and a dilated left common carotid artery with a retrograde flow; minor ventricular septal defect with a normal ductus venosus triphasic flow.\nWe performed fetal echocardiography, which demonstrated a mild cardiomegaly with a left deviated 72-degree heart axis, normal aspect of the four-chamber view, a small membranous ventricular septal defect, and ductal aortic coarctation; the ductus venosus flow was normal (, and ). In addition, we identified an aneurysmal structure measuring 1.63/1.25/1.16 cm with turbulent Doppler flow, situated above the emergence of the pulmonary trunk and continued by a dilated vascular structure that bifurcates in the cervical region; the aneurysm seemed connected to the left pulmonary artery as well. A dilated left subclavian artery was also suspected (, and ).\nIn the context of complex cardio-vascular malformations, the patient requested the termination of the pregnancy by drug-induced abortion.\nThe hands-on dissection of the fetus revealed a set of abnormalities that could stand as an anatomical basis for what has been found during the ultrasound examination.\nThe first and the most pronounced aspect was the distention of the whole venous system of the neck and mediastinum. The specimen presented a linguo-facial vein that described a rather sinuous pathway alongside the inferior margin of the mandible (). Both the linguo-facial vein and the external jugular vein appeared with a markedly increased caliber, around 4–5 times larger than expected for this gestational age. Both left and right jugular veins and the right subclavian vein were assessed as three times larger than usual, respecting the normal relations to the neighboring structures ( and ).\nThe confluence between the left jugular and subclavian vein into the left brachiocephalic vein was observed to be very dilated to superior and inferior, extending above the superior margin of the omohyoid muscle as well as below the inferior concavity of the aortic arch. Moreover, on the inferior side of the enlarged brachiocephalic vein, a vessel could be observed descending lateral to the left vagus nerve and communicating with the left pulmonary artery. The left pulmonary artery was observed to be dilated as well, around twice as normal ().\nRegarding the great vessels of the heart, there are some anomalies to be discussed. A narrowing of the aortic arch was identified distally to the emergence of the left subclavian artery ( and ). A large, patent ductus arteriosus was found, ending right at the narrowing level observed in the aortic arch (ending right at the coarctation level) (). The left subclavian artery was dilated as well, sizing as much as the ascending aorta and the aortic arch, creating the illusion of a terminal branch rather than a lateral one ( and ).\nHeart analysis concluded no distinct changes in heart architectural formation for this gestational age. Atrioventricular and ventriculoarterial concordance was noted. Atria and ventricles were increased in relation to the mediastinum. Surprisingly for an aortic coarctation, the right atrium was not found to be enlarged.
A healthy 5-year-old boy was referred to the pediatrics department of Shahid Beheshti School of Dental Medicine, Tehran, Iran, with the chief complaint of a painful swelling located in the mandibular left buccal region ().\nThe patient’s dental history indicated that the first primary molar had received conventional pulpotomy treatment one year before and the left second primary molar had a defective Class II amalgam filling with recurrent caries. Clinical examination revealed marked grade I mobility of the mandibular left first primary molar and a palpable expansion of the buccal plate with crepitus on the mandibular left primary molar region extending 2×2 cm. The panoramic radiograph showed a round radiolucent unilocular lesion with smooth and well defined borders, extending 22×23 mm in the periapical area of the mandibular left primary first molar. The expansion of the lesion had pushed the permanent first bicuspid very close to the lower border of the mandible (). Based on the patient’s history and clinical and radiographic examinations, the differential diagnosis of the lesion was radicular cyst or dentigerous cyst and the treatment plan was surgical enucleation of the lesion. Other treatments required for the patient consisted of pulpotomy and restoration of teeth number A, B, I, J, K and T, extraction of tooth number S and band and loop space maintainer to maintain the E space in the mandible.\nConsent form was signed by the patient’s parents and the patient was scheduled for the surgery. At the day of surgery, standard disinfection protocols were followed and the area was anesthetized by block and infiltration injection of Lidocaine HCl with 1:100000 epinephrine. Initially, a needle biopsy was taken from the lesion and the result of aspiration was a light-yellow odorless liquid that was immediately sent to lab for cytopathologic evaluation. The results approved the cystic nature of the lesion. An incision was made from the left canine to the left second primary molar along the gingival margin and the site was exposed. The buccal cortical plate was considerably thin at the area over the lesion that had to be removed, but the lingual plate was left intact. The cystic lining was then enucleated and was sent for histopathologic examination. A decision was made by the surgeon and the pediatric dentist at the time of surgery to extract the first primary molar because it was involved with cystic lining and the prognosis was considered to be very poor. The surgical site was then rinsed with normal saline and after the bleeding was controlled, the flap was sutured back and primary closure was achieved. The patient was advised to use iboprufen 200mg every 4 hours in case of pain until pain relief. Seven days later, the patient came back for post-surgical examination and suture removal. The histopathologic features were inconsistent with the clinical diagnosis of radicular cyst. The cystic cavity was lined by varying thickness of nonkeratinized stratified squamous epithelium with arch shaped appearance and exocytosis in the underlying connective tissue that was severely infiltrated by chronic inflammatory cells. Extravasated RBCs, hemosiderin pigments and Russell bodies were also seen (). At 3 months recall, clinical and radiographic examination was performed to evaluate the healing process (). Oral examination revealed good healing of soft tissues and radiographic examination showed that the bony lesion seemed to be healing and there was reduction in size compared to the preoperative radiographs. The first bicuspid was also in an improved position. The patient was then scheduled for the remaining required dental treatment and was also referred to an orthodontist for orthodontic evaluation. At 20 months recall radiographic and clinical evaluations indicated successful treatment ().
A 6-hour-old female neonate born from a 22-year-old Para 1 mother after term pregnancy. The newborn had protrusion of mass per vagina since birth; she also has a defect at the lower back with no discharge since the time of birth. Additionally the mother has noticed deformity on both legs and feet which barely move. The baby was active since the time of delivery and is sucking well and she passed meconium. The mother had two ANC visits and was vaccinated according to the national schedule and she reported the whole course of the pregnancy as uneventful. She did not have any known medical illness and has never taken any medication during the entire course of the index pregnancy except iron folate which was prescribed during the ANC visit.\nShe had spontaneous onset of labor and the membrane was ruptured spontaneously intrapartum. She gave birth to a 2330-gram female neonate after 5 hours of labor at a local health center. Baby had good APGAR score. Health care providers at the health center noticed defect at the back of the baby and referred her to Hawassa University Comprehensive Specialized Hospital (HUCSH) with the diagnosis of spinal bifida.\nDuring the initial evaluation at HUCSH the baby was active, vital signs were in the normal limits, and all neonatal reflexes were intact. There was 4x4 cm pink mass protruding through the introitus, cervical os is noted at the tip of the mass, the external genitalia appears normal, no discharge or bleeding from the mass, and the mass was reducible digitally and increases in size when the baby cries (see ). There was also 4x4 cm defect at the lumbosacral region the major portion of which is covered with skin while the lower edge is open, no discharge from the mass (see ). Additionally the newborn had bilateral club foot deformity (see ).\nOn investigation, the complete blood count was normal, creatinine level was 0.4mg/dl, and transfontanelle ultrasound scan shows mild dilatation of the lateral and third ventricles with an index of “mild hydrocephalus” and abdominal ultrasound was normal.\nAfter obtaining consent from the parents, under aseptic technique, the baby was catheterized, the vaginal mass was reduced digitally, and bandage was applied from the lower abdomen, both buttocks and legs were strapped in the bandage to the level of the mid-thigh leaving an opening at the anal orifice for passage of stool (see ). The bandage was removed after 72 hours at which time the mass was completely reduced and there was no recurrence of the prolapse afterwards (see ). Unfortunately one day after the removal of the bandage the baby started to shoot fever and had difficulty of sucking, on examination she had tachycardia and tachypnea and was febrile, she had depressed reflexes, the lower border of the meningocele got ruptured, her fontanels were bulged, and she was diagnosed with ruptured myelomeningocele and meningitis. The plan was to start her on antimeningitis drugs and repair the spinal cord defect after improvement but the parents insisted and went home against medical advice frustrated by her multiple anomalies. We communicated with the parents after her discharge and were informed that the baby died 7 days after she went home, but there was no recurrence of the prolapse throughout her stay.
A 61-year-old man went through a pelvic fracture due to wall collapse and was diagnosed with posterior urethral rupture. Five months after the accident, he came to our department to seek for urethral reconstruction.\nWe retrieved his medical history after injury from the medical record system in the orthopedic department. After the injury, the patient went through acute urinary retention (). Computed tomography (CT) scan showed edematous lower limbs and perineum (), purpura, and obvious local tenderness. The condition of the scrotum and testicles was not recorded. CT scan showed a comminuted fracture of the pelvis and sacrum (). The catheter balloon was not located in the bladder (). At that time, the orthopedists asked a urologist for a consultation on the acute urinary retention of the patient. The patient was then diagnosed as having a pelvic fracture complicated with acute urinary retention caused by posterior urethral rupture. Cystostomy and external pelvic fixation were performed.\nThe patient then came to the urology department to seek for urethral reconstruction 5 months after injury. Physical examination revealed a tough, round palpable mass in his right inguinal region, with a size of about 42 cm. The right scrotum was shrunken whereas the other side was normal. After further questioning the medical history, the patient confirmed that his bilateral testicles were palpable in scrotum and denied history of retractile testes before the accident, and the right scrotum was found empty after he was discharged from the hospital last time. He supposed the testicle had been completely destroyed during the accident. The patient was old, had no fertility requirements, and still had erectile function, so he did not take the initiative to mention it to his doctor.\nPelvic floor ultrasonography () was done during transurethral injection of normal saline, and presented that the penile part of the urethra was naturally dilated and the mucosa was smooth. However, the membranous urethra was like the shape of the mouth of a bird. There was no dilatation of membranous, bulbar, and prostatic urethra (indicating posterior urethral rupture). Color Doppler ultrasound image of right inguinal mass () presented a moderate echo with a size of 4.11.2 cm in the subcutaneous fat layer, with clear boundary, intact shape, and blood flow signals, which was consistent with a normal testicular echo. Reexamination of the pelvic CT showed that the right scrotum was empty and asymmetrical to the left (). The patient was diagnosed with membranous urethral rupture and right traumatic testicular dislocation.\nAfter adequate antiinfection treatment, fiber cystourethroscopy, urethral reconstruction, and orchiopexy for the dislocated testicle were performed under general anesthesia. The posterior urethral atresia was confirmed by fiber endoscopy through the external urethral orifice and cystostomy catheter. An invert “Y” incision was made in the perineum. Then the urethra was exposed and separated. Then we excised the scar tissue around the urethra, and the defect length was measured at about 1 cm. After eight stitches of tension-free anastomosis of the proximal and distal urethra, the incision was sutured. Negative pressure drainage was retained. Then, an oblique incision of about 3 cm was made in the right inguinal region, and a well-formed dislocated testicle was exposed outside the subcutaneous inguinal ring with no rupture of the tunica albuginea and any hematoma. We separated the spermatic cord and vas deferens, pulled the testicles out of the scrotum, and fixed them with three stitches to prevent from torsion.
The patient is a 58 years old Iraqi male that referred to our center three months after an unsuccessful operation for removing a subhepatic mass, probably adrenal mass. He experienced general weakness, fatigue, and weight loss in the last several months. The patient BMI was 20 kg/m2. In physical examination, vital signs were normal. In abdominal examination, a scar of previous surgery in the right subcostal area (subcostal incision) with near 15 cm length was noted. In hematological examination, Hb was 11 g/dl. In serum biochemistry, renal function tests, electrolytes, and bilirubin were all within normal limits. Only mild elevations of hepatic enzymes were noted. Serum and urinary catecholamines were normal. Serum levels of cortisole and aldosterone were normal. In CT scan that was done with intravenous and oral contrast (), a large mass located in the subhepatic area and intervening with the upper pole of the right kidney was noted. In the report of previous operation that was done in Iraq, tumorectomy was failed due to severe adhesions of the mass to the peripheral tissues. This mass was isolated from the liver. The patient scheduled for right adrenalectomy with the midline laparotomy incision. After opening the layers, the mass was located in the lodge of upper pole of the right kidney. It was severely adherent to the peripheral tissues, due to severe adhesion of the mass to the subhepatic area, its differentiation from the kidney or adrenal was difficult. Due to severe bleeding from the subhepatic bed subcapsular resection of the mass with the adherent kidney performed. Its total size was 14 cm × 10 cm and it weighted 2250 grams. The final Histopathological examination of the mass revealed, renal cell carcinoma, clear cell type, Fuhrman nuclear grading 2 () with invasion to Gerotas fascia and involvement of ipsilateral adrenal which originated from the upper pole of the kidney as exophytic growth pattern (T3N0M0). The postoperative course was uneventful and the patient discharged home at fourth postoperative day uneventfully. The patient was well one year after operation without any chemo or radiotherapy.
A 3-year-old male patient was brought in for anterior crossbite treatment. Facial photographs revealed a straight profile and a normal facial midline. Intraoral, dental cast, and 3D model examinations showed mesial terminal plane relationships on both sides and a moderate crossbite in the anterior segment (Fig. a). No symptoms of temporomandibular disorder were detected. His parents stated that the child had an incorrect nursing habit of lying flat while drinking from a bottle and had a functional mandibular protrusion habit. In this case, there was no evidence of a family history of Class III malocclusion. Based on these findings, the patient was diagnosed with a Class III malocclusion, moderate anterior crossbite, and occlusal interference.\nBased on the data described in the two clinical cases, the first treatment option was a conventional appliance with steel wire. However, both patients were unwilling to wear the appliance because it was uncomfortable and even painful. In response, the new clear removable appliance was chosen. We designed and fabricated new clear aligners following the treatment protocol mentioned above and started treatment as early as possible. After several hours of adaptation, patients were able to speak and eat with the appliance. According to their parents' statement, the new appliance was easier to wear than a conventional appliance and comfortable for the child during the period of home treatment. After 2–6 months of treatment, the crossbite was basically corrected. The patients’ upper anterior teeth were proclined, and the anterior crossbite was corrected. Then, the subsequent aligners were designed with thinner bite splints, allowing the posterior teeth to erupt gradually. The overall treatment time for the primary dentition was 4–8 months.\nAfter clinical examination, normal overbite and overjet in the primary dentition were achieved using this new appliance. Proper condylar position was achieved by eliminating occlusal interference and repositioning of the mandible, preventing future temporomandibular disorders. Based on a comparison between T3 records and T1 records, dental and soft tissue relationships were improved (Figs. , ). Questionnaire results (Table ) showed that during the therapeutic process, the treatment was safe and comfortable for the children and saved time for the parents. After the appliance was removed, regular dental visits were suggested for long-term observation of oral health.
