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A 15-year old Caucasian girl was referred by her own general practitioner after a suicide attempt with symptoms of sadness, anxiety, and obsessive–compulsive behavior. The intake was together with her parents. She was struggling in school, despite her very supportive family. No drug abuse or other psychiatric symptoms were found. She told the counselor she tried hard, but felt that she could not keep up in school; it was never good enough, no matter how hard she tried. The counselor estimated that the school level was appropriate for the level of intelligence of the patient.\nShe had periods when her self-esteem was very low. During these periods she spent hours on her appearance, focusing on her hair and makeup. Her hair fell out as a result of these sessions. She could not stop herself, and always ended with self-harm. This in turn lowered her self-esteem and increased the experienced stress. She was locked in a downward spiral. Gradually her mood disorder worsened and made her passive. She no longer wanted to go to school and meet friends, but passed hours in front of the mirror. She attempted to end her life.\nWe hypothesized on the basis of the girl's stress complaints that she felt school, parents, and friends expected too much of her. After a neuropsychological assessment the testing showed that she had a disharmonic intelligence profile with an IQ of approximately 80 (using the Wechsler Intelligence Scale For Children-III ()), inconsistent at all factor levels. We classified a mild intellectual developmental disorder in the conceptual and practical domain, which explained the structural struggle with the standard school curriculum instructions. We educated parents and school on how instructions might fit in better with her learning abilities and style. Her preferred method of learning new things was being shown how to do it, as opposed to having it explained to her. This led to significant stress reduction and positive school experiences. In the process her self-esteem improved, the experienced stress decreased, and her mood improved. CBT was adjusted to her learning style and was used to reduce her obsessive–compulsive behavior. |
A 47 year-old African American female with a past medical history of hypertension, bronchial asthma, former smoker for 10 years (quitting 15 years prior) and end stage renal disease on hemodialysis, presented to our institution to be evaluated for kidney transplantation. She denied shortness of breath on exertion, chest pain, palpitations, or dizziness. She described her exercise tolerance as 3–4 blocks limited by fatigue. Physical examination revealed a well developed female, not in any distress. She was afebrile with a blood pressure of 138/76 and a heart rate of 71. Her electrocardiogram demonstrated characters of left ventricular hypertrophy, nonspecific T wave abnormalities, and a prolonged QTc of 534 (). Transthoracic echocardiography revealed an ejection fraction of 55% to 60% with left ventricular wall thickness markedly increased to 16.2 mm, mild systolic and diastolic flattening of the ventricular septum consistent with right ventricular volume and pressure overload. Furthermore, there was mild to moderate dilatation of the right ventricle, left atrium and right atrium with moderate to severe tricuspid regurgitation. Her pulmonary artery systolic pressure was estimated at approximately 40 mmHg plus right atrial pressure with a small pericardial effusion (). She was sent for an outpatient dobutamine stress echocardiogram, primarily for risk stratification before renal transplantation. The test was terminated as the patient did not achieve the target heart rate. However, pulmonary systolic pressure was increased to 60–65 mmHg plus right atrial pressure with leftward displacement of the interatrial septum consistent with increased right atrial pressure. She was referred for CT coronary angiography which revealed a malignant interarterial course of her right coronary artery (RCA) originating from the proximal part of the left main coronary artery (LMCA). The artery traversed between the aorta and right ventricular outflow tract with secondary ostial and proximal narrowing. The LMCA trifurcated with presence of a ramus intermedius. There was evidence of pulmonary arterial hypertension with enlarged pulmonary arteries, right-sided chambers, and thickening of the free wall of both ventricles (, ). She went for cardiac catheterization for better evaluation, which revealed non obstructive coronary artery disease at the distal left circumflex artery (LCX) and proximal RCA (). Cardiothoracic surgery was consulted to evaluate the treatment for RCA course and conservative medical management was decided. |
A 59-year-old man with a history of type 2 diabetes and smoking (an average of 20 cigarettes per day) but no history of high blood pressure and heart disease underwent video-assisted thoracoscopic esophagectomy for squamous cell carcinoma (stage PT1N1Mx) of the distal esophagus with an esophageal reconstruction using a gastric tube through the posterior mediastinal route 14 months previously. The surgical resection margins were negative, and right lung nodule metastasis was not observed. The adjuvant radiotherapy (chemoradiotherapy for the original esophageal tumor area and lymphatic drainage area) and chemotherapy were completed. At 7 months postoperation, the patient occasionally experienced episodes of epigastric discomfort. A gastrointestinal endoscopic biopsy revealed a gastric ulcer on the posterior wall of the distal part of the gastric tube, but his vital signs were stable and routine blood, liver, and kidney function test results were within the normal ranges. This patient did not receive continuous treatment with a proton pump inhibitor. Five months previously, he experienced epigastric discomfort again and had blood in the stool. Gastrointestinal endoscopic examination revealed a round deep ulcer, 1.5 mm in diameter, in the gastric body, with visible blood vessels in the middle (Fig. A and B). However, there was no evidence of recurrence over the 1 year following the operation. Two days after the endoscopic examination, he suddenly experienced massive hematemesis and unstable circulatory dynamics, with a blood pressure of 90/45 mm Hg and a pulse of 150 beats/min. Blood tests revealed severe anemia, with hemoglobin (Hb) of 50 g/L and hematocrit of 15.3%. Infusion was performed to treat critical hemorrhagic shock but was ineffective. We informed the patient and his family members of the situation, and once written informed consent to treatment was provided, we rushed him to the operating room.\nBecause of the patient's unstable circulatory dynamics, we classified him as high risk for open surgery and decided to use a stent graft repair as the first feasible option for urgent treatment. Diagnostic angiography and endovascular stent graft repair were performed under local anesthesia. After access was obtained via the femoral artery, a 4-Fr catheter was placed into the abdominal aorta, and we found that contrast medium permeated into the gastric cavity through a fistula between the abdominal aorta and gastric tube at the 11th thoracic level (Fig. ). Based on this, we made a diagnosis of AGF resulting from a peptic ulcer, and this diagnosis was further confirmed by high pressure angiography combined with computed tomography (CT) imaging. We considered the AGF to be the primary cause of upper gastrointestinal bleeding. Thereafter, angiography was performed in the aortic arch by puncturing the left femoral artery and entering the marked pigtail tube, and the AGF was located. A stent (COOK ZTEG-2PT-32–160) was then passed through the right femoral artery into the aorta, positioned on the fistula location and released. After stenting, the graft was in good condition, no contrast medium was permeating into the stomach, and hematemesis ceased (Fig. ). The patient's circulatory dynamics also stabilized immediately following the procedure, and he was then transferred to the intensive care unit (ICU) where he was kept for 1 day before being returned to the general ward (Fig. ). The full procedure was performed under the guidance of digital subtraction angiography (DSA). Subsequent treatments included pumping norepinephrine, blood transfusion, infusion booster, sufentanil analgesia, and ceftriaxone. Also, omeprazole and octreotide were continuously administered to inhibit gastric acid secretion. The stent implantation, Hb concentration, and vital signs of this patient were relatively stable, and the patient did not have hematemesis, melena, or any other active bleeding. Considering that this patient had a tumor and was in a hypercoagulable state, in order to avoid thrombosis, he did not continue antibleeding treatment. At 2 months postoperation, in order to monitor the patency of the stent and active bleeding and related complications, we recommend that patients should have endoscopic review, but this patient and his family declined. Contrast-enhanced CT revealed no extravasation and no other complications such as hematemesis, infection, and pseudoaneurysm (Fig. ). Four months after surgery, the patient died of organ failure caused by retroperitoneal lymph node metastasis and multiple intrahepatic metastases, with no postoperative bleeding linked to the endovascular stent graft repair. |
A 14-year-old boy, who was initially examined for continuous coughing, was referred to our hospital owing to an anterior mediastinal mass identified on chest X-ray (). Systematic examination revealed a 20 × 10 cm sized mass at the right anterior mediastinum that involved the right pulmonary vein and elevated levels of alpha fetoprotein (AFP), a tumor marker, at 3825 ng/ml. Needle aspiration was performed, but only necrotic tissue could be collected. We did not analyze the karyotype since the symptoms suggesting the Klinefelter syndrome were not observed. Therefore, a clinical diagnosis of malignant teratoma was made, and three courses of cisplatin-based chemotherapy were administered because AFP levels continuously increased. After chemotherapy, AFP levels decreased, although the size of the tumor did not change, as evident in a computed tomography (CT) scan. Next, tumorectomy and total right lung extraction were performed. The pathological diagnosis of the extracted tumor was malignant teratoma with areas of yolk sac tumor (). No cancer cells were found at the edges of the area where the tumor was removed. Two courses of cisplatin-based postoperative chemotherapy were administered until AFP levels were normal.\nAfter 3 months of follow-up, the patient experienced pain in his right hip joint while AFP was still normal. The 99mTc-methylene diphosphonate bone scan showed increased tracer uptake at the left forehead and right hip joint (). Head and pelvic MRI also revealed signs of metastasis at the left forehead and right hip joint. Abdominal CT showed a metastatic region in the liver. Bone biopsy was performed at the left forehead. The pathological diagnosis was metastatic malignant melanoma originating from an immature teratoma of mediastinum (). Large heteromorphic cells with melanin were found in the original mediastinal malignant teratoma by retrospective re-examination. This suggested that a section of the malignant melanoma in the original malignant teratoma, which was composed of various components, metastasized. Because of the very fast disease progression, after consultation with the family, aggressive treatment was discontinued, and palliative therapy was provided. He died 15 months after diagnosis of the original malignant teratoma. |
Our patient was a 78-year-old Danish woman with a history of hyperthyroidism for 15 years caused by a toxic multinodular goiter. The condition was uncomplicated and treated with thiamazole. She was also receiving statin therapy for hypercholesterolemia. She had had successful knee replacement surgery 7 years before the current admission. She lived alone, was fully mobile, and was not dependent on care.\nIn the 5 weeks prior to admission, she had had two episodes of syncope/presyncope. The first time she did not lose consciousness and attributed it to orthostatism. Her general practitioner ordered blood tests and referred her to an otorhinolaryngologist. The second episode happened 4 weeks prior to admission. She had been drinking two glasses of wine and subsequently felt dizzy and experienced loss of consciousness for up to 1 minute. She was immediately attended by family members, who did not observe convulsions or neurological deficits. She regained consciousness rapidly and was admitted to our hospital, where the results of a clinical examination, including a full neurological evaluation, routine blood tests, and electrocardiogram (ECG), were normal. A urine rapid test was positive for leukocytes and nitrite, and a subsequent culture revealed the presence of Escherichia coli. The patient was discharged the same day without treatment.\nThe day prior to admission, family members could confirm that the patient was doing well and went to bed feeling well. A granddaughter was visiting the patient, and the next morning she found the patient lying unconscious on her bed as if she had collapsed and fallen backward onto the bed. The ambulance paramedics reported an initial Glasgow Coma Scale (GCS) score of 6. The patient was admitted to the emergency department, where she was examined by the doctor on call along with a consultant in infectious medicine. The patient was evaluated with a GCS score of 12. She was found febrile with a rectal temperature of 39.4 °C, but without neck stiffness. She had pinpoint pupils and brief twitching of the left-side extremities but no convulsions. The patient was considered confused, unable to recall her date of birth, but she could otherwise respond with a whispering voice. She denied complaints of headache or other pain.\nThe result of a 12-lead ECG was normal, as was continuous ECG monitoring. The patient had no cardiac murmurs or clinical findings suggestive of pneumonia. Her ear examination was without signs of otitis.\nBlood tests revealed marginal leukocytosis with 9.35 × 109/L neutrophils. All other biochemistry results were normal, including a C-reactive protein level of 4 mmol/L. Arterial blood gas analysis revealed slight hyperventilation with a partial pressure of carbon dioxide of 4.0 kPa. The patient’s lactate level was 0.7 mmol/L.\nOn the basis of primary suspicion of stroke, the patient was seen by the neurologist on call, who found a bilateral horizontal nystagmus, unstable eye movements, and suspected right-sided gaze paralysis. On suspicion of a stroke affecting the pons, a computed tomographic scan including angiography was performed 2.5 hours after admission and was described as without signs of stroke or occlusions. Lumbar puncture was performed 4.5 hours after admission. The patient’s CSF pressure was 8 cm H2O, and the result of CSF analysis was normal (leukocyte cell count 4 × 106/L, glucose 4.4 mmol/L, protein 0.23 g/L, no erythrocytes). CSF samples were sent immediately for microscopic analysis, the result of which was negative for bacteria. Meningitis was considered highly unlikely, and the patient was transferred to the neurology department, where 5.5 hours after admittance she was started on empirical sepsis treatment with cefuroxime 1500 mg three times daily. After 7 hours, treatment with clopidogrel and acetylsalicylic acid was initiated.\nThe next morning, 22 hours after the patient had been found unconscious, three of three CSF cultures yielded growth of pneumococci. A few hours later, blood culture results also became positive for pneumococci. The patient was stable and fully conscious with only minimal confusion.\nUpon the discovery of pneumococci in the cultures, the patient was immediately started on ceftriaxone 4 g once daily, 3 million U of benzylpenicillin six times daily, and dexamethasone 10 mg four times daily. The pneumococci were penicillin-susceptible, and the patient was treated for 14 days and discharged after 16 days. The course of disease was uncomplicated, except for influenza confirmed by polymerase chain reaction on day 10 of admittance. The patient’s influenza was treated with oseltamivir phosphate (Tamiflu; Genentech, South San Francisco, CA, USA) 75 mg twice daily.\nThe patient was seen in the outpatient clinic 8 days after discharge, where her daughter complained of worsening of her mother’s hearing deficit, impaired balance, and fatigue. The patient was referred to an otorhinolaryngologist. Audiometry revealed a bilateral moderate perceptive hearing loss. The patient’s impaired balance was described as a long-standing problem with only minor aggravation after the admission.\nThe patient’s neurological examination result was normal, apart from marginally unstable walking and hyperreflexia of the left upper extremity. A brain magnetic resonance imaging scan showed brain atrophy with ventricular enlargement and, on the basis of diffusion-weighted images, a small brain infarction in the right corona radiata was suspected (Fig. ). The patient was recommended prophylactic antihypertensive and antithrombotic therapy and pneumococcal vaccination, and she was discharged to the care of her general practitioner 3 months after hospital discharge. Shortly after discharge, the patient resumed all daily activities. She had no memory of the day of admittance but remembered she had felt cold and shivering before going to bed the night before admission. She also remembered getting up in the morning to find her bathrobe because she was feeling cold when she remembers suddenly falling backward. |
A 55 year old man was diagnosed with anemia by his family physician towards the end of 2017. In February of 2018, an upper GI endoscopy and colonoscopy were performed which showed a right colon cancer. CEA was 40.4. CT of the chest, abdomen, and pelvis was obtained in February of 2018. This showed a mass 7 cm in greatest diameter that was immediately adjacent to the undersurface of the right liver and contiguous with numerous loops small bowel and the lowest part of the second portion of the duodenum (). There were mildly prominent mesenteric lymph nodes but no evidence of distant metastatic disease.\nIn February of 2018, the patient underwent a single port laparoscopic right colon resection. The surgeon described the tumor as stuck to the undersurface of the liver with adhesions taken down without difficulty. On histopathologic examination, the tumor was PT3N0M0 with 0/16 positive nodes. Perineural invasion was identified. The cancer was moderately to poorly differentiated. Systemic chemotherapy was not recommended.\nIn June of 2018, the patient began noting problems with digestion and pain after eating. CEA had increased to 940 ng/ml. Repeat CT scan showed multiple nodules within the greater omentum compatible with peritoneal metastases. The liver, kidneys, and ureters were normal. There was marked stranding within the right colon resection site (). Bowel loops proximal to the prior ileocolic anastomotic site were moderately dilated and fluid-filled. A mass was noted in the abdominal wall at the laparoscopic port site. There was also a mass in the rectovesical space immediately adjacent or invading the right and left seminal vesicles.\nIn July of 2018, the patient underwent exploratory laparotomy. The posterior surface of the liver was layered by cancer. The undersurface of the right hemidiaphragm was covered by tumor nodules. The pelvis contained a 4 cm tumor mass invading into the seminal vesicles. The old ileocolic anastomosis was involved and needed to be resected. The umbilical port site was infiltrated by cancer. Near complete resection was possible by cytoreductive surgery.\nIntraoperatively, the patient was treated with HIPEC-melphalan []. This postoperative course was unremarkable. The histopathology showed adenocarcinoma compatible with the primary tumor in all tissues submitted. The peritoneal cancer index was 28 and the CC score was 2 []. The resection was judged to be a palliative effort and its major goal was to relieve intestinal obstruction and to prepare the patient for rapid initiation of palliative systemic chemotherapy.\nNeither of our patients was evaluated by the MDT preoperatively. Their preoperative and intraoperative management was evaluated in an attempt to improve the outcome of subsequent patients. |
An 8-year-old male child presented to the Department of Cardiology with uncontrolled hypertension. He had undergone PTRA and stenting with 5 mm × 15 mm balloon expandable metallic stent at 2 years of age for 90% ostial stenosis of left renal artery. His blood pressure remained well controlled for the next 6 years. On admission now, his blood pressure was between 150/90 and 160/110 mm of Hg, on three antihypertensive drugs. A computed tomogram (CT) angiography revealed left renal artery stent fracture at the origin and 70% ostial stenosis with irregularity of the renal artery till the bifurcation []. Renal scintigraphy with 99 m DTPA (diethylene triamine penta acetic acid) scan showed glomerular filtration rate (GFR) of 69 ml/min with a right to left ratio of 75:25. A repeat PTRA was attempted and confirmed the presence of renal artery stent fracture []. However dilatation of the stenosed segment could not be done because of tight stenosis. He was referred to our department for surgical revascularization of left kidney. Autotransplantation of the left kidney was planned in view of the CT showing irregularity of the renal artery till the bifurcation. After laparoscopic dissection and retrieval, left kidney was perfused with Eurocollin's solution. On bench dissection, the stent containing stenosed part of the renal artery was excised which on further cut section showed that the fractured stent incorporated into the vessel wall and developed intimal hyperplasia []. The bifurcation of the renal artery was found to be intact. The autograft was implanted in the right iliac fossa with the renal artery anatomosed end to end with right internal iliac artery, renal vein anastomosed end to side with right external iliac vein and ureter implanted into dome of bladder with modified Lich's technique. The postoperative course was uncomplicated. His blood pressure gradually became normal. At 4 years after surgery, serum creatinine was 1.0 mg/dl, blood pressure was 110/70 mm of Hg and DTPA scan showed equal perfusion of both kidneys with total GFR of 90 ml/min. |
We report a case of a 51-year-old man diagnosed with NF1, addressed to our consultation for oral pain in his wisdom tooth 48. During anamnesis, the patient informed us about cutaneous café au lait spots since childhood, and that at puberty cutaneous neurofibromas located on the head, abdomen, back, and arms appeared, followed by the occurrence of oral neurofibromas around the age of 20. NF1 was then diagnosed and multiple cerebral MRI were carried out without revealing any lesion. Furthermore, he related having been diagnosed with idiopathic thrombocytopenia during childhood which required high dose corticosteroid treatment leading to side effects such as weight gain and increased hairiness. At the age of 31 years, he sustained surgical excision of multiple neurofibromas in the head and neck region in France. He also mentioned a tibial fracture which occurred five years earlier while skiing which led to an osteoporosis diagnosis, treated by intra-venous denosumab monoclonal antibody (Prolia) twice a year since then.\nThe clinical examination revealed numerous cutaneous neurofibromas on the neck and back of the head as well as on the abdomen and the back, arising to the gluteal fold (). Apart from the infection related to the tooth 48, the intra-oral examination disclosed vestibular gingival nodules on the right side, measuring about one centimeter in diameter, covered by normal mucosa; the anterior one had a round aspect in comparison to the posterior one which had an elongated appearance most certainly due to the dental occlusion (). The patient had however, no complaint in regard of those intra-oral nodules. A facial cone beam CT was performed and revealed no intra-osseus lesions linked to NF1.\nTooth 48 was removed. However, its infection was not in relation with NF1. Two excisional biopsies of the maxillary lesions were also performed to assess a diagnosis and to allow the patient to have better oral hygiene. The histopathological analysis confirmed the diagnosis of oral neurofibromas in the context of NF1 ( and ). The postoperative course was uneventful. Follow-up 11 years later showed no recurrence of the intra-oral lesions as well as no new lesions. |
An asymptomatic 9-year-old male had a past medical history of interventricular communication due to interventricular septal defect in infancy, which spontaneously closed without residual regurgitation. The child was referred for preoperative cardiac evaluation due to auscultated systolic murmur in view of orthopedic surgery (removal of a foreign body on the left foot). It is of paramount importance to point out, that the absence of a remarkable cardiac murmur does not exclude any severe cardiac disease. His routine biochemical parameters were within normal rates.\nIn the electrocardiogram (ECG) of 12 leads of the child at rest, there were observed negative T in the V1-V3 leads, which is a normal variant of the electrocardiogram in children and young women. It was also observed biphasic T in V4 which is sometimes present in the normal pediatric ECG.\nEchocardiography revealed normal left ventricular contractility, with ejection fraction (EF) >65%. The internal dimensions of the left abdomen were increased with age. Its end-diastolic diameter (LVIDd) was 44 mm with normal values for the age and body weight of the child up to 41 mm. The thickness of the left ventricular wall at dilation was normal (intraventricular septum [IVSd] 7 mm). The left atrium showed mild dilation.\nThe echocardiography also showed ventricular communication that has been closed in infancy and insignificant leak through it. Also, multiple pathological flows emerged in the area of the interventricular septum that gave the impression of multiple remaining muscular type of interventricular communications (Figure ). Inside the myocardium of the left ventricle, elongated flows were detected which were also considered as potential communications.\nIn addition, the pulmonary artery stem developed a diastolic flow which, as it turned out, was due to an anomalous outflow of the left circumflex coronary arterial branch from the pulmonary artery. Due to the above findings in the echocardiography examination it was decided to perform cardiac catheterization and coronary angiography.\nCatheterization of the right heart chambers from the right femoral vein was performed percutaneously. Retrograde catheterization of the aorta, coronary arteries and left ventricle from the right femoral artery was then performed percutaneously (Figure ).\nDuring oximetry that was performed in the catheterization, the arterial blood saturation was normal. The ratio of pulmonary blood flow (Qp) to systemic blood flow (Qs) was calculated according to Fick principle (Qp / Qs = 1), which indicated no significant left-right blood shunt.\nNormal outflow of the left anterior descending artery (LAD) from the left atrium of the Valsalva and abnormal outflow of the left circumflex coronary artery (LCX) from the pulmonary artery (PA) (anomalous left circumflex coronary artery from pulmonary artery [ALCAPA]) was detected after injection of the contrast agent into the protrusion of the LAD (Figure ).\nRight coronary angiography showed a predominant right coronary artery (RCA). There was a retrospective imaging of the left parietal and pulmonary artery through an extensive lateral coronary network between the RCA and the LCX (Figure ).\nRCA is the predominant coronary artery as the posterior descending branch emerges from it. We observed also that there was an extensive collateral circulation network between the right coronary artery and the LCX which is outlined retrospectively by this rich blood supply. The pulmonary artery from which LCX emerges was then outlined retrospectively.\nMyocardial stress echo and nuclear scintigraphy were finally performed. A myocardial stress echo test revealed asymptomatic ischemia with a decrease in the ST interval in leads II, III, aVF, V4-V6 indicating possible development of ischemia in the lower and lateral wall of the left ventricle.\nThe child was operated at the age of 11, two years after the initial diagnosis, due to parental refusal to consent to the risks of an open-heart surgery, in terms of mortality and morbidity. Finally, the child underwent successful reimplantation of LCX in the aorta. It is remarkable and it is extremely rare for an asymptomatic child to find an abnormal outflow of circulating LCX from the pulmonary artery. |
A 56-year-old lady who worked as a nursing sister presented with the chronic low back pain. She had attended in August 2016 with left-sided renal colic and this had picked up as a lesion in the right upper quadrant which was 8–9 cm in size and mainly cystic. MRI revealed thick enhancing cystic wall lesion occupying the right retroocular space [].\nShe described the pain as aching sensation coming from her spine around the thoracolumbar region and the right side had more discomfort than the left side. She had no neurological symptoms or signs related to this although she was a bit overweight (BMI 36) and lower lumbar spine facet joint tenderness. She had no breathing difficulty and no problems with hiccups or coughing episodes.\nShe had underlying medical problems of the previous BCC in face and uterine fibroid.\nOn review, she had been suffering from on-going aching sensation in right side of the thoracolumbar region. Recent MRI showed an increase in size of the lesion [].\nAlthough initially the lesion was thought to be under the diaphragm, carefully study of the CT and MRI revealed that this was in fact above the diaphragm and arising from the right T11 nerve root [ and ]. A biopsy revealed this as schwannoma.\nConsidering her symptoms and the large size, removing this lesion was discussed. Various operative approaches were explored. Considering that the lesion was low in the mediastinum behind the liver and kidneys, it was decided that a thoracoabdominal approach would be the optimal.\nShe underwent a right thoracoabdominal approach for giant t11/12 nerve sheath tumor excision under GA. The procedure was performed via reverse L shaped incision over chest and mid upper abdomen. Abdomen and chest were entered and diaphragm was incised. After mobilizing the liver, adrenal and kidneys, and gross total excision of the tumor which lay above the diaphragm was done.\nIn the postoperative period, unfortunately, she experienced wound gaping at the epigastric area over 10 cm length without obvious sign of infection. The wound was closed back after washout on 14th postoperative and she went home 2 days after that was her 16th postoperative day.\nPostoperative imaging showed good excision of the tumor with no residuum found [ and ].\nHistology showed WHO Grade 1 Schwannoma. |
A 42-year-old male visited our department for the first time in October 2011 with a chief complaint of multiple facial fractures. He had no past history or family history. As for the present history, he fell down from a bicycle and was hit severely in his left face in October 2011. He visited a nearby general hospital and was diagnosed with facial fractures. Then, he was referred to our department.\nRadiographic imaging demonstrated fracture lines at the anterior and posterior walls of the left maxillary sinus and zygomatic arch and revealed a mass at a right preauricular area, well delineated radiopaque ().\nHistory taking revealed that his mother had noticed the mass when he was three years old, but there was no symptom. Since he hoped extraction, the right preauricular mass was resected at the same time with reduction and fixation of multiple facial fractures under general anesthesia.\nGeneral physical examination revealed average body constitution with excellent nutritious status and no abnormality. Extraoral findings revealed a depression at the cheek on the left, anesthesia, and palpebral subconjunctival hemorrhage on the left, but there was no motility disorder of the left eyeball, double vision, or occlusal deviation. A bone-like hard mass about 10 mm in size was palpable subcutaneously at the right preauricular area. The mass was well delineated without adhesion to the adjacent tissue. The surface of the skin showed a healthy color, and the mass developed no symptom or growing tendency from the childhood ().\nIn the oral cavity, there was no abnormal finding, and the excretion and nature of saliva were normal.\nTentative diagnosis of maxillary fracture and preauricular benign tumor was made, and the right preauricular mass was resected at the same time with open reduction and fixation of multiple left facial fractures. A horizontal skin incision was made about 1 cm from the top of the mass, and the mass was dissected from the adjacent tissue. The mass had no adhesion with the adjacent tissue, and it was resected en bloc.\nThe resected specimen was hard and yellowish white 10 × 9 mm in size, and the cross section showed a coarse and bone-like surface (). Dental X-rays showed an image of patchy calcification in the mass (). |
An 88-year-old female presented to the emergency department with abdominal pain and vomiting. She was mildly tender in her right upper quadrant but was focally more tender in her right lower quadrant. Her blood results showed an elevated white cell count of 12.9 × 109 l–1 and a neutrophil count of 10.4 × 109 l–1. Furthermore her C-reactive protein level was 34 mg/L. An abdominal ultrasound showed multiple calculi within the gallbladder. Gallbladder wall thickness was not assessed. A non-contrast CT scan, due to the patients impaired renal function, was then ordered as the patient’s physical exam findings were not in keeping with a diagnosis of cholecystitis.\nThe CT showed abnormal dilatation of the proximal small bowel with a possible transition point in the left iliac fossa. Pneumobilia was also noted due to prominence of the hepatic biliary tree (). No evidence of a ductal calculus was observed. The CT scan was reported as showing features of a bowel obstruction with a transition point in the left iliac fossa. The pneumobilia was reported as being possibly an indicator of previous sphincterotomy. There was no evidence of appendicitis or another inflammatory process in the right iliac fossa.\nThe following day, while proceeding with conservative management, the treating team requested a small bowel follow through to investigate the obstruction. After further review and discussion with the treating team it was revealed that there was no history of an endoscopic retrograde cholangio-pancreatography or cholecystectomy. An addendum was added to the CT report, suggesting that the appearances were indicative of a gallstone ileus from a choledochoduodenal fistula () with a non-calcified gallstone possibly being present at the transition point in the left iliac fossa ().\nThe patient underwent a laparotomy and a gallstone was found obstructing the distal jejunum. A 41 mm gallstone was removed by vertical enterotomy, later confirmed on histological diagnosis. The patient had an uneventful recovery in hospital and was discharged a few days later to a regional hospital for rehabilitation. A cholecystectomy or fistula closure was not performed and she was not referred for further surgical follow-up on discharge, likely due to the patient’s advanced age and medical comorbidities. |
A 32-year-old white woman, who was undergoing transgender body modifications and no previous medical history, presented to our emergency department (ED) with a chief complaint of right forearm pain, associated with redness and blistering. The patient was a tattoo fanatic who underwent solar branding on her right forearm approximately 2 weeks before presentation. The patient first noticed the blistering of the branded area 3 days after her body modification. Over the next few days, the entire branded area became very warm, swollen, painful to touch, and developed cellulitis with weeping blisters. Given her dire situation, the patient finally sought medical care in the local urgent care center. The patient was diagnosed with a superficial infection of her right forearm full thickness burn and prescribed oral clindamycin. The patient continued to develop painful blisters for the next week and did not notice any improvements in her cellulitis despite the antibiotic treatment, prompting her to come to our ED for a second medical opinion.\nDuring her physical examination, the patient was noted to have painful blisters, redness, and swelling of the right forearm and hand (Figure ). She did have a full range of motion of the hand; however, there was mild pain noted on the flexion of the hand. There were no signs of sepsis, and her vital signs and her laboratory panel which included white blood cell count were all within normal limits. The rest of the physical examination was also unremarkable. The patient was admitted to the Burn Surgery Service for intravenous (IV) antibiotics and possible operative interventions.\nThe patient was started on IV clindamycin 600 mg every 8 hours, and once adequate pain control was achieved, a bedside debridement was performed in the Burn Unit. The following day, the patient was taken to the operating room for tangential excision of her wound and split-thickness skin autograft (STSG) placement onto her right forearm. The left anterolateral thigh was chosen as the donor site. The grafted site was dressed with xeroform, followed by a layer of bacitracin ointment and then wrapped with Kerlex and Coban dressings. A right forearm elbow splint was fitted and placed by the occupational therapy service for further graft protection. Postoperatively, the patient was transferred back to the floors without complications, and antibiotics were stopped. The right forearm dressing was taken down on postoperative day 4 to evaluate the STSG. The entire graft was viable and taken. Subsequently, the graft staples were removed on postoperative day 5.\nThe patient recovered exceptionally well and was discharged home a few days later. Two weeks later, the patient returned to the burn clinic for a follow-up appointment. The skin graft and the donor site healed remarkably well (Figure ). The patient reported no pain and had a full range of motion of the hand and wrist. The patient was discharged from the burn service and advised to use over-the-counter moisturizer or lotion as needed. |
A 77-year-old woman presented to our Accident and Emergency department with a one-year history of a painful right hip replacement. She complained of difficulty in weight bearing and a gradually diminishing walking distance such that in the four weeks prior to admission she was essentially housebound. Furthermore she reported her hip pain had progressed to an extent that it was present at rest and on occasion woke her up from sleep at night.\nShe had been reviewed six months previously by her local GP who had started her on high dose oral steroids for a provisional diagnosis of polymyalgia rheumatic under the guidance of the rheumatologist. There was no history of trauma.\nHer past medical history included osteoarthritis, iron deficiency anemia, and a right cemented Exeter total hip replacement performed three years previously.\nOn examination she appeared generally unwell and frail. She was apyrexial with no evidence of any localized features of erythema, swelling, redness, or skin changes around the affected hip. There was severe tenderness on palpation around the greater trochanter. Her active range of motion of the right hip was limited to 20° flexion with all other movements being severely restricted. Passive range of motion was also limited.\nThe blood results showed a haemoglobin of 86 with an elevated WCC 32.5, CRP 246, and ESR 100. Plain X-rays of the hip were normal () and further cross-sectional CT imaging demonstrated a cystic mass closely related to the hip prosthesis suggesting an inflammatory pathology (). The bone scan using Te99 and SPECT (Single Photon Emission Computed Tomography) showed a large an area of intense hypervascularity at the medial aspect of the right proximal femur (Figures , , and ). The MRI scan revealed huge lesion with solid components and areas of liquefaction necrosis involving the periarticular hip region (Figures and ). In view of the fact that the patient has got clinical signs and symptoms of sepsis and the imaging investigations pointing towards and infective pathology a provisional diagnosis of periprosthetic hip infection was made.\nA hip aspiration was performed in theatre under image intensifier guidance with strict aseptic conditions. The aspirate revealed slightly turbid fluid. The following results including Gram stain, culture, and sensitivity were negative. Postaspiration intravenous antimicrobials were commenced. However the patient continued to remain unwell despite antimicrobial therapy with a progressively increasing WBC and CRP count. The decision was therefore taken to explore and perform open biopsy of the pelvic collection and soft tissue mass. At surgical exploration, a 4 cm × 6 cm cavity was identified. Deep tissues were noted to be oedematous, necrotic, and inflamed, but macroscopically they were not typical findings in keeping with an abscess cavity. Specimens were sent for histology and microbiology. All direct culture and sensitivity specimens from the hip biopsy were negative. Histopathology of the specimen demonstrated sheets of atypical lymphoid cells with a hint of plasmacytoid differentiation (). Immunostains showed the neoplastic cells to express CD138, MUM 1, PAX5, and EBV ISH (Figures , , , and ). They demonstrated patchy weak expression of CD56 and CD30. They were negative for CD20, CD79, CD19, cyclin D1, ALK, HHV8, CD2, CD3, CD4, CD5, CD8, CD33, CD43, EMA, Cytokeratin, and S-100. In situ hybridization and immunostaining for immunoglobulin heavy and light chains appeared polytypic and was difficult to interpret. Therefore a PCR based analysis of B cell clonality was performed which showed clonal IG Kappa gene rearrangements consistent with clonal B cell population. In the context of a metallic hip replacement, the features were those of diffuse large B cell lymphoma with chronic inflammation.\nDue to clinical condition of the patient and poor prognosis, in liaison with the haematological team who advised that the patient was in the terminal phase of her illness, appropriate management would be palliation. The patient was transferred to a hospice for continuing care and support and the patient passed way after only two weeks of admission to hospice. |
Ocular pressure waveforms from the right eye of a 49-year-old white male subject were digitally recorded during training to use the PASCAL DCT. Custom software was provided by the manufacturer for continuous recording. It was noted that the waveform had an unusual shape consistent with an early ventricular contraction every other beat []. It was also noted that the OPA was > 9 mmHg. Blood pressure (BP) was 138/64 and heart rate was 64 beats/min. A digital recording of the patent's OPA during Valsalva was then performed []. It was noted that the OPA became significantly lower during Valsalva, and the waveform became more regular in appearance.\nThe subject then presented for a thorough examination by a cardiologist. He was known to have congenital absence of the pericardium, which was diagnosed 12 years prior when he was evaluated for nonexertional chest pain.[] Past medical history was significant for repair of a right inguinal hernia at age 27. He took omeprazole 20 mg p.o. daily for gastroesophageal reflux disease. On the review of systems, he reported generalized fatigue for 6 months, paroxysmal nocturnal dyspnea and orthopnea for 2 months, and a sensation of a bounding pulse in the neck for several weeks. He denied chest pain or lower extremity edema. Family history was significant for acute ascending aortic dissection in his younger brother at age 43.\nPhysical examination revealed an early descending diastolic murmur. The lungs were clear to auscultation. Holter monitor testing for 24 h revealed a baseline rhythm of normal sinus with first degree atrioventricular block and intermittent episodes of sinus tachycardia, sinus bradycardia and sinus arrhythmia, including isolated premature atrial complexes, premature ventricular complexes and ventricular bigeminy []. Echocardiography estimated left ventricular (LV) ejection fraction to be 50–55%. There was normal right ventricular systolic function and at least moderate aortic regurgitation []. Mild dilatation of the aortic root was noted on the echocardiogram. Cardiac magnetic resonance imaging demonstrated severe aortic insufficiency and a dilated left ventricle with a calculated LV ejection fraction of 42%. The ascending aorta was dilated with a maximum dimension of 4.3 cm at the level of the sinuses and 4.1 cm in the mid ascending aorta. Absence of the pericardium and posterior rotation of the heart were again noted.\nPreoperative diagnoses included congenital absence of the pericardium, annulo-aortic ectasia with aortic root aneurysm and symptomatic, severe aortic insufficiency with LV dysfunction. He underwent surgery to replace the ascending aorta and the aortic valve with a pericardial tissue graft. A Bentall procedure was performed with a composite graft composed of a 32 mm Valsalva graft and a 29 mm Carpentier-Edwards PERIMOUNT Magna valve. Re-implantation of the coronary artery ostium was also performed.\nThe subject's postoperative course was uneventful. His symptoms of dyspnea and orthopnea resolved. Postoperative electrocardiogram readings revealed a progressive decrease in the frequency of arrhythmias, with the patient predominantly in normal sinus rhythm []. OPA was measured at 1 month and 6 months postoperatively []. The average values for OPA were 2.0 mmHg at 1 month and 3.5 mmHg at 6 months. The mean IOP at the 1 and 6 month postoperative visits was 13.3 mmHg and 12.2 mmHg, respectively. The waveform revealed occasional ectopic beats at both postoperative visits; however, the frequency of these and the amount of time in bigeminy was drastically reduced [Fig. and ]. Postoperative two-dimensional echocardiography demonstrated resolution of the aortic regurgitation []. |
A 54-year-old man presented at emergency with a complaint of sudden, severe, and generalized abdominal pain. The pain was persistent without any radiation mentioned from the patient. The heart echo and the laboratory tests were normal. Computed tomography angiography (CTA) showed a type B aortic dissection, originated distally of the left subclavian artery and extended to the left iliac artery. The celiac trunk was also dissected, while the superior mesenteric and both renal arteries were supplied by the true lumen ( and ).\nA conservative management strategy was chosen, with administration of anti-hypertensive treatment and in-hospital observation. The patient’s condition remained stable and on the fifth day after diagnosis he was discharged from hospital and endovascular restoration of the type B dissection was scheduled for 2 weeks later, in a sub-acute phase.\nAfter four days, the patient presented again, with generalized abdominal pain and two episodes of vomiting containing food material. An emergency CT angiography was performed, showing that the type B aortic dissection was similar, but the Celiac trunk dissection had aggravated, although blood supply was preserved through the hepatic and left gastric arteries. One of the branches of the splenic artery was no longer patent, and a splenic infarct was detected (). There were no differences in laboratory test results compared to the previous admission.\nAn urgent intervention decision was taken, in order to avoid organ ischemia. Careful observation of the CT angiography identified the Arc of Buhler anatomical variation (). This random finding suggested that the blood supply could be maintained, since the flow from superior mesenteric artery was intact. Taking into consideration this fact, we decided to reject the option of a bypass of the celiac trunk but the intervention through Thoracic Endovascular Aortic Repair (TEVAR) couldn’t be postponed, as the dissection was progressing. A 43 x 37 x 200mm stent graft (LifeTech endograft) was placed distally to the left subclavian artery, covering the entry tear, while its distal edge was proximal to celiac trunk. Despite the fact that the celiac trunk was occluded to an important degree, blood supply to the organs was maintained with absence of clinical deterioration due to malperfusion.\nPostoperative recovery was uneventful and the patient was discharged from hospital after 6 days. The results of CT angiography after one month were satisfactory enough, but unfortunately he died in a car accident 2 months later. |
A 29-year-old man with no history of preexisting diseases presented with acute upper gastrointestinal bleeding in a local primary hospital. Endoscopy revealed a submucosal tumor located shortly distal of the papilla of Vater, bulging under the mucosa and forming a partly intraluminal mass. The mucosa showed a central ulceration, this being the origin of the massive bleeding. Because of persistent bleeding that could not be controlled by endoscopic interventional treatment, an emergency laparotomy was performed at the local primary hospital. At laparotomy, an encapsulated mass originating from the duodenal wall at the proximal third portion of the duodenum was identified, reaching to the pancreatic head. A ligation of tumor-feeding vessels was successfully performed to control the bleeding. After the emergency treatment, the patient was referred to our university hospital for further therapy.\nComputed tomography visualized the tumor of the duodenum with a diameter of 1.8 × 2.3 × 2.5 cm (Figure ). The scan showed no metastases. 2 days after the initial emergency surgery, the patient underwent definitive surgery at our institution. No recurrent bleeding occurred since the emergency ligation of tumor-feeding vessels.\nA relaparotomy was performed. The tumor was located in the proximal third portion of the duodenum, 3 cm distal of the ampulla of Vater (Figure ). It originated from the duodenal wall and protruded as a roundly shaped mass into the near of the pancreatic head. No infiltration of the pancreas or other adjacent organs was found, there were no suspicious lymph nodes. There were no signs of duodenal ischemia related to the haemostatic vessel ligation done at the first emergency operation. The tumor was treated by a limited resection of the distal second, third and fourth part of the duodenum, the proximal resection margin was located just distal of the ampulla of Vater. The bowel continuity was reconstructed by a latero-terminal duodenojejunostomy (Figure ) located opposite to the ampulla of Vater in order not to induce a stricture of the papilla.\nThe postoperative course was uneventful and the patient was discharged on postoperative day 13.\nIn the opened specimen, the tumor diameter was 2.5 cm (Figure ). The distance to the proximal resection margin was 0.5 cm; overall length of the duodenal segment was 9 cm. Histology revealed a GIST with a typical spindle cell pattern of the tumor cells (Figure ). There was focal necrosis. The main tumor mass was located subserosal. The tumor had a thin fibrous capsule, and it reached the muscularis mucosae, without penetrating it. There was a regular duodenal mucosa covering the tumor. Immunohistochemistry showed a strong positivity for KIT (CD117) and CD34, while desmin and smooth muscle actin were negative (Figure ). Mitotic activity was < 5/50 high power fields. No formal lymph node dissection had been performed, and as expected no lymph nodes were detected in the resected specimen.\nAs tumor diameter and low proliferative activity indicated a low risk of malignancy and recurrence, there was no indication for an adjuvant therapy with Imatinib, a tyrosine kinase inhibitor. |
A 17-year-old female patient was referred to the Infectious Diseases Ward from a district hospital because of prolonged fever lasting for 14 days. In the patient history, in the evening of the first day of the onset of symptoms the patient reported a temperature of 40°C, pain in the lower limbs and muscular pain. The next day, the patient was without fever but developed a sore throat and had enlarged submandibular lymph nodes. The patient was consulted by her family doctor, who diagnosed a throat infection and prescribed second-generation cephalosporin (cefuroxime axetil) and ibuprofen. After a few days, the sore throat was relieved, although the fever remained. Five days later, the patient went to a gynecologist with the symptoms of intense lower abdominal pain during her menses; a gynecological examination revealed no abnormalities. On the basis of her fever for the previous 8 days and not responding to antibiotic treatment, the patient was admitted to hospital for further evaluation.\nOn being admitted, the patient was in a generally good state, with good contact and orientation, no pathological changes on the skin, a body mass of 60 kg, height of 170 cm, body mass index (BMI) of 19.33, blood pressure of 110/70 and without any abnormalities determined during the physical examination. Past medical history of this patient was as follows: at the age of 5, she was hospitalized because of an infection of the oral cavity, urticaria and edema of the joints. At the age of 13, the patient was admitted once again because of tonsillitis accompanied by another episode of edema of the joints. At the age of 14, the patient underwent tonsillectomy. The girl was then consulted by a rheumatologist – but the diagnosis of a connective tissue disease was not established. The consulting hematologist did not recommend bone marrow examination. The current process of diagnostic laboratory tests revealed elevated inflammatory markers: ESR, CRP, PCT. Serum ferritin concentration was slightly elevated, and serological markers indicated previous infection with Epstein-Barr virus (EBV), cytomegalovirus (CMV) and Mycoplasma pneumoniae. Immunoelectrophoresis showed increased concentrations of α-globulins. Both antinuclear antibodies (ANA) and anti-neutrophil cytoplasmic antibodies (ANCA) were negative. On three separate occasions, blood culture did not cultivate any bacteria. Also urine culture was negative. The results are presented in .\nChest X-ray, abdominal ultrasonography, and echocardiography did not reveal significant abnormalities. During hospitalization, episodes of fever with skin rash and musculoskeletal pain of the lower limbs, which responded well to 1 g paracetamol administered intravenously, were observed. Procalcitonin concentrations continued to increase, despite antibiotic therapy, a lack of a bacterial source of infection and the patient’s generally good status between episodes of fever ().\nC-reactive protein concentrations decreased during therapy, starting from 191 mg/l to 18 mg/l (normal values 0–5 mg/l) on antibiotics, and further to the normal range on steroid treatment. Procalcitonin analysis was performed by the VIDAS B.R.A.H.M.S PCT immunoassay, BioMérieux, Paris, France). On the 15th day of illness, the patient complained of vision loss. However, subsequent ophthalmologist examination and computed tomography of the head did not reveal any pathologies. On the 10th day of hospitalization (23rd day of the disease), edema of the feet, ankles, and knees appeared, with edema of the feet persisting for the next few days. On the basis of the clinical picture and after excluding any other possible causes of fever, especially infections, the patient was diagnosed with adult onset Still’s disease (AOSD); steroid therapy was initiated, and antibiotics were discontinued. The procalcitonin concentration of 13 ng/ml (normal values < 0.5 ng/ml) was normalized after 5 days of steroid therapy. The patient was discharged under ambulatory rheumatologic supervision. |
A 67-year-old female with past medical history of congenital deafness presented to the emergency room with complaints of right-sided facial droop and right upper extremity weakness, tingling, and numbness. These symptoms were sudden in onset and lasted for a few minutes. Symptoms had completely resolved at the time of presentation. She did not have a history of any atherosclerotic risk factor including hypertension, diabetes, or hypercholesterolemia. The initial set of vital signs were normal; routine laboratory tests including complete blood count and basic metabolic panel were unremarkable. A computed tomography (CT) of the head without contrast as well as a magnetic resonance imaging (MRI) of the brain with and without contrast did not show any acute intracranial hemorrhage or infarction.\nThe patient was diagnosed with TIA, and further investigations were planned to determine the etiology. A magnetic resonance angiogram (MRA) of the head and neck with and without contrast did not show any arterial flow limiting stenosis or occlusion. A transthoracic echocardiogram (TTE) with bubble study using agitated normal saline contrast was performed and was found to be normal. Patient's heart rhythm was monitored with continuous cardiac monitoring, and no arrhythmias were noted during her stay at the hospital. At this point, the patient was identified as having cryptogenic TIA, having failed to determine the precise etiology from routine workup. Patient was started on aspirin therapy and discharged from the hospital on day 3 with further outpatient workup planned. Outpatient workup for hypercoagulability showed a high factor VIII activity of 153%, which potentially put her at increased risk of venous thromboembolism. However, this test was performed just one week after the thrombotic event and was hence difficult to interpret. Subsequently, a transesophageal echocardiogram (TEE) was performed that revealed a patent foramen ovale with right-to-left shunt. This raised the concern for paradoxical embolism as the cause of patient's TIA. Lower extremity duplex venous ultrasound showed no evidence of deep vein thrombosis. However, magnetic resonance venogram (MRV) of pelvis showed compression of the left common iliac vein just after its origin, which was suggestive of May-Thurner syndrome (). There was no evidence of venous thrombosis on the MRV. May-Thurner syndrome was recognized as the probable source of paradoxical embolism causing TIA in the patient. The patient was eventually referred for percutaneous PFO repair, which was performed without any complications. The patient had been regularly followed yearly at the cardiology clinic for 5 years now. She remains in good health with no further episodes of TIA. |