A 54-year-old Japanese woman visited our ophthalmology department after experiencing proptosis, lid swelling, diplopia, and retro-orbital pain in her left eye lasting for 1 day. She had a medical history of poorly differentiated adenocarcinoma of the stomach, which had metastasized to her ovary and mesentery, diagnosed 2 years earlier. She had undergone four regimen courses of chemotherapy, yet these had failed and she thus received palliative treatment. There were metastases to subcutaneous tissue of her neck and thoracic bone marrow 3 months before her initial visit to our ophthalmic department. She had been admitted to our hospital 5 days previously without symptoms in either eye. She had undergone stenting in her esophagus against eating difficulties but she lived a self-reliant life at home.\nAt her first visit, an external examination showed lid swelling, red coloration, and proptosis of her left eye. A motility examination revealed an adduction deficit of − 4.0 and an abduction deficit of − 1.0. Ophthalmological examinations revealed a best-corrected visual acuity of 20/20 and an intraocular pressure of 15 mmHg in both eyes. No abnormal findings were found in the anterior segment. Her pupils were equally reactive without any relative afferent pupillary defect. A funduscopic examination showed partial optic disc edema in her left eye (Fig. a). No choroidal masses or striae were noted.\nA CT scan performed 10 days before her initial visit to our ophthalmology department revealed enlargement of the left medial rectus muscle. Retrospectively, similar findings were seen on a CT scan performed 3 months previously, and had worsened in the interim. Yet, a CT scan that had been performed 6 months previously showed no remarkable findings (Fig. ). There was no enlargement of other extraocular muscles and no swelling or mass lesion in other orbital tissues during the 6 months. So, gastric cancer metastasis to the medial rectus muscle of her left eye was suspected. Radiation therapy for metastasis to the subcutaneous tissue of her neck and thoracic bone marrow was effective; she received a total of 20 Gy/5 courses of radiation therapy to the orbit. A few days after completion of radiation therapy, lid swelling, red coloring, and pain disappeared. Two weeks post-radiation therapy, a motility examination revealed an adduction deficit of − 4.0 and Hertel’s exophthalmometry measurements with a 108-mm base were 14 mm (right eye) and 19 mm (left eye). At 1.5 months post-radiation therapy, a motility examination revealed an adduction deficit of − 2.0 and Hertel’s exophthalmometry measurements (108-mm base) were 14 mm (right eye) and 13 mm (left eye). A posterior ocular segment examination showed a normal left optic disc (Fig. b). She died 3 months after her initial presentation to our ophthalmology department.
This patient is a 29-year-old white woman from the USA with a medical history significant for severe IBS-D (diagnosed at age 12) and anxiety disorder. In July 2015, she presented with severe bleeding hemorrhoids secondary to IBS, which required hemorrhoidectomy and anal sphincterotomy in August 2015. The week before the surgery she developed pharyngitis and was treated with azithromycin, which resulted in mucousy diarrhea and abdominal discomfort. She tested negative for C. difficile antigen and toxins at that time.\nA week after surgery, she developed a perirectal abscess that had formed at the site of the sphincterotomy and was prescribed orally administered ciprofloxacin. Despite moderate symptom improvement, in September 2015 she required an abscess incision and drainage procedure and Penrose drain insertion. Prior to the surgery she was given a single dose of clindamycin. An additional 2-week course of ciprofloxacin and metronidazole was then prescribed. In late September 2015 she was admitted to the hospital for two nights due to further complications related to the abscess and was then diagnosed as having a perianal fistula.\nIn November 2015, she was prescribed clindamycin for an episode of group C streptococcal-positive pharyngitis. In late November 2015, she was also diagnosed as having Ehlers–Danlos syndrome, which according to her medical record may partially explain the poor wound healing from the perirectal abscess. In December 2015, her fistula required an anus seton placement. She was treated with multiple courses of ciprofloxacin and metronidazole off and on from December 2015 to January 2016.\nIn January 2016, following up on her recurrent pharyngitis, she was diagnosed as having chronic tonsillitis which led to tonsillectomy. In February 2016, 2 weeks after the surgery she was prescribed clindamycin. At the beginning of March 2016, she was diagnosed as having bacterial vaginosis and was prescribed orally administered metronidazole. A week later she was diagnosed as having vaginal candidiasis and was prescribed orally administered fluconazole. In April 2016, she complained of dysuria and was prescribed ciprofloxacin. After 2 days, when urine analysis results came back negative, she was asked by her physician to stop the treatment.\nIn June 2016, she presented for follow-up with ongoing diarrhea and abdominal pain. She was diagnosed as having C. difficile diarrhea, her antigen and toxins laboratory results were indeterminate, and a toxigenic strain was confirmed by polymerase chain reaction (PCR). She was prescribed a 6-week course of orally administered vancomycin. After a week of treatment her symptoms worsened, and following discussion with her gastroenterologist her treatment was switched to a 2-week course of metronidazole. Hours later, she was admitted to the hospital for a 4-day period for colitis. Her C. difficile antigen and toxin test returned negative during her admission. She received intravenously administered metronidazole treatment during her hospitalization. Her symptoms improved during her hospital stay, with 1–2 soft bowel movements a day. At discharge her metronidazole course was stopped and she was again prescribed vancomycin, which she took for over a month. She continued to experience GI irregularity (3–5 bowel movements a day) beyond what she had experienced secondary to her IBS prior to her surgeries. In March 2017, she was prescribed rifaximin for 2 weeks to treat chronic diarrhea.\nIn November 2017, she was prescribed a series of clinical intestinal tests (SmartGut™, uBiome Inc., San Francisco, USA) with the instructions to administer the test at home whenever she was experiencing a noticeable change of GI symptoms, then follow-up with her health care provider to discuss the results. This sequencing-based test requires that patients use a sterile swab to transfer a small amount of fecal material from toilet paper into a vial containing a lysis and stabilization buffer that preserves the microbial DNA for transport by mail back to the laboratory for processing, which involves DNA extraction, 16S ribosomal RNA (rRNA) gene amplification, and sequencing []. She first used this test in November 2017, about a month after completing a 2-week course of rifaximin. The results revealed a number of microbial organisms that were outside the healthy reference ranges, but she was negative for all pathogenic organisms included in the test, including C. difficile (Fig. ).\nBetween November and December 2017, her GI symptoms worsened considerably; her daily bowel movements increased from 3–4 to 6–10, stool consistency became more mucous-like and gelatinous, and she was experiencing more pain with defecation. She re-tested with SmartGut™ test again in January 2018. Her results continued to reveal a number of microbial organisms outside the healthy range and, this time, her sample also indicated the presence of C. difficile (Fig. ). She immediately contacted her primary care provider, who re-tested her for C. difficile and confirmed indeterminate CDI by antigen and toxins A and B. Additional PCR testing at a regional laboratory confirmed the sample was positive for a toxigenic C. difficile strain. As a result of testing, her clinician started her on fidaxomicin; her symptoms improved rapidly. By April 2018, she had returned to her baseline in regard to her IBS-related GI symptoms with no blood in her stools.\nIn addition, the second SmartGut™ sample was tested for toxins A and B by sequencing at uBiome Inc. laboratory in San Francisco, USA, which resulted positive for both and confirmed the toxigenic nature of the C. difficile strain.
We present the case of a 45-year-old Caucasian man suffering from psoriasis for more than 20 years at the time of the first visit. No relevant comorbidities were found at history collection, apart from benign prostatic hyperplasia, for which he is treated with tamsulosine. He was initially diagnosed and followed-up by dermatologists, who treated him with topical corticosteroids and salicylic acid for skin involvement and systemic corticosteroids for resistant lesions as needed ().\nThe skin component had always been <30% of the total body surface area (BSA). The patient reported distress in personal and social relationships with consequently reduced HRQoL. After ten years, he started experiencing dactylitis of feet and recurrent arthritis of the small joints of hands, shoulders and knees, together with inflammatory morning stiffness lasting around one hour, with negative rheumatoid factor (RF); thus fulfilling ClASsification criteria for Psoriatic ARthritis CASPAR criteria for PsA []. He was referred to the rheumatology outpatient clinic of another hospital and treated with indomethacine and systemic corticosteroids as needed for about five years, and then achieved complete remission of joint symptoms. Three years later, because of a flare of joint involvement (episodes of peripheral arthritis and dactylitis of the small joints of hands and feet once per week) and skin involvement, he was started on methotrexate 10 mg weekly with folate supplementation, in combination with cyclosporine 200 mg daily, with reduced extension and severity of skin lesions and decreased frequency of arthritis flares to once per month. However, he did not reach complete remission. Shortly after initiation, due to a 2-fold increase of liver enzymes (hepatitis excluded based on abdomen ultrasound and hepatitis B and C viral profile), methotrexate was reduced to 7.5 mg weekly []. The reduced methotrexate regimen led to normalization of the liver enzymes but was ineffective in controlling both skin and joint involvement. Two years later, due to elevated arterial pressure values (up to 150/90 mmHg) and serum creatinine increase >30% of baseline value (up to 1.4 g/L), cyclosporine was stopped.\nThe following year, he referred to our outpatient clinic for skin and joint flare. At referral, physical examination demonstrated mild psoriasis of trunk, arms and legs, with a psoriasis area severity index (PASI) score of 5.1 (), dactylitis of the third digit of the left foot and arthritis of the third interphalangeal joint of the left hand, which, together with a normal C reactive protein (CRP), activity visual analogue scale (VAS) and pain VAS of 4 and 4.5 respectively, accounted for a disease activity in psoriatic arthritis (DAPSA) score of 12 (low disease activity) [].\nLaboratory tests showed no relevant alterations. Radiographic assessment of hands and feet showed no erosions and no signs of axial involvement were found at magnetic resonance imaging (MRI). Ultrasound imaging of the third metacarpophalangeal (MCP) joint showed inflammation of the joint and of the peritendon of the extensor tendon (a).\nConsidering that the patient failed to respond to two DMARDs (cyclosporine and methotrexate), that he refused a parenteral drug, that he presented mild skin and joint involvement, no bone erosions, dactylitis and no axial involvement, apremilast was chosen [,,]. At baseline, the EQ-5D questionnaire reported altered HRQoL, with some difficulties in motility, no difficulties in self-care and some difficulties in usual activities, moderate pain/discomfort and moderately anxious/depressed mood. Patient’s global health (GH) VAS was 60 and pain VAS was 45 (). Furthermore, the patient was screened at baseline for CV risk; carotid arterial doppler ultrasound demonstrated only intimal thickening of the left bulb and 24 h blood pressure monitoring demonstrated mild systolic and diastolic daily hypertension for which he started lercanidipine 10 mg/daily. At baseline, total cholesterol (TC) and triglyceride (TG) serum levels were 173 mg/dL and 113 mg/dL respectively, and TC/high density lipoprotein (HDL) ratio was 4.94 (). After 4 weeks, the extension and severity of erythema and infiltration of psoriatic plaques were substantially reduced (PASI 2.5, ). Furthermore, the patient reported a marked improvement of joint involvement with no further episodes of arthritis or dactylitis, although inflammatory morning stiffness persisted (DAPSA 6.0). At the 2-month follow-up, the patient reported several side effects such as mild headache, dizziness and hypotension and, due to further amelioration of the skin and joint involvement he stopped apremilast, which was restarted shortly after, due to worsening of psoriasis (PASI 3.0) and occurrence of a new joint flare (DAPSA 10). At the time he was still taking methotrexate 7.5 mg weekly. At the 6-month follow-up he reported no arthritis flares and no inflammatory morning stiffness. At physical examination, the third MCP joint was swollen but not tender (DAPSA 3), and ultrasound imaging showed reduction of joint and soft tissue inflammation (b). A further improvement of skin involvement was observed and reported by the patient (PASI 1.2, ). During the follow-up, we observed an amelioration of the serum lipid profile already after 4 weeks, with a 5.8% reduction of TC and a reduction of TC/HDL ratio, and after 12 months, a further reduction of TC, LDL and TG values of 15.6%, 25.7% and 17.7% respectively, and a 20% increase of HDL levels compared to baseline values. TC/HDL ratio reached the favorable value of 3.48, as shown in . We even observed a 5% weight loss—although our patient already had a normal baseline weight and body mass index (BMI)—reaching a final normal weight with a normal body mass index (BMI 21.5). After 12 months, the clinical manifestations were stable. We therefore decided to stop methotrexate, but the patient experienced a slight relapse of both skin and arthritis (PASI 2.5, DAPSA 6), shortly after. Therefore, although dosage was low (7.5 mg), we reintroduced methotrexate, leading at the 18-month follow-up to stable minimal disease activity with PASI 75 and joint remission (PASI 1.2, DAPSA 2) [,] and a dramatic improvement in HRQoL ().
A 6 year-old girl presented with a recurrent urinary tract infection (UTI) had an abdominal ultrasonography (US) which revealed an enlarged left duplex kidney with a mildly prominent lower moiety collecting system. Voiding cystourethrography was normal without post voiding residual urine. She was referred for the radionuclide renography to see the drainage pattern of the left kidney.\nTc-99m mercaptoacetyltriglycine (MAG3) renal scintigraphy was performed according to F -15 protocol (). Oral hydration (10 ml/kg) started 30 minutes before the study and intravenous furosemide (2 mg) was given 15 minutes before the intravenous administration of 1 mCi (37 MBq) of MAG3. Imaging was performed from posterior projection using GE XRT gamma camera equipped with a low energy parallel-hole general purpose collimator at 140 keV energy peak with a 20% symmetrical energy window. Dynamic acquisition at 64x64 matrix was acquired 1 second per frame for 1 minute for the renal perfusion and 60 seconds per frame for 29 minutes for the renal function. An Entegra (GE medical systems) workstation was used for image processing. The dynamic planar images of MAG3 renal scintigraphy showed more prominent activity in lower moiety than upper (). The region of interests and the time activity curves are shown in and , respectively. The time to peak parenchymal uptake of upper and lower moieties of left renal duplex system was at 2.5 minutes for each and at about the third minute both curves showed a gradual decline indicating the beginning of excretion and patency of the collecting systems (). At the fifth minute, the slope of the activity curve of upper moiety increased, indicating a more rapid excretion followed by a focal rise of activity in lower moiety until the 10.5th minute (). Diuretic was injected before the study, intravenous line was washed with saline just after the injection of radiopharmaceutical and there was not any patient motion, so the increase of the activity in the lower moiety was explained by “yo-yo” reflux. The drainage of both moieties was complete until the end of the study excluding the presence of an obstruction.\nThe patient is well after the last episode of UTI that was treated with antibiotics, on regular follow up. Also a long term suppression antibiotherapy was given. Proper wiping technique following the toilet use and frequent urination were advised for conservative management. Surgical treatment was not indicated.
A 29-year-old woman (gravida 1, papa 0) was referred to our hospital at 31 weeks of gestation because of a suspected presence of a posterior fossa cyst identified during a routine third-trimester ultrasound examination. The patient and her husband’s medical records were unremarkable. Maternal serum screening for Down’s syndrome in the second trimester represented low risk and the ultrasound at 12 and 20 weeks of gestation revealed no abnormalities.\nUltrasound examination was repeated in our hospital and revealed a cystic mass measuring 2.16 × 1.05 cm in the posterior midline portion of the fetal brain (). However, color Doppler showed no vascularity in or around the mass. There were no ventriculomegaly or other extracranial abnormalities, nor were there any signs of fetal hydrops or other anomalies.\nFetal magnetic resonance imaging (MRI was recommended to get more information. MRI at 32 weeks gestation showed a isolated triangle occipital mass measuring 2.5 × 2.4cm along the posterior surface of the brain with high signal intensity on T2 weighted MRI with occipital lobe compressed forward and superior longitudinal sinuses dilated (). A fetal dural sinus thrombosis at torcular was suggested.\nAfter a genetic counseling session where all the possible fetal outcomes were explained fully to the parents, they decided to continue the pregnancy. At 39 weeks of gestation, fetal MRI was repeated which showed the thrombosis had shrunk significantly without causing any brain compression (). There were no complications encountered during the pregnancy and the baby was born via a Cesarean section at 40 + 2/7 weeks of gestation. Apgar scores were 10 at 1 minute and 5 minutes after birth. The female neonate was referred to NICU for further observation. The neonate weighted 3450 g and her head circumference was 35.2 cm which was within the normal range. No abnormalities were found during the physical exam. There was no evidence of hypercoagulability (plasma prothrombin time (PT) and INR, activated partial thromboplastin time (APTT) and APTT-R, d-dimer, Protein C, Protein S, anticardiolipin were all within the normal range). Three days after birth, MRI and MRV was performed which showed a further shrinkage of the thrombus () and the cerebral venous drainage was not compromised. The infant was discharged 7 days after birth and no physical or neurological abnormalities were observed at 8-months during a follow-up.