The patient was a 56-year-old female who came to us during an episode of mania and psychosis while receiving treatment at an addiction treatment center where she was taking a phenobarbital taper for chronic lorazepam use. She displayed symptoms of aggressive posturing, verbal abuse to staff, delayed response, and racing thoughts. She did not describe suicidal thoughts. She had 4 prior psychiatric hospitalizations. At age 33, she exhibited depression, anxiety, and paranoia that lead to her first hospitalization. At age 44, she attempted suicide via acetaminophen overdose. Her first reported manic episode was at age 45, during which bipolar type 1 disorder was considered as her differential and subsequently diagnosed. Her symptoms accompanied delusions during this episode, without suicidal ideation. Her most recent hospitalization was at age 49 for depression and paranoia with delusions of being wiretapped and people reading her mind. At age 55, the patient presented with optic neuritis and diagnosed with MS after a lumbar puncture showed oligoclonal bands.\nFamily history revealed depression in father and alcohol use disorder in mother. Past medical history described an acute onset dizziness when moving eyes left to right or vice versa and when standing up from a lying position.\nNeurology consult found no focal or lateralizing findings. MRI analysis showed greater than 15 foci of T2 hyperintensity within white matter where some lesions were within periventricular and juxtacortical white matter of both cerebral hemispheres, consistent with a demyelinating disease. A single focus of enhancement in the posterior corona radiata was suggestive of active demyelination. No demyelinating signs were seen in the thoracic spine. However, no significant difference was seen compared to previous MRI.\nDuring the present hospitalization, patient's prior bupropion was reduced due to concern for further mania activation. Lithium 600 mg twice a day was prescribed for mood stabilization. Risperidone 0.5 mg at bedtime was prescribed for ongoing delusions. Patient was not taking scheduled steroids prior to admission. After 8 days of hospitalization, patient's mania improved but demonstrated atypical features, such as absence of pressured speech, grandiosity, risk taking or sleep pattern changes. Per a family member's report, patient stated that she was in a movie and that everyone else was acting around her. Patient requested discharge to continue treatment in an outpatient setting. |
A 50-year-old female with a history of smoking and hypertension developed the acute onset of left upper extremity paresthesias and weakness lasting approximately 1 to 5 minutes with jaw numbness for another 5 minutes. All her symptoms resolved by the time she arrived at the hospital, and she was normotensive with no neurologic deficits on examination. A CT scan obtained in the emergency room showed a small chronic cortical insult of the ipsilateral temporal lobe, which was presumed to be unrelated. MRI of the brain showed no acute ischemia. MRA of the head and neck was then obtained and showed an anomalous anastomosis of the right vertebral artery to the internal carotid artery segment 2.0 cm distal to the right common carotid artery bifurcation (Figure ). Formal digital subtraction angiography confirmed this anatomic variant originating from the right internal carotid and terminating as the basilar artery. Injection of the right, internal carotid thus demonstrated filling of the entire right hemisphere and posterior circulation (except the posterior inferior cerebellar artery (PICA)) from the right internal carotid. The absence of posterior communicating arterial flow was noted bilaterally. The vertebral arteries terminated at the PICA bilaterally. Just proximal to the ICA-PPHA bifurcation, there was a 1.3 cm length of plaque resulting in greater than 60% diameter stenosis (Figures -). Given the clinical implications of atheroembolic disease affecting the only supply to the patient's brainstem and the technical difficulty of surgical endarterectomy with difficult anatomy, the patient was entered into the treatment arm of the SAMMPRIS trial and underwent endovascular stenting. After placement of a SpiderFx™ embolic protection device into the PPHA, a 7 x 10 x 40 mm Protege stent was deployed successfully across the stenotic segment and resulted in complete restoration of luminal diameter without the need for angioplasty (Figure ). The patient tolerated the procedure well, was maintained on antithrombotics, and incurred no further ischemic symptoms in long-term follow up with the last angiogram one-year post-treatment showing stable resolution of stenosis and ulcerated plaque. |
A 33-year-old male patient came to the hospital complaining of headache that had been occurring for two weeks prior to his visit. One week before visiting the hospital, he received a cervical facet joint block and drug treatment in another hospital, but the pain continued so he visited the pain clinic at our hospital. In the first medical examination, the patient said that he did not have any unusual past history. The headache began at the left occipital region and then gradually spread to the posterior neck, where numbness and aching developed; there was no paresthesia. Additionally, during the same period, the patient had blurred vision as a symptom. The physical examination performed at the time of the hospital visit appeared to be normal.\nAt first, it was suspected to be a general cervicogenic headache, so a bilateral greater occipital nerve block was performed as a diagnostic block and his progress was monitored. Three days later in the clinic, the ache in the head area had slightly decreased, but the numbness had spread from the face to the posterior neck, both shoulders, and both arms. In addition, during the physical examination performed this time, the Lhermitte sign appeared to be positive. Hence, a more detailed past history was examined, and it was found that 15 years ago the patient's eyesight suddenly failed so he was diagnosed with optic neuritis, and during the same period, he also suffered from myelitis. Secondary headache due to central pain was suspected from the progress of the disease, past history, and physical examination, and thus C-spine MRI was performed. As a result, an extensive intramedullary high signal intensity was observed from the medulla oblongata to the C5 (). Visual acuity test, color vision test, visual field test, and fundus examination performed in ophthalmology to check for anomalies in the optic nerve all appeared to be normal, but in the subsequent goggle VEPS (visual evoked potentials), a bilateral ocular or prechiasmal lesion and suspicious bilateral upper brainstem lesion were observed. In addition, the oligoclonal band IgG appeared negative in the CSF analysis. There were no abnormalities in the brain MRI, and the NMO-Ab test was negative.\nBased on the past medical history and test results above, the patient was diagnosed with neuromyelitis optica, and steroid pulse therapy was performed for 5 days in which 1 g of solumedrol was given on each day. Subsequently, the neurological symptoms and MRI observations improved, so the drugs were changed to oral steroids and the patient was discharged (). |
A 24 year-old female, teacher by profession, with normal birth and developmental history, presented with a history of generalized tonic clonic seizures since three years and frequent episodes of bizarre posturing of limbs followed by unresponsiveness since one and half years.\nShe was well until three years back when she had the first episode of sudden loss of consciousness with repetitive tonic clonic movements of all four limbs and tongue bite. This was not associated with any prodrome, aura or any postictal automatisms or autonomic symptoms. The episodes were short lasting (usually about a minute) and not followed by any focal neurological deficit and the frequency being two to three episodes per month. There was no history of myoclonic jerks or any definite relation to sleep deprivation or menstrual cycle and no family history of seizures. MRI of Brain and a routine EEG done prior to coming to this hospital were normal. At some other hospital, based on a diagnosis of Juvenile Myoclonic Epilepsy, sodium valproate with a maximum total dose of 1250 mg/day and clobazam with dose of 20 mg/day, was started. As per the patient the seizure frequency had not changed significantly on treatment. She had gained weight and also developed menstrual irregularities on medications and hence she left all her medications nearly an year ago.\nNearly one and half years ago, she started to have episodes which were more prolonged with a gradual progression of symptoms. She would start having bizarre, flinging movements of all four limbs and neck, without tonic clonic movements, jaw clenching, tongue bite or incontinence. At the onset of the episode she would be partially responsive but would lapse into drowsiness over the next few minutes. These would be precipitated whenever she would delay her meals or miss meals but had no relation to exercise or movement. Also the mother used to feed her in this state of reduced responsiveness and over next few minutes she would gradually improve. There was history of amnesia for these episodes As a result the mother had started giving her meals every two to three hours and even wake her up at midnight to feed her. The duration of these would range from few minutes to about half an hour. She had also started feeling hungry more frequently and had gained weight in the last one year. During the same period she also continued to have generalized tonic clonic seizures.\nA provisional clinical diagnosis of generalized tonic clonic seizures, probably idiopathic generalized epilepsy with Non epileptic attacks, was made.\nShe was admitted for evaluation. On examination she was found to be obese. Rest of her general physical examination including the vitals revealed no abnormality. Examination of her nervous system including higher mental functions, cranial nerves, sensorimotor system and extrapyramidal system were all normal.\nThe patient was instructed to miss her meals and concomitant video-EEG monitoring was done.\nA typical attack was induced after 2 hours. Her extremities became cold and she started sweating profusely. She was confused but able to respond to questions. Almost simultaneously she started having involuntary, unpurposeful, non-rhythmic, asynchronous bizarre movements of her limbs, abnormal posturing, opisthotonous posturing, tongue darting, facial grimacing and limb thrashing. There were no tonic or clonic components. A random blood glucose level was done by finger prick method and was found to be 24 mg% (normal 55-100 mg%). She was infused intravenous dextrose and within a few minutes she regained consciousness and the movements subsided.\nRegular monitoring of her blood glucose revealed spontaneous asymptomatic hypoglycemia at several occasions per day (as low as 35 mg%), precipitated by fasting. However, the attacks occurred only when her blood sugar decreased to < 25 mg%.\nOne episode of generalized tonic clonic seizure with tongue bite, lasting for a minute was witnessed in the ward during the morning hours. A random blood sugar done at that time was 45 mg%.\nIn view of the abnormal movements being a combination of chorea, ballism and dystonia with prolonged duration, no temporal relation to movement or any form of startle, we diagnosed them as being Paroxysmal non-kinesiogenic dystonic choreoathetosis. As a possible etiology for symptomatic recurrent hypoglycemia and weight gain, Insulinoma was suspected. Serum fasting insulin levels were done and were found to be markedly elevated in the range of 106 IU/L (Normal lab values 2.6-24.9 IU/L). MRI of abdomen with contrast revealed a hypoechoic mass lesion in the distal pancreas, size 2 × 3 cm with no evidence of local or distant metastasis, possibly Insulinoma Figures and .\nEEG done during the episode showed intermittent sharp waves and spike and wave discharges in the period preceding the episodes. Blood sugar levels were also done simultaneously and the electrographic discharges were found to appear only during hypoglycemic periods and subside when normoglycemia was restored. Interictal EEG record, however, was normal.\nShe was given frequent small meals and was given intravenous dextrose only if she became symptomatic. She underwent a laparotomy and was found to have a 3 × 3 cm firm tumor in the postero-superior aspect of distal pancreas adherent to the splenic vein, without any distant metastasis. A distal pancreactectomy with splenectomy was performed. Histopathology of the mass confirmed the diagnosis of insulinoma with an uncertain malignant potential, with the pancreatic resection margin and lymph nodes free of the tumor.\nPresently she is doing well after the surgery and has been totally symptom-free since last 6 months. She has been off all antiepileptic drugs with no recurrence of seizure or abnormal movements. |
A 37-year-old Hispanic woman without a significant past medical history presented to our hospital with a one week history of shortness of breath, which had acutely worsened over the past few days. She stated that she required two pillows to breathe while lying down. She also stated that she could no longer work at her childrens' day care center, as even walking at a relaxed pace for more than two minutes would cause her to be short of breath, although the shortness of breath would improve with rest. She also complained of a nonproductive cough for two days before admission. She denied any trauma, chest pain, palpitations, nausea and vomiting, diarrhea, abdominal pain, or fevers. Review of systems was remarkable for a one month history of bilateral lower extremity edema.\nShe denied any significant past medical history and had not been taking any medication before hospital admission. She remotely recalled that her last physician, whom she had not seen in the previous eight years, had mentioned to her that she might have hypothyroidism, but this was not further investigated. She denied use of tobacco, alcohol, or any other recreational drugs.\nPhysical examination revealed an obese, pale woman in mild distress from shortness of breath, but able to speak in full sentences. Her vital signs on arrival showed an oral temperature of 36.8°C, heart rate in the range of 81 to 93 beats per minute, blood pressures ranging from 140 to 173 mm Hg systolic and 94 to 121 mm Hg diastolic. Her breathing was 18 to 22 times per minute, with a digital pulse oximetry saturation of 86% while breathing air, which subsequently improved to 92% when she was given oxygen therapy via a face mask. Her neck examination showed no nodular thyroid or thyroid masses. There was no lymphadenopathy. Dullness to percussion was found over the lower portion of her left hemithorax, accompanied by decreased vocal fremitus, as well as decreased breath sounds on auscultation. The chest examination was normal over her right hemithorax. Cardiac examination revealed no murmurs, rubs, or gallops, but heart sounds were generally distant. Abdominal examination was unremarkable, with no signs of ascites or masses. Lower extremities revealed 2+ pitting edema to the level of the knees bilaterally.\nHer electrocardiogram showed low voltage throughout all leads with no other ST-segment or T-wave abnormalities. A chest radiograph showed an enlarged cardiac silhouette and bilateral pleural effusions, with more fluid on the left than on the right. A computed tomography (CT) angiogram of the chest did not reveal pulmonary thromboembolism and confirmed the presence of a large left pleural effusion with associated compressive atelectasis, as well as a moderate pericardial effusion (Figure ).\nAdmission laboratory values demonstrated a leukocyte count of 6.3 × 109/L (normal, 3.5 to 11.0 × 109/L) with a hematocrit of 36% (normal, 36% to 46%). A chemistry panel was remarkable for a creatinine of 1.4 mg/dL (124 μmol/L; normal, 44 to 106 μmol/L). Troponin T cardiac enzyme was slightly elevated at 0.02 μg/L (normal, <0.01 μg/L), and beta-natriuretic peptide was elevated at 1325 ng/L (normal, 0 to 450 ng/L). Urine alaysis was significant for 2+ protein. A fasting lipid panel showed a total cholesterol of 179 mg/dL (4.64 mmol/L; normal, 3.65 to 5.15 mmol/L); triglycerides, 186 mg/dL (2.10 mmol/L; normal, <1.70 mmol/L), HDL, 25 mg/dL (0.65 mmol/L; normal: >1.01 mmol/L), and an LDL of 117 mg/dL (3.03 mmol/L; normal, 1.55 to 3.34 mmol/L). The liver panel showed a total protein of 7.0 g/dL (70 g/L; normal, 60 to 80 g/L) and albumin at 3.3 g/dL (33 g/L; normal, 35 to 50 g/L) with normal transaminases. Lactate dehydrogenase (LDH) was 302 U/L (normal, 112 to 220 U/L). Given her complaints of fatigue, a check of thyroid-stimulating hormone (TSH) and a free T4 levels were also carried out. The results of those tests showed the her TSH to be 181.90 mIU/L (normal, 0.27 to 4.2 mIU/L) and her free T4 to be <0.1 ng/dL (<1.29 pmol/L; normal, 0.9 to 1.7 pmol/L).\nThoracentesis on hospital day two revealed cloudy yellow fluid (Figure ) with 129 × 109/L white blood cells (23% neutrophils, 23% lymphocytes, 54% monocytes); a lactate dehydrogenase (LDH) level of 170 U/L, with a pleural fluid LDH-to-serum ratio of 0.56; a protein level of 5.6 g/dL (56 g/L), with a pleural fluid protein-to-serum ratio of 0.8; albumin of 2.8 g/dL (28 g/L); cholesterol of 81 mg/dL (2.10 mmol/L), with a pleural fluid cholesterol-to-serum ratio of 0.5; and triglycerides of 442 mg/dL (4.99 mmol/L), with a pleural fluid triglycerides-to-serum ratio of 2.4. These findings were thought to be compatible with an exudative chylous effusion. A 10-French chest tube was placed by our interventional radiologists the fourth day of her hospitalization. A follow-up chest CT after drainage of the effusion performed the seventh day of hospitalization showed a nonspecific retrocrural density that our radiologists thought to be compatible with swelling or inflammation related to her myxedema.\nTo investigate the etiology of her chylous effusion, she underwent nuclear medicine lymphatic scintigraphy on the following day to look for possible thoracic duct injury. The study showed normal tracer uptake throughout the lymphatic system without any evidence of accumulation to suggest leakage or trauma.\nShe was prescribed levothyroxine, 100 μg, slowly escalating to 150 μg orally per day for treatment of her hypothyroidism, along with a low-fat diet, and over the course of two weeks, her chest-tube drainage progressively decreased (Figure ), and her fatigue and dyspnea subjectively improved. Her antithyroid peroxidase antibody was found to be elevated at 97.1 kIU/L (normal, <40 kIU/L), compatible with autoimmune thyroiditis. The chest tube was removed after ten days, and she was discharged home.\nAt two and three month follow-up visits, her fatigue had significantly improved, and she had returned to regular employment. Her TSH achieved a normal level (1.35 mIU/L; normal, 0.27 to 4.2 mIU/L). Follow-up chest radiographs and CT studies at two, three and six months showed near-complete resolution of the left chylous effusion, with complete resolution of the mild pericardial effusion. The retrocrural density seen on earlier CT studies has remained unchanged. At one year follow-up, no recurrence of her pleural effusion or clinical signs of malignancy have occurred. |
A 68-year-old man with a medical history of hypertension was referred to our hospital with progressively increasing lower back and left leg pain despite analgesic treatment. Preoperative imaging revealed degenerative spinal stenosis from L2 to L5 with spondylolisthesis at L3 to L4 and L4 to L5 (). Preoperative neurological examination detected no motor or sensory deficits. The patient did not report any bowel or bladder function loss at that time. He also had no history of prior anticoagulation therapy, and preoperative coagulation studies were normal.\nThe patient underwent L2 to L5 decompressive laminectomies and L3 to L5 instrumented posterolateral spinal fusion with autograft bone. No dural tears occurred intraoperatively. After achievement of excellent hemostasis, a surgical drain was placed at the time of closure. Intraoperative blood loss was approximately 500 mL.\nOn postoperative day 2, the drain was removed, and progressive physical therapy was started. His postoperative course was unremarkable. His symptoms resolved, and he was discharged on day 14. That same evening he felt a sensation of lower abdominal bloating.\nThe next morning (postoperative day 15) he presented to the emergency room complaining of difficulty in urinating. His bladder was found to contain approximately 500 mL of urine. He did not report any leg or back pain. Physical examination revealed no motor or sensory deficits. Emergent lumbar magnetic resonance imaging (MRI) visualized a T2 high weighed epidural mass at the surgical site flattening the thecal sac from L4 to L5 (). We immediately performed surgical evacuation manually with irrigation and in the process discovered a large consolidated hematoma (4.5 cm × 2 cm), which we removed (). No obvious source of bleeding could be identified. We placed an epidural drain in the surgical site before closure, which we kept there for 5 days. Postoperative MRI showed complete resolution of the epidural hematoma (). After evacuation, his symptoms immediately disappeared, and his urinary function recovered completely. We allowed him to walk again after the removal of drain, and he was discharged home on day 10. |
A 28-year-old male working as a tractor driver was brought to the Emergency surgical ward by his wife with complaints of cutting off at the left side at the base of his scrotum with a kitchen knife.\nHis history revealed that patient has been taking arrack for the last 6 years. He started with 200 ml of arrack and slowly increased the amount to 1l over a period of 6 years as he was not getting the desired effect he used to get with 200 ml. On occasions, when he did not have the money or opportunity to drink, he used to experience dysphoric state including severe anxiety, palpitations sweating, restlessness, and tremors of hands. These symptoms used to subside upon taking alcohol.\nHe also did not have any control over the amount of alcohol he is taking and the money he is spending on it once he started to drink. He also neglected his duties both at work and at home due to this habit, for which he had frequent quarrels with his wife and has also ended up in financial crisis with a big debt. Due to above mentioned problems two days before admission, patient decided to stop drinking alcohol and stopped all of a sudden and he has developed abnormal behavior like talking to self, not feeding self, not recognizing family members, and talking irrelevantly. He started to complain that he is hearing voices of male and female when no one around.\nOn the morning of the day of admission, patient went to the kitchen and cut his scrotum at the base with a knife on left side. The dimensions 2×3 inches with a depth of 1 inch. After amputation, patient did not talk to anyone and sat all alone. His wife after one hour saw blood stains on floor and on his groins and brought him to casualty. After suturing the wound, the patient was referred to psychiatry op. The Mental status examination of the patient showed that the patient is not co-operative, not oriented to time, place, and person, hence cognitive functions could not done. He was diagnosed as Mental and behavioral disorders due to alcohol withdrawal state, complicated with delirium. The patient was admitted in psychiatry ward, all the investigations done like complete blood picture, liver function tests, blood urea, serum electrolytes, and Computed Tomography (CT) scan brain to rule out any organic cause. They started on tablet Chlordiazepoxide 200 mg in divided doses, tablet Thiamine 150 mg both given orally in divided doses to prevent Korsakoff's psychosis and injection Lorazepam 2 mg IM on sos basis to prevent any agitation. The patient became oriented on the 4th day of admission and then stared to complain of pain at injury site for which he was started on Non steroidal Anti Inflammatory Drugs. Mental status examination then done showed that the patient's cognition was normal.\nThere were no adverse effects like infection of the injury or cutting of the cord or vas deferens and the wound completely healed in a period of 3 weeks. For next ten days, we slowly tapered off the Chlordiazepoxide and on the 10th day of admission, patient was discharged on tablet Disulfiram. The patient is on regular follow-up now for last 8 months and completely abstinent from alcohol. |
A female infant, one of di-amniotic di-chorionic twins was born at 23-week gestation to a 34-year-old, G3, P2, mother who had received prenatal care. The pregnancy was a product of in vitro fertilization. She presented in preterm labor and had a spontaneous vaginal delivery which was complicated by placental abruption. The baby had a birth weight of 460 grams. The Apgar scores were 1, 1 and 6 at 1, 5 and 10 minutes respectively. She received one dose intra-tracheal surfactant soon after birth and did not require additional doses. The initial chest radiograph revealed respiratory distress syndrome along with pulmonary interstitial emphysema. She was mechanically ventilated for three weeks followed by non-invasive ventilation for another five weeks. Then she needed supplemental blended nasal cannula oxygen at one liter per minute (LPM) with oxygen between 22% to 26% after that and she was also on inhaled fluticasone. The patient had medical management of a symptomatic hemodynamically significant patent ductus arteriosus from the 15th to the 17th day. Subsequently echocardiograms at 3 weeks, 9 weeks and at 14 weeks of age showed that the ductus arteriosus remained closed and there was no evidence of pulmonary hypertension. The patient received parenteral nutrition for 5 weeks till enteral feeding by orogastric and nasogastric feeding tube was fully established beyond that. At 12 weeks of age she had signs of mild cholestatic liver dysfunction and was noted to have intermittent hypoglycemia. The calories in her feeds were increased to 26 calories per ounce and her feeds had to be infused over an hour to manage that. Further workup with levels of insulin, cortisol, growth hormone, betahydroxy butyrate, free fatty acids, c peptide and acyl carnitine profile revealed only a mildly elevated insulin level. She had an adequate response to ACTH stimulation test but had a poor glucose response to glucagon administration. As the work up was interpreted as transient hyperinsulinism by her clinicians, she was started on diazoxide at 5 mg/kg/day with some improvement in blood glucose levels. The clinical team felt that maintaining euglycemia was essential prior to sending her home and sequentially increased the dose by 5 mg/kg till a maximum of 15 mg /kg/day was reached. At this point she remained euglycemic but her cardiorespiratory status rapidly declined. This deterioration was initially thought to be due to fluid retention and she was started on diuretics. With worsening respiratory failure and hypotension, she needed significant ventilator and ionotropic support with vasopressors. She was in FiO2 of 1 and after 12 hours of conventional mechanical ventilation had to be changed to a high frequency oscillating ventilation. Her echocardiogram at 16 weeks of age (9 days after starting diazoxide) revealed pulmonary hypertension, tricuspid regurgitation (TR) with the TR jet indicating near systemic pulmonary arterial pressures, and a small ductus arteriosus with bidirectional flow. There was no evidence of right ventricular dilatation, hypertrophy or dysfunction. At this point her diazoxide was discontinued (9 days after starting). She was started on inhaled nitric oxide (iNO) at 20 ppm with prompt response in oxygenation and her FiO2 requirements. Her oxygenation index improved from 38 to 27 over 4 hours and to 19 over 24 hours. She did not receive any additional pulmonary vasodilators. She was euglycemic, when maintained on intravenous alimentation. After seven days of mechanical ventilation, when her oxygen requirements were less than 50% her iNO was slowly weaned every six hours and she was completely off the iNO nine days after its initiation. Follow up echocardiograms showed that her pulmonary arterial hypertension resolved over two weeks and the ductus had spontaneously closed. She was again weaned off mechanical ventilation but required nasal cannula 100% oxygen at 0.2 LPM. At this point she was noted to have significant cholestasis and hepatic dysfunction. Her liver was just palpable 1–2 cms and she did not have hypertriglyceridemia or lipidemia. A genetic panel using next generation sequencing for neonatal cholestasis and alpha one antitrypsin testing were negative. Her liver function markedly worsened necessitating a referral for liver transplant at the age of six months. A liver biopsy done was inconclusive and she succumbed while waiting for the procedure at the age of eight months. |
A 19-year-old female patient was referred to the Department of Oral and Maxillofacial Surgery by the Department of Oral Medicine for the diagnosis and management of a painless “swelling” in the left buccal mucosa. The intraoral swelling was noticed by the patient 1 year back. According to the patient, the swelling had recently enlarged interfering with her mastication. No changes of size or development of swelling during meal times were reported. The patient was in apparent good health and her medical history was non-contributory.\nClinical examination showed a 3 × 2 cm swelling in the left buccal mucosa, which was freely movable. Intra-orally, it was covered by a mucosa of normal texture and color. On digital palpation, the swelling was non-tender, non-fluctuant, and rubbery in consistency. The swelling had well-defined margins and was not fixed to the underlying as well as overlying structures. There was no sign of hypoesthesia or paresthesia of the area involved.\nA computerized tomography (CT) scan with enhancement was performed to confirm the existence of a well-demarcated mass lying anterior to the masseter muscle []. The lesion was solid, without any relation to the mandible. The remainder of the head and neck scan did not reveal any other pathology or lymph node enlargement. Although it was believed that magnetic resonance imaging could give more precise and accurate information for the diagnosis and management of the lesion, the patient refused any further imaging study.\nThe structures of oral cavity can be affected by numerous pathological conditions that could be developmental, inflammatory, or neoplastic in origin. Lesions in the oral cavity may be present for a longer period of time before the patient seeks any medical intervention, which is usually as a result of interference with swallowing or speech.\nBased on the clinical presentation of the swelling, it appeared that the mass originated from the underlying mesenchymal tissues as opposed to the overlying mucosa. Our differential diagnosis was broken down into the general categories of reactive lesions, benign neoplastic lesions, and infectious origin. Specifically, our differential diagnosis of this soft tissue mass of buccal mucosa included lesions of minor salivary gland origin (benign vs. malignant salivary gland tumor, mucocele), vascular origin (hemangioma), non-odontogenic soft-tissue infection, hematomas, an intramasseteric abscess, masseteric hypertrophy, benign neoplasms of smooth muscle origin (leiomyoma), and reactive and neoplastic neural lesions (traumatic neuroma, neurofibroma, neurilemoma).\nCystic lesions-like dermoid cysts were clearly excluded by the CT findings, because fluid in the cysts would not have demonstrated enhancement by the contrast material.\nNo history of trauma excluded the possibility of hematoma.\nTraumatic neuroma was also considered. However, traumatic neuromas typically occur in the mental foramen region, tongue, and lower lip. One-third to one-fourth of these lesions are painful and are usually associated with trauma. The patient had no history of trauma. Benign neural neoplasms such as neurofibroma and neurilemoma may present as smooth-surfaced nodules similar in color to the adjacent normal mucosa. Approximately 25-45% of schwannomas or neurilemomas occur in the head and neck region, with tongue and buccal mucosa representing the most frequent intraoral lesions. Therefore, benign neural tumor was considered in one of the differential diagnosis.\nSalivary gland tumors account for 1% of neoplastic lesions in the head and neck region.[]\nA salivary gland neoplasm could have been a possibility, because the lesion was in close proximity to both parotid gland and minor salivary glands of buccal mucosa. Although the parotid gland is by far the most common site for salivary gland neoplasms, accounting for 64-80% of all cases, CT scans of this patient failed to reveal any parotid gland enlargement. Approximately 11-15% of minor salivary gland tumors occur in buccal mucosa. These typically present as painless, slow-growing, firm, frequently mobile swellings.[] Taking into account the size of the lesion in the present case, the possibility that this could represent either a high-grade mucoepidermoid carcinoma or adenoid cystic carcinoma of minor salivary gland origin was considered.\nInfections and inflammatory lesions of perioral tissues may also present as a swelling in the buccal mucosa. Acute infections, such as Ludwig angina or cellulitis, which may derive from an odontogenic focus of infection, osteomyelitis, were not considered in the differential diagnosis because of the chronic nature of the condition.[] In addition, fever, malaise, and pain would normally accompany the clinical presentation of an acute infection, and tenderness to palpation and fixed or fluctuant overlying skin would be apparent in the clinical examination. In addition, an intramasseteric abscess was unlikely, because there was no redness of skin or oral mucosa over the mass and the patient had no associated symptoms of fever or pain.\nMasseteric hypertrophy is a benign condition characterized by an enlargement of the bilateral masseter muscle that appears as a homogenous enlargement on CT. In the present case, CT showed the lesion as a unilateral mass.\nMucoceles of the minor salivary glands are rarely >1.5 cm in diameter and are always superficial. Mucoceles found in deeper areas are usually larger. Mucoceles can cause a convex swelling, depending on the size and location, as well as difficulties in speaking or chewing.[] Mucoceles can appear at any site of the oral mucosa containing salivary glands. The two types of mucoceles are more commonly found at different sites. Extravasation mucoceles appear frequently on the lower lip, whereas retention mucoceles appear at any other location of the oral cavity. The lower lip is the most frequent site for a mucocele, as it is the most probable place for a trauma, especially at the premolar level. A study of 312 patients showed 230 lesions on the lower lip (73.7%), with the tongue as the second most common location (15.4%).[] These locations are followed by the buccal mucosa and palate and are rarely found in the retromolar region and posterior dorsal area of the tongue. Mucocele of buccal mucosa is a superficial lesion and, in the present case, the lump was deep in origin.\nHemangiomas frequently appear as reddish-blue lesions. Hemangiomas of the head and neck occur commonly in infancy and childhood, especially in girls. Hemangiomas in the masseter muscle are relatively rare, and approximately 90% of these lesions develop before the age of 30 years. However, this group of lesions was excluded from our differential diagnosis because the lesion did not blanch with pressure and needle aspiration was negative.\nLipomas are slow-growing, nearly always benign, adipose tumors that are most often found in the subcutaneous tissues.[] These tumors may also be found in deeper tissues such as the intermuscular septa, the abdominal organs, the oral cavity, the internal auditory canal, the cerebellopontine angle, and the thorax.[] Some lipomas are believed to have developed following blunt trauma. Lipomas usually present as non-painful, round, mobile masses, with a characteristic soft, doughy feel. The overlying skin appears normal. Lipomas can usually be correctly diagnosed by their clinical appearance alone. In the present case, the mass was mobile, non-tender, and rubbery in consistency, but non-slippery. Lipoma was also considered to be one of the differential diagnosis.\nNeedle aspiration yielded neither blood nor any other fluid. An incisional biopsy was performed under local anesthesia, during which, profuse bleeding was noted. The patient did not have a history of bleeding disorders and was not taking anticoagulant or other medication that could interfere with coagulation. Histopathological examination of the specimen revealed cystic spaces with papillary projections, fibrocollagenous tissue, and skeletal muscles, which was not conclusive of any typical pathology. An excisional biopsy was performed under local anesthesia. After clinical and radiological diagnosis, the patient was planned under conscious sedation. Intra-oral incision or parallel to occlusal plane was given. Submucosally lesion was identified and dissected bluntly from rest of the tissue, particularly in the region of masseter muscle. Whole of the lesion was removed and, after achieving hemostasis, the wound was closed primarily. The excised tissue was submitted for histopathological evaluation []. The lesion appeared macroscopically as an enucleated soft tissue lesion without evidence of bony involvement. Histopathological examination revealed increased vascularity. Endothelial cells arranged in multiple papillary-like projections within the major lumen. Dilated vascular space lined by plump endothelial cells with multiple anastomosing channels were seen. Papillary projections were proliferating into the lumen of the major vessel and surrounded by muscles of the vessel wall. No increased mitotic activity was evident [Figures and ]. Since there was no dysplasia in the endothelial lining, the possibility of angiosarcoma histologically was also ruled out. Attempt of thrombus formation was evident in the lumen of many vessels.\nImmunohistochemical staining was performed using an avidin-biotin method. Immunoreactivity for factor VIII was seen positive in the papillary structure of the cystic lining [].\nThe final diagnosis of Masson's hemangioma was made after extensively reviewing the slides and also after reviewing the archival literature. The patient was followed for several months and was found to be disease-free, without any evidence of local recurrence. |
A 64-year-old man sustained right thigh and knee injuries when he had a road traffic accident in 1982. Right knee operation was performed to fix the proximal tibial fracture. He was then put on skeletal traction in a hospital for a few months and recovered gradually. Afterwards, he noticed right thigh deformity and right lower limb shortening. He was otherwise asymptomatic and resumed his duty as a manual worker.\nHe had his first orthopaedic consultation in 2009 and complained of gradual onset of right knee pain for 4 years. He could manage the knee pain with soft knee brace and analgesic initially. In 2012, his right knee pain worsened and he could walk unaided for 20 minutes only. He needed banister assistance when going upstairs and downstairs.\nPhysical examination showed mild right genu varum of about 10 degrees. There were one long medial parapatellar scar and one short anterolateral proximal tibial scar. Medial joint line was tender. Right knee active range of motion was from zero to 100 degrees. Posterior sagging, anterior and posterior drawer tests, and varus and valgus stress tests were all negative. Both tibial segments were equally long. His right femur was one centimeter shorter at the level below the greater trochanter. Palpable deformity was noted at the anterolateral aspect of proximal third of his right femur. There was no flexion contracture of the right hip. Right hip could be flexed to 60 degrees. Right hip abduction range was 20 degrees which was about half of the left hip abduction. Right femoral neck anteversion was about 20 degrees which was similar to the left hip.\nStanding scanogram of right lower limb () showed right femoral subtrochanteric fracture malunion, old tibial plateau, proximal fibular shaft fracture, four metal staples in the medial tibial plateau, and tricompartmental osteoarthritis of the right knee. When the anatomical axes of proximal and distal femoral segments were drawn on the anteroposterior (AP) () and lateral radiographs () of the right whole femur, the center of rotation of angulation (CORA) was inside the bone and corresponded to the obvious apex of angulation. This implied that neither a translation deformity nor multiapical angular deformity was present. Computer tomography from right hip to right knee () was performed to see any rotational deformity.\nWhen planning the distal femoral bone cut by drawing perpendicular line to the mechanical axis of right femur in AP and lateral radiographs, the origin of lateral collateral ligament was likely jeopardized and the anterior cortex of distal femur had to be significantly notched to be put in the femoral component. Therefore, intra-articular correction of this extra-articular femoral deformity and osteoarthritis of knee would not be successful. Femoral osteotomy and TKA would be necessary. One-stage operation was performed in August 2012 in order to achieve good relief of knee pain and satisfactory lower limb alignment. The apex of deformity was at the subtrochanteric region of the right femur and long gamma 3 nail (Stryker) was chosen to fix the osteotomy at this CORA to allow immediate postoperative full weight-bearing walking. Posterior stabilized TKA (Triathlon, Stryker) was done using Stryker's OrthoMap Articular Surface Mounted (ASM) Knee Navigation because conventional intramedullary guide would not be possible.\nHe was last followed up in the clinic in April 2014. He had no pain at his right thigh and knee. He could walk unaided and unlimited. His active right knee range was from zero to 110 degrees (). The function score was 80 and his right knee score was 97. Standing scanogram () showed healed femoral osteotomy and normal mechanical axis of right lower limb. |
A 44-year-old male with history of a biopsy proven spinal osteochondroma (Figure ) presented with a one-year history of left hip pain and a slowly enlarging left hip mass. He reported an indolent onset of left hip discomfort with progressive loss of range of motion over the year. He walked unassisted and took no medication for pain at the time of presentation. His pain was localized primarily to the left groin, and was worst with flexion/extension of the left hip. He reported occasional left anterior thigh burning pain that is worst with prolonged sitting. He denied radiation of pain down past the calf or weakness of the leg or foot. He presented with a firm prominence along the lateral groin. His physical examination showed a palpable, irregular fullness in the left groin that was minimally tender to palpation. Hip flexion was to 80° but limited by pain. He had hip extension to the neutral position. There was no inguinal adenopathy. His radiographs of the left hip (Figures and 3) showed extensive soft tissue calcifications surrounding the hip joint associated with erosion of the femoral neck and mild left hip degenerative changes. The radiographic differential diagnoses included synovial chondromatosis and synovial chondrosarcoma.\nHe subsequently underwent a CT scan, which demonstrated extensive calcifications adjacent to the left hip with asymmetric soft tissue enlargement and mild degenerative changes of the hip joint (Figure ). The masses were mostly anterior and medial, and the conglomerate measurement was approximately 13 x 7 cm without apparent extension into the pelvis. The differential diagnosis was unchanged, so the patient underwent CT-guided biopsy. He then underwent MRI using a 3T Skyra MRI (Siemens AG, München, Germany) for further evaluation. He underwent open subtotal debulking of his disease because his disease was felt to be too bulky and not amenable to arthroscopy. Both the percutaneous biopsy and final histologic diagnosis from the resected specimens were thought to be most consistent with synovial osteochondromatosis, with no evidence of atypia or cellularity.\nApproximately one year later, his residual disease progressed and resulted in limiting his range of motion. He underwent a left total hip arthroplasty via a posterior approach with simultaneous debulking of disease. The final histologic diagnosis from the resected femur and intra-articular bodies was synovial osteochondromatosis with erosive change in the femoral head/neck. Approximately two months postoperatively, he developed new foci of calcification around the hip joint on his postoperative radiographic studies (Figure ), consistent with recurrent disease. Worsening symptoms prompted a repeat CT scan six months later. The CT scan demonstrated extensive intra-articular disease extending into the iliopsoas bursa (Figure ) and around total hip arthroplasty as well as a new soft tissue nodule with foci of calcification in the left gluteus maximus, consistent with an additional focus of disease (Figure ).\nThe patient underwent repeat extensive surgical debulking of his disease. The final histological diagnosis from the excised tumor was grade 1 chondrosarcoma arising from synovial chondromatosis. |
A 65-year-old male patient with no significant medical history presented to our hospital for a huge mass on the frontal area that was bleeding easily. The mass has been slowly growing for 22 years, became huge and affected the vision. The lesion became ulceronecrotic four months prior to presentation and enlarged rapidly after a trial of incision and drainage at another institution.\nPhysical examination showed a 17-cm dome shaped mass present on the forehead with central necrosis and bleeding ().\nNo evidence of regional lymph node reaction was found in the head and neck region and no additional lesions were noted on physical examination. The rest of examination was normal.\nComputed tomography scan showed a heterogeneous large mass containing multiple foci of calcifications and multiple concentric vessels which enhanced significantly. No intra-cerebral extension was seen. No lymphadenopathy was present. These findings were suggestive of either lymphangioma or cavernous hemangioma.\nUnder local anesthesia, the patient underwent incisional biopsy of the large ulcerating lesion. The histological diagnosis was consistent with proliferating trichilemmal cyst. The patient subsequently underwent, under general anesthesia, a complete resection of the tumor with 1cm margins from the necrotic skin. Absence of the anterior table of the left frontal sinus was noted after tumor excision and the defect was reconstructed with a corrugator muscle flap. Finally, multiple rotation flaps were used to close the defect ().\nDefinitive histopathological study came out as complete excision of PTT (17×12×6 cm) with no evidence of malignancy ().\nAfter 3 years, patient presented with recurrent left supraorbital mass measuring 3 cm ().\nThe mass was totally resected with 2 mm margins and sent directly for pathology as a frozen section due to the fear of malignancy. Pathology was consistent with proliferating trichilemmal cyst with no signs of malignancy and the margins were free of tumor. So, the defect was reconstructed with local flap. Definitive pathological result confirmed the complete excision of PTT with no signs of malignancy. To note that adjuvant treatment was not given.\nPatient was followed biannually for the first year and then annually for 15 years. At each visit, an extensive clinical exam was done and patient showed no signs of local recurrence or distant metastases with long term follow-up. |
A 32 year-old African-American female had previously presented to Children’s of Alabama at age 15 in Birmingham, Alabama with a cavitary pneumonia and was treated with clindamycin and cefuroxime intravenously for 10 days. Due to the severity of the infection, an immunologic workup was performed, and although the patient was mildly lymphopenic (absolute lymphocyte count 900 μl−1), the lymphocyte subset distributions were normal. Immunoglobulin levels were all below the normal ranges for age, and she was diagnosed with CVID. The patient received 2 monthly doses of intravenous IgG replacement therapy (IVIG) and recovered from her pneumonia but was subsequently lost to follow-up for 16 years.\nAt the time of her diagnosis with septic arthritis during hospital admission at 32 years of age, the patient had a history of weight loss, malaise, nausea and vomiting, diarrhoea and swelling in left knee and right wrist dating from a knee injury as detailed below. During the intervening years she had suffered chronic ear infections, chronic sinusitis, and axillary skin abscesses and had experienced several dental abscesses requiring tooth extraction and antibiotics over the preceding 2 years. She also suffered from occasional episodes of oral candidiasis/thrush. Eighteen months prior to admission, the patient had suffered trauma to the left knee and 9 months later received treatment by an orthopedist that included arthroscopy and arthrocentesis that yielded no fluid for diagnostic testing. At that time she was also beginning to have intermittent left ankle swelling and low-grade fevers. About a year after her knee injury she began experiencing pain and swelling intermittently in her left thumb and wrist. Two months prior to admission the pain and swelling in the right wrist increased significantly with the appearance of nodules around the joint. At time of admission, radiographs revealed severe destructive changes in the wrist and knee, as well as left ankle (). Initial aspiration of joint fluid from the knee had been nondiagnostic. On admission joint fluid obtained from the right wrist was grossly purulent (216 120 WBC μl−1, 77 % neutrophils, and 186 000 RBC μl−1). She was treated empirically with intravenous vancomycin and ceftriaxone, and doxycycline was added on hospital day 13 due to continued negative microbiologic test results and continued fevers and joint pain. She continued to run intermittent fevers as high as 103 °F, and aspiration of left knee fluid on day six of admission revealed 47 500 WBC μl−1 but routine microbial cultures and Gram-stain were negative from both joint aspirations. Laboratory evaluation revealed profound agammaglobulinemia (IgM <20 mg dl−1, range 43–215; IgA <6, range 59–371; IgG <200, range 678–1425; IgE <1 IU ml−1, range 6–216), and there was B cell lymphopenia noted on flow cytometric analysis of patient’s peripheral blood lymphocytes (2 %, range 6–19 %; absolute numbers 27 µl−1, range 100–500). Other lymphocyte populations were normal in distribution and number, and T cell antigen recall responses were also normal (Supplementary Table).\nAs multiple bacterial cultures remained negative for 2 weeks, patient samples were submitted for PCR of 16S rRNA domain IV-V followed by next generation sequencing (NGS) of PCR product (University of Washington, Molecular Diagnosis Microbiology Section) in an attempt to identify the presumptive microbial infection. Additionally, samples were submitted for culture to the UAB Diagnostic Mycoplasma Laboratory. Culture of joint fluid in SP4 broth and agar was performed, and large colonies with a fried-egg shape typical of mycoplasma species were observed on the agar plate after 16 days of incubation at 37 °C. Then 16S rRNA NGS analysis revealed the organism to be . Diagnosis was further confirmed on the UAB clinical isolate by PCR, amplifying the full length of the 16S RNA gene followed by Sanger sequencing.\nAntimicrobial susceptibility testing (AST) was carried out on the patient’s isolate in the UAB Diagnostic Mycoplasma Laboratory to determine the minimum inhibitory concentration (MIC) of different antimicrobials by broth microdilution methods standardized for other human mycoplasma species []. The following MICs were obtained: levofloxacin (4 µg ml−1), doxycycline (0.016 µg ml−1), clindamycin (0.063 µg ml−1), and erythromycin (2 µg ml−1). Using MIC interpretive breakpoints for , the isolate would be considered susceptible to doxycycline, and clindamycin and resistant to levofloxacin. There are no MIC breakpoints for erythromycin for since this mycoplasma is innately resistant to 14 and 15 membered macrolide antibiotics, but the erythromycin MIC of 2 µg ml−1 would be considered resistant using the interpretive criteria for []. On hospital day 20 her antibiotics were changed to oral moxifloxacin, 400 mg daily. At discharge after a total of 40 days hospitalization, the patient was given an extended course of oral moxifloxacin 400 mg daily over 4 months together with monthly IVIG (25 grams, 0.5 gm kg−1), infusions of which had been given three times during her hospitalization. The patient developed recurrent colitis over the course of treatment (diagnosed on hospital day 33 using a DNA molecular assay which detects the presence of the pathogenicity locus (PaLoc)), but her joint inflammation improved and the infection cleared leaving her with significant disability in the affected joints.\nOver the last 6 years since she has been able to get uninterrupted monthly treatment of IVIG, she has not had any other severe infections. She has recovered some function of her wrist and was able to get a left knee total replacement 2 years ago. Recent studies showed she has nearly zero B cells in her peripheral blood, and a primary immunodeficiency panel of 207 genes including 32 associated with CVID did not find any significant variants. She has not yet had exome sequencing. |
A 60-year-old man, referred to the orthopaedic outpatients department in July 1997, presented with a painful right hip of several months' duration. Examination revealed a grossly reduced range of movement in the right hip. His previous medical history included gout, controlled by 300 mg allopurinol. Radiographs of the pelvis revealed severe osteoarthritic changes of the right hip and a normal left hip.\nHe was kept under review for one year, during which his pain increased and in August 1998 he underwent a right Exeter cemented total hip replacement via a posterior approach. Immediate postoperative recovery until hospital discharge was uneventful, with radiographs of the hip showing no problems. At a routine 6-week follow-up it was noted that although pain free he was making slow progress. There was no leg length discrepancy and the range of movement of the right hip was good, with 100° of flexion, 30° of abduction, 15° of internal rotation and 20° of external rotation. However, the muscular strength was reduced in comparison with his left hip and he had an unsteady gait. He was referred to a physiotherapist and made good progress with improvement in hip strength. Follow-up at one year revealed him to be making excellent progress, and at two years post-operatively he was discharged, entirely symptom free and very happy with his surgical result.\nThe patient did not see his general practitioner about hip pain until he next presented in November 2006 complaining of pain in the left groin. An anterioposterior radiograph of the pelvis revealed degenerative changes in the left hip and a dislocated right total hip replacement (Figure ). The dislocated femoral component had formed a neoacetabulum within the ilium, in which it was freely articulating (Figures and ). He remained pain free on this side, had 5 cm of true leg length shortening, with a good range of movement, and was very pleased with his hip replacement. He was later placed on the waiting list for a left total hip replacement. |