Presenting concerns This is a case of a 22-year-old white female who presented at 34 weeks with preterm premature rupture of membranes. She had a history of two prior cesarean deliveries. A left ovarian tumor, not noted during the course of her current pregnancy, was noted on cesarean delivery. Current pregnancy When she presented with preterm premature rupture of membranes, repeat cesarean section was performed again. At the time of surgery, another 8 cm mass on her left ovary was diagnosed intraoperatively. This mass was not diagnosed prior to surgery. A second trimester ultrasound was performed that did not show any ovarian cyst. During the surgery, the mass appeared tan with punctate focal hemorrhage. The mass was unable to be separated from her left fallopian tube, so the mass, the left fallopian tube and part of the left ovary were all removed. We were able to leave a small amount of residual ovarian tissue. Pathologic description noted a multiloculated and cystic mass with clear mucinous fluid consistent with mature cystic teratoma (Figure ). Her postoperative course was uncomplicated. Previous pregnancy Her antecedent pregnancy was complicated by right ovarian torsion at 13 weeks secondary to an ovarian tumor. A right salpingo-oophorectomy was performed. The right ovary was sent to pathology and noted to be infarcted with no viable tissue from the mass to make a definitive diagnosis (Figure ). During that same surgery, an 8 cm, irregularly shaped mass with a focally hemorrhagic surface was noted on the left ovary. A left ovarian cystectomy was performed, and final pathology showed a benign mucinous cystadenoma (Figure ). At the time of cesarean in the same pregnancy, a metachronous mucinous cystadenoma was noted on her left ovary; cystectomy and partial left oophorectomy were performed. Follow-up and outcomes Following her third cesarean delivery, this patient was surprised to learn that she had developed another ovarian cyst in pregnancy necessitating removal. She was concerned about the function of
A 73-year-old woman with a history of well-controlled type 2 diabetes mellitus and hypertension presented to the clinic with visual loss in both eyes in July 2008. The vision was 20 / 200 in both eyes. There were no other abnormalities in the anterior segments of the eyes, other than visually significant cataracts, bilaterally. The fundus examination with the indirect ophthalmoscope revealed the presence of a moderate nonproliferative diabetic retinopathy and macular edema in both eyes. Optical coherence tomography showed macular thickening in both eyes with a taut posterior hyaloid membrane of the macula in the right eye. In July of 2008, phacoemulsification and intraocular lens implantation were performed, as well as vitrectomy with removal of the posterior hyaloid and internal limiting membranes, as well as endolaser treatment of the right eye. At the end of the vitrectomy, IVTA (4 mg) was administered. One week later, phacoemulsification, intraocular lens implantation, and IVTA administration (4 mg) were performed on the left eye. The postoperative clinical course was fair. In August of 2008, the vision improved slightly to 20 / 100 with improvement of the macular edema in both eyes.\nThree-months later, in October of 2008, the patient presented with 'cloudy vision' in both eyes that developed two weeks previously. The visual acuity was 20 / 200 in the right eye and counting fingers at 30 cm in the left eye. The intraocular pressure (IOP) was 30 mmHg in the right eye and 42 mmHg in the left eye. The slit lamp examination demonstrated fine keratic precipitates of the corneal endothelium, 3+ inflammatory cells, 2+ flare in the anterior chamber, and moderate vitreous haze in both eyes. The fundus examination revealed white-yellow, necrotic peripheral retinal lesions in the superotemporal quadrant of both eyes ().\nAs bilateral acute retinal necrosis was suspected, starting intravenous treatment with acyclovir was considered. However, the patient refused hospital admission and further treatment. Moreover, azotemia resulting from diabetic nephropathy limited the use of acyclovir. Therefore, antiviral treatment was not started and the patient was closely monitored. A diagnostic aqueous humor sample for polymerase chain reaction (PCR) analysis was obtained. PCR was carried out to test for varicella zoster virus, herpes simplex virus type 1 and 2, and CMV. Topical anti-glaucomatous and steroid medications were used for treatment.\nThe PCR results, available seven days later, confirmed the presence of CMV. The infectious disease consultant reported that systemic CMV infection was not possible and recommended antiviral therapy with ganciclovir for the CMV retinitis. However, one week had elapsed and the IOP and inflammatory reaction of the anterior chamber and vitreous had decreased, the peripheral retinal lesion did not increase, and the macula was not threatened in either eye ().\nThe CMV retinitis was likely caused by the local (intraocular) immunosuppression caused by the IVTA and the immunosuppression might have reduced over time (already 3 months since the IVTA was administered in both eyes). The CMV retinitis did not threaten the macula. Since starting treatment with ganciclovir was not urgent, the patient was closely followed without any anti-CMV medications. The plan was close observation while maintaining the topical anti-glaucomatous and steroid therapy.\nIn November of 2008, two months after the initial presentation, the inflammatory reaction of the anterior chamber appeared to be resolved and the vitreous haze was much improved in both eyes. The IOP was 24 mmHg in the right eye and 28 mmHg in the left eye with topical application of timolol and dorzolamide. The necrotic retinal lesion regressed slightly without any progression of the margin of the lesion in both eyes.\nIn April of 2009, six months after initial presentation, the visual acuity was 40 / 200 in the right eye and 20 / 200 in the left eye. The anterior chamber reactions had resolved and the inflammatory retinal lesions regressed completely with the subsequent development of retinal pigmentary changes in both eyes. A mild vitreous haze was still present in the left eye ().
A 44-year-old female patient complained of pain on her right knee after fast walking for two months. On physical examination, the range of motion was limited due to the pain for flexion more than 90°. There was no effusion. The patient had medial joint line tenderness, and a McMurray test elicited pain on the medial joint line. Radiographs of the right knee showed lateral joint space widening, high fibular head, and also increased concavity and subchondral sclerosis of the medial tibial plateau (Fig ). MRI of the right knee showed a discoid medial meniscus with a horizontal cleavage tear (Fig and ) and a discoid lateral meniscus with no tear (Fig and ).\nArthroscopic examination of the right knee confirmed the presence of discoid medial and lateral menisci. The medial meniscus was incomplete discoid conformation and had a horizontal tear (Fig ), whereas the lateral meniscus was completely discoid and had no tear (Fig ). The discoid medial meniscus had an anomalous insertion to the anterior cruciate ligament (ACL). We found also a pathologic medial patellar plica with fibrotic, thickening, and tear. There was cartilage fasciculation on the medial facet of patella. We performed partial meniscectomy of incomplete discoid medial meniscus and resection of pathologic medial patellar plica. We did not perform surgical procedure to the discoid lateral meniscus since the patient had no symptom and no tear. The patient had no limitation of motion or pain 2 years after operation.\nFrom the medical history, it was revealed that seven years before, when she was 37 years old, she complained about pain in her left knee for three years. The pain had become worse during walking. The patient was referred to our hospital under the diagnosis of medial meniscus tear. Physical examination of the left knee showed swelling and medial joint line tenderness. A McMurray test revealed pain with external rotation. Radiographs showed widening of lateral joint space, and a high fibular head, and increased concavity of medial tibial plateau (Fig ). Arthroscopic examination of the left knee confirmed the presence of incomplete discoid medial meniscus with a flap and horizontal tear, and incomplete discoid lateral meniscus. We performed a subtotal meniscectomy on the medial meniscus and reshaping of the lateral meniscus. Unfortunately, the patient and our hospital did not keep her MRI and arthroscopic picture, so we were unable to present those here. However, we could present the original scan of senior author hand writing operation report describing the operative finding of incomplete discoid medial meniscus with flap and horizontal tear and incomplete discoid lateral meniscus (Fig ).\nOn the last follow-up, seven years after the first operation and six months after the second operation, patient had no complaint and satisfied with the result. The radiological examination showed that left knee has apparent varus deformity. It could be due to the prior surgery.
The patient was a 38-year-old male with a history of ankylosing spondylitis. He had no prior hip surgeries. The patient presented with severe limping on both sides. The ROM on both left and right hip was zero secondary to bilateral hip ankyloses. The patient could hardly walk and moved by twisting his whole lower body to move forward or backward. A decision was made to perform bilateral THA on the patient (Fig. A–C).\nBoth left and right hips were completely fused, making dislocation impossible. As a result, in the preoperative plan it was decided to perform the segmentation of the femoral head and acetabulum as a single bone. The three acetabular align points that should had been set at the acetabulum were set at the great trochanter and acetabular rim (Fig. E). And in the registration step of the operation, the circumference of the joint (great trochanter, femoral neck, acetabulum rim) was registered instead of the acetabulum (Fig. I).This is an unusual process, and a significant deviation from standard protocol, which required specific surgeon approval; however, given the circumstances, it was deemed necessary to successfully plan and perform the surgery on this patient. Without being able to dislocate the hips, the exposure was very limited. Therefore the exposure was extended by about 1cm on the superior side and more superior soft tissue was stripped in order for a wider spread of registration, especially the superior area to the acetabulum rim. In such cases, extra caution needs to be taken to spread the registration points as far apart from each other as possible. This will ensure adequate registration despite the involvement of large surface area for matching. Other than the eight verification spheres, a manual check was performed to make sure the rotation was locked correctly. After we were certain the surface matching was successful, the hip was dislocated by osteotomy of the femoral neck in situ and then standard Mako procedure was followed (Fig. I–K).\nThe patient underwent conventional bilateral TKA 15 days after the bilateral Mako THA. During follow up at 3 months post-operation, both hips can be fully extended and flexed up 45° with assistance. Patient reported no pain and could walk with walking frame (Fig. L). The HHS 6 months after THA was 62 points (left and right).
A 61-year-old male with a past medical history of hypertension, hyperlipidemia and coronary artery disease with the remote stent in the right coronary artery (RCA) presented with sustained tachycardia. He had suffered an acute ST-segment elevation myocardial infarction one month earlier and had undergone emergent RCA stenting with a drug eluting stent. Following that index admission, he had been discharged but presented three days later with a cardiac arrest, from which he was resuscitated. He had been noncompliant with his dual antiplatelet therapy and was found to have RCA in-stent thrombosis requiring repeat stenting with a drug eluting stent. After a good neurological recovery, he was again discharged and subsequently enrolled in cardiac rehabilitation. During the rehabilitation session, he was found to have sustained tachycardia with associated nausea and lightheadedness but no symptoms of palpitation, chest pain or dyspnea. He was treated with intravenous adenosine in the ambulance and allegedly had some brief sinus rhythm, but arrived in the emergency department in a regular tachycardia with hemodynamic stability. The electrocardiogram showed tachycardia at the rate of 176 beats per minute which was regular with narrow complexes in most leads and a maximum QRS duration of 115-120 milliseconds in leads I and aVL, with slurring of the QRS upstroke. There was an abrupt R-wave transition between leads V1 and V2 (as in Figure ). The arrhythmia failed to respond to intravenous diltiazem but resolved after 150 mg of intravenous amiodarone. The sinus rhythm electrocardiogram demonstrated a significantly different QRS complex than during tachycardia, both in axis and without the slurred QRS upstroke in leads I and aVL (Figure ).\nThe patient was admitted to coronary care unit and was continued on intravenous amiodarone infusion. A subsequent electrophysiological study was performed using single, double and triple extra stimuli from both the right ventricular apex and outflow tract. A non-clinical rapid monomorphic ventricular tachycardia (VT) was induced with double extra stimuli, with resultant hypotension and lightheadedness. Ventricular burst pacing was delivered and converted this VT into the tachycardia with which the patient presented to the hospital. This was clearly ventricular in origin, with AV dissociation. Additional burst pacing terminated this arrhythmia. The patient underwent subsequent placement of a dual-chamber implantable cardioverter-defibrillator.
We present the case of a twelve year old male patient, with no relevant past medical history, who suffered a high power fire weapon injury to his left upper arm during a street armed robbery. He presented extensive loss of soft tissues, including part of the proximal third and midshaft of the humerus, part of the biceps muscle, and complete loss of subcutaneous fat and skin over the posterolateral aspect of the arm. He was assisted by local paramedics who applied a splint and proximal tourniquet to the extremity and initiated hydric reanimation during transportation to our third level trauma center. At arrival, the patient was found hemodynamically stable, with a grossly contaminated wound over the aforementioned location, so a formal surgical exploration was indicated.\nDuring this initial procedure extensive irrigation and debridement of nonviable tissue were performed, followed by external fixation of the humerus, aiming for a definitive reconstructive procedure during a second intervention (A). After broadly explaining the risks and possible outcomes of attempting an extremity saving procedure, the patient and his family decided to accept the operation. During the reconstruction, bilateral peroneal grafts were harvested to reconstruct the damaged bone, and were fixated with a titanium metal plate to the proximal and distal humeral remnants in the upper arm (B). Also, an ipsilateral dorsoepigastric pediculated flap, with a cutaneous island of 18 × 7 cm was designed and utilized for coverage of the defect (C, 2 and 3).\nThe patient evolved uneventfully, maintaining stability on the upper extremity and was discharged home, presenting appropriate integration of the flap during follow up visits. He attended rehabilitation and gradually reintegrated to his daily activities. Functionally the patient presented suboptimal outcomes, with subtle limitation of the range of movement caused by inadequate integration of the bone grafts. One year later, we decided to re intervene the patient to perform a formal shoulder joint replacement to improve functional outcomes. The operation was carried out successfully and after one year the patient has improved his functionality, being capable of performing full ad/abduction, extension and flexion of the shoulder, elbow and wrist joints. Sensibility remains partially compromised, with intermittent disesthesias over the area covered by the flap, but there are no signs of atrophic changes over the arm and forearm muscles ().
A 5-year-old girl was admitted to our Emergency Department after an episode of acute interscapular back pain occurring without trauma and followed by bilateral upper and lower limb weakness.\nHer family history included 8 Italian members harboring the same homoplasmic m.3460G > A mtDNA (Table , Fig. ). All the family members presented headache poorly responsive to NSAIDs and, except for the girl and her mother, visual loss due to optic nerve pathology. The patient’s medical history was unremarkable.\nThe patient’s vital signs were normal and stable. At the neurological examination, her cognitive functions were normal. There were no cranial nerve lesions. She was unable to stand and walk and had more severe weakness in the right lower limb. She also presented weakness in her upper extremities and difficulties with fine hand movements, particularly in the right hand. The right patellar and Achilles tendon reflexes were brisk. The plantar reflex was positive in the right foot. She presented impaired bladder control. No alterations in touch or pain sensitivity were present in the trunk and limbs. No concomitant signs of infection or inflammation were present, and no such signs had been reported in the previous weeks. Analyses of CSF pressure, glucose, protein, cell count, viral PCR, and culture were normal, and oligoclonal bands were absent (see timeline of events and treatment in Fig. ). The patient underwent a spinal cord MRI; the results showed hyperintense signal alterations in T2-weighted sequences and restricted diffusion in diffusion weighted imaging (DWI) sequences in the anterior portion of the cervical and dorsal spinal cord, suggesting anterior spinal artery territory involvement (Fig. ). Computed tomography angiography (CT) imaging showed no arterial dissection or other vessel abnormalities. Visual evoked potentials were normal. Somatosensory evoked potentials in both legs showed decreased conduction velocities. Motor evoked potentials showed lower amplitude for cortical derivation, prolonged latency in the upper limbs and normal in the lower limbs. Central conduction time was increased in the upper limbs and normal at the lower limbs. Complete autoimmunity and thrombophilia screening were unremarkable. Testing for anti-AQP4 antibodies was negative, and anti-MOG antibodies were not significantly elevated. A cardiologic consultation and echocardiography identified normal heart and aorta features. At the ophthalmological assessment, pupillary reactions were normal, the fundus oculi did not reveal pathological signs, and color vision was not affected. The visual acuity was 9/10 bilaterally. The digital visual field test displayed a mild defect in the peripheral portion of the visual field that was more evident in the left eye. Optical coherence tomography, visual evoked potential and electroretinograms were normal. As the child grows, her visual function will require careful monitoring, particularly when she reaches the adolescence.\nAfter obtaining written informed consent consistent with the principles set forth in the Declaration of Helsinki, total DNA was isolated from the patient’s peripheral blood according to standard protocols. Full-length mitochondrial DNA sequencing was conducted according to a previously described protocol [], and detected the haplogroup H27 was detected in the proband. The following variants were detected in the homoplasmic state: m.41C > T, m.73A > G, m.263A > G, m.1438A > G, m.3460G > A, m.4769A > G, m.8860A > G, m.11719G > A, m.15326A > G, m.16129G > A, m.16316A > G, m.16519 T > C. Based on the presence of the m.11719G > A and m.16316A > G nucleotide variants, the patient is predicted to belong to the H27 haplogroup. The m.3460G > A transition, a major mutation associated with LHON, was verified in blood-derived DNA of other family members by PCR-RFLP analysis.\nAcetylsalicylic acid was administered at low doses (2.5 mg/kg daily), and high doses of methylprednisolone (20 mg/kg daily) were empirically administered for three days. Although no proven treatments for LHON are available, early treatment with idebenone is thought to limit the progression of the disease; the patient was administered 45 mg of idebenone three times daily. Within two days following this therapy, clinical manifestations improved, and the patient regained bladder control and the ability to ambulate; within one week, the girl also recovered nearly normal strength in both arms. A control spinal cord MRI together with 1H-MRS to study the lactate peak was performed 10 days after hospital admission; the results showed complete regression of the alterations and no abnormal metabolites (Fig. ). The patient continued outpatient rehabilitation, and her motor functions improved, resulting in an almost completely normalized neurological examination after 2 months and preserving these achievements at follow-up one year later.