A 73-year-old male visited a previous doctor with a chief complaint of progressive leg edema and dyspnea on exertion 1 year prior. The patient had a history of spasmodic angina pectoris, midbrain infarction, and chronic renal failure. Computed tomography (CT) showed multiple hepatic cysts compressing the right lung, right side of the heart, and IVC. He underwent percutaneous aspiration for the largest cyst in segment eight of the liver; however, the leg edema did not improve, and the cyst flared up within a month. He was referred to our hospital for further investigation and treatment.\nBlood tests at the time of our visit revealed no significant findings other than mildly decreased renal function (Table ). CT showed multiple liver cysts, with the largest cyst in segment eight measuring 13 cm in diameter and compressing the right side of the heart and the right thoracic cavity (Fig. a). The IVC was bilaterally compressed by multiple liver cysts in the caudal lobe (Fig. b), implying blood flow obstruction. The cysts were also present in the hilar region of the liver, compressing the portal bifurcation and causing stenosis of the right branch of the portal vein (Figs. c, a, b). Angiography showed that the IVC was markedly stenotic, with an 11-mmHg pressure gradient on the central and peripheral sides of the IVC stenosis (Fig. a). Furthermore, superior mesenteric artery angiography showed that the portal vein enhanced the antegrade flow; however, left gastric vein–left renal vein shunt developed (Fig. b). No intestinal edema was found on imaging studies. Considering these imaging findings, pre- or post-hepatic mild portal hypertension (PH) may have occurred with the IVC compression.\nBased on the above test results, we concluded that the IVC stenosis due to the liver cysts was the cause of the leg edema and dyspnea on exertion and hence decided to treat the liver cysts with fenestration. To confirm the hemodynamic status of the IVC, intraoperative angiography with CT during hepatic arteriography, CT during arterial portography, and angiography were performed in the hybrid operating room to allow IVC and PV angiography before and after liver cyst fenestration. Moreover, portal vein pressure (PVP) and central vein pressure (CVP) were continuously monitored intraoperatively as indices of hemodynamics in relation to the release of IVC stenosis. Furthermore, if the above methods did not improve the IVC stenosis, we were prepared for the dissection of the hepatic caudate lobe from the IVC and the placement of a metallic stent in the vessel.\nThe surgery was performed in a supine position under general anesthesia. To monitor PV pressure, a 15-Fr intravascular catheter (ANTHRON® P-U CATHETER; TORAY Medical, Japan) was inserted and fixed peripherally in the jejunum vein. CVP was defined as the pressure measured at the peripheral side of the IVC stenosis and measured by inserting a sensor through the right femoral vein. After mobilization of the left lobe of the liver, we fenestrated the largest lesion in segment eight of the liver. The CVP did not change before and after the fenestration of this lesion. After mobilization of the right lobe, the lesions on both sides of the IVC were identified under ultrasonographic guidance, and the cysts were fenestrated on the left and right sides of the IVC. Before and after fenestration, the CVP decreased from 5 to 1 mmHg, and the PVP decreased from 22 to 17 mmHg. The intraoperative changes in CVP and PVP are summarized in Table . Angiography findings and improvement of CVP confirmed that IVC stenosis was released after fenestration (Fig. a). Intraoperative CT findings also confirmed that the cysts that were causing the stenosis had been fenestrated (Fig. b-d). The cyst fundus was cauterized using a bipolar device, and a leak test was performed from the gallbladder duct to confirm that there was no bile leakage, finally completing the surgery. The operation time was 334 min, and the amount of bleeding during the surgery was 767 mL. The patient was discharged on the 10th postoperative day. His weight markedly decreased from 63.0 kg just before the surgery to 58.8 kg at the time of discharge. Six months after the surgery, the CT scan showed no obvious recurrence of the cysts (Fig. a-c). No leg edema recurrence or weight gain was observed. |
A 36-year-old male patient with a 6-year history of chronic kidney disease of unknown etiology was brought to the emergency department due to neurological impairment that started during the previous hour characterized by altered mental status while he was walking down the street. On examination he was stuporous, with poor response to external stimuli. The patient was admitted to the hospital and vital signs and capillary glucose were determined. Glucose levels were 20 mg/dl and increased to 42 mg/dl after a 50 ml infusion of 50% dextrose. During his stay his mental status recovered after glucose levels were returned to normal parameters, requiring high doses of intravenous glucose. After his stabilization he was transferred to the internal medicine department. We confirmed that the patient was not taking medications that would cause hypoglycemia. On physical examination he was somnolent and pale, with slight oedema in both legs. A new episode of symptomatic hypoglycemia was observed while he was receiving an infusion of 20% dextrose. The infusion rate at that moment was 10.416 ml/hr. The infusion was not being weaned off or was an acute disruption. It caught our attention that although the patient was on a 20% dextrose infusion, he continued with hypoglycemia. Blood samples were taken and the results were abnormal ().\nAlthough hypoglycemia could be explained by chronic kidney disease, the diagnosis of insulinoma was considered, so a computed axial tomography with double contrast was taken but unfortunately there were no abnormal findings. During the patient's hospital stay he received several treatments that failed to achieve proper glucose control. We used ascending doses of diazoxide up to 600 mg/day with a poor response. In order to reduce episodes of hypoglycemia, we started with low doses of octreotide and found a good response that created tolerance quickly, so we decided to increase the dose by 0.1 mcg/kg/h always observing the same phenomenon. We decided to suspend this treatment when we reached 0.4. mcg/kg/h. Finally, we decided to maintain a continuous infusion of 50% dextrose with which we achieved serum glucose levels between 120 and 160 mg/dl. Magnetic resonance imaging and an endoscopic ultrasonography were performed but no conclusive data on any structural pancreatic disorder were obtained. In order to locate the tumor, we performed selective intra-arterial pancreatic stimulation with hepatic venous sampling at 0, 20, 40, and 60 seconds. High insulin levels were obtained after a selective injection of 0.025mEq/Kg calcium gluconate in the proximal splenic and gastroduodenal arteries () (). With these results, the patient was scheduled for surgery. During the procedure, bimanual palpation of the pancreas was performed, as well as a pancreatic ultrasound in which no tumor could be identified. The body and tail of the pancreas were resected. For two days the patient had an adequate glycemic control but after a couple of days, he presented with hypoglycemia again. The macroscopic pathology report did not show any tumor compatible with insulinoma; however, on microscopic examination pancreatic islets with elongated cells and clear cytoplasm compatible with nesidioblastosis were seen (). Unfortunately, during his stay at the intensive care unit, the patient developed late-onset hospital-acquired pneumonia and, in spite of treatment, he developed sepsis followed by septic shock which ultimately caused his death. |
A 74-year-old female presented to a local hospital with severe left lower extremity pain and oral thrush. In the emergency department, laboratory data revealed anemia, thrombocytopenia of 10,000 platelets/microliter, and elevated white blood count of 235,500 leukocytes/microliter with 97% blasts concerning for leukemia. Bone marrow biopsy confirmed a new diagnosis of acute myelogenous leukemia (AML). She was subsequently transferred to a major tertiary referral center for management.\nOn further evaluation, she continued to have worsening left lower extremity pain with physical exam notable for cyanosis of all five toes, erythema to the midshin, and decreased sensation to touch but intact posterior tibial and dorsalis pedis pulses detected using a Doppler device. Bilateral lower extremity ultrasound revealed a left popliteal artery occlusion at the level of the popliteal fossa and acute bilateral deep vein thrombosis (DVTs). The patient was started on a heparin infusion for acute DVT and given nonopioid adjuvants (scheduled oral acetaminophen, scheduled oral gabapentin, lidocaine patch, and ketamine injection as needed) as well as oral and intravenous opioids to control her pain. Despite escalation of pain regimen up to 200 oral morphine equivalents (OME), she continued to experience 10/10 pain at rest and was unable to sleep at night. Palliative care service was consulted for further management.\ntGiven her worsening pain, a regional anesthetic technique was considered. Due to her critical thrombocytopenia and continuous heparin infusion, she was not a candidate for neuraxial anesthesia. Consideration was given to the patient's multiple risk factors for peripheral nerve block including bleeding, infection due to severe immunocompromised state, and potential for masking a developing compartment syndrome or a worsening of her CLI. After an extensive discussion with the patient and her family regarding the high risk nature of an invasive procedure, the decision was made to proceed with a trial of continuous popliteal nerve catheter for the patient's refractory pain. A left popliteal nerve catheter was placed by the anesthesia acute pain service under ultrasound guidance using classic landmarks with an initial bolus dose of 30 mL of 0.25% bupivacaine followed by an infusion of 0.1% bupivacaine at 10 mL per hour. The patient reported near immediate pain relief with pain score decreasing to 0/10. She was able to sleep through the night, transitioned off intravenous narcotics, and only required minimal oral narcotics for breakthrough pain (less than 60 mg OME per day). In , we display average patient-reported pain scores for each hospital day, utilizing a numeric pain scale ranging from 0 (no pain at all) to 10 (worst pain possible). In , we display the total 24-hour OME consumed by the patient for each hospital day. We manually converted the reported opioid dosage for each hospital day to its respective OME using the Mayo Opioid Converter Online Tool (; accessed June 2019).\nSubsequently, the patient had a complicated hospital course. A follow-up CT scan showed increased clot burden to the level of the aorta, and she underwent an attempt at surgical revascularization of the affected limb with thromboembolectomy (). For the surgery, a left femoral artery cutdown was performed and the thrombus was found within the distal aorta, left common iliac artery, left external iliac artery, profunda femoris, and left popliteal artery. A transverse left common femoral arteriotomy was performed with retrograde thromboembolectomy of the left common iliac artery and the external iliac artery. A second transverse arteriotomy for anterograde thromboembolectomy from the left popliteal artery was also performed. A completion angiogram demonstrated patency of the aforementioned occluded vessels.\nUnfortunately, the left popliteal artery acutely rethrombosed in the postanesthesia care unit, resulting in an emergent return to the operating room for a second attempt at revascularization. During this surgery, there was thrombosis in the left distal superficial femoral artery and the left proximal popliteal artery. Balloon angioplasty and stenting of the left distal superficial femoral artery and left proximal popliteal artery were performed, and a completion angiography demonstrated patency and inline flow down to the left posterior tibial artery. Although the second surgery initially appeared to be successful, the patient again experienced rethrombosis of the popliteal artery the next morning. Following discussion with the patient, her family, and the multidisciplinary care team, further surgical intervention was felt to be unlikely to provide significant benefit and not to be in accordance with her goals of care. The peripheral nerve catheter had been removed at the time of the first surgery, and the patient's severe ischemic leg pain returned. A new ultrasound-guided left popliteal nerve catheter was placed with near immediate pain relief. She was then transitioned to comfort care with the catheter in place and ultimately died five days later, likely due to complications of AML. Consent was obtained from the patient's family to publish this report. |
We report a case of a 75 year old woman addressed to Allergology Department of our hospital in January 2014 for recurrent episodes of angioedema since the age of 66, with progressively increased severity and frequency. It was first considered to be induced by treatment with angiotensin-converting-enzyme inhibitors (ACEI) for mild hypertension, but she continued to have angioedema attacks for the next 6 years after discontinuation of ACE, with progressive aggravation during the last year. The previous multiple evaluations by many specialists in other hospitals did not succeed to give a clear diagnosis and treatment.\nThe patient had no relevant medical history and took no medication, except ACEI that was stopped some months after angioedema onset. No relation with possible allergic stimuli could be identified and she had no clinical manifestations between attacks. Angioedema was painful, not accompanied by urticaria or abdominal symptoms, located variably to neck, arms or buttocks, without facial involvement during 6 years. The attacks occurred at weeks or months intervals and usually lasted between 48 and 72 h, irrespective of corticosteroids and antihistamines treatment usually administered. The frequency of attacks had progressively increased from one at 2–3 months intervals to almost weekly during the last year before presentation. The last angioedema attack, determining emergency hospitalization in September 2014, was more severe and prolonged, accompanied for the first time by laryngeal edema and respiratory symptoms. The extended medical evaluation, including complete blood tests for inflammation, allergy, autoimmunity and cancer, were all negative (Table ). Full body CT scan and bone marrow examination were normal. No criteria for lymphoproliferative, mieloproliferative or autoimmune diseases could be found. Measurement of C1 inhibitor (C1INH) in plasma showed significantly decreased level at all measurements, with low activity ranging from 58 to 4% and constantly low C4 (Fig. ). Complement fraction C1q plasma level was measured twice and had normal value. Genetic tests were not performed, given the patient advanced age and lack of family history of angioedema, which are against HAE. A spontaneous mutation in SERPING 1 gene is noticed in up to 25% cases of HAE without family history, but we considered this probability very low in our case, due to late onset of angioedema.\nTreatment of angioedema attacks before hospitalization consisted of antihistamines and systemic corticosteroids, which proved to be ineffective. Since no pathogenic therapy with C1INH concentrate, antagonists of bradikinin receptors (icatibant) or selective inhibitor of plasma kallikrein (ecallantide) was available in 2014, we first initiated daily prophylactic treatment with tranexamic acid for 3 months, with no amelioration. During the more severe attack with laryngeal edema, in September 2014, we have switched to attenuated androgen danazol, given 400 mg the initial dose, reduced to 200 mg daily after 1 week and then to 100 mg daily. The clinical evolution was very good, no angioedema attack occurred since the introduction of danazol. C1INH and C4 plasma levels increased after 2 weeks of treatment and became normal after 1 month. After some months, the patient decided herself to discontinue danazol for short time, in order to check effects and to taper the minimal dose. Serum C1-INH and C4 plasma levels were measured after 2 and 4 weeks and showed significant lower levels, but no angioedema attack occurred during this period of time. She therefore restarted danazol prophylactic therapy at a minimum of 50 mg dose daily, ongoing after 3 years. The patient was closely monitored during the next 3 years, with complete clinical and laboratory control twice a year. We took into consideration the possible side effects of danazol, mainly dislipidemia, haematological and liver malignancies and any other complications or concomitant diseases. The clinical evaluation was very good, except two episodes of pulmonary cysts infection, remitted with broad spectrum antibiotherapy, which could not be related to danazol treatment. The clinical outcome of angioedema after 3 years of danazol treatment is very good, with no attacks or other related symptoms. No clinical or laboratory sign of any disease that could induce C1 INH deficiency occurred. No relevant side effects of danazol were noticed. The patient has an improved quality of life due to therapeutic compliance and general management plan. |
A 47-year-old female was found to have two masses in the right breast following a screening mammogram, as well as subsequent breast ultrasound and MRI. Ultrasound-guided core biopsies showed a benign fibroadenoma, as well as a fibroadenoma with associated atypical lobular hyperplasia, apocrine metaplasia, and microcalcifications. Excisional breast biopsy showed an ER-positive fibroadenoma with involvement by foci of ductal carcinoma in situ within less than one millimeter from surgical margins and atypical lobular hyperplasia (ALH). The E-cadherin immunostain demonstrated loss of membranous expression in the ALH, while stains for p63 and SMMHC demonstrated retained myoepithelial cells throughout the lesion. Following a discussion regarding treatment options, the patient underwent a right partial mastectomy and axillary sentinel lymph node biopsy, with adjuvant radiation of the right breast. Pathology from this surgery showed no residual tumor, and no tumor was identified in the two sentinel lymph nodes.\nAbout one month following the completion of adjuvant radiation of the right breast, the patient underwent a thyroidectomy and pathology showed multifocal papillary thyroid cancer. Thyroid ultrasound prior to the thyroidectomy showed enlargement of both lobes, with heterogeneous echogenicity, and was suspicious for small calcification of the lower pole of the right lobe. This was followed by radioactive iodine therapy. About two and a half weeks later, she noticed edema, mild pain, and erythema of the right breast. The patient had previously experienced significant right breast skin changes as a result of her adjuvant breast radiation, including a dermatitis and skin hyperpigmentation; however, the radiation dermatitis had healed prior to the radioactive iodine therapy. We suspected the patient's new symptoms were due to radiation recall dermatitis. However, we decided to evaluate the patient for possible inflammatory breast carcinoma, and incisional biopsies of the right breast were performed. Pathology revealed moderate chronic perivasculitis with edema of the dermis and no evidence of malignancy (Figures and ). The patient was closely monitored, and her symptoms were noted to have improved on subsequent evaluation. |
A 73-year-old Caucasian man with a history of smoking and alcohol abuse presented to the Emergency Department (ED) complaining of the abrupt onset of diplopia and mild slurred speech upon awakening at 8 a.m. He initially thought the symptoms were due to fatigue. Approximately 4 hours after his initial symptoms, he noted minimal right-sided weakness and came to the ED. On admission, the patient was awake and alert but was slightly confused and not oriented to place. Physical examination revealed mild dysarthria, a partial left 3rd cranial nerve palsy with mild abduction of the left eye and a 4 mm pupil that was minimally reactive, with a briskly reactive 2.5 mm pupil on the right. He had a mild right central 7th nerve palsy as well as mild right upper extremity weakness with a pronator drift. Sensory exam was intact to pin prick and touch. Computed tomography (CT) scanning of the head was obtained urgently and showed no hemorrhage or acute ischemic changes. An urgent computed tomography angiogram (CTA) was performed that demonstrated a tortuous tip of the basilar artery with "possible aneurysm vs. clot". A second incidental 5 mm aneurysm was seen in the Middle Cerebral Artery. As the patient was clinically stable, and it was unclear if there was a clot in an underlying basilar aneurysm, he was admitted to the neurological intensive care unit (ICU) for observation. He was placed on aspirin (325 mg) at that time.\nOver the next 8 hours, the patient became progressively obtunded and developed a 2/5 quadriparesis, a complete left 3rd cranial nerve palsy, and complete right ophthalmoplegia. At approximately 14 hours after the onset of symptoms, he was urgently intubated for airway control, decreasing mental status and loss of gag reflex. He was brought to the endovascular suite for angiography and possible IA tPA for a progressive basilar thrombosis despite the known aneurysm. A near occlusion was found at the basilar tip after the origins of the superior cerebellar arteries, which was associated with a large, complex calcified basilar tip aneurysm, and which was likely a nidus for clot formation. From a position just proximal to the occlusion, 10 mg of tPA was infused in a pulsatile fashion over 30 minutes. Angiography showed improved flow through the distal basilar artery, with no change in the appearance of the calcified dilation of the basilar tip or extravasation of dye (Figure ). The patient was sent to the ICU. Diffusion magnetic resonance imaging (MRI) the following morning showed multiple tiny diffusion bright lesions in the cerebellum, thalamus and midbrain. This was consistent with an aborted basilar artery occlusion with evidence of ischemia throughout the entire basilar artery vascular territory (Figure ). On examination the following afternoon, the patient had near complete resolution of symptoms, and was discharged to rehabilitation 9 days later with a mild right 6th cranial nerve palsy. He was discharged on Warfarin with an International Normalized Ratio (INR) of 2.0 as the ischemic changes seen on MRI were minimal, suggesting a low risk of hemorrhagic conversion. No dye extravasation was seen from the aneurysm. It was felt that he was at high risk for recurrent thrombosis. The plan was for non-emergency endovascular coiling of the basilar tip aneurysm 2 months after discharge; however, the patient was lost to follow-up and did not return to the vascular clinic. |
The patient in question was a 90-year-old female who suffered with a destructive form of erosive rheumatoid arthritis. At the time of her presentation she was living alone in a 3-bedroom flat with a substantial package of care and had become in recent months almost wheelchair bound.\nAt the age of 76, she had a left Neer shoulder hemiarthroplasty for avascular necrosis of her humeral head following a dislocation. For 10 years she had satisfactory level of function and was pain-free; however, at the age of 86, she was experiencing pain in her shoulder as was seen in the out-patient clinic. Clinically, she had severe rotator cuff dysfunction with anterior and superior displacement of the prosthesis, but no intervention was recommended at this time.\nThe patient then represented at the age of 89 with a 6-week history of her left shoulder becoming increasingly painful and stiff and with a small discharging wound over the posterior aspect of her left shoulder.\nOn presentation, the patient was apyrexial with normal observations and felt systemically well. There was a 1 cm sinus over the posterior aspect of her shoulder in the supra-spinatus fossa just medial to the acromium process. The wound was discharging moderate amounts of serous fluid which was causing the patient some distress and also skin irritation.\nShe was seen by her GP who organised some investigations including inflammatory markers and X-rays (see ). When compared to previous X-rays, a grossly abnormal left shoulder was seen with the dislocation of the humeral head. There was a loss of the lateral 3rd of the left clavicle with bony destruction of the clavicle and glenoid. There was overlying soft tissue opacification containing gas, and the overall appearances were suggestive of aggressive osteomyelitis.\nHer blood tests that were taken at the time were normal, WCC 6.2, ESR 25, and an CRP of 2. Swabs and cultures were taken at this initial presentation and came back with no growth.\nShe was then taken to theatre for exploration of the sinus, and large seroma was found; a bony prominence was seen and was identified as what remained of the clavicle which had been the cause of the perforation to the skin; this was trimmed back to prevent recurrence, and good soft tissue coverage was achieved. Tissue and fluid samples taken intraoperatively were cultured and later demonstrated no growth. |
A 57-year-old female presented with an abdominal swelling over the last 6 months, and pain during the last 2 months. She also had a prolapsed uterus, which was diagnosed 3 months ago. She had no co-morbidities. A history of radiation exposure or any previous surgery was absent. On examination, a large abdominopelvic lump was palpable, measuring about 25 cm in diameter. The lower border of the mass could not be felt. Routine investigations were within normal limits. The contrast-enhanced computed tomography (CECT) of the abdomen and pelvis scan showed a large abdominopelvic mass with homogeneous density and enhancement, predominantly on the right side, displacing the small bowel and urinary bladder to the left. The abdominal and pelvic CECT scan showed a large abdominopelvic mass with homogeneous density and enhancement after contrast injection, predominantly on the right side, displacing the small bowel and the urinary bladder to the left. The CECT also showed multiple small lesions in the abdomen, with a large right iliac mass abutting the right iliac vessels ( and ). There was no bowel or any other visceral infiltration. The imaging findings were consistent with the diagnosis of a retroperitoneal liposarcoma.\nOn exploratory laparotomy, there was minimal free fluid in the abdominal cavity. A multilobulated soft tissue tumor measuring approximately 20 × 15 cm was seen arising probably from the mesentery or from the bowel wall of the right colon. Another mass measuring 8 × 6 cm was seen in the right iliac fossa abutting the iliac vessels (). Multiple nodules of up to 3 cm were seen in the entire abdomen, and they appeared to be attached to the colon.\nThe patient underwent a right hemicolectomy with en bloc excision of the mass, excision of right iliac fossa mass with hysterectomy and pelvic floor repair. The postoperative period was uneventful. The patient is currently doing well (3 months post-surgery) and is on regular follow-up. The excised mass was sent for histopathology.\nThe right hemicolectomy specimen included part of the terminal ileum, the cecum, the appendix, the ascending colon, and the proximal part of the transverse colon. The respective lengths of the parts of bowel were 13.5 cm (terminal ileum), 5 cm (cecum), 8.5 cm (appendix) and 14 cm (rest of the colon). The same container contained the en bloc mass along with multiple separately lying nodules. The en bloc resected specimen showed multiple nodules and exophytic masses attached to and hanging from the colon. Most of the masses were found to hang by a tiny pedicle, simulating appendices epiploicae of variable size, while only a few appeared not to have any stalk. These nodules and masses ranged in size from 1 cm to 22.5 × 16.5 x 10 cm in its largest dimension. The cut surfaces of the smaller nodules were fatty and yellow with thin strands of fibrous septa dividing these nodules into fatty lobules. The thickness of the septa was greater in the larger nodules, and the even larger ones showed a grayish white and dominantly fibrous cut surface with only a few interspersed fat lobules. In places, the larger masses showed indentation/cleavage, and these areas appeared to be the zone of fusion of two or more adjacent exophytic masses, and the cut surfaces of such masses showed the same ().\nIt appeared to be the coalescence of multiple adjacent appendices. The largest nodules showed central myxoid areas and scattered calcific specks. All these masses appeared to arise from the appendices on gross morphology. In addition, the cecum showed a bulge measuring 2.4 × 0.8 × 0.6 cm, and on cutting open showed a mass with a fibrofatty cut surface protruding into the cecum. No mucosal pathology was noted.\nThe specimen was extensively sampled to demonstrate the pathology, and a spectrum of pathological changes was documented. The smallest nodules (both attached and freely floating) showed multiple lobules of mature adipocytes with thin fibrous septa. The larger the masses, the more the thickness, complexity, number of septa, and number of septal and atypical adipocytic cells and lipoblasts increased (- and ). The large nodules showed only a few lobules of mature fat and dominantly fibrocollagenous areas, giving rise to a sclerosing pattern (). The largest nodule showed well-differentiated morphology with central areas of myxoid degeneration and atypical spindle cells in a myxoid matrix ().\nThe septal cells were moderate to highly pleomorphic, singly scattered, spindle-shaped with hyperchromatic nuclei, and inconspicuous to occasional prominent nucleoli and wispy eosinophilic cytoplasm (). Many of these cells displayed bizarre morphology, and giant cells and floret-like cells were also noted (). The larger nodules also showed atypical adipocytes with hyperchromatic nuclei and many scattered univacuolated and multivacuolated lipoblasts (). Mitotic figures were occasional (2–4/10 high-power field); necrosis was absent. Immunohistochemistry for MDM2 () and S100 showed nuclear positivity in the atypical septal cells, lipoblasts, and many of the mature-looking adipocytes.\nCD34, Desmin, SMA, and βcatenin were negative in the tumor cells. The cecal bulge showed an endoluminal liposarcoma arising within the submucosa and stretching the overlying mucosa. The remaining ileum, ileocecal valve, and the colon did not reveal any mucosal pathology. The diagnosis of well-differentiated liposarcoma, adipocytic and sclerosing subtypes was rendered with the suspicion that the exophytic masses appeared to arise from the appendices epiploicae, which was consistent with the diagnosis of liposarcomatosis. The spectrum of changes was mostly corroborated by the size of the mass with the larger mass showing thicker septa, fewer number of adipocytes, and an abundance of atypical septal cells, atypical-looking adipocytes, and lipoblasts. Only few of the smaller nodules showed a morphological pattern similar to large nodules in terms of septal thickness and complexity. |
The patient is an 18-year-old African American male with a documented history of Prader-Willi syndrome with mild intellectual disability and type I diabetes mellitus (DM) who presented to the emergency department (ED) multiple times over the course of two months after attempting suicide via multiple methods.\nThe patient went shopping with his mother and became upset that he was not getting a high carbohydrate meal. He then became behaviorally dysregulated later on and refused to step out of the car. Later that evening, he refused to take his insulin. He was then found by his mother at nighttime somnolent and reported overdosing on his home insulin. This event led to him being brought to the hospital. Psychiatry was consulted to assess for continuous suicidal thoughts and guide disposition. The mother ended up enacting the durable power of attorney (DPOA) for the patient. The patient refused to take any psychotropic medications, and the team recommended that the patient would not benefit from acute inpatient psychiatric admission at this time; the patient was then discharged.\nThe patient's mother brought him to the ED for an assessment of violent aggressive behavior. The patient denied any aggressive episodes and was not able to recall any. He did admit to having a displacement reaction in which he used to hit his pillow when he got upset.\nAccording to the mother, the patient was living at a homeless shelter. Prior to that, he was living with his sister until she killed herself, and he was not able to live in that house as he kept experiencing flashbacks. The team once again did not see the benefit of admitting him to an acute inpatient psychiatric unit as he was not at risk of harming himself or others at the time of assessment. He was discharged back to the homeless shelter.\nThe patient was admitted to the ED, and psychiatry was consulted as the patient attempted to wrap a laptop cord around his neck and attempted to jump out of a moving vehicle. He was agitated in the ED requiring as-needed meds for agitation (i.e., haloperidol and lorazepam).\nThe patient told his treatment team that his mother triggered this episode but was unable to elaborate on it further. Due to this suicide attempt and continuous suicidal ideation, the patient was admitted to the acute inpatient psychiatric hospitalization involuntarily for safety.\nHe was started on aripiprazole 5 mg orally and was placed on 1 : 1 observation due to being a potential victim risk. He was given a diagnosis of unspecified depressive disorder, as he had been experiencing some depressive symptoms since age 12. During his stay at the inpatient unit, he did not endorse perceptual disturbances, depression, and anxiety, nor did he exhibit agitated behavior. He was then discharged to the homeless shelter.\nThe patient appeared his stated age, mildly obese with apparent gynecomastia. He exhibited intermittent eye contact. His speech was normal during conversation but was childish in tone with noticeable changes in pitch between phone call and conversation.\nThe patient has a history of multiple inpatient psychiatric hospitalizations in different facilities in the area. No formal diagnosis of depression, primary thought disorder, or bipolar disorder was given in the past. His primary symptoms have always been in relation to his behavioral dysregulation and compulsive/impulsive eating.\nHe was diagnosed with Prader-Willi syndrome at the age of 13 years. He did not receive therapeutic treatment (e.g., growth hormone); had sleep difficulties, hyperphagia, and developmental delays (as per the mother), and lack secondary characteristics.\nThere was no outpatient psychiatric follow-up. The patient refused to take psychotropic medications; however, he has tried guanfacine, hydroxyzine, and fluoxetine. The length of these trials is unknown. Fluoxetine exacerbated his suicidal ideations. He was prescribed trazodone for sleep. According to the chart review, he has been on escitalopram (two trials with unknown outcomes) as well. He has no substance abuse history.\nHe did poorly in school, requiring an individualized education plan (IEP) for graduation. There were reports of behavioral disturbances related to being bullied in high school. He has been unable to hold a job and lives with his mother who is also his support.\nDuring the past three admissions to the ED, the patient was admitted to the inpatient psychiatric hospitalization only once. There was an absence of follow-up in the outpatient setting after his discharge. |
A thirty-six year old female was referred to our hospital with a 16-year history of right rib pain and recurrent lower respiratory system infections. The patient had multiple computed tomography scans in the past, but the diagnosis was never established with success as intravenous contrast was never used. The patient had no other symptoms at presentation and no abnormality was found on physical examination. The only abnormal laboratory value was a slight increase in the white blood cell count, which was 14,000 cells/mm3. A chest radiograph was obtained and was normal. The diagnosis was established with a computed tomography (CT) scan of the chest with intravenous contrast. The test revealed a mass in the right lower lobe (measuring 4.5 × 3.8 × 2.9 cm). An aberrant artery (3 mm in diameter) arose from the abdominal aorta and flowed into the lesion (see Figures and ). The patient was diagnosed with intralobar pulmonary sequestration and surgical exploration and treatment with video-assisted thoracoscopic surgery was planned.\nAfter getting the appropriate consent for the operation and administering general anesthesia and intubation, the patient was placed in a full left lateral decubitus position with the table flexed at 30-degrees at the level between the nipples and the umbilicus in order to have better exposure of the right intercostals spaces. A 10 mm zero-degree thoracoscope was inserted in the right pleural cavity through a port site placed in the sixth intercostal space on the midaxillary line. Two additional port sites were placed in the fifth intercostal space on the posterior and anterior midaxillary line, respectively. The port sites were chosen with a possible thoracotomy in mind. The VATS exploration revealed immediately a mass in the base of the right lung. Upon retraction of the mass, an aberrant artery coming through the diaphragm and leading into the mass was identified (see Figure ). The artery was carefully dissected from the surrounding tissues and divided using an endothoracic stapling device (Endopath 45, Ethicon Endo-Surgery, Inc., Cincinnati, Ohio). The same device was used in order to perform a tailored wedge resection of the right lower lobe, removing this way the pulmonary sequestration. Inspection of the rest of the lung revealed normal pulmonary parenchyma.\nAfter closing the port sites and inserting a chest tube, the patient was extubated and was transferred to the surgical intensive care unit for observation. Her recovery was uneventful and the chest tube was put on water seal the next morning and removed on post-operative day 2. The patient was discharged the third post-operative day and until today, about five months after the operation, remains in excellent condition. Final pathology revealed intralobar pulmonary sequestration. |
We present the case of a 57-year-old man who presented to the outpatient clinic with a 6-month history of intermittent attacks of colicky abdominal pain mainly in the right hypochondrium, radiating to the right shoulder. There was no significant past medical history. The only family history of note was that his mother had been diagnosed with breast cancer at the age of 68.\nGeneral examination was unremarkable and all observations were stable. Abdominal examination revealed no palpable masses or organomegaly. There was mild tenderness in the right upper quadrant and epigastric region with no rebound tenderness and Murphy’s sign was negative. Initial laboratory investigations including full blood count, liver function tests, and serum amylase as well as kidney function tests were all normal.\nBased on the history and clinical findings, a provisional diagnosis of gallbladder stones was suspected, so an abdominal ultrasound scan was performed. The results confirmed the clinical diagnosis, showing chronic calculous cholecystitis with a normal calibre common bile duct.\nThe patient underwent elective laparoscopic cholecystectomy which revealed an intrahepatic gallbladder, so the operation was converted to conventional open surgery through a Kocher incision. The gallbladder was found to be intrahepatic with its fundus on the anterior surface of the liver ().\nRetrograde cholecystectomy was carried out after resecting through the liver to separate the gallbladder from its bed (). The cystic duct was very long (about 8 cm) and resected flush to the common bile duct as a small stone was impacted at its proximal end ().\nAfter haemostasis, a size 16 tube drain was left in the operative bed, and mass abdominal wall closure was carried out. On the second post-operative day, the patient was able to tolerate fluids and a light diet, and his post-operative bloods were all within normal range. The drain only drained 40 ml of serous fluid, so it was removed. The patient mobilized well and was discharged home after 3 days.\nThe patient was seen in the outpatient department 1 month later. He had continued to recover well without any recorded complications. |
A 52-year-old woman presented with pain and active function loss in her left shoulder, and was admitted to our hospital in June 2017. Her shoulder problem had started 3 months earlier, and there was no history of trauma or fracture. At first the pain was intermittent and bearable, but then gradually increased. On examination, there were no positive signs except for localized pain. A radiologic examination on 3 April 2017 found no destruction of the shoulder (Fig. ). Pain at the shoulder joint became gradually aggravated, together with the appearance of shoulder joint dysfunction. Two months later, physical examination revealed mild swelling of the shoulder, and markedly restricted shoulder and elbow motion. Mild distal nerve function defects appeared gradually. Radiography on 3 June 2017 showed that the head of the humerus had disappeared within the past 2 months (Fig. ), which was confirmed by magnetic resonance imaging (Fig. a, b).\nThe patient is a healthy, active individual with no history of weight loss, anorexia, or fever during this period. General and systemic examination findings were within normal limits. Routine laboratory investigations were also normal, including levels of serum calcium, phosphate, alkaline phosphatase, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate. An open biopsy of the lesion revealed that the bony tissue had been replaced by fibrous connective tissue, and small areas of bony trabeculae with occasional osteoclasts were visible (Fig. ). There was no evidence of malignancy or tuberculosis. Because of the lack of any clinical findings or supporting data for other causes, the features were confirmatory of GSD. Computed tomography of the shoulder joint (Fig. ) revealed a bony defect of the glenoid cavity.\nWe performed surgery involving glenoid cavity amplification with an autologous iliac bone graft and a reverse total shoulder arthroplasty. The grafts were from autologous iliac bone (Fig. , Fig. ). A deltopectoral approach was used on the shoulder, and necrotic and dissolving bone tissue was removed. Reconstruction of the glenoid was carried out with autologous iliac bone and installation of reverse shoulder prosthesis. Postoperatively, the arm was placed in a sling for 3 months. Passive elevation and external rotation were allowed 2 weeks after the operation. Three months later, sling use was discontinued, and active range of movement was initiated. Six months after surgery, the patient is pain-free with more than 90° of active abduction, 100° of forward flexion, and 30° of shoulder posterior extension. She also has good functional use of her shoulder (Fig. , Fig. ). |
In 2013, a 73-year-old man presented to our clinic with ocular irritation, pain, and visible tumor mass in the anterior chamber of his left eye. He gave a past ophthalmic history of same eye pterygium surgery a year earlier that was performed elsewhere and an unsuccessful postoperative steroid treatment due to presumed anterior uveitis. On examination, the vision was 20/35 in the affected eye, and 20/20 in the fellow eye, which was healthy. The intraocular pressure was normal in both eyes. A white solid mass was seen in the left anterior chamber covering the nasal part of the iris, and infiltrating the corneal endothelium (-). On gonioscopy, the tumor was blocking 2 clock hours of the angle. Both fundi were unremarkable, and the submandibular and periauricular lymph nodes were normal. Iridectomy was performed as the first line treatment to remove a part of the tumor adjacent to the iris and for histopathological diagnosis. However, the residual tumor tissue was still present in the anterior chamber and deeper layers of the cornea. The histopathological report revealed T3N0M0 invasive squamous cell carcinoma originating from the conjunctiva with the signs of HPV infection (koilocytosis) (-). The margins of resected tissue were infiltrated with tumor cells. A month later, conjunctival mapping biopsy was undertaken, which demonstrated no conjunctival involvement, and adjunctive ruthenium plaque brachytherapy was performed to eradicate the residual tumor tissue (-). The conjunctiva was cut approximately 7 mm from the limbus, and the medial rectus was disinserted to create the opening for the 20 mm CCB plaque as well as to irradiate a wider part of the conjunctiva adjacent to the limbus. The plaque was placed in the nasal part of the conjunctiva covering approximately 6 clock hours of the corneoscleral junction to ensure coverage of all remaining tumor. A dose of 86 Gy at a depth of 1.2 mm was delivered over 24 hours.\nAt 4.5 years post-treatment, the affected eye is painless with the vision of 1.0. The intraocular part of the tumor had completely regressed; no orbital or conjunctival recurrence was noted. There was, however, a sectorial cortical cataract (-) that was removed during uneventful phacoemulsification surgery 6 months ago. No distant metastases were observed at the last follow-up visit. |
A 35-year-old woman of Turkish origin presented to our outpatient clinic with increasing gait and stance disturbances of 2 or 3 years’ duration. She reported tingling pain in the feet and lower legs as well as an impairment in detecting cold temperatures as a sign of small fiber involvement. In addition, the patient had recurrent shortness of breath. She had previously been diagnosed with a severe polyneuropathy of unknown origin. Besides the neuropathy, her medical history was empty. She denied gastrointestinal or visual disturbances.\nShe was a daughter of nonconsanguineous parents from Turkey. Her family history was empty regarding neuromuscular disorders. She has four brothers and two sisters. She is the mother of two healthy children (ages 5 and 7 years).\nHer clinical examination revealed a distal as well as left pronounced tetraparesis, hyporeflexia with absence of Achilles tendon reflexes, hypesthesia of the lower legs reaching the patellae and pallanesthesia at the medial malleoli. No compound motor unit potential could be measured on her lower extremities of the tibial or peroneal nerves bilaterally. Motor conduction studies of her upper extremities, including investigation of both the median and ulnar nerves, were unremarkable. No sensory nerve action potentials could be obtained on the upper or lower extremities. Signs of denervation in the distal muscles of the lower extremities, as well as signs of chronic neurogenic reorganization, were detected by electromyography. The extensive polyneuropathy work-up and rheumatology assessments did not produce any pathological results. No indication for a paraneoplastic origin of the disease was discovered. Standard screening methods did not reveal an associated monoclonal gammopathy of undetermined significance or diabetes. However, proteinuria was discovered in the 24-hour urine collection, indicating renal involvement. Because she had dyspnea, a cardiac work-up was performed. In this clinical examination, minor signs of chronic heart failure were found, and we classified her as New York Heart Association functional class II. Magnetic resonance imaging of her heart revealed early left ventricular hypertrophy with a normal ejection fraction of 60%. Echocardiography showed an increased thickness of the interventricular septum of 15mm and a slightly impaired longitudinal left ventricular function. The general weakness might also have been associated with an involvement of the autonomic nervous system with low systolic blood pressure (generally 90mmHg or lower). The patient did not complain about visual disturbances. However, upon examination, opacity of the vitreous body with signs of visual loss was detected in both eyes.\nA sural nerve biopsy was performed and processed using standard protocols\n[]. Paraffin-embedded nerve tissue was processed for Congo red staining. The stain depicted small but distinct endoneural deposits of amyloid (Figure \nA) with a bright green birefringence under polarizing filters (data not shown). These deposits stained positive with antibodies against transthyretin (TTR) (Figure \nB). Elastica van Gieson staining allowed visualization of collagen fibers and showed a strong endoneural fibrosis (data not shown). Immunohistochemistry against neurofilament and p-phenylenediamine-stained semithin sections revealed peripheral nerve fascicles with severe loss of myelinated fibers (about 95% compared to healthy controls) and only few remaining myelinated fibers (Figures \nC and\nD). Single fibers with acute myelin degradation could be observed. Electron microscopy confirmed the severe axonal neuropathy with few remaining myelinated fibers with signs of axonal atrophy, few myelin debris and denervated Schwann cell bands (Figures \nE and\nF). Additionally, a severe loss of unmyelinated fibers could be detected. Single amyloid deposits were demonstrated by ultrastructural analysis. There were no signs of axonal regeneration.\nIn the genetic analyses of the TTR gene, we found a heterozygous missense mutation: c.221A>G (GAG>GGG; p.Glu74Gly). The four coding exons of TTR, corresponding to coding cDNA nucleotides c.1 to c.444, including flanking sequences, were amplified by PCR and screened for mutations by direct sequencing (reference sequence: TTR ENSG00000118271 with transcript ENST00000237014). Mutation designation according to standard naming conventions of the Human Genome Variation Society\n[], which uses numbering beginning at the Met initiation codon, includes a 20–amino acid signal sequence and may be different from that reported in the literature. The alternative mutation designation for the mutation detected in our patient is p.Glu54Gly.\nTreatment with tafamidis was initiated after TTR-FAP was diagnosed\n[], but our patient’s neuropathy was aggravated nonetheless. An orthotopic liver transplant was performed 10 months later, but the patient died as a result of intra-operative complications. Three of her four brothers and three sisters have since presented to our outpatient clinic. A 36-year-old brother tested positive for amyloid in a subcutaneous fat aspiration test. Further family members in Turkey have been evaluated for the mutation. One cousin was found to be a mutation carrier; unfortunately, we have no further information on his health condition. |
In 2006 November, a 27 year old diagnosed HbE-Beta thalassaemic Sri Lankan Muslim male patient presented to the medical ward with rapidly progressive weakness of both lower limbs for about 1 week duration and ended up in total paralysis of both lower limbs with double incontinence. It did not progress to the upper limbs or respiratory muscles. On examination he had spastic paraparesis with a sensory level at thoracic 8 (T8) level. At that time his haemoglobin was around 5 g/dl and had a number of blood transfusions which settled his paralysis.\nIn 2007 November, once again he presented to the neurology ward with similar symptoms. Physical examination revealed a young male with a characteristic facies with frontal bossing, prominent malar prominences, short upper limbs and multiple skeletal deformities. He was severely pale and icteric as well. Splenectomy scar was there and liver was moderately enlarged (5 cm below costal margin). Higher functions and cranial nerves examinations were normal. Motor system examination revealed normal upper limbs with increased tone in lower limb muscles and complete loss of power in all muscle groups of the both lower limbs. Deep tendon reflexes were exaggerated in both lower limbs with bilateral extensor planter response. Superficial abdominal reflexes were absent and there was a complete sensory loss below T8 level.\nOn admission he had haemoglobin of 6.5 g/dl with normal white cell and platelet counts. All the biochemical investigations were normal except high serum bilirubin indirect fraction and very high level of serum ferritin (2488 ng/mL). X ray thoracic spine did not provide any clues of compressive lesions. MRI of the thoracic spine revealed multilevel, lobulated soft tissue mass in the extradural space extending from T5 to T8. The spinal cord and thecal sac was severely compromised with anterior displacement of the cord due to pressure effect on dorsal cord suggesting extra- medullary haemopoiesis.\nThe patient had undergone number of packed cell transfusions with iron chelating therapy (Intravenous desferioxamine). With blood transfusions the lower extremity power improved gradually and regained sphincter control once his haemoglobin level comes around 10 g/dl. Post transfusion MRI of thoracic spine revealed the reduction of the size of soft tissue mass with minimal pressure effect on the cord, which further strengthening the diagnosis of cord compression due to extramedullary haemopoiesis to overcome the anaemia. |
A 20-year-old female patient reported to our institute with a swelling on the zygomatic area on the right side of the face. The swelling was noticed 10 years ago, which remained asymptomatic and had gradually increased to the present size of 6 cm in diameter causing asymmetry of the face []. Another bony hard swelling was also seen in relation to the left mandible extending laterally to the symphysis area []. In both swellings, the color of the surface of the skin was normal and not attached to underlying tissue. There was no local raise in temperature over the swelling.\nThe intraoral examination in relation to the maxillary right quadrant revealed a bony hard swelling involving the buccal [] and palatal aspects of the canine area, extending up to the maxillary tuberosity. The maxillary left quadrant revealed a bony hard swelling involving the palatal aspect of the premolar extending up to the molar region. The mandibular left quadrant revealed a hard swelling leading to obliteration of the vestibular sulcus from the canine to the first molar region [].\nThe orthopantomograph of the maxillary right quadrant revealed mixed, radiopaque [ black arrows] and radiolucent areas [ blue arrows] with diffuse, ill-defined margins obliterating the maxillary sinus, extending from the infraorbital region to the alveolar region. The medial aspect revealed opacity extending into the nasal cavity and laterally extending to the zygomatic arch and posteriorly up to the maxillary tuberosity.\nThe maxillary left quadrant revealed an impacted second premolar [ orange arrow] present at the apex of the first premolar and radiopacity [ black arrows] was seen surrounding the impacted premolar. The mandibular left quadrant showed radiopacity [ black arrows] in relation to apical areas of the mesial and distal roots of the retained deciduous second molar. In addition, the mandibular left quadrant demonstrated a unilocular radiolucent area with sclerotic margin in relation to the premolar and molar regions extending to the crown of the impacted second premolar at the lower border of the mandible [ orange arrows]. The mandibular right quadrant demonstrated unilocular radiolucency in relation to the molar area [ blue arrow]. The complete blood picture and serum alkaline phosphatase was performed and was found to be within the normal range.\nOn the basis of clinical and radiological findings, dentigerous cyst was suspected involving the impacted 35. Incisional biopsy was performed on the maxillary right quadrant and provisional diagnosis of cemento-ossifying fibroma was given. Surgery was planned and performed for all three quadrants under general anesthesia.\nThe surgical specimen received showed multiple bits of tissue that were white in color with a gritty surface. It was hard in consistency in relation to the maxillary right quadrant [], maxillary left quadrant [] and mandibular left quadrant []. In addition to this, an impacted permanent second premolar was seen in relation to the maxillary and mandibular left quadrant. The mandibular left quadrant contained soft tissue, which was firm in consistency, along with a retained deciduous second molar. In case of the retained deciduous molar, the radiographic picture of fused radiopacities at the mesial and distal root apex was not evident as the retained tooth was devoid of such attachments and the roots of the tooth were well defined, as shown in the inset of .\nMicroscopic features in relation to the maxillary right quadrant exhibited trabeculae of immature bone and irregular basophilic cemental masses in a moderately cellular fibrous connective tissue stroma []. The periphery of the section exhibits normal vital bone. There is no connective tissue separating the immature trabeculae from the normal bone. The cemental masses appear to be fusing with the trabeculae of the bone and with themselves to form larger masses [].\nThe decalcified tissue section in relation to the maxillary left quadrant showed trabeculae of immature bone and irregular basophilic cemental masses in a highly cellular fibrous connective tissue stroma exhibiting whorling around the cemental masses and the immature bone []. The immature bone appears to be curvilinear, resembling ginger roots. Cemental masses appear to be fusing with trabeculae of the bone and with themselves to form larger masses [].\nThe mandibular left quadrant showed a cyst-like area with irregular basophilic cemental masses in a background of less-cellular fibrous connective tissue []. The cystic lumen is lined by non-keratinized odontogenic epithelium resembling reduced enamel epithelium [inset; ]. The epithelium shows proliferation, with the underlying connective tissue being infiltrated with lymphocytes and plasma cells. Other areas showed fibrous connective tissue stroma with cemental masses []. |