A 48-year-old gentleman was diagnosed as a case of left-sided RCC in June 2004, which was treated with radical nephrectomy and adjuvant radiotherapy at an outside hospital. In October 2006, he presented to our hospital for second opinion in view of progressive metastatic deposits in both the adrenals, lungs, and mediastinum. He was started on interferon (INF-α, 5 MIU daily subcutaneous doses), which he continued till December 2007. Subsequently, he presented with swelling in throat in March 2009. It was biopsied and confirmed to be metastases from RCC. He was advised external beam radiotherapy for the base tongue lesion but refused treatment due to personal reasons. In December 2009, he presented again with increasing difficulty in swallowing and alteration in voice. He was barely able to swallow liquids orally and had lost 2-3 kg of weight. On clinical examination, he had a large (5 × 5 cm) exophytic swelling in the oropharynx with epicenter in the right base of tongue []. It involved the tonsillar fossa and adjacent soft palate on right side, crossing midline. Tongue mobility was normal. There was no palpable cervical lymphadenopathy. Computerized tomography (CT) scan of the face, neck, thorax, abdomen, and pelvis was suggestive of widespread visceral and osseous deposits apart from the base tongue lesion []. No adenopathy was noted (which corroborated with secondary from RCC as it is uncommon for large oropharyngeal primary of squamous carcinoma to present without neck nodes). In view of predominant symptom of dysphagia and exophytic growth, he was first planned for palliative radiotherapy with escalating doses followed by Sunitinib. The patient received radiotherapy to a dose of 50 Gy in 25 fractions over 5 weeks with limited portals. Protracted regimen (prolonged fractionated course of radiotherapy over 5 weeks as compared to the most commonly used short course of palliative radiotherapy) was chosen expecting sustained and relatively quicker relief of symptoms. By the end of treatment, he was able to swallow semisolids without difficulty. The base tongue lesion showed significant regression at radiotherapy conclusion []. Subsequently, he was referred to medical oncologist for targeted therapy. Till the time of last follow-up (3 months postradiotherapy), durable response at the base tongue lesion was documented but unfortunately he was yet to start targeted therapy due to financial issues.
A 45-year-old woman presented to medical attention for abdominal pain, episodic diarrhea, and a mild weight loss (<10% body weight). Informed consent was obtained from patients included in the study. All procedures performed were in accordance with the ethical standards of our institutional research committee. She underwent a colonoscopy that showed a 4 cm stenosis of the sigmoid colon, covered with pale mucosa that could not be passed by a conventional colonscope (13.2 mm) but could be passed with an enteroscope (9.2 mm) with which the cecum could be reached; the mucosa of the other colonic tract, as well as the mucosa of the terminal ileum, were unremarkable. Random ileal and colonic biopsies were obtained, which were unremarkable as well. Histology of the stenosis showed mild inflammation, mild stromal fibrosis, and architectural disruption. Subsequently, she underwent a computed tomography scan, which showed a mild thickening of the terminal ileum and sigmoid colon. The finding of thickened terminal ileum was further confirmed by a subsequent magnetic resonance imaging enterography, suggestive for Crohn disease (CD) localized at the terminal ileum. Due to the discrepancy of the radiological findings with the initial endoscopic examination, a further colonscopy with retrograde ileoscopy was performed, showing no visible endoscopic signs of inflammation and confirming the sigmoid stenosis. The repeated ileal and colonic biopsy sampling was again not diagnostic, showing a nonspecific pattern of inflammation affecting the sigmoid colonic specimens.\nThe physician in charge of the patient, in view of her clinical history and the radiologic finding of intestinal thickening, reckoned that the patient was affected by CD with terminal ileum and sigmoid colon localization and proposed a therapy immunomodulators: corticosteroids 1 mg/kg for 12 weeks together with antitumor necrosis factor (TNF) infliximab 5 mg at weeks 0, 2, and 6, followed by infliximab alone. After 6 months of treatment with infliximab (5 mg/kg every 8 weeks as maintenance), an endoscopic reevaluation was scheduled, showing a resolution of the stenosis in the sigmoid colon and a normal-appearing terminal ileum. The marked improvement of the sigmoid inflammation together with the finding of fibrosis on the corresponding histology further convinced the physician in charge of the patient in favor of a diagnosis of CD, and the anti-TNF treatment with infliximab 5 mg/kg was continued. After 3 months (9 months after the beginning of anti-TNF), the patient was admitted for obstructive symptoms. A computed tomography suspected neoplasia of ileocecal region. The patient underwent an uneventful ileocecal surgical resection. The surgical specimen consisted of a portion of 8 cm of terminal ileum with the cecum and the vermiform appendix. The ileal wall showed an increased consistency and a diffuse thickening, with cystic-hemorrhagic foci in the perivisceral fat. The ileal mucosa was corrugated and showed a polypoid structure with a wide base (1.5 cm maximum diameter) originating from the submucosa and determining ulceration of the mucosal layer. The ileocecal valve, the cecum, and the appendix were normal, except for a mild lipomatous filling of the submucosa.
An 82-year-old Caucasian woman with no major past medical history was referred to our center after undergoing biopsies of longstanding pubic skin lesions. The patient was otherwise feeling well and had no symptoms. Physical examination revealed a 2-cm ulcerated lesion on her pubis (shown in Fig. ). There were no palpable inguinal lymph nodes, and examination of the genital parts was normal.\nThe biopsy results from another medical center came back with a diagnosis of poorly differentiated squamous cell carcinoma on all the samples. The excision was incomplete, and therefore this patient was addressed for further management.\nThe patient's case was discussed at our multidisciplinary meeting, and wide local excision was decided. The histological report of the reexcision revealed a lesion with a depth of 4 mm in the dermis reaching the limit of the hypodermis, with tumoral cells infiltrating the dermis and harboring nuclear atypia and high mitotic activity. The lesion was ulcerative with a fibrinous leukocytic exudate. Vascular emboli were reported, and clear margins of 1 cm were achieved (shown in Fig. ). Immunohistochemistry showed positive staining for carcinoembryonic antigen and cytokeratin (CK) 7 on the luminal ductal borders. p40 and CK5 and 6 were also positive on the whole cell population, and epithelial membrane antigen was heterogeneously positive. Those characteristics were consistent with the diagnosis of EPC. TNM classification was pT2.\nGiven the presence of vascular emboli, a staging CT scan of the thorax, abdomen, and pelvis was performed, which showed an image compatible with pelvic carcinomatosis. An additional FDG-PET scan was then requested, which displayed a hypermetabolic left inguinal lymph node and focal uptake in the right colon (shown in Fig. ). Further investigations were conducted, which included a total colonoscopy and an inguinal left node biopsy. The ultrasound-guided node biopsy revealed distant infiltration of the porocarcinoma, whereas the endoscopy showed a large polyp which could not be endoscopically resected. Therefore, right colectomy combined with left inguinal and left iliac node dissection was performed, but the pathological findings showed no metastatic involvement. An additional ultrasound-guided resection was carried out to remove the targeted left hypermetabolic inguinal node, whose histology eventually revealed EPC and confirmed the disease's metastatic setting. The patient has been undergoing close follow-up for the last 4 months without any sign of relapse.
A 47-year-old woman with severe neck pain radiating to the scapula and motor weakness was referred to our emergency room after a car accident. Her symptoms developed immediately after the neck injury and at once she experienced acute dorsal pain. She had no previous history of anticoagulant therapy or hematological coagulopathy, and was in good health before this episode. On neurological examination, she was alert and fully oriented. No neurologic abnormalities were found in cranial nerves or the cerebellar system. However, right side hemiparesis was present with a muscle power of upper limb Grade III and lower limb Grade IV+. Tendon reflexes were absent in right limbs and were slightly hyperactive in the left side. There was no bruise or petechia, and bladder and bowel functions were intact. Paresthesia and slightly reduced sensation to pinprick, light touch, and vibration sensations were noted below the level of the C5 dermatome on the right side. The hematological parameters including platelet count and function of the coagulation cascade were all within normal ranges. Under diagnosis of a cervical lesion, such as, acute disc herniation, emergent radiological studies, including an MRI scan of the cervical spine were performed. Simple radiographs and a computed tomography scan were normal. However, cervical spine MRI revealed a right posteriolateral EDH, from C3 to C5, severely compressing the spinal cord and showing edematous changes (). Urgent laminectomy and hematoma removal was performed 5 hours after symptom onset. Beneath the ligamentum flavum, liquid dark blood with solid clots was detected and evacuated (), but no active, discrete bleeding point was identified. Hemostasis was achieved without difficulty. No tumor or vascular abnormality was found in the epidural space. Histopathological examination of the blood clots revealed fibrin deposition but no evidence of malignancy. The right side hemiparesis improved markedly after surgery. The day after surgery, muscle power on the right side had fully recovered and she was able to walk without assistance. MRIs taken 10 days after surgery revealed that the cervical EDH had completely resolved (). The patient was in good health and free of any neurological deficits at her 6-month follow-up.\nThis article is about an individual case without the approval of an ethical research. We obtained informed consent from the patient.
The patient was a 63-year-old woman with a past medical history of lymphedema of the right lower extremity for the past 2 years and diagnosis of angiosarcoma of the right lower extremity status post right above knee amputation. The patient also had surgical re-vision of the right knee stump due to recurrent disease. Ultimately there was metastasis to lymph nodes, bone, lung, and adrenal glands status post radiation and chemotherapy. Other history included recent right lower lobe segmental pulmonary embolism and bilateral lower extremity deep vein thrombosis treated with rivaroxaban, recent resolution of a large right-sided pneumothorax, morbid obesity for her entire recorded adult life with body mass index (BMI) of 41.6, hypertension, type 2 diabetes mellitus, hyperlipidemia, and no other surgical history. The patient was admitted to the intensive care unit (ICU) from an inpatient rehabilitation facility for worsening shortness of breath. She was found to be in atrial fibrillation and respiratory distress. A review of systems was unable to be obtained due to the patient’s mental status.\nOf note, the patient had an extensive history of radiation and chemotherapy for metastatic angiosarcoma of the right lower extremity and was being followed by a vascular surgeon for lymphedema for the last 10 years. She could not tolerate paclitaxel or doxorubicin due to acute hypersensitivity reaction. She was initiated on monotherapy treatment with gemcitabine approximately 4 months before the presentation and completed six rounds of treatment. The patient remained on monthly denosumab, a RANK ligand inhibitor, for osseous metastasis. The histomorphologic features and immunohistochemistry profile of the right anterior leg soft tissue were consistent with the diagnosis of angiosarcoma with a high Ki-67 mitotic index (45%) and with cells positive for CD34, CD31, and factor VIII (pictures not shown). The positron emission tomography (PET) scan revealed metastatic disease to lymph nodes, bone, lung, and adrenal glands ().\nOn presentation, the patient had a blood pressure of 86/53 mm Hg, a pulse of 132 beats/min, temperature of 36.6 °C, respiratory rate of 15 breaths/min, oxygen saturation 98% on bilevel positive airway pressure (Bi-PAP) with FiO2 of 60%, and BMI of 41.6. On the physical exam, she was non-alert and lethargic. Rhonchi were auscultated over the lung fields bilaterally. There was minimal +1 left lower limb edema in contrast to the worse 3+ edema of the right lower stump with serous discharge from the surgical incision consistent with wound dehiscence. There were no signs of purulence, erythema, or cellulitis associated with the wound. The rest of the physical examination was unremarkable.\nThe complete blood count showed an elevated white blood cell count of 16.5 × 103/µL and low hemoglobin of 9.4 g/dL (). Chest X-ray showed bilateral effusions, for which she underwent thoracentesis with pleural fluid analysis showing groups of malignant cells with positive CD34 and partial CD31 and epithelial membrane antigen positivity supporting the diagnosis of metastatic angiosarcoma (-).\nThe patient presented with worsening shortness of breath, primarily consistent with acute hypoxemic respiratory failure in the setting of underlying metastatic pulmonary disease and malignant pleural effusion. She was also found to be in atrial fibrillation with a rapid ventricular response. She was subsequently given cardizem bolus and infusion and was intubated due to persistent respiratory distress. She was started on empiric antibiotic therapy with vancomycin and a combination of piperacillin and tazobactam. She continued to require ventilation support for oxygenation.\nThe hematology-oncology service was consulted, and the patient was found to have significant progressive decompensation of underlying metastatic pulmonary disease from angiosarcoma of the right lower extremity, likely causing respiratory failure and cardiac instability. She was not a candidate for any additional therapy options for the underlying metastatic disease. The prognosis and goals of care were discussed with the patient’s family and the decision was made to transition to hospice care. The patient was made comfortable and ultimately passed away in the ICU.
The patient was a 64-year-old man with destructive injury of both lower extremities due to a machine accident. His left ankle and heel bone, along with the soft tissue, were torn off, and the peripheral blood supply and sensation to the toes of the left foot were lost. We amputated his left leg below the knee. The left fibular head was retained after the left foot operation. Two months later, the patient came to our hospital for additional treatment after debridement of necrotic tissue operations twice. There was still approximately a 20 ×10-cm area on the right foot and right external ankle exposing the distal fibula fractures. X-ray showed that the right external ankle bony defect and the right medial ankle mortise widened; fortunately, the left fibular head was retained after left foot amputation (Fig. ). We reconstructed the right ankle using vascular anastomosis of the fibular head and a flap taken from the left stump. The Ethics Committee of the Second Affiliated Hospital of Soochow University approved the study.\nWe used an ultrasonic Doppler instrument (Model: ES-1000SPM Hayashi Denki Co., Ltd, Hong Kong China) to find the perforating branch of the peroneal artery before surgery, and near that location we created a flap approximately 20 × 10 cm in size according to the right foot wound (Fig. ). Surgery was performed with the patient under general anesthesia. The patient was placed in the supine position on the operating table with the left knee joint slightly bent and the left lower limb stump in internal torsion. The incision began in the popliteal space, swept down to the fibular head, and continued along the lateral peroneus muscles to the stump extremity. We cut the skin and subcutaneous tissue, then separated the peroneus longus and soleus muscles. We found the nervus peroneus communis on the inner posterior margin of the biceps femoris tendon and dissociated it for protection. The peroneal perforator was located in the intermuscular space between the peroneus longus and triceps surae muscles, then we freed the peroneal artery and 2 accompanying veins. We cut off the biceps femoris tendon, fibular collateral ligament and other muscle tissue attached to the fibula, extracted the fibular head, and then divided the superior tibiofibular joint. On release of the tourniquet, bleeding spots on the fibula and residual muscle tissue showed that the blood supply was satisfied. Therefore, we could then cut off the vessels and take the fibular head (Fig. ). We sewed the biceps femoris tendon and fibular collateral ligament with deep fascia around the lateral tibial plateau to avoid instability of the knee joint. We completely debrided bone splinters and necrotic soft tissue from the lower right limb and smoothed the edges. The free fibular head was fixated to the distal fibula with a fibular plate. The reconstructed inferior tibiofibular joint was reinforced with 3 lag screws before anastomosis of the vessels in the transplanted fibular head with the recipient area. Lastly, we made a suture flap in the wound and inserted rubber drainages (Fig. ).\nDue to necrosis of the skin and soft tissue of the distal flap 7 days after the operation, part of the reconstructed external ankle bone was exposed, so we transferred the sural neurocutaneous vascular flap to repair the skin and soft tissue defects of the lateral malleolus. X-ray examination 3 months after the operation showed that the fracture had a bony union and the shape of the transplanted fibular head was similar to the normal external ankle. At 12-month follow-up, walking function had been recovered with the help of a left artificial limb (Fig. ). According to the Baird–Jackson scoring system, the curative effect was satisfied.