A 70-year-old man was being followed for myasthenia gravis and diabetes. Periodic CT incidentally detected a splenic arterial aneurysm that had enlarged over time. The true aneurysm extended almost the full length of the splenic artery, arising from the root to the splenic hilum. Interventional radiology was excluded from the therapeutic options because of risks associated with arterial recanalization, ischemic complications, and cost effectiveness. Therefore, we performed elective surgery with aneurysmectomy and splenectomy. The splenic artery was cut at its root without disturbing celiac arterial flow. After dissecting the pancreatic parenchyma to detect the intact portion of the dorsal pancreatic artery branching from the aneurysm, this artery was also cut. Because dense pancreatic capsule was adherent to the calcified aneurysmal wall, the aneurysm required dissection from the pancreatic parenchyma. The pancreatic parenchyma was preserved with its drainage vein, although the pancreatic parenchyma was widely exposed secondary to the aneurysmectomy (Figure ). Operative time was 250 min, and blood loss was 1907 ml.\nThe patient subsequently developed postoperative intractable pancreatic fistula, and refractory symptoms affected his postoperative course. Peak amylase level in the drainage discharge was 32197 U/L. Although intraperitoneal drainage continued, optimal tube drainage (i.e., intentional placement along the distal pancreas) was not an option because a percutaneous approach was too difficult. Fistulography via the intraperitoneal drain on POD 13 revealed intractable pancreatic fistula in the distal pancreas (Figure ); therefore, we performed endoscopic retrograde pancreatic drainage on POD 15. Intraperitoneal drainage with retrograde pancreatic drainage was continued for 36 days with intermittent antibiotics until POD 40. However, intractable pancreatic fistula resulted in an abscess and localized peritonitis secondary to bacterial infection. Contrast CT on POD 59 detected an intraperitoneal abscess in the distal pancreas (Figure ).\nOn POD 63, we initiated CLL of the abscess cavity via the transgastric route. The abscess cavity was punctured endoscopically under ultrasound guidance, and a transgastric path into the abscess cavity was made. Transnasal infusion was initiated, and transgastric drainage tubes were placed endoscopically (Figure ). CLL was instituted via transnasal continuous infusion and endoscopic transgastric drainage (Figure ). The abscess cavity could be effectively lavaged, and the recovery rate of injected contrast dye was approximately 70% because of the concurrent transgastric drainage tube.\nFrom POD 63, saline irrigation was continuously injected via the transnasal infusion tube. A total of 1000 ml of saline per day was slowly irrigated into the abscess cavity, and amylase levels in the drainage discharge decreased immediately (139 U/L). Fistulography on POD 70 revealed that the abscess resolved after 1 wk of CLL, and the refractory pancreatic fistula was no longer identifiable. Therefore, we removed the transnasal infusion and retrograde pancreatic drainage tubes. The patient was discharged on POD 72 with only the transgastric drainage tube remaining in place. His postoperative course was categorized as Clavien–Dindo grade IIIa[], and he was in good health 9 moafter surgery. |
A 77-year-old woman visited a local clinic because of persistent cough for the past 6 months. A tracheal tumor was suspected on imaging and she was referred to us for further evaluation and treatment. Her medical history included hypertension and atrial fibrillation, for which she was treated with medications. Her height was 156 cm and weight was 56 kg. Respiratory noise was audible on respiration. There was no enlargement of surface lymph nodes. Additionally, there were no abnormalities in blood biochemistry and tumor marker levels were normal. Radiography showed a mass in the trachea. Computed tomography (CT) and magnetic resonance imaging (MRI) findings revealed a 1.5 cm large border and a smooth surface nodule shadow on the posterior wall of the thoracic trachea approximately 5 cm below the glottis. Mass formation with a major axis of 1.0 cm in diameter extending from the trachea to the esophagus was observed. Based on MRI findings, the border with the surrounding organs was clear and it was diagnosed as primary tracheal tumor invasion (Fig. a, b) Furthermore, bronchoscopy showed a smooth-surfaced submucosal tumor with abundant neovessels, which accounted for 80% of the cross-sectional area of the trachea (Fig. ). Biopsy was not performed, because there was a strong possibility of airway obstruction due to bleeding. Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed accumulation with a maximum standardized uptake value of 2.9 for the lesion and the possibility of malignancy could not be excluded (Fig. ). The patient was not in a state of respiratory distress and tumor resection using a bronchoscope was at risk of bleeding, we decided to remove the tumor by tracheal sleeve resection, because the patient had symptoms and was at risk of airway obstruction. Regarding anesthesia, the percutaneous cardiopulmonary support (PCPS) was put on standby in consideration of the possibility of suffocation due to intubation difficulties and bleeding during intubation. There were no problems during anesthesia except for the operation of the intubation tube. Surgery was performed via median sternotomy. The thymus and surrounding structures were separated to expose the trachea, and then five tracheal rings, including the tumor, were removed by sleeve resection. Separation from the esophagus was relatively easy. After resection of the trachea, the airway was secured by intubation. End-to-end anastomosis of the trachea was performed using 3-0 monofilament synthetic absorbable suture material. A continuous suture was initiated in the membranous portion. The orally inserted tracheal tube was then advanced to the distal portion to secure the airway and interrupted suture of the portion of the tracheal cartilage was performed. The anastomosis site was covered with the thymus (Fig. ). Five tracheal rings were excised for excision of one or more tracheal rings above and below the tumor. Based on the results of intraoperative pathology, it was judged that there was no tendency for tumor infiltration and additional resection was not performed. The postoperative course was favorable. There has been no recurrence for 2 years after surgery. The resected specimen showed a white–pink tumor (maximum diameter of 23 mm) protruding into the tracheal lumen and the membranous portion (Fig. ). Pathological examination showed a mass of hyperplastic atypical cells with spindle-shaped nuclei arranged in bundles running in various directions in the submucosa. On immunostaining, the tumor was positive for vimentin, CD34, and Bcl-2, and negative for α-smooth muscle actin (α-SMA). Based on these findings, the tumor was diagnosed as an SFT (Fig. , ). There were no malignant findings and the resected stump was negative. The postoperative course was favorable and there has been no evidence of recurrence for 2 years after the surgery. |
An 80-year-old Caucasian male of Italian descent presented to the emergency room with a two-week history of progressive left thigh pain and swelling. The patient denied any history of trauma and stated that the pain was aggravated by weight-bearing. He denied any fevers, chills, night sweats, constitutional or neurological symptoms. He had no drug allergies and his only medication was a statin. Past medical history included hypercholesterolemia, open cholecystectomy, and prostate cancer requiring resection remotely in the past.\nThe patient’s surgical history was significant for six previous operations for a left intertrochanteric femoral fracture complicated by an infected non-union. An infectious diagnosis had been proven with Staphylococcus aureus cultured from previous operations. His most recent surgery occurred one month prior to his presentation to the emergency room and involved removal of a left trochanteric fixation nail and excision of a pseudoarthrosis, followed by an osteotomy of the left proximal femur nonunion and fixation with a proximal femoral locking plate (Figure ). No complications were encountered during that surgery or afterwards, and the patient had been discharged home several days after surgery with instructions to avoid bearing weight on the left leg.On physical examination, the patient was hemodynamically stable and afebrile. A general physical examination, including lymph nodes, was unremarkable. Inspection revealed pronounced firm swelling of the left thigh with slight erythema. The surgical incision on the lateral aspect of the left thigh was clean and dry. There was slight tenderness to palpation over the swollen area and left hip and knee range of motion was restricted secondary to pain in the left thigh. The patient was neurovascularly intact in his left leg. Lab results, including erythrocyte sedimentation rate, C-reactive protein, white blood cell count, and hemoglobin levels were all within normal range. Left hip radiographs showed only generalized soft tissue swelling of the left thigh with no signs of hardware failure. Computed tomography (CT) scanning of the pelvis and left thigh was suggestive of the diagnosis of a psoas abscess (Figure A, B).After obtaining blood and urine cultures, the patient was admitted to hospital and broad-spectrum intravenous antibiotics were started based on recommendations from a surgical infectious disease specialist. The patient underwent incision and drainage for the left thigh abscess that same day using the previous lateral hip incision. No pus was noted intraoperatively, but a collection of thick white gelatinous tissue was noted underneath the muscle fascia of the left thigh anterior compartment and was observed to extend medially and laterally (Figure ). Tissue specimens were sent for gram stain, aerobic and anaerobic cultures, acid fast testing, fungal cultures and cytology. The wound was then irrigated with normal saline and antibiotic beads and a hemovac drain were placed inside the wound. Intravenous antibiotics were resumed post-operatively and all cultures were negative. When swelling did not resolve within a few days after surgery, a repeat CT of the pelvis showed residual features of the presumed iliopsoas abscess extending into the left thigh. On postoperative day two, the patient underwent a second look incision and drainage through a combined ilioinguinal and lateral hip approach. A mass was noted close to the left iliac wing encasing the left femoral nerve. The femoral nerve was freed from the lesion without complication and the mass was then removed, debulked, and sent for frozen section. The frozen section revealed acute and chronic inflammation. The remaining tissue was sent for microbiology and histopathology. The wound was irrigated with normal saline and a hemovac drain was again placed before wound closure. Postoperatively, the patient was re-evaluated by the surgical infectious disease service, who proceeded to discontinue IV antibiotics and advised initiating anti-tuberculosis therapy. This decision was based on a diagnosis of exclusion, given the negative bacterial cultures, the anatomic site of the abscess and the gross pathological features of the biopsied tissue. All screening tests for tuberculosis were negative. CT scans of the chest and abdomen were performed as part of a metastatic work up, but revealed only a large right-sided kidney cyst. The patient improved and was discharged home with oral anti-tuberculosis therapy to be taken until definitive pathology results were available. Two weeks later, final pathology reports supported the diagnosis of a diffuse large B-cell lymphoma. Anti-tuberculosis drugs were discontinued and a medical oncologist was consulted to initiate chemotherapy.\nDiffuse large B-cell lymphoma is the most commonly occurring lymphoma, estimated to account for 35% of all lymphoma cases globally []. It typically presents in patients older than 60 years, has an aggressive natural history, and is primarily treated with systemic chemotherapy []. They typically develop in nodal tissue with less common development in extra-nodal tissue with estimates of approximately 1.6% []. Extra-nodal sites include the gastrointestinal tract, orbit, respiratory tract, skin, bone, thyroid, central nervous system and (less commonly) the soft tissues [,].\nLymphomas can also involve any part of the musculoskeletal system and occasionally can manifest as a primary soft tissue mass that can be mistaken for infection or a soft tissue sarcoma [-]. Soft tissue lymphoma accounts for approximately 1% of malignant soft tissue neoplasms and are much more likely to present as non-Hodgkin’s lymphoma rather than Hodgkin’s lymphoma []. Most patients presenting with soft tissue lymphomas are older than 50 years, with no predilection to either sex [].\nAlthough poorly understood, it has been postulated that primary soft tissue lymphomas may be the result of chronic antigen stimulation in an individual with a deficient immune system. In addition to inflammatory connective tissue disorders and viruses, other potential causes of a prolonged local inflammatory environment in patients undergoing orthopedic surgery include shedding of local metal ions and debris from metallic implants, radiation from radiographs and CT imaging, surgical trauma and infection.\nPre-clinical evidence examining the potential carcinogenic properties of metal implants found a trend towards sarcoma development in rats after 30 months when implants with a high content of cobalt, chromium or nickel were used. There was also an increase in bone-associated lymphoma development in rats with these metal implants []. It is believed that the local accumulation of metallic debris can cause damage to the cell membrane of macrophages with subsequent release of pro-inflammatory mediators as well as the formation of immunogenic complexes, both of which can stimulate immune system responses [,].\nNumerous studies have explored the topic of increased cancer risk in individuals with metal implants, but have shown discordant conclusions. Gillespie et al. [] followed a cohort of total hip arthroplasty (THA) patients and found that the incidence of lymphatic and hematopoietic malignancies was nearly twice that expected for the general population. Similarly Visuri and Koskenvuo [] also looked at THA patients and observed an incidence of lymphomas and leukemias that was three times what was expected. However, more recent studies have shown no increase in risk of cancer [-]. There are approximately thirteen recorded instances of lymphoma at sites associated with metal implants in the literature (Table ).\nChronic inflammation as a result of infection is a possible contributing factor in the development of lymphoma, as implied by studies of non-infectious consequences of osteomyelitis [-]. Cheuk et al. [] also make this link, arguing that lymphoma associated with osteomyelitis shares characteristics of that associated with metallic implants. These characteristics include the fact that these solid tumors arise in the context of chronic inflammation, occur in a confined space, and have a long latency period between the onset of inflammation and the development of lymphoma.\nTypically, soft tissue lymphomas present in patients older than 60 years as enlarging soft tissue masses associated with swelling or pain. In typical cases, there are usually no B symptoms such as weight loss and fever [,]. Our case features a similar presentation with an enlarging thigh mass associated with pain with the absence of B symptoms. Although the patient showed no identifiable active cause of immunocompromise, he had several risk factors for chronic antigenic stimulation including several surgeries involving metallic implants and prolonged infection after his index surgical procedure.\nDespite the presence of some risk factors, the malignancy in this case could also have been coincidental. Cases have been reported of patients who underwent total joint replacement and were found to have lymphoma on routine histopathological exams [,]. One study looking at retrieved femoral heads that were to be used for bone allograft found that 14 of 852 heads were highly suspicious for low-grade B-cell lymphoma. With a median of 7.2 years follow-up, one patient developed a B-cell lymphoma in a lymph vessel in the inguinal region on the contralateral side []. |
A 7-year-old girl, injured by an oncoming vehicle while she was on a tricycle, was admitted to the local hospital with a complete traumatic amputation of her left lower limb for 5 h. She was transferred to our hospital after pressure dressing of the wound and transfusion in the local hospital. Physical examination indicated that the patient’s blood pressure was 80/50 mmHg and the pulse rate was 128 per minute. She was conscious, and her left lower extremity below the hip completely lost and the wound dressing unopened. Her blood pressure rose to 100/60 mmHg after transfusion. Debridement was performed 2 h after admission. During the surgery, we noticed that the left lower extremity beyond inguinal area was totally absent, and the area from the sacroiliac joint to the pubic bone was exposed. There were leaves and other foreign matter in the wound. The rectum, vagina and the bladder were exposed, and there was a 4 cm long laceration of the anal sphincter. The wound did not communicate with the abdominal cavity and there was no injury to the viscera. Left iliopsoas were completely avulsed from the original site and a large cavity was present in the retroperitoneal space below the diaphragm. The left common iliac artery and vein was transected and thrombosed 1 cm distal to its origin. Debridement was performed, the proximal ends of the left common iliac artery and vein were ligated and the wound was washed repeatedly. Due to the extensive skin loss, the wound was partly closed and the rest left open and covered with Vaseline Gauze after operation. Diverting colostomy was performed and thorough irrigation of the distal colon segment was conducted to prevent continued fecal contamination of the pelvic wound. The wound remained open initially, the granulation tissue gradually matured and the wound became self-contracted and closed spontaneously 6 weeks after the injury. The patient could walk with her crutches at the 12-month follow-up after the procedure (Figure \n). |
In April 1996 this 78-year-old woman was diagnosed with a poorly differentiated lobular carcinoma in the upper outer part of the left breast. Clinically it measured 15 mm. She was treated with conservative breast surgery and level III axillary node clearance. Tumor margins were clear and all lymph nodes were negative. Tamoxifen was commenced at 20 mg OD.\nA course of postoperative radiotherapy was arranged due to breast conservation surgery. She received 46 Gy in 23 fractions over 35 days using paired glancing megavoltage fields. This treatment was completed in July 1996 and was followed by a boost to the site of excision of a further 12 Gy in 4 fractions over 7 days using 9MEV electrons. Chemotherapy was discussed but the patient did not want to pursue this option.\nShe remained in regular follow up with no evidence of local or regional recurrence. In June 2005 during a routine follow up appointment she reported a 2 cm area of purple skin lesion at the lower inner quadrant of the left breast (figure ), with a history of it being there for about 3 months. It was associated with peau d'orange and soft tissue thickening extending from the lower inner quadrant across the midline. Mammography was not performed, as she was too tender. Ultrasound showed no evidence of a lesion deep to the abnormality. Freehand core biopsies were taken of the area. Pathology demonstrated the presence of malignancy in keeping with high-grade angiosarcoma.\nStaging investigations including CT chest and abdomen, CXR and bone scan none showed any evidence of metastasis. She had wide surgical excision and reconstruction with a Vertical Rectus Abdominus Myocutaneous flap (VRAM flap). She has completed her first year follow up without any recurrence.\nHistological examination following routine processing and staining with haemotoxylin and eosin shows malignant tumor (figure ). Although this tumor is predominantly solid, comprising highly pleomorphic cells (figure ) and areas with spindle cell morphology (figure ) neoplastic vascular channels are also identified (figure ). Immunohistochemical staining for CD31, CD34 and factor viii confirm endothelial differentiation both within the solid tumor component and the recognizable vascular channels. The possible differential diagnoses of carcinoma and malignant melanoma are excluded by the negativity of other stains (MNF and S100 respectively). The histological features are those of high grade angiosarcoma.\nHistology from the original breast excision biopsy (figure ) shows a lobular carcinoma, with strong oestrogen receptor positivity. This shows no similarity to the newly arising angiosarcoma. |
A 32-year-old white woman, who was undergoing transgender body modifications and no previous medical history, presented to our emergency department (ED) with a chief complaint of right forearm pain, associated with redness and blistering. The patient was a tattoo fanatic who underwent solar branding on her right forearm approximately 2 weeks before presentation. The patient first noticed the blistering of the branded area 3 days after her body modification. Over the next few days, the entire branded area became very warm, swollen, painful to touch, and developed cellulitis with weeping blisters. Given her dire situation, the patient finally sought medical care in the local urgent care center. The patient was diagnosed with a superficial infection of her right forearm full thickness burn and prescribed oral clindamycin. The patient continued to develop painful blisters for the next week and did not notice any improvements in her cellulitis despite the antibiotic treatment, prompting her to come to our ED for a second medical opinion.\nDuring her physical examination, the patient was noted to have painful blisters, redness, and swelling of the right forearm and hand (Figure ). She did have a full range of motion of the hand; however, there was mild pain noted on the flexion of the hand. There were no signs of sepsis, and her vital signs and her laboratory panel which included white blood cell count were all within normal limits. The rest of the physical examination was also unremarkable. The patient was admitted to the Burn Surgery Service for intravenous (IV) antibiotics and possible operative interventions.\nThe patient was started on IV clindamycin 600 mg every 8 hours, and once adequate pain control was achieved, a bedside debridement was performed in the Burn Unit. The following day, the patient was taken to the operating room for tangential excision of her wound and split-thickness skin autograft (STSG) placement onto her right forearm. The left anterolateral thigh was chosen as the donor site. The grafted site was dressed with xeroform, followed by a layer of bacitracin ointment and then wrapped with Kerlex and Coban dressings. A right forearm elbow splint was fitted and placed by the occupational therapy service for further graft protection. Postoperatively, the patient was transferred back to the floors without complications, and antibiotics were stopped. The right forearm dressing was taken down on postoperative day 4 to evaluate the STSG. The entire graft was viable and taken. Subsequently, the graft staples were removed on postoperative day 5.\nThe patient recovered exceptionally well and was discharged home a few days later. Two weeks later, the patient returned to the burn clinic for a follow-up appointment. The skin graft and the donor site healed remarkably well (Figure ). The patient reported no pain and had a full range of motion of the hand and wrist. The patient was discharged from the burn service and advised to use over-the-counter moisturizer or lotion as needed. |
A 52-year-old woman presented with pain and active function loss in her left shoulder, and was admitted to our hospital in June 2017. Her shoulder problem had started 3 months earlier, and there was no history of trauma or fracture. At first the pain was intermittent and bearable, but then gradually increased. On examination, there were no positive signs except for localized pain. A radiologic examination on 3 April 2017 found no destruction of the shoulder (Fig. ). Pain at the shoulder joint became gradually aggravated, together with the appearance of shoulder joint dysfunction. Two months later, physical examination revealed mild swelling of the shoulder, and markedly restricted shoulder and elbow motion. Mild distal nerve function defects appeared gradually. Radiography on 3 June 2017 showed that the head of the humerus had disappeared within the past 2 months (Fig. ), which was confirmed by magnetic resonance imaging (Fig. a, b).\nThe patient is a healthy, active individual with no history of weight loss, anorexia, or fever during this period. General and systemic examination findings were within normal limits. Routine laboratory investigations were also normal, including levels of serum calcium, phosphate, alkaline phosphatase, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate. An open biopsy of the lesion revealed that the bony tissue had been replaced by fibrous connective tissue, and small areas of bony trabeculae with occasional osteoclasts were visible (Fig. ). There was no evidence of malignancy or tuberculosis. Because of the lack of any clinical findings or supporting data for other causes, the features were confirmatory of GSD. Computed tomography of the shoulder joint (Fig. ) revealed a bony defect of the glenoid cavity.\nWe performed surgery involving glenoid cavity amplification with an autologous iliac bone graft and a reverse total shoulder arthroplasty. The grafts were from autologous iliac bone (Fig. , Fig. ). A deltopectoral approach was used on the shoulder, and necrotic and dissolving bone tissue was removed. Reconstruction of the glenoid was carried out with autologous iliac bone and installation of reverse shoulder prosthesis. Postoperatively, the arm was placed in a sling for 3 months. Passive elevation and external rotation were allowed 2 weeks after the operation. Three months later, sling use was discontinued, and active range of movement was initiated. Six months after surgery, the patient is pain-free with more than 90° of active abduction, 100° of forward flexion, and 30° of shoulder posterior extension. She also has good functional use of her shoulder (Fig. , Fig. ). |
A 22-year-old male patient reported to the department of oral medicine and radiology with the complaint of swelling on the left side of the face for the past 6 months and reduced mouth opening for the past 20 days. The patient's history revealed that the swelling was initially smaller in size and has increased slowly to the present size over the period of 6 months. The patient also stated that his mouth opening was approximately four fingers 20 days back, which has now reduced to approximately two fingers over a period of 20 days. His dental history revealed that he had visited a private dental clinic 10 days back for the same complaint where he was advised orthopantomogram (OPG) and magnetic resonance imaging (MRI) of the left side of the mandible and was referred to our institution for further treatment. His medical history was not significant. On extraoral examination, single diffuse swelling measuring approximately 3 cm × 4 cm was present on the left side of the mandibular angle region, extending anteroposteriorly from the level of corner of the mouth till the posterior border of the ramus of the mandible and superoinferiorly from the level of the ala-tragus line till the lower border of the mandible. The overlying skin appeared normal in color []. On palpation, the swelling was afebrile, nontender, and firm to hard in consistency. The interincisal opening was approximately 18 mm. Intraoral examination revealed inflammation in the retromolar area, with clinically missing left mandibular third molar. There was obliteration of the buccal vestibule in relation to the mandibular left second molar due to cortical expansion in the same region. There was slight tenderness in the same region. No signs of paresthesia were present. A provisional diagnosis of dentigerous cyst with the mandibular left third molar was given. Orthopantomogram (OPG) showed large well-defined multilocular radiolucency involving the left mandibular angle region and ramus, extending from the periapical region of the mesial root of the mandibular left second molar till posterosuperiorly up to the coronoid process and neck of the condyle along with distoangularly impacted third molar []. MRI revealed poorly marginated heterogeneous lesion in the left mandibular ramus measuring approximately 56 mm × 33 mm × 30 mm, which was predominantly hyperintense on T2 and Short-TI Inversion Recovery (STIR) and hypointense on Short-TI, suggestive of ameloblastoma of the left mandibular ramus []. The patient was advised biopsy from the same region. The histopathological report revealed multiple pieces of tissue showing odontogenic epithelium (luminal epithelium) overlying fibrous connective tissue stroma, with some areas of inflammatory aggregates, and in one area, numerous cholesterol clefts were also appreciated under low-power view, whereas, under higher magnification, cystic epithelium in few areas showed preameloblastic cells with dark hyperchromatic and elongated nuclei associated with star-shaped cells, suggestive of stellate reticulum-like tissue. The connective tissue stroma comprised of loose-to-dense bundles of collagen fibers with plump to spindle-shaped fibroblasts. Inflammatory infiltrate predominantly comprising lymphocytes was evident. Thus, the overall histopathological picture was suggestive of infected UA []. The patient was referred to the department of oral surgery for further treatment where segmental resection of the left side of the mandible was advised. The patient was posted for surgery under general anesthesia, and left segmental resection of the mandible with disarticulation was done followed by reconstruction with stainless steel condylar prosthesis []. The patient is under regular follow-up for more than 1½ years with no signs of recurrence. |