A 53-year-old female presented with gradually progressive diminution of vision in her left eye for 2 months. She had a history of diabetes mellitus for 20 years for which she is taking insulin. Her most recent HbA1C was 7.5%. She had no history of hypertension or renal problems. She had no past ocular history. Examination revealed a corrected distance visual acuity (CDVA) of 20/40 in her right eye and 20/100 in the left. Anterior segment examination showed nuclear sclerosis in both eyes. Posterior segment examination revealed intraretinal hemorrhages in all 4 quadrants indicating severe nonproliferative diabetic retinopathy in both eyes with clinically significant macular edema in the left eye confirmed by fluorescein angiography (). Spectral domain optical coherence tomography (OCT) of the macula was done and revealed multiple cystic spaces, mild subfoveal neurosensory detachment, and diffuse retinal thickening with a central subfield macular thickness of 332 μm in the left eye (). The right eye showed only few cystic spaces with minimal thickening. OCTA (Optovue, Inc., Fremont, CA, USA) was done in both eyes and showed areas of capillary nonperfusion in the superficial capillary plexus (SCP) of the maculae of both eyes (Figures and ). Three monthly intravitreal bevacizumab injections were done to treat the macular edema in the left eye. One month following the last intravitreal injection, CDVA improved to 20/60 in the left eye and was stable in the right eye. OCT showed improvement of the macular edema in the left eye (). OCTA was performed in both eyes and showed decreased vascular density of the SCP of the left eye compared to pretreatment OCTA while a mild increase was noted in the vascular density of the SCP of the untreated right eye (Figures and ). The patient was then followed up without requiring further intravitreal injections and 4 months following the last intravitreal injection OCTA was repeated in both eyes and showed improvement of the vascular density of the SCP of the left eye with unchanged SCP in the right eye (Figures and ). CDVA was 20/60 in the right eye and 20/100 in the left. Clinical examination revealed retinal neovascularization in the right eye with clinically significant macular edema in both eyes. OCT showed increased center-involving macular edema in both eyes with recurrent neurosensory detachment in the left eye (). Three monthly intravitreal bevacizumab injections were then done for treatment of proliferative diabetic retinopathy in the right eye and the macular edema in both eyes. One month following the last intravitreal injection, CDVA was 20/40 in the right eye and 20/60 in the left, OCT showed decreased macular thickness in both eyes (), and OCTA revealed decreased vascular density of the SCP of both eyes (Figures and ). Changes in the deep capillary plexus closely followed those in the SCP at all stages.
In July 2014, a 59-year-old Japanese female with no significant past history presented to our hospital with a lump and pain in her right breast that had gradually worsened for 3 years and 3 months. She had never undertaken any mammography breast cancer screenings, although it was offered through the local government of Minamisoma City. In April 2011, she first noticed a lump in her right breast. It continued to gradually enlarge, and in September 2012, pain appeared in her breast, leading to the first disclosure of her symptoms to her general practitioner, whom she had frequently (3–4 times per year) visited for antimicrobial treatment for recurrent urinary tract infections since March 2010. However, she was falsely diagnosed with intercostal neuralgia due to her pain, without consideration of the lump. She did not seek a second opinion, reporting that she trusted the judgment of her doctor. From this event, she continued to see her general practitioner, while ignoring her deteriorating symptoms. She had knowledge of breast cancer, and suspected that it may have caused the lump, yet avoided thinking about it. Her general practitioner retired in December 2012, and another physician immediately took over. However, while she saw the new physician twice in 2013, she did not disclose her breast symptoms. In July 2014, 1 month after her lump abruptly enlarged, she first presented to our hospital, where postdisaster specialized breast cancer care had restarted in August 2011.\nAt initial examination, her lump was 2 cm in diameter, fixed to her chest wall. The pathological examination showed tumor cells of invasive ductal carcinoma, with positive estrogen receptor, negative progesterone receptor, and negative human epidermal receptor type 2. None of the further examinations revealed any lymph node swelling or distant metastasis. She was diagnosed with stage IIIB right breast cancer. Because of her intense pain, surgery was prioritized before medical therapy. In August 2014, a right mastectomy and axillary lymph node dissection was conducted. The surgery revealed that the tumor had invaded the right pectoralis major muscle, part of which was resected en bloc. The pathological diagnosis was stage IIIB right breast cancer, with negative margins. Adjuvant chemotherapy of FEC, consisting of 5-fluorouracil, epirubicin, and cyclophosphamide, for 4 cycles, followed by tri-weekly paclitaxel for 4 cycles, was administered after the surgery. In May 2016, she continues hormonal therapy with aromatase inhibitor, with no relapse or adverse effects.\nAdditional history taking revealed that the patient was exposed to social isolation after losing contact with her daughter and friends in the aftermath of the March 2011 disasters, which likely resulted in lessened opportunities to seek advice about her changed health status. Before the disasters, she had lived by herself since the death of her husband. Her daughter and her family, living in the same city, frequently visited her and provided social support. Additionally, she regularly kept in touch with her friends in the neighborhood. After the disasters, the patient, her daughter's family, and her friends were physically unharmed. However, the daughter's family evacuated to another city, approximately 80 km away, in fear of radiation exposure. The patient hesitated to relocate with them, eventually deciding to remain because she did not want to become a burden to them in the already difficult postdisaster period. This concern is also what led her to rarely call them on the phone after their evacuation, or contact her neighborhood friends. She did not use any social networking services.
A 23-year-old man presented with progressive swaying to either side while walking for five months and progressive headache associated with vomiting for four months prior to presentation. There was no history of seizures, diplopia or similar illness in family members. Examination revealed bilateral papilloedema and bilateral cerebellar signs. The patient was initially evaluated by a general practitioner at local hospital with computed tomography (CT) of the head. It revealed a midline posterior fossa mass lesion, isodense on non-contrast CT and enhancing heterogeneously on contrast injection with obstructive hydrocephalus. He was subsequently referred to our hospital. The patient was first seen at our hospital two months after the initial CT scan. Magnetic Resonance Imaging (MRI) of the brain done about 2 months after initial CT showed increase in the size of the tumor which was isointense on T1WI and T2WI and enhancing heterogeneously on gadolinium contrast injection. The lesion was completely intra-fourth ventricular []. Another lesion was noted in the septum pellucidum extending to the frontal horn of the left lateral ventricle causing obstruction of the foramen of Monro on the left side []. It had the same signal characteristics as that of the tumor. This lesion was not present in the initial CT scan [], which was done two months prior to the MRI. There were no other lesions seen on craniospinal MRI.\nIn view of severe symptoms of raised intracranial pressure, he initially underwent ventriculo-peritoneal shunt placement followed by midline sub-occipital craniectomy and near total excision of the tumor except for the part adherent to the brainstem. Post-operatively, he recovered well.\nHistopathological examination showed a cellular glial neoplasm consisting of proliferating ependymal cells arranged in compact sheets and several intervening perivascular rosettes. The cells exhibited brisk mitotic activity (6 to 7 in 10 high power fields) with areas of necrosis []. These features suggested a diagnosis of anaplastic ependymoma (grade III).\nThe patient was subsequently referred to radiation oncologist for adjuvant therapy.
A six-month-old Caucasian male with no significant past medical history presented to the pediatric emergency department for a chief complaint of frequent unusual eye movements as reported by the parents. The patient began having intermittent rotary nystagmus approximately three weeks ago, and since then it progressed and became more continuous, worsened by changes in position or flashing lights from toys brought in his visual field. The parents also noticed the patient sleeping more than usual, only staying awake for an hour at a time, and had a subsequent severe regression in milestones, with an inability to roll over or sit unsupported.\nInitial workup in the emergency department included a CT brain without contrast, which showed evidence of thickening of the bilateral optic nerves with associated masses and diffuse bilateral ventriculomegaly (Figure ). This finding was concerning for possible bilateral optic pathway gliomas, at which point a dedicated MRI of the brain and orbits was recommended, along with further genetic workup for possible neurofibromatosis I.\nMRI of the brain and orbits confirmed these findings, showing diffuse enlargement of the optic tracts extending from the lateral geniculate nuclei to the optic chiasm and into the bilateral optic nerves, with a mass demonstrating 270º encasement of the bilateral supraclinoid internal carotid arteries and near circumferential encasement of the bilateral A1 segments of the anterior cerebral arteries (Figures , ).\nThese imaging findings confirmed the suspicion of bilateral optic pathway glioma, and as a result, the patient was admitted to the hospital and evaluated by both neurosurgery and pediatric hematology-oncology. Neurosurgery did not recommend any surgical intervention at the time, and the hematology-oncology team recommended proceeding with chemotherapy. His genetic testing results showed no evidence of clinically significant variants of neurofibromatosis I or II, suggesting a sporadic bilateral optic pathway glioma. The patient was then discharged and was followed up on an outpatient basis by both ophthalmology and pediatric hematology-oncology for maintenance chemotherapy with carboplatin and vincristine.
A 21-year-old man presenting rectal bleeding visited our hospital. Based on initial laboratory results, his hemoglobin was mildly decreased of 12.7 g/dL, but no additional abnormalities were found. A colonoscopy indicated multiple colon polyps with hyperemic features (). Histological examination revealed hamartomatous polyps with cystically dilated glands, which were surrounded by stroma and showed prominent inflammatory cell infiltration (). Upper endoscopy identified the presence of other lesions and revealed numerous reddish polypoid lesions in the whole stomach (). To confirm small bowel involvement, capsule endoscopy was conducted and it showed numerous polypoid lesions in the jejunum and the ileum (). Endoscopic removal of gastric and colonic polyps was done. We planned the surveillance with upper endoscopy and colonoscopy every 1 to 2 years, and also planned the evaluation of small bowel every 2 to 3 years.\nThe patient's 47-year-old mother had a history of subtotal gastrectomy due to gastric polyposis with anemia 15 years earlier. She had also undergone a right hemicolectomy because of ascending colon cancer 2 years previously. A colonoscopy performed during that time indicated a pedunculated polyp, which is similar to the type of polyp found in her son (). The histological analysis of the polyps showed hamartomatous features that were consistent with juvenile polyposis () Upper endoscopy revealed multiple erythematous polyps in the stomach including cardia which are similar to her son ().\nGiven the clinicopathological findings and the family history, the diagnosis was FJP. Subsequently, written informed consent was obtained from both patients to perform genetic testing, and screening was performed to determine the presence of a germline mutation in the SMAD4, PTEN, and ENG genes in both patients. All coding exons and the flanking intronic regions of the SMAD4, PTEN, and ENG genes were amplified by polymerase chain reaction on a thermal cycler (Applied Biosystems, Foster City, CA, USA) using primer pairs designed by the authors. Direct sequencing was performed using the same primers on the ABI Prism 3130 Genetic Analyzer using the BigDye Terminator Cycle Sequencing Reaction Kit (Applied Biosystems). The sequences were analyzed using the Sequencher program (Gene Codes Corp., Ann Arbor, MI, USA) and compared with the reference sequence. The direct sequencing analysis revealed a novel nonsense mutation in the SMAD4 gene in both the mother and the son. The mutation, a C to G transition at position 1239 in exon 10, resulted in premature stop codon at tyrosine 413 (p.Tyr413*) (). To our knowledge, this mutation has not been described previously. The mutation was not detected in the PTEN and ENG genes.
A 44-year-old previously healthy Asian female reported to the neurology clinic with complaints of episodic, persistent, uncomfortable needle-like sensations in her genitalia extending to the anal area and the tip of the coccyx. She had been suffering from these symptoms for approximately 11 years. She had difficulty describing the actual nature of this phenomenon, which, according to her, was more of an irritation and discomfort than actual pain. The discomfort was aggravated during rest and periods of inactivity, particularly at night. It responded briefly to mefenamic acid tablets for four to five hours. She also reported the pain was worse before her monthly menstrual cycle but that sexual activity did not affect her discomfort. Sometimes, the discomfort was intense and would wake her from sleep causing severe discomfort and resulting in difficulty sitting; walking would relieve her symptoms partially. She was better in the morning, but the symptoms appeared again at end of the day. She had multiple gynecology and dermatology consults with no relief or clear diagnosis. She was prescribed antifungal creams, including fluconazole and ketoconazole, topical steroids (betamethasone and hydrocortisone), and mefenamic acid for pain relief. All failed to bring relief. She also consulted a homeopathy practitioner, who labeled her as suffering from a chronic skin disease and prescribed oral and topical medication. She took it for a few months but stopped it due to lack of relief. Ultimately, due to the distress and anguish associated with the condition, lack of response to various treatments, and social embarrassment, she stopped visiting doctors for some time. However, during a recent visit, the consulting gynecologist suspected it was not a dermatological or gynecological problem and referred her for a neurology consult.\nThere was no history of back pain, sensory symptoms in the legs, urinary incontinence, or neurological disorder, such as Parkinsonism or restless leg disorder. She had a stable and happy marital life of 22 years and had three children. She has used no substance of abuse or recreational drugs. Her neurological and gynecological examinations, as well as magnetic resonance imaging (MRI) of the spine, were normal (Figure ). Similarly, her biochemical profile (including complete blood count, urine analysis, serum urea, and creatinine) and an ultrasound examination of abdomen and pelvis were normal.\nConsidering her history of circadian rhythm variations in symptoms and aggravation on rest, RGS was considered the most likely cause of her symptoms. After a detailed discussion with the patient, she was prescribed 0.5 mg of ropinirole once at night. It was increased to twice daily after seven days, to which she responded well and reported a dramatic relief of symptoms after 14 days. The dose was increased to 1 mg in the morning and 0.5 mg at night, which led to further improvement. On her third follow-up after two months, she reported taking her medication regularly, which led to almost no episodes of discomfort, with exception of one or two times. The dose of ropinirole was maintained for one month. At the three-month follow-up, she reported only one episode of pain and itching, when she missed one dose of her medicine for a few days; otherwise, she had no complaints and reported no side effects of the medication.
A 50-year-old male was admitted to our hospital for optimal control of blood sugar. The patient had been diabetic for 1 year and was being treated with an oral hypoglycemic agent. A recent blood sugar profile indicated a poorly controlled state, with glycated hemoglobin of 9.6%. The patient had a 30-year smoking history. In the previous month, the patient had been hospitalized via the emergency department to evaluate acute chest pain and minimally elevated serum cardiac troponin I. Coronary angiography conducted under the impression of unstable angina revealed an intermediate lesion at mid-left anterior descending artery (LAD). The patient was treated with medical therapy. On discharge, the patient received a beta-adrenergic blocker and isosorbide dinitrate, but ceased taking the nitrate several days later because of severe headache.\nOn the 5th hospital day in the morning, the patient developed excruciating chest pain followed by drop of systolic blood pressure to 70 mm Hg. Heart rate was 69 beats/min, and breathing rate was 28/min with 98% oxygen saturation on nasal oxygen (3 L/min). The patient was acutely ill looking, with a pale expression and profuse perspiration. Physical examination revealed fine rales on the bilateral lung field. However, cardiac murmur, friction rub, hepatomegaly, and peripheral edema were not present. An electrocardiogram showed wide QRS complex with right bundle branch block type morphology, and ST-segment elevation in multiple sites including inferior and anteroseptal leads (). Chest radiography revealed evidence of pulmonary edema in both lung fields (). The patient was first treated with crystalloid fluid resuscitation and an infusion of an inotropic agent (dopamine). Initial assessment of the patient suggested the possibility of cardiogenic shock either from right ventricular infarction complicating acute inferior myocardial infarction or from extensive myocardial infarction due to proximal occlusion of left coronary artery. Because of the profound hemodynamic instability, the patient was immediately transferred for cardiac catheterization. On arrival at the catheterization room, the patient developed ventricular fibrillation () and was successfully resuscitated by immediate cardioversion without apparent neurological sequelae. A coronary angiogram revealed a severe spasm of the epicardial arteries, resulting in diffuse and near total occlusion of the proximal right coronary artery (RCA), subtotal occlusion of the left circumflex artery (LCX), and non-occlusive significant narrowing of the proximal LAD (). The coronary lesions were promptly relieved by intracoronary administration of isosorbide dinitrate () with resolution of the chest pain as well as ST-segment elevation. Vital signs rapidly stabilized. The next day, transthroacic echocardiography revealed regional wall motion abnormality of LAD territory. Serum troponin-I peaked at 22.78 ng/mL and was normalized after 3 days. The patient was treated with amlodipine at a dose of 5 mg per day and with isosorbide dinitrate. Since discharge, the patient has been followed-up regularly in the outpatient clinic without any complaints of chest pain.
A 67-year-old Caucasian female with glipizide- and metformin-treated diabetes mellitus and enalapril-treated arterial hypertension presented for open reposition and Zuggurtung fixation of a fracture of the olecranon. The elbow was fractured in a bicycle accident and had been conservatively treated for 14 days prior to surgery. The patient had suffered from diarrhoea and signs of confusion in the period between the trauma and surgery, but had been free of gastrointestinal or neurological symptoms during the week before surgery. The patient had not sought professional medical advice for these symptoms. When the patient presented for surgery, she was lucid, without any gastrointestinal symptoms and otherwise physically well. The preoperative laboratory work-up tests were without pathological signs (Table ). At the operating ward, 1 g paracetamol, 100 mg diclofenac, 10 mg oxycodone and 25 mg meclizine were administered orally as premedication. The anaesthesia was induced with propofol and fentanyl. The airway was secured by orotracheal intubation after which the anaesthesia was maintained with sevoflurane. The time of surgery was 31 min and the total anaesthesia duration was 70 min. After uncomplicated surgery and anaesthesia, the patient was transferred to the postoperative unit from where the patient went home after 3 h accompanied by her next of kin. During the first postoperative evening and night, the patient’s next of kin observed that the patient had problems with articulating words followed by increasing anxiety and confusion. At the next morning, the patient only spoke unrecognizable words, and by the afternoon, she could not walk. Approximately 24 h after leaving the hospital, the patient presented at the emergency ward. Respiration and circulation were normal and the patient was afebrile. A computed tomography (CT) scan of the brain was performed as well as an analysis of the cerebrospinal fluid, with both indicating no pathological signs. A neurological consult ordered an electroencephalogram (EEG) and a magnetic resonance imaging (MRI) of the brain to be performed as soon as possible at a secondary hospital to which the patient was referred. At arrival to the secondary hospital on the second postoperative day, the patient did not respond verbally, could not open her eyes spontaneously and the best motor response was withdrawal of the limbs on pain stimulation (Glasgow Coma Scale, GCS 9). It was noted that plasma creatinine was elevated to 313 μmol × L-1. Except for an elevated C-reactive protein (CRP) at 76 mg × L-1, all blood analyses were in the normal range, including haemoglobin, leucocyte and platelet count. On the night between the second and third postoperative day, the patient suffered a generalised tonic-clonic seizure. The patient was transferred to the intensive care unit, where she, was orotracheally intubated and mechanically ventilated. A second CT scan of the brain showed no bleeding or ischaemic signs. The patient was respiratory and circulatory stable and did not require an increased oxygen fraction or vasoactive drugs. The patient was anuric, was not icteric and did not have any pathological signs on the skin. Blood cultures were drawn and an antibiotic was given based on a body temperature of 38.9°C and tachycardia. The patient was started at 4 g piperacillin-tazobactam 3 times per day intravenously (i.v.). The cultures did not show any bacterial growth. Because the patient had elevated serum potassium of 5.8 mmol × L-1 and hyponatraemia of 131 mmol × L-1, blood was analysed for serum cortisol on the suspicion of Addison’s disease. Accordingly, the patient was given 100 mg hydrocortisone i.v. Cortisol in serum was found to be 796 nmol × L-1 and thus the suspicion of Addison’s disease was refuted. The patient was now anaemic with a haemoglobin of 86 g × L-1 and thrombocytopenic with a platelet count of 31 × 109 × L-1. Lactate dehydrogenase was elevated to 21.5μkat × L-1 and haptoglobin low at 0.07 g × L-1, which indicate haemolysis. The blood film showed schistocytosis., On the third postoperative day, plasmapheresis was started on the indication of HUS/TTP. One plasma volume was replaced daily for four days and continuous veno-venous haemodiafiltration was performed between plasmapheresis treatments. The patient’s haemoglobin and platelet count improved during treatment (Table ). The same positive development was seen for creatinine. Neurological status improved slowly and on the seventh postoperative day, GCS was 6 with withdrawal of the limbs on pain stimulation. Polymerase chain reaction (PCR) revealed enterohaemorrhagic E. coli (EHEC) in faeces. The serotype was non-O157, produced verotoxin type 2 and was eae-gene negative. Blood analysis of Adamts-13 protein activity showed normal levels and antibodies against the protein were not observed. Signs of multiple small ischaemic fronto-temporal cortical lesions were noted on an MRI scan, as well as lesions in the circulus Willisi and the basilar artery with narrowing and more distal dilatations of the vessels. Analyses of anti-neutrophil antibodies and anti-neutrophil cytoplasmic antibodies were negative. The neurological consult assessed the clinical picture together with the MRI findings as thrombotic microangiopathy. The patient continued to show slow improvement in neurological status with spontaneous eye opening and the ability to move all limbs, although with substantial weakness. The patient could make eye contact on instructions on the 20th postoperative day but was still anuric and in need of intermittent haemodialysis and mechanical ventilatory support. On the 21st postoperative day, the patient suffered a generalised tonic-clonic seizure followed by deep coma (GCS 3). EEG showed generalised deeply suppressed activity. A joint decision was made with the next of kin to withdraw all treatment. The patient died 6 h later.
HG is a 60-year-old, well-educated man who suffered a right parietal hemorrhage four years before this study. He had a first neurological and neuropsychological examination within a week after his stroke, which showed partial sensory loss of the left arm, left inferior quadrantanopia, moderate spatial neglect and visual-constructive deficits. While hospitalized, the patient had a series of partial epileptic seizures whose focus was localized to the right parietal lobe. He quickly recovered arm sensation and part of his deficits of spatial cognition, though he was unable to take up driving and work due to persistent fatigue. At the time of this study his neuropsychological assessment revealed a slight, but significant bias on line bisection, visual-constructive deficits as well as increased reaction times to stimuli shown in his left visual hemifield and in visual search (), consistent with his report of slight spatial neglect in activities of daily living (e.g., orienting in new environments, bumping occasionally into people on his left, searching for his keys). A magnetic resonance imaging (MRI) scan performed two years after his hemorrhage showed local damage in the superior part of the posterior parietal cortex and underlying white matter (). The patient was seen three years following his hemorrhage for a routine examination. At this time, he still had partial left inferior quadrantanopia, though the central ~10° of vision were preserved (). He had been without seizures for more than two years, but continued to receive anticonvulsant medication (carbamazepine).\nIllusory perception was detected when we tested extinction on a PC screen with a simple paradigm (black disks shown unilaterally or bilaterally for 176 ms). Our expectation was that HG would be capable of detecting unilateral stimuli in both hemifields, but would eventually fail to report the left stimulus when a competitive item appeared simultaneously on the right (which would correspond to an extinction phenomenon). Preliminary testing showed that the patient always saw a stimulus presented in the right hemifield, but also reported an additional stimulus at the mirror position in his left hemifield. This illusory contralesional stimulus appeared and disappeared simultaneously with the target, could be seen with the left or right eye and had similar size as the target.\nThe patient gave written informed consent before participating in this study, and our investigation was approved by the Ethical Commission of the Canton of Geneva.
A 6-year-old boy with no pathological history accidentally fell from the top of an approximately 3 m climbing pole and injured his right extended elbow and wrist joint. Due to pain and deformity in the right elbow and wrist joints, he visited our hospital. Swelling and a dinner fork deformity of the right wrist joint and pronounced swelling of the right elbow joint were observed. No skin damage was observed. No findings of nerve injury or arterial injury were obtained in the right upper limb. Radiography revealed lateral dislocation of the radial head, a fracture of the proximal ulnar metaphysis, and mild bending deformation at the fracture site. In addition, fractures of the distal radius and ulna, as well as dorsal displacement of the distal fragment, were seen (). Thus, the patient was diagnosed with Bado type III Monteggia injury with ipsilateral fracture of the distal radius and ulna.\nManual reduction under nerve block was attempted on the day of injury. However, because it was difficult to maintain the reduction of the radial head, as shown in , open reduction and percutaneous procedures were performed under general anesthesia. A Kirschner wire was inserted, percutaneously, from the olecranon into the ulnar diaphysis. When the Kirschner wire was in place, the dislocation of the radial head immediately showed good reduction. Further, open reduction and fixation of the fractured distal radius and ulna were performed with Kirschner wires (). A long-arm cast was used for external fixation with the elbow in 90° flexion and the forearm in an intermediate position.\nTwo weeks after surgery, callus formation at the fractured bone was observed. Therefore, the cast was removed, and range of motion (ROM) exercises of the elbow and wrist joints were initiated. Since bone union was achieved at 6 weeks postsurgery, the Kirschner wires were removed. Pain, ROM limitation, and lateral instability were not observed in the elbow or wrist joints at 3 months after surgery. Additionally, plain radiographs taken at the same time showed a radially convex curvature at the proximal portion of the ulna and lateral subluxation of the radial head (). However, a gradual correction in the outward displacement of the radial head was observed during the 3-year follow-up.\nTwenty-one years after surgery, the patient returned to our hospital for another disorder. At that time, we obtained informed consent to perform an examination and take radiographs of the previous Monteggia injury. Neither spontaneous pain, pain during exercise, tenderness, nor ROM asymmetry were observed (). The biocompatibility of the radiocapitellar joint was good, and no malunion was found in the distal radius and ulna (). The patient reports that he has been working as a computer programmer and performs weight training as a hobby without limitations.
We here describe a 28-year-old female patient who was diagnosed with scimitar syndrome a few days after birth. Scimitar syndrome is a very rare anomaly described to occur in about two of 100,000 births, of which females are affected in a twofold predominance. This syndrome is defined by the presence of a scimitar vein that provides an abnormal venous drainage of the right lung into the inferior vena cava. In addition, several other cardiopulmonary anomalies have been described in association with a scimitar vein (reviewed in []) including but not limited to hypoplasia of the right lung with consecutive dextroposition of the right heart, pulmonary sequestration, perimembranous ventricular septal defect, and a persistent ductus arteriosus. All these abnormal findings were present in the case described here.\nAt the age of five months, the girl underwent surgical repair to redirect the anomalous lung veins via patch into the left atrium, closure of the ventricular septal defect, and ligature of the ductus arteriosus. The pre- and postoperative situations are illustrated in . The postoperative cardiac catheterization on the day of the operation showed complete obstruction of the right-sided pulmonary venous return and missing anterograde perfusion of the right lung. Due to the complex anatomical situation no further surgical interventions were performed and the patient remained stable with slightly impaired functional capacity for over 20 years.\nAt the age of 28, hemodynamic assessment by right heart catheterization confirmed complete occlusion of the right pulmonary artery and elevated pulmonary pressure (mean pulmonary arterial pressure (mPAP) of 29 mmHg with normal wedge pressure (PAWP) of 14 mmHg). Long-term oxygen therapy and PH-specific treatment with an endothelin-receptor antagonist (bosentan) were established. The course during the following years was characterized by frequent pulmonary infections but the clinical status remained stable under treatment with bosentan, diuretics, and oral anticoagulation. At the age of 34, a new episode of severe pulmonary infection with subsequent respiratory failure, septic shock, and multiorgan failure occurred. Despite maximal therapeutic efforts, the patient deteriorated further and died one day after deescalation of treatment to a palliative concept.\nAt autopsy, complete occlusion of the redirected right pulmonary veins and hypoplasia of the right lung were found. The lungs had dense consolidation suggesting diffuse alveolar damage as a consequence of pulmonary infection and acute respiratory distress syndrome. Massive dilatation and hypertrophy of the right ventricle was found (“cor pulmonale”) indicating severe pulmonary hypertension and right heart failure. Microscopic analysis () showed pulmonary veno-occlusive disease with prominent pulmonary capillary hemangiomatosis of the left lung. In particular, thickening of the alveolar septa due to capillary proliferation and congestion and massive iron deposition in the alveolar spaces secondary to venous obstruction were found (). Moreover, arterialization and intimal fibrosis of venules in the interlobular septa with subtotal luminal occlusion were described (). Conversely, the right lung was characterized by a normal alveolar architecture (). Right-sided pulmonary arteries showed alterations probably due to pressure overload with intimal proliferation and hypertrophy of the medial layer () but no signs of PCH or PVOD, indicating a reactive, unilateral process of the left lung.
A 46-year-old male patient presented to our emergency department with a history of generalized abdominal pain of 7 days' duration. The pain had become more localized to the right lower abdomen for the last 2 days. There was a history of constipation lasting for 3 days. There was no vomiting and he did not have any chest or abdominal complaints in the past. There were no known co-morbidities. There was no history of recent trauma or surgery. On physical examination, he was febrile (101 Fahrenheit) and had tachycardia. Abdomen was distended and the liver dullness was obliterated. There was generalized abdominal tenderness in addition to rebound tenderness in the right iliac fossa. The bowel sounds were absent. The haemogram showed leucocytosis (11000/Cu mm). Chest X-ray showed free air under the diaphragm (Fig ) and abdominal X-rays showed a markedly dilated transverse colon. A preoperative diagnosis of hollow viscus perforation with peritonitis was made and the patient was taken up for emergency laparotomy. On laparotomy, there was caecal perforation with faecal peritonitis (Fig ). There was marked dilatation of the caecum, ascending colon and transverse colon up to the level of splenic flexure of the colon. The descending colon was collapsed and there was no mass or band causing the obstruction. The dilated transverse colon was followed and it became evident that it was entering the pleural cavity through a postero-lateral defect in the diaphragm (Fig ). A dilated loop of transverse colon was found in the chest cavity with obstruction at the level of the defect. This loop along with its mesentery was viable and brought down into the abdominal cavity by enlarging the defect in diaphragm (Fig ). The defect was primarily repaired in one layer with interrupted sutures of No-1 prolene and a left intercostal tube drain (ICD) with negative pressure was placed. The caecal perforation was managed by intracaecal placement of a Foley urethral catheter of 20 French to establish a tube caecostomy. In the postoperative period, ICD was removed on the 5th postoperative day. The patient developed mild infection at the laparotomy wound which was treated by conservative regimen. The caecostomy tube was removed after 3 weeks and the patient was subsequently discharged from the hospital.
A 60-year-old female came to the diagnosis of the oral disease department of Shahed Dental School for dental treatment with a chief complaint of lack of masticatory capability and poor esthetic for the past 20 years. Her medical history was significant for osteoporosis and congenital heart disease (CHD). Her current medication was the daily use of ASA as an antithrombotic, Digoxin for CHD, Enalapril for her hypertension, and vit D. Also, she had been medicating with Famotidine and Omeprazole for the last two years due to her masticatory hypofunction and digestive problems. Her medical practitioner advised that her dental treatment should be limited to noninvasive dental procedures with minimal trauma. The patient revealed that there was an absence of the lower and upper anterior teeth after exfoliation of deciduous teeth and the history of mandibular fractures 25 years ago in a motor vehicle collision. In extraoral examination, the prominent frontal and parietal, hypertelorism, depressed nasal bridge, mandibular prognathism, and maxillary hypoplasia were observed (). Also, the patient was able to move shoulders in front of the chest associated with underdeveloped or absent collarbone ().\nIntraoral examination revealed a long span bridge in maxilla from tooth #15 to #25 and multiple missing permanent teeth in the anterior and posterior regions of the mandible. A panoramic radiograph and CBCT were captured (Figures and ).\nRadiographic findings revealed multiple impacted permanent teeth in the anterior region of maxilla and anterior and posterior regions of the mandible as well as the fracture of the left angle of the mandible which had been fixed with wire. The patient was diagnosed with cleidocranial dysplasia evidenced by clinical and radiographic findings. After consulting with a maxillofacial surgeon, the extraction of all teeth (erupted and unerupted) and replacement by implants was not suggested due to the medical conditions of the patient including osteoporosis and CHD. The most conservative and minimally invasive treatment recommended by the surgeon. Therefore, removable partial denture (RPD) was planned to restore both arches. The situation was explained to the patient in detail. A treatment plan including RPD after extraction of hopeless teeth was chosen by the patient, which was the same to therapeutic team choice. The long span maxillary bridge was removed and the tooth #15, 13, 23, 24, 25, 36, and 38 were diagnosed hopeless. The extraction of these teeth was performed under local anesthesia and with minimal trauma. Socket preservation was done with bone graft materials to assist regeneration and healing, and to avoid severe bone loss, particularly in the site of tooth #38. On the other hand, the tooth #26, 27, and 46 were saved due to their stable conditions, increasing the retention and support of the removable partial prosthesis ().\nAfter 3 months, a new OPG was captured, which showed no significant mandibular and maxillary bone resorption ().\nPreliminary impressions were made for both arches using irreversible hydrocolloid impression material (CA37; Cavex Holland BV, Haarlem, Netherlands) in a stock tray. Impressions of both arches were poured to obtain study casts by dental plaster type 2 (Pars Dandan, Iran). Acrylic custom trays were made using auto polymerizing acrylic resin (Bisico-Germany). In the second visit, both custom trays were border molded using green stick compound, the definitive impression was recorded with Panasil initial contact light impression material (Kettenbach, Germany), and final casts were poured in type 3 dental stone (Pars Dandan–Iran). Acrylic resin base and wax rims were fabricated, and maxillomandibular relationships were recorded by facebow. Maxillary and mandibular diagnostic tooth arrangements were prepared to evaluate phonetic and esthetic, teeth position, and to create maxillomandibular relationship. Afterwards, acrylic resin partial dental prostheses were processed from heat-polymerized acrylic resin (Kulzer, Germany) with a heat-cured permanent silicone soft liner (silicone based detax, Germany) and delivered (Figures and and ).\nWrought wire retentive arm was designed on tooth #27 and 46 to elevate the retention of partial dentures. In order to minimize the microbial/fungal colonization of liners and prolong their life, the patient was trained in two sessions on how to maintain the oral and denture hygiene in good condition. Also, the patient was advised to use prostheses except when asleep. An appointment was scheduled after a week for the final adjustment (Figures and ).\nThe patient was examined clinically and radiographically every 3 months, throughout a year. Her quality of life and mastication function was improved significantly, and the patient was satisfied. The prognosis of the mandibular and maxillary natural molars tooth #26, 27, and 46 were excellent. The maxillary and mandibular partial denture did not need to be relined after a year.
Ms X, a 55-year-old married Caucasian woman was admitted to psychiatry ward following an overdose of prescribed medication in conjunction with alcohol. This was her first contact with psychiatric services although she had been on citalopram for a long period from her general practitioner. She experienced low mood, suicidal ideation, reduced appetite, poor sleep, and tearfulness following her divorce and removal of her adopted son from her care. She had a difficult childhood and was sexually abused by her father over a long period of time. The sexual abuse was associated with high negative emotions and a lot of anger. About 3 years ago, she suffered from seizures and cerebrovascular accident resulting in weakness in right leg and dysphasia. Couple of months ago, she started drinking alcohol in moderation in order to cope with her feelings of distress and low mood due to divorce and her son's removal from her care. There was no history suggestive of any illicit drug abuse in this lady.\nDuring her current admission, Ms. X disclosed that she had multiple and distressing spontaneous orgasms since her childhood following sexual abuse by her father. These orgasms would generally be coupled with her feeling angry on any account. However, she stopped experiencing these orgasms with the initiation of citalopram but would experience them again if she did not take citalopram for a few days. Unfortunately, when she was hospitalized following cerebrovascular accident about 3 years ago, citalopram was discontinued in error and she started re-experiencing these multiple orgasms. Apart from hypertension, she did not have any other risk factors to have cerebrovascular accident at the age of 52. She could have up to 70 orgasms in a day and felt totally exhausted. She did not demand citalopram out of embarrassment and shame for 3 months and continued to suffer till she was restarted on citalopram following which the orgasms disappeared.\nMs. X was diagnosed to be suffering from adjustment disorder brief depressive reaction based on her current psychosocial stressors and symptoms (F43.20 of ICD-10). She settled fairly quickly on the ward and improved in her low mood and stopped feeling suicidal and was discharged on citalopram (40 mg daily) and her other medications for her physical health problems (bisoprolol 10 mg mane, levetiracetam1 g twice a day, tramadol 100 mg twice a day).
A 54-year-old Caucasian female proceeded to our institution with epigastric pain, nausea and vomiting along with pain located around the lumbar area lasting for one week. No previous surgical history or commorbidities existed. Clinical examination did not reveal any palpable abdominal masses or abdominal tenderness and the patient’s vital signs were within the normal spectrum. Blood test detected hypercalcemia (serum calcium: 10.2 mg/dL) and parathyroid hormone level of 111.8 pg/mL. All the findings in conjunction with the clinical presentation lead to the assumption that the patient had primary hyperparathyroidism (PHPT).\nThen, an ultrasound was performed but it was negative for any thyroid or parathyroid abnormalities. Subsequently, the thoracic and abdominal CT revealed a soft tissue in the anterior mediastinum 7 × 1 cm. Additional Tc-99m-MIBI scintigraphy followed, which detected an ectopic adenoma located in the lower anterior mediastinum, on the left of the median line (). Following these, a mid-sternal thoracotomy was finally scheduled.\nDuring the operation, after the thoracotomy, surgeons attempted to detect deep into the mediastinum the parathyroid adenoma according to the preoparative localization. Indeed, the mediastinal mass was detected on the left of the median line, at the anterior mediastinum, in front of the anterior surface of the pericardium and close to the left pericardiophrenic vessels and the left phrenic nerve (). The adenoma was covered by a thin fibrous capsule. When surgeons removed the capsule, a dark red mass of 7 × 2.8 × 1 cm was finally revealed (Figs. and 4 ). The detailful preoperative localization of the present mediastinal adenoma which was in close relation with various anatomical structures of the thorax, reduced effectively the difficulty of the mass excision and the potentiality of accidental surgical injuries which may lead to thoracic bleeding and subsequent obstructive symptoms.\nThen, the operation continued in the usual fashion and a drainage was placed into the left side of the thoracic cavity. The patient was discharged the 5th postoperative day with instructions, when the drainage was finally removed.\nHistology of the mass confirmed the diagnosis of ectopic parathyroid adenoma that was composed predominantly of oxyphil cells arranged in an acinar pattern. Serum calcium level was 2.60 mmol/L and iPTH 17.6 pg/mL 12 h after the operation. Serum calcium and iPTH remained normal after 6 months’ follow-up.
A 71-year-old Caucasian female with heart failure and a history of heparin induced thrombocytopenia type 2 (HIT) presented for placement of an off pump HeartMate II. Her baseline activated clotting time (ACT) was 124 seconds. A 50 mg bivalirudin bolus was given followed by continuous infusion of bivalirudin titrated to 1.25 mg/kg/hr to achieve an ACT of 326 seconds after induction of general anesthesia and insertion of invasive lines. We have previously described our off pump LVAD implantation technique\n[]. A median sternotomy was performed surgically. Aortic and right atrial venous cannulas were placed. A partial occlusion clamp was placed across the ascending aorta and the outflow graft was anastomosed to the ascending aorta and then attached to the device likely to reduce time for initiation of mechanical support after LV apex coring. At this time, the LVAD device was primed and de-aired with retrograde blood flow from the outflow graft for approximately 20 minutes. The ACT at this time was 324 seconds. The heart was fibrillated and the apex was cored out. There was no evidence of the thrombosis in the left ventricular apex, but a fresh clot was noted in the inflow cannula of the LVAD pump, which was flushed out. However, another clot was found inside the LVAD pump that was unable to be flushed out. During this critical time, we urgently converted the procedure to on pump by using CPB. In addition, an additional 40 mg bivalirudin bolus was given, and we increased the infusion rate bivalirudin to 2 mg/kg/hr. The ACT was checked 20 minutes later, and it was 728 seconds. A new LVAD device was subsequently placed. After being weaned from the CPB, the patient became coagulopathic, which was thought to be from the additional dose of bivalirudin and she required significant transfusion of blood products. The patient’s chest was closed with only four wires and she was taken to the Intensive Care Unit after the surgical procedure. She returned to the operating room for mediastinal washout 2 days after her original procedure. She was later discharged from the hospital without complications, and was doing well at home at 16-month follow-up.
A 69-year-old man, with hypertension and past history as heavy smoker and chronic alcoholic for 40 years, presented with several episodes of transient weakness involving left lower extremity that lasted for 6 months. Each episode occurred once a week, and lasted approximately for 10-20 seconds. The initial magnetic resonance imaging (MRI) from another hospital revealed multifocal stenosis of cerebral artery including left proximal internal carotid artery (ICA), right proximal external carotid artery (ECA), right middle cerebral artery (MCA) at the M1 segment, and occlusion of right ACA at the A2 segment without any evidence of cerebral infarct in the diffusion weighted imaging. Despite the medical treatment with warfarin for one month, there was an increase in the frequency of transient ischemic attack (TIA) in the left leg. Follow-up MRI revealed acute focal infarct in the right frontal periventricular white matter corresponding to the territory of right ACA (). The rate of occurrence of TIA was once or twice a day despite combined medical treatment with warfarin and aspirin.\nThe patient visited our hospital one month after the first experience of infarct. On presentation, no apparent neurological deficit was found. Cardiac evaluation did not reveal any remarkable findings. Cerebral angiography revealed multifocal atherosclerotic luminal irregularity in both the ICA, especially moderate stenosis of the right supraclinoid ICA segment. Right ACA was occluded at the A2 segment, but collateral flow from right MCA and posterior cerebral artery (PCA) was minimal (). Basal and acetazolamide-stressed brain perfusion single photon emission computerized tomography (SPECT) with 99mTc-hexamethylpropyleneamin oxime was performed to evaluate the patient's hemodynamic status. Brain SPECT showed decreased perfusion and decreased vascular reserve in the right frontal lobe ().\nOral anticoagulant and antiplatelet agent were discontinued 1 week prior to surgery. After 2 months from experience of cerebral infarct, revascularization surgery was performed. After confirmation of sufficient length and integrity of parietal branch of the STA in preoperative ECA angiography, we planned end-to-side anastomosis between ipsilateral STA and distal ACA. Under general anesthesia, curvelinear skin incision was made along the parietal branch of the superficial temporal artery (STA), and then extended to the contralateral frontal just behind the hairline. The parietal branch of the STA were saved and prepared for anastomosis to the distal ACA. The frontal branch of the STA with galeal flap was saved as well during the preparation in case of adverse events, such as graft failure (). The length of parietal branch of the STA was 11 cm from the bifurcation of the STA. After right paramedian craniotomy and opening of the dura, the cortical branches of the right ACA were observed. We chose one of the cortical branches as a recipient artery, in which no flow was detected on the intraoperative Doppler. After confirmation of sufficient length and good pulsation of the parietal branch of the STA, end-to-side anastomosis between STA and ACA was performed (). Anterograde flow from the STA and flow diversion to the recipient artery was confirmed on the intraoperative Doppler.\nThe TIA of the left leg disappeared immediately after surgery. Oral antiplatelet agent (aspirin) was resumed on the first postoperative day with a dose of 100 mg/day. The patient was discharged with no apparent neurological deficit. Six months after the operation, follow-up cerebral angiography showed obvious flow through right STA to the territory of the right ACA and decreased leptomeningeal collateral flow from right PCA (). Brain SPECT revealed improved perfusion and vascular reserve when compared with preoperative imaging (). The patient received follow-up care for 30 months after surgery without occurrence of any remarkable events.
A 45-year-old male presented with the complaints of a small swelling on his right groin which had rapidly increased in size over the preceding 3 months. He had been prescribed some medication by a local physician after which he developed painful bullous lesions over upper part of his right thigh and left inguinal region. He came to our institute and was initially evaluated by dermatologists. A thorough examination revealed bilateral, hard, multiple, mobile, inguinal lymph nodes, and multiple small nodular lesions over the left half of glans penis. On further enquiry, the patient revealed that he had an erythematous patch over glans without the nodular lesions for the past 4 years. A skin biopsy from the groin was suggestive of bullous pemphigoid, and fine needle aspiration cytology of bilateral inguinal lymph nodes revealed metastatic SCC. He was subsequently referred to us for the suspicious lesion on the glans penis []. A wedge biopsy of the largest nodule on glans was performed which showed features of a microinvasive SCC where there was invasion of the superficial dermis and vascular tumor embolization [Figure and ]. There was dominant mononuclear inflammatory cell infiltration along the deeper aspect of the tumor. The tumor cells in the superficial portion were dis-cohesive resulting in cleft formation. Following the biopsy report, contrast-enhanced computed tomography scan of chest and abdomen revealed multiple enlarged necrotic lymph nodes in bilateral external and internal iliac, obturator, and inguinal locations, the largest being in the right hemipelvis measuring 2.5 cm. Two ill-defined subpleural nodules were seen in the anterior basal segment of the right lower lobe and another in lateral basal segment of the left lower lobe. Fluorodeoxyglucose (FDG) positron emission tomography scan showed FDG avid left supraclavicular, abdominal, retroperitoneal, pelvic, inguinal nodes, and also multiple lytic skeletal lesions [Figure –]. A final diagnosis of microinvasive SCC penis with extensive lymph nodal and skeletal metastasis was made. He was subsequently planned for TIP (paclitaxel, ifosphamide, cisplatin)-based chemotherapy in view of extensive metastasis. He received 6 cycles of chemotherapy and is presently undergoing radiotherapy.
A previously healthy 2-year-old female presented to the ED with her mother for neck pain and limited neck rotation that had started that day. At the time of her first evaluation, she had normal vital signs, normal movement of her neck, no lymphadenopathy, a normal neurologic examination, and rhinorrhea. The mother expressed concern that her symptoms were occurring secondary to an impact to her neck when she struck a table the prior day. Both traumatic and viral causes of torticollis were considered in this patient. Due to the presence of rhinorrhea and the lack of neurologic or traumatic findings, the patient was diagnosed with a presumptive viral infection and discharged with instructions for supportive care and routine follow-up.\nThe patient re-presented to the ED with her father two days later with complaint of inability to rotate her head to the right. She was receiving acetaminophen at home without improvement in her symptoms. Her behavior and appetite were unchanged from her baseline. In the ED, her vital signs were again normal and her exam was notable for head deviation to the left with increased tone of the left sternocleidomastoid and multiple enlarged, non-tender, matted lymph nodes in the left anterior cervical chain. A review of systems was notably negative for any recent fevers, changes in appetite or weight, rashes, or bruising. Her neurologic examination was normal and the remainder of her examination, including auscultation over the carotids and a pharyngeal examination, was unremarkable. She showed some improvement with ibuprofen and diazepam and was discharged with a presumptive diagnosis of viral vs traumatic torticollis.\nThe patient then presented to her primary care provider four days later with persistent inability to rotate her neck. Her exam was unchanged at that time and she was discharged with a diagnosis of viral lymphadenitis and instructions for continued supportive care and a follow-up appointment four days later. At her follow-up appointment her symptoms persisted, and because her oral intake had decreased she was referred for admission. Her admission labs, including a complete blood count with differential, were normal. A computed tomography (CT) of the soft tissue of the neck with contrast was obtained and initially read as unremarkable. She remained hospitalized for four days for pain control and physical therapy without improvement in the range of motion of her neck. On the fourth day of her hospitalization, she developed weakness in her left arm and increasing lethargy. This prompted re-examination of her initial CT, where a possible spinal cord tumor was noted (, panel A). Follow-up MRI showed an extramedullary tumor extending from the level of the second cervical vertebra (C2) to the seventh cervical vertebra (C7) with associated cord edema (, panel B).\nGiven her declining neurologic status, she was given dexamethasone for the cord edema. A cervical mass biopsy and bone marrow biopsy done 72 hours later were both inconclusive. Repeat MRI showed complete resolution of the mass with the steroids. Based on this response to steroids, a presumptive diagnosis of leukemia/lymphoma was made and the patient was discharged on dexamethasone. Four days later, she presented to the pediatric ED again, this time with left facial droop and weakness of the upper and lower extremities on the left side. Examination was notable for swelling of the right wrist and left leg. A lytic lesion was noted on radiograph of the right ulna. An iliac biopsy was again non-diagnostic. On the second day of her hospitalization, she also developed subcutaneous scalp nodules. Biopsies and flow cytometry of the nodules were consistent with B-cell lymphoblastic leukemia/lymphoma. The patient was started on chemotherapy and is currently doing well, with strength in her extremities improving with physical therapy.
A 69-year-old man visited Asan Medical Center for dysphagia and a gradually growing huge anterior neck mass of 10 years duration. He had no clinical symptoms related to thyroid dysfunction and had been repeatedly treated at another institution with radiofrequency ablation for his cystic neck lesion. Fine needle aspiration cytology at that time showed squamous cells and many inflammatory cells, leading to a diagnosis of branchial cleft cyst. Neck computed tomography on this occasion showed a 13×11 cm cystic lesion with irregularly thick walls involving the left lobe of the thyroid gland; the lesion also contained a strongly enhanced solid portion, septation, and calcification (). Fusion whole body positron emission tomography fluorine-18 fluorodeoxyglucose was performed, showing abnormal uptake only by the neck mass (). The esophagus, stomach, and larynx were unremarkable on esophagogastroduodenoscopy. The patient underwent total thyroidectomy with neck lymph node dissection. Macroscopically, a huge mass measuring 14×13×8 cm had replaced the entire left lobe of his thyroid gland, with the mass also involving the right lobe. Multiple cystic spaces measuring up to 8.2 cm in the greatest dimension were present in the central portion of the mass and were filled with dirty necrotic fluid. The solid part of the mass was well demarcated, round in shape and had tan, firm, and smooth cut surfaces. Although the mass abutted the thyroid capsule, there was no extension to the surrounding soft tissues or other organs (). The tumor consisted histologically of two well-delineated components. The center contained a cystic lesion lined by atypical squamous epithelium. The squamous cells showed unequivocal pleomorphism and extensive keratinization, resulting in a diagnosis of squamous cell carcinoma with cystic degeneration (). The peripheral part of the mass was composed of a solid growth of thyroid follicles. The two components were in direct contact, but they did not show areas of transition (). The follicular lesion was cellular and displayed microfollicular and trabecular growth patterns. The follicle cells contained abundant eosinophilic cytoplasm and mildly atypical round nuclei with smooth contour. Small nucleoli were frequently seen, whereas mitotic figures were rare (). The peripheral boundary of the follicular lesion was surrounded by a thick fibrous capsule, and showed a generally expansile, but partly invasive, growth pattern (). Immunohistochemical examination showed that the squamous cell carcinoma was positive for p63, thyroid transcription factor 1 (TTF-1), and p53 and had a 20% Ki-67 labeling index (-). The follicular carcinoma component was diffusely positive for galectin-3 (), TTF-1, and CD56 () and negative for p53, cytokeratin 19 (), and HBME-1 (), and the Ki-67 labeling index was 2%. Of the 18 dissected cervical lymph nodes, none was found to contain a metastatic tumor. The patient was lost to follow up 1 month later, but Korean National Health Insurance data show that he survived for 15 months after surgery.
A 58-year-old Korean woman was referred for progressive blurred vision in her right eye for the previous 3 months. She was admitted for acute left cerebellar and right basal ganglia infarction 1 week prior and received acetylsalicylic acid therapy (100 mg once a day) in the neurology department. She had a 20-year history of hypertension and a 2-year history of type 2 diabetes mellitus, but was not currently taking any medication. On initial ophthalmic examination, visual acuity was 20 / 63 in the right eye and 20 / 20 in the left eye. Intraocular pressure (IOP) of the right eye was 30 mmHg and 10mmHg in the left eye. Slit-lamp biomicroscopy of the right eye showed iris neovascularization (NVI) and gonioscopy revealed 360 degrees of angle neovascularization (NVA) (). The anterior segment of her left eye was normal but fundus examination revealed a single peripapillary flame hemorrhage temporally and narrowing of the arterial vessels. Funduscopic examination of her right eye showed scattered retinal hemorrhage along the inferotemporal vein and ischemic edema in the inferior parafoveal area which was supplied by the small branches of the inferior retinal artery with atheroma ().\nFluorescein angiography (FA) of the right eye showed significant delayed filling of the branches of the inferior retinal artery in the ischemic area. The foveal avascular zone was widened and the superior border was irregular with moderate leakage of dye from the arterioles. A wide area of capillary nonperfusion in the distribution of the inferotemporal vein was also noticed, but choroidal perfusion was normal in the right eye. In her left eye, arteriolar tortuosity and moderate leakage was found near the flame hemorrhage (). FA was consistent with BRAO combined with BRVO in her right eye and the impending state in her left eye.\nCarotid Doppler sonography and echocardiogram showed no evidence of systemic conditions associated with multiple emboli and thrombosis. Her laboratory data including lipid profile, blood coagulation test, and serum homocystein were normal except for blood glucose.\nWe immediately injected intravitreal and intracameral bevacizumab (0.4 mg/0.05 mL) in her right eye. The next day, we performed scatter photocoagulation in the nonperfusion area. One week after the injection, the NVI and NVA had regressed, and the IOP was 12 mmHg with topical antiglaucoma medication (dorzolamide/timolol fixed combination). One month later, visual acuity returned to 20 / 20 in her right eye and the IOP was 17 mmHg with topical antiglaucoma medication (dorzolamide/timolol fixed combination).
A 12-year-old boy with acquired immunodeficiency syndrome (AIDS) diagnosed at 9 years of age and on ART for the last three years, presented with a history of gradual onset of rapidly progressive difficulty in ambulation for the past six months. The child subsequently walked with support and then progressively lost ability to sit or to use his limbs for activities of daily living. The patient became dependent on family members for all his activities. This was also associated with the reduction in verbal output for the same duration, which was limited to bisyllables at presentation. He was studying in 7th standard and developed declining in scholastic performance.\nThe patient gave a history of non-rhythmic, brief, involuntary and sudden jerks, involving upper limbs followed by lower limbs leading to dropping of objects for the past three months. This initially used to happen for four to five times a day but its frequency gradually increased and later happened once in every 10-15 seconds. These jerks used to persist in sleep. No other tonic-clonic or focal seizures were reported. There was no loss of consciousness, bladder or bowel symptoms, or visual deterioration.\nHe is the youngest of five siblings born out of a non-consanguineous seropositive couple with a normal birth and development history until 11 years of age. He had an exanthematous fever at 5 years of age, suggestive of measles which resolved without any immediate complications. The child was diagnosed with HIV at 9 years of age, one year after his parents were diagnosed. There was no prior history of blood transfusions or sexual abuse. The possibility of perinatally acquired infection was considered as perinatally acquired HIV infection present with symptoms only in adolescence and early adulthood as has been described in the literature. His CD4 count was 204 before initiation of ART. This child was initiated on triple-drug ART regime – Zidovudine, Nevirapine, and Lamivudine which was well tolerated. CD4 count increased to 419 before child developed neurological symptoms. Clinically, at presentation, his World Health Organization (WHO) staging for HIV virus was stage 2.\nOn examination, the child was conscious, alert, and oriented with normal vital parameters. There were multiple healed scars evident on his forehead and scalp, attributed to injuries sustained after dropping attacks. A cranial nerve examination, including the fundus, was unremarkable. Motor examination revealed 3/5 power in all four limbs and normal tone with normal deep tendon reflexes. No meningeal signs, involuntary movements, or cerebellar signs were evident. His gait and posture could not be tested as he was not able to stand without support.\nLaboratory evaluation revealed normal complete blood counts and normal renal and liver parameters. His CD4 count was 429. Electroencephalography of monopolar montage revealed generalized pseudo-periodic discharges at an interval of nine to 12 seconds (Figure ). Cerebrospinal fluid cytology, glucose levels, and protein levels were normal. The measurement of specific antibodies by enzyme-linked immunosorbent assay revealed that measles immunoglobulin G antibodies were markedly elevated in the cerebrospinal fluid at 1:625 (normal range, <1:5). Magnetic resonance imaging revealed enhancements of affected regions in the early course of the disease in T1-weighted scan. T2-weighted scans show nonspecific hyperintensities and fluid attenuated inversion recovery sequences (Figure ).\nHence the clinical picture, electroencephalogram, and cerebrospinal fluid patterns were pathognomonic for a diagnosis of SSPE.\nThe child received sodium valproate and clonazepam, after which the frequency of the child’s drop attacks initially abated, but later increased again. Other treatment options such as interferon and Isoprinosine were not considered because of financial constraints. The patient was on follow-up for two months but did not show any significant improvement.
A 46-year-old woman visited the emergency room with left hemiparesis and dysarthria. She was diagnosed with an intracerebral hemorrhage (ICH) at the right basal ganglia by brain computed tomography (CT) (). Three days later, a chest X-ray revealed a diffuse haziness in both lung fields (). Her pulmonary status was further complicated by an inability to cooperate with respiratory toilet and total opacification of both lungs on the chest X-ray. She was intubated due to aspiration pneumonia. Her condition was worsened despite intravenous administration of culture-specific antibiotics and repeated sputum drainage. A tracheostomy was performed two weeks later, and the patient was ventilated via a low pressure tube inserted through the second tracheal ring. Thereafter, she stabilized and began to improve.\nThree months after the tracheal intubation, massive bleeding was detected from the tracheal stoma on tracheal suction and the patient vital signs became unstable. Her blood pressure was below 80/60 and with 40% oxygen saturation. The tracheostomy tube was replaced with a flexible endotracheal tube and the endotracheal tube cuff was inflated until adequate ventilation and bleeding control was achieved. Portable fiber optic bronchoscopy could not identify the source of the hemorrhage at the anterior wall of the midtrachea. A thoracic CT scan demonstrated that the tip of the endotracheal tube was in contact with the tracheal wall in the back of a tracheoinnominate artery (). Suspecting TIAF, we brought the patient to the interventional radiology suite. Emergent aortic arch angiography was performed through the right common femoral artery. Catheterization of the aortic arch did not locate the fistula, but upon selective catheterization of the innominate artery, the fistula was clearly visible near the origin of the common carotid artery (). Embolization of the innominate artery using coils was not recommended by the interventionalist because of an occlusion of the right common carotid artery. We also were worry of unexpected results, such as ischemia caused by occlusion of the common carotid artery.\nThe operative procedure to control the fistula and maintain adequate blood flow to the brain consisted of first performing a right aorta-axillary artery bypass using a synthetic graft, followed by resection of the 3 mm fistula and oversewing the divided ends of the innominate artery. The defect in the trachea was approximated with 3-0 Vicryl sutures, and autologous tissue was interposed. Control of the fistula was confirmed by thoracic CT angiography with three-dimensional (3D) reconstruction ().\nPostoperatively, the patient exhibited no evidence of graft infection or erosion and recurrent bleeding. The patient's focal neurologic deficits remained unchanged. She was discharged after three months and continued to do well.
A 5-year-old girl who was diagnosed with acute lymphoblastic leukemia and had been receiving maintenance chemotherapy was admitted to hospital with left knee pain and swelling. She had been on chemotherapy for two years. Her maintenance chemotherapy regimen consisted of dexamethasone, mercaptopurine and methotrexate. An initial evaluation revealed the presence of swelling, tenderness and reduced motion of the right knee joint because of pain. She was afebrile and did not have hepatosplenomegaly or lymphadenopathy. Her white blood cell count was 0,6×109/l, absolute neutrophil count was 0,3×109/l. Erythrocyte sedimentation rate was 82 mm/hour. Joint MRI revealed findings consistent with nonspecific severe inflammation of synovia (). We stopped her maintenance chemotherapy and performed bone marrow aspiration to see if it was a relapse of leukemia and its bone involvement, but bone marrow was completely in remission. After the consultation with the orthopedia department we decided to perform biopsy from the joint and bone. Biopsy was performed from distal femur and synovia. Surgical debridement of the joint was also performed at the same time.\nBone and synovium specimens examined in the pathology department were consistent with chronic granulomatous Aspergillus synovitis but no speciation was performed. Voriconazole was initiated immediately with a loading dose of 6 mg/kg intravenous every 12 h for two doses and because she was a pediatric patient we decided to follow by 6 mg/kg every 12 h. We also assessed our patient for other organ involvements for invasive aspergillosis but there were no other sites of infection evident on physical examination. A high resolution computed tomography (HRCT) was done to search for a pulmonary source of Aspergillus but it was normal. Hemocultures were negative. Galactomannan test was studied twice a week just after the diagnosis and tests results were negative. We could not find any evidence for a source which could cause a hematogenous or contiguous spread for Aspergillus. Most likely, our patient had isolated Aspergillus synovitis. On the 11 th day of intravenous voriconazole her white blood cell count increased to 2,1×109/l and absolute neutrophil count increased to 1×109/l. During this period she didn’t develop fever in spite of neutropenia On the 15 th day of voriconazole we began to achieve clinical improvement. The response to voriconazole was followed with roentgenograms and also joint MRI. On the 29 th day of treatment her joint MRI was done and it demonstrated remarkable regression in findings of synovitis supporting the good response to voriconazole. At the end of the first month with voriconazole treatment we also began to continue her maintenance chemotherapy. She received intravenous voriconazole for 62 days. A control roentgenogram which was repeated on the 62 nd day supported the clinical improvement. At this time we decided to switch intravenous voriconazole to the oral formulation and we discharged her home on oral voriconazole. She came to hospital once a week for physical examination and complete blood count control. During this period she received the rest of the maintenance therapy and completed her chemotherapy for leukemia. Control roentgenograms were seen approximately once a month and a control MRI was repeated in the 5 th month of treatment with voriconazole. Seven months after the diagnosis of synovitis and initiation of voriconazole follow-up MRI of the knee showed no signs of infection and the treatment was completed. The last MRI did not show any signs of bone loss or destruction in the joint either. Our patient was treated for three months with intravenous voriconazole followed by oral form for the next four months for a total of seven months. We did not put her on prophylaxis because her chemotherapy was completed. At follow-up six months after treatment she remainded well without evidence of infection recurrence.

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