A 65-year-old male patient came to the hospital with multiple traumatic brain injuries caused by a motorcycle accident. He had a history of diabetes and asthma, for which he had been pharmacologically treated for several years. He also had a history of open surgery to treat peritonitis 3 years before. Upon arrival at the hospital, he was in a lethargic state (Glasgow coma scale; E3V5M5), with a loss of direct and indirect light reflexes due to damage to the right optic nerve. Skull X-ray and cranial computed tomography (CT) showed comminuted fractures of the skull and multiple areas around the skull base, a Le Fort type II fracture of the facial bones, multiple comminuted fractures of the nose and around the orbit, and a small amount of pneumocephalus in the anterior recess (). Plastic surgeons decided to delay operating on the facial fractures because of severe edema of the face and orbit, and the patient was treated conservatively at the bedside. On day 8 following the trauma, the patient complained of severe headache and nausea, and CT performed on day 8 revealed a large tension pneumocephalus with a tented configuration and noticeably enlarged shadows of air compressing both sides of the frontal lobe (). As an emergency treatment, a rupture of the dura mater in the area of the right anterior cranial fossa was sutured using a subfrontal approach, and fixation surgery was performed on fragments of the comminuted fractures. Four days later, open reduction and fixation surgery were performed on the multiple facial fractures in the department of plastic surgery. On cranial CT performed 2 weeks postsurgery, pneumocephalus was no longer observed (). Subsequently, the patient did not have any specific abnormal symptoms and was discharged in a favorable condition. Two months after surgery for tension pneumocephalus, however, the patient returned to the hospital with primary complaints of memory loss, problems in walking, and urinary tract dysfunction. On cranial CT taken at that time, a communicating hydrocephalus was observed with an Evans ratio of 41% (). Ventriculoperitoneal shunting was performed, after which the symptoms greatly improved. The skull and abdominal X-rays and cranial CT performed post surgery confirmed that the shunts were well positioned in the ventricles and the abdomen (). The patient again returned to the hospital approximately 4 weeks after the procedure, due to a swelling at the shunt insertion site on the right side of the abdomen. An abdomen CT revealed that the shunt had moved from the intraperitoneal space into the abdominal subcutis, and formed a pseudocyst filled with CSF (). Surgery was performed to reinsert the shunt into the intraperitoneal space, and currently (3 months after the last procedure), the patient does not have any abnormalities and is in good condition. |
This is a 72-year-old Caucasian male with a past medical history of hepatitis C-related cirrhosis (status post-liver transplantation 14 years prior), with recurrence of HCV genotype 1a, untreated, who was admitted three times over 3 months for recurrent episodes of small bowel obstruction. During the first two admissions, the patient presented with early satiety, abdominal pain, and inability to pass flatus or have bowel movements, which led to him being treated conservatively with nasogastric tube decompression and intravenous fluids with clinical improvement. The patient presented 4 days after discharge from his second hospital admission with similar abdominal pain complaints, decreased appetite, and increased abdominal distention. The patient denied nausea, vomiting and was able to pass flatus. However, he had no bowel movements for three days. His white blood cell count, electrolytes, and liver function tests were all within normal limits. CT of the abdomen and pelvis showed dilated loops of small bowel concerning ileus versus small bowel obstruction (). Due to the failure to improve symptoms after 9 days from conservative management, the patient was taken for exploratory laparotomy with lysis of adhesions and peritoneal biopsy.\nIntraoperatively, it was noted that there were chronic fibrinous inflammatory changes to both the bowel and the mesentery. There was a trabeculated rind covering all of the bowels. The thickened bulky adhesions covered the terminal ileum, with a few bands at both the terminal ileum and the cecum. All bowel proximal to the terminal ileum’s thickened rind area was dilated, and a small amount of the ileum, and the large bowel, were decompressed. Some of the bands and adhesions near the ileocecal junction were taken down. The surgeon stated they could not safely dissect the peritoneum from the small bowel; therefore, the procedure was terminated without significant lysis of adhesions ().\nInterestingly, the peritoneal biopsy results revealed fibro adipose connective tissue with focal extensive fat necrosis and dystrophic calcifications, fibrosis, and scattered chronic inflammation with no malignancy signs most compatible with encapsulating peritoneal sclerosis ().\nThe patient had a postoperative course complicated by worsening abdominal pain and distension, minimal bowel movements, and poor appetite. Therefore, to increase nutritional status, total parenteral nutrition (TPN) was initiated. Despite being maintained on minimal pain medications, the patient failed to improve symptomatically even after approximately 22 days post-operatively. After reviewing the literature, the decision was made to start Tamoxifen 10mg daily, titrated up to 10mg PO BID after 1 week. The patient was weaned off TPN and improved symptomatically, and was discharged uneventfully. At the 4-month postoperative follow-up, the patient has been doing well. Although the patient still has constipation episodes relieved with laxatives, he has not had any more episodes of small bowel obstruction since the initiation of the Tamoxifen. He is always maintained on 10mg twice a day. |
A 16-year-old man was struck by a car while riding a bicycle and was taken to the emergency room with chief complaints of chest pain and dyspnea. He was mentally alert and his vital signs included a blood pressure of 130/83 mmHg, pulse of 93 beats per minute, respiratory rate of 22 breaths per minute, body temperature of 36.6°C, and 90% oxygen saturation. The patient presented with an ill appearance, decreased respiratory sounds in both lungs, and acute pain in his right chest. From the portable cervical lateral view, deep neck subcutaneous emphysema was observed in the soft tissues of the anterior cervical spine; chest images revealed subcutaneous emphysema in the right chest. Chest CT was performed immediately after confirming that the patient’s paroxysmal events appeared to be stable. From the axial view, mediastinal emphysema was observed in the right lung contusion and main bronchus areas along with a small amount of bilateral pneumothorax in the apex of the lung (). The main bronchial laceration was clearly seen from the coronal view, but there was a mistake of not checking the view (). Despite the oxygen supply, the patient complained about worsening dyspnea and developed cyanosis, diaphoresis, and palpitations along with symptoms of anxiety. His oxygen saturation level decreased to 80%. The hypophonesis of both lungs appeared to have decreased, suggesting the progression of tension pneumothorax. An 18-gauge needle was used to perform emergency needle aspiration between the second and third ribs on both thoraces and chest tubes were inserted on both sides of the chest. However, the oxygen saturation level still did not improve and the deep neck subcutaneous emphysema in the cervical spine and chest wall were aggravated; therefore, an endotracheal tube was inserted, after which gas analysis of the arterial blood revealed a pH of 7.29, pO2 of 69 mmHg, pCO2 of 51 mmHg, and 91% O2 saturation. Despite the endotracheal tube, the oxygen saturation did not improve and the subcutaneous emphysema progressed. Conscious sedation was performed and a ventilator was attached; however, evacuation of air through the chest tubes remained poor and the oxygen levels did not fully recover. Subsequently, a self-inflating bag was used to supply oxygen. Despite this, the air deflation was not smooth and there was large resistance to the ventilations. Suspecting an error in the placement and the function of chest tubes, a third tube was inserted in the intercostal space between the fifth and the sixth ribs on the right side. The patient’s oxygen level briefly rose to 90%, but the resistance during bagging persisted. A suction tube was inserted but did not go deeper than 25 cm from the incisor. In order to identify the bronchial injury, a portable bronchoscopy was performed. The endotracheal ends were almost blocked by blood clots. A guide wire was used to remove the blood clots and a right bronchus proximal rupture was confirmed. A double lumen intubation tube (35-Fr left Broncho-Cath, Mallinckrodt, St Louis, MO, USA) was then inserted to the left bronchus (). The patient’s oxygen level increased to 95% and his other vital signs stabilized. The patient was referred to the thoracic surgery department. He was hospitalized in the intensive care unit and an emergency operation was performed. During the operation, a right bronchus rupture was discovered 2 cm directly under the carina. An end-to-end anastomosis with interrupted suture was performed using 3.0 Vicryl. The patient recovered after the operation and was discharged from the hospital after 21 days without complications. |
A 75-year-old woman was referred to our hospital because of her mild exertional dyspnea and cardiac murmur. She had a long history of hypertension: 5 years earlier, she underwent a coronary angiographic study at our hospital because of her chest pain; no significant stenosis was found except for some minimal narrowing that was noted at the middle portion of the left anterior descending artery. During the same period, the precordial echocardiographic study revealed a slight left atrial enlargement and mild mitral regurgitation, but the quality of imaging resolution was poor and any further evaluation was not performed. The patient reported that she had been doing well until a few weeks earlier when she started suffering from a shortness of breath during mild exercise. Three days before her referral to the hospital, she had visited a general practitioner to get an evaluation of her symptoms. The doctor heard the murmur during the examination of the heart and he found blunting of the right costophrenic angle on the chest X-ray, so he referred her to us for further evaluation.\nAt the time of her second presentation, the patient had light dyspnea and a harsh pansystolic murmur (grade 3/6) was heard at the apex of the heart. The blood pressure was 140/80 mmHg. The EKG showed a sinus rhythm with nonspecific ST-T segment abnormalities. Echocardiography (Vivid 7, GE) revealed left ventricular hypertrophy and dilatation of the left atrium. The ventricle was not dilated and it had good contractility. There was also a very eccentric mitral regurgitation, but the morphology of the valve and the severity of the mitral regurgitation could not be precisely determined due to the poor image quality of the patient's test results.\nTransesophageal echocardiography (TEE) clearly showed that the mitral valve was composed of two distinct orifices that were separated by a central fibrous bridge. It is interesting that each orifice had its own chordae tendineae tethered to a different papillary muscle (). The transgastric short axis view revealed a larger posteromedial orifice and a smaller anterolateral orifice (). The posteromedial orifice was competent without any evidence of regurgitation or stenosis. At the level of anterolateral orifice, the color Doppler revealed moderate to severe eccentric regurgitation due to the flail posterior leaflet (). No other congenital cardiac anomalies were noted except for the persistent left superior vena cava that was demonstrated by injecting agitated saline at the antecubital vein of the left arm (). The patient became asymptomatic with the administration of angiotensin-converting enzyme inhibitor and diuretics. She was scheduled for long term follow-up and prophylaxis was recommended for any potential infectious endocarditis. |
A 55-year-old man with hypertension and diabetes presents with new awareness of blurred vision OD for two months. The visual acuity was 20/25 OD and 20/20 OS. Goldmann perimetry demonstrated an inferior altitudinal visual field loss OD. The pupil examination showed a relative afferent pupillary defect (RAPD) OD. Ophthalmoscopy showed a pale optic nerve OD. The eye exam was normal OS. A diagnosis of “optic atrophy” OD was made and the patient was instructed to return in 3 months for a repeat visual field. The patient returns in 3 months and the vision is now counting fingers OD. Magnetic resonance imaging of the head and orbit showed optic nerve sheath enhancement OD consistent with an optic nerve sheath meningioma.\nOptic atrophy is not a diagnosis. The clinician should document a cause for the optic atrophy and if no etiology is established a neuroimaging study and other evaluation should be performed. In a 55-year-old vasculopathic male the most likely etiology for a unilateral optic neuropathy with optic atrophy is non-arteritic anterior ischemic optic neuropathy (NA-AION). The “anterior” in NA-AION refers to optic disc edema and the absence of documented disc edema phase should prompt consideration for evaluation for alternative etiologies. In this case the patient experienced acute awareness of his visual loss OD rather than acute onset and the disc edema phase was not seen. Although serial visual field and follow up for a presumptive diagnosis of optic atrophy due to NA-AION is reasonable the clinical plan should be well documented in the chart, the patient should be informed of the possibility for a compressive or other etiology, and imaging and other evaluation should be pursued if the patient has progressive signs or symptoms (as in this case).\nLee et al. previously reported on the diagnostic yield for the evaluation of isolated and unexplained optic atrophy. This study was a retrospective review of all charts with the diagnosis of optic atrophy. Included patients were adults with isolated, but unexplained, optic atrophy and patients were excluded if they were children, had incomplete or inadequate documentation of the findings, had non-neurologically isolated optic atrophy (e.g., other localizing findings), or had a history (e.g., prior neuroimaging study showed a compressive lesion, prior ischemic optic neuropathy) or examination (e.g., central retinal artery occlusion) evidence for an etiology for the optic atrophy. A total of 1110 charts with the diagnosis of optic atrophy were reviewed from the two participating institutions (368 from the University of Cincinnati and 742 from the University of Iowa). Of these 1110 charts, 91 (8%) with isolated unexplained optic atrophy were included, and 1019 charts (92%) were excluded. Of the 91 cases included 18 (20%) had an underlying compressive lesion causing optic atrophy and 73 (80%) cases had no etiology for the optic atrophy on neuroimaging. Of the 18 patients with abnormal neuroimaging studies (e.g., meningioma, pituitary adenoma, or craniopharyngioma) 11 had bilateral and 7 had unilateral optic atrophy. Five of the 18 patients had progressive visual loss, 3 had hemianopic visual field loss, and 11 were younger than 50 years old. This study demonstrates that the risk of missing a compressive lesion in unexplained optic atrophy is high (20%) and that clinicians should not make the diagnosis of optic atrophy alone without an underlying proven or postulated mechanism. |
We present a case of a 34 year old woman who was diagnosed as bone lymphangiomatosis in the femur and tibia. The patient had undergone surgery for the lower limb discrepancy. Epiphysiodesis distal of the right femur was done at the age of 12 years and left femoral lengthening at the age of 14 years. Since, the age of 21 years the patient has had symptoms of lymphangitis, with an acute inflammatory process in the left thigh and leg as well as signs and symptoms of phlogosis, pain, edema, functional impairment and a fever of 39.5°C. Laboratory tests showed leukocytosis with left shift. Throughout the years, she suffered an additional thirteen episodes of lymphangitis, which was bacterial in some cases.\nAt the age of 29, the patient underwent curettage and filling of the femoral and tibial cavities with freeze-dried bone and autogenous cancellous bone from the iliac crest. As a result of these procedures, it was observed that this was a particular case of dysplasia as there was an accumulation of lymph in the interior of the cavity as well, which suggested lymphatic dysplasia or bone lymphangiomatosis. Reabsorption of the bone grafts was later observed, which coincided with two additional episodes of lymphangitis.\nThe differential diagnosis included neoplastic and proliferative bone marrow disorders, especially histiocytosis; as well as benign lesions such as solitary bone cysts, fibrous dysplasia, fibrous bone defects, cystic fibrous osteitis resulting from hyperparathyroidism and congenital generalized fibromatosis. She was diagnosed by histopathological study and she was treated by percutaneous osteoplasty and was followed for 2 years and 10 months in the femur and 2 years and 3 months in tibia.\nThe radiographic imaging preosteoplasty showed large areas of medullary resorption and cortical thinning of distal femoral and proximal tibial epiphysio metaphysial region [], CT scans revealed decreased trabeculation and endosteal scalloping with cortical thinning []. Magnetic resonance imaging (MRI) scans revealed diffuse lymphangiomatosis of bones and soft-tissues [].\nHistological sections stained with H and E, which revealed dense fibro-connective tissue with an abundance of large-caliber vascular channels []. Said channels were lined by flat monostratified epithelium, which tested positive for D2-40 (antibody that specifically stains lymphatic endothelial cells) through a specific immunohistochemical technique []. The postoperative images show cavities filled with acrylic cement in tibia (December 2010) [] and femur (May 2010) []. After over 2 years of followup (2 years and 10 months in the femur and 2 years and 3 months in tibia) there are no signs of disease progression at this level in the radiographic images []. |
An 18-year-old female with a past medical history of migraine headaches presented to the hospital with chief complaints of persistent nausea, vomiting, inability to tolerate oral feeds, and significant weight loss for the past 2 months. The patient reported that she vomited after every meal, and vomitus mainly consisted of undigested food particles without blood. The patient also had intermitted constipation; however, she had no diarrhea. She also observed substantial weight loss despite good appetite but was unable to quantify the weight loss. The patient did not take any medications for her migraine headaches and her last flare-up was about 1 year ago. She reported multiple outpatient visits for similar complaints in the last 2 months; however, on this visit with her primary care provider, she was referred to the hospital. On clinical evaluation, she had stable vital signs. Physical examination revealed dry mucous membranes and abdominal examination was positive for mild epigastric tenderness along with decreased bowel sounds. The patient was started on intravenous (IV) fluids. Laboratory investigations such as complete blood count and comprehensive metabolic panel were ordered, which revealed a low hemoglobin level of 10.2 g/dL, hematocrit 30.2%, serum sodium 132 mEq/L, and serum potassium 3.3 mEq/L. An abdominal X-ray did not reveal mechanical obstruction. Due to concerns for gastroparesis (GP) as the underlying etiology, a Gastric Emptying Study was ordered, which revealed markedly delayed gastric emptying time as the tracer was distributed in the stomach on initial views with activity not appearing in the duodenum until 45 minutes and emptying half time was not reached with 77% of initial gastric contents remaining at the end of the examination. Hence, a diagnosis of GP was established. The patient was started on metoclopramide, which led to improvement of her presenting symptoms, and she was eventually discharged home with recommendations to taper the doses of metoclopramide as needed.\nOne month after discharge, the patient presented to the hospital with similar complaints as her initial presentation despite metoclopramide therapy. Laboratory investigations were similar to initial hospitalization and revealed hemoglobin level of 10.7 g/dL, hematocrit 31.9%, serum sodium 133 mEq/L, and serum potassium 3.2 mEq/L. An abdominal X-ray was ordered, which revealed dilated bowel segments with high burden of stool in the colon without obvious mechanical obstruction. A diagnosis of severe GP and chronic intestinal pseudo-obstruction (CIPO) was established. Over the next month, the patient was given an extensive trial of numerous prokinetic agents such as mirtazapine, ondansetron, pyridostigmine, octreotide, and promethazine, but she failed to show clinical improvement. With failure of medical therapy, a nasojejunal feeding tube was placed for nutrition; however, the patient noted worsening nausea despite the slow feeding rate of 10 mL/h. Hence, after detailed discussions with the patient, a decision was made to start her on total parenteral nutrition (TPN) after which she was transferred to a tertiary center for higher level of care. At the tertiary care center, she was continued on TPN and underwent extensive evaluation. Laboratory investigations were within normal limits except mild elevation of the liver enzymes. Additionally, antinuclear antibody, extractable nuclear antigens panel, paraneoplastic antibody panel, and acetylcholine receptor binding antibodies were found to be negative, thereby ruling out an autoimmune etiology. A right upper quadrant ultrasound revealed the presence of gallbladder sludge and normal liver architecture. An esophagogastroduodenoscopy (EGD) and colonoscopy were found to be negative. A small bowel follow-through ruled out mechanical obstruction. Magnetic resonance enterography and computed tomography angiography of the abdomen were noted to be unremarkable. Furthermore, a deep rectal biopsy was performed, which was negative for amyloidosis. However, antroduodenal manometry, a diagnostic tool for GI motility disorders, showed paucity of contractile activity with very low amplitudes highly suggestive of a myopathic etiology. This was followed up with a laparoscopic full-thickness intestinal biopsy, which revealed decreased actin staining in the circular muscles of the muscularis propria of small intestine consistent with a diagnosis of HVM. During the course of the hospital stay, the patient developed recurrent bacteremia, the source of which was identified to be the central venous catheter used for TPN. She was treated with appropriate antibiotics, which led to resolution of the bacteremia. Eventually, she underwent isolated small intestine transplant and was started on mycophenolate 500 mg daily, everolimus 5 mg daily, and prednisone 10 mg daily. This led to eventual resolution of her presenting symptoms, and she was discharged home. The patient continues to be asymptomatic and follows up with Gastroenterology and Transplant Surgery regularly. |
We present the case of a 64-year-old Caucasian female who was treated at our department because of the deformity of her left foot. Because of this deformity, she was completely disabled wearing ready-made shoes. She felt pain in the forefoot on dayly basis. Medial side of the thumb and dorsal side of the second toe were sometimes inflammed including secretion. Four years earlier, an orthopaedic surgeon advised her surgery, which among other things included the amputation of the second toe, and the patient had not consented. On several occasions, she underwent physical therapy consisting of analgesic and anti-inflammatory procedures and exercises to strengthen the muscles of the foot and lower leg. The physical therapy she performed had no positive effect. In her medical history, there were no serious diseases; she was being treated for hypertension at the time.\nClinical examination verifies the pronounced deformation of the left forefoot in terms of the valgus position of the thumb, crossover second toe, and third claw toe, with a distinct contracture of involved joints ().\nShe had painful plantar callosity in the projection of the third metatarsal head. The X-ray of her left foot showed a severe deformity of the second toe and its overlapping and complete dislocation of the second and third metatarsophalangeal joints. X-ray also showed a high degree of valgus position of the thumb with severe arthrosis of the first MTP joint with medial osteophytes ().\nAfter preoperative preparation, we performed the surgery in regional anesthesia (spinal block) with antibiotic prophylaxis. On the thumb, planned resection of the base of the proximal phalanx is made (classic intervention by Keller). Due to the extreme rigid deformities of the second toe and because of complete dislocations in the second MTP joint, we made a decision to do complete extraction of the proximal phalanx of the second toe. On the third toe, we did a resection of the head of the proximal phalanx, and actually we performed resection arthroplasty of the third MTP joint. Because of the painful plantar callosity in the projection of the third metatarsal head, we performed an oblique subcapital osteotomy of the third metatarsal bone (procedure by Helal) (Figures and ).\nWe had an idea to do the same operative procedure on the second metatarsal bone, but because of the risk of circulatory collapse, we made a decision not to do that procedure in the same time.\nThe postoperative recovery was normal. From the first postoperative day, full weightbearing with a maximum load on the left heel was allowed. On the second postoperative day, she was discharged to home care, and on the twelfth postoperative day, the sutures were removed. Two weeks after surgery, full weightbearing without any restrictions (to the pain tolerance) was allowed. Physical therapy started two weeks after surgery, lasted three weeks, and was performed three times a week. The therapy consisted of stretching the toes and ankles to achieve a normal range of motion, anti-inflammatory procedures and lymphatic drainage in order to reduce swelling of the forefoot, and muscle strengthening exercises. A control radiograph showed the correct position of the thumb and the second and third toes, including the place of the third metatarsal osteotomy (); three months after surgery, she was able to wear all kinds of shoes including thong slippers ().\nThe only complaint about her left foot was a mild plantar pain in the projection of the second metatarsal head after walking on longer distances. Despite these subjective symptoms, she was very satisfied with the status of the operated foot and the final result of operative treatment. |
A 41-year-old female suddenly developed headache and nausea. She visited a local physician, and was referred to one of our hospitals. On admission, neither consciousness disturbance nor neurological deficit was observed. Her past history included scoliosis since birth; however, other diseases such as infection or connective tissue disease were not noted. A routine blood examination was not remarkable. Further, the examination of antigens and antibodies for infectious diseases also revealed no abnormality. Computed tomography (CT) demonstrated SAH [], and three-dimensional CT angiography (3D-CTA) showed a dilated M2 portion of the right MCA accompanied by two small notches []. Magnetic resonance imaging (MRI) performed 4 months prior to the episode had already revealed the irregular wall of the right M2 []. MRI performed at admission showed the same findings as those of 3D-CTA []. Cerebral angiography revealed the dilated M2 of the right MCA []. The wall of the dilated portion was relatively smooth. Angiography neither showed a double lumen, pearl and string sign nor the retention of contrast medium. Based on these radiological findings, the lesion was diagnosed as a fusiform aneurysm, and delayed surgical intervention was planned. Repeated angiography was performed on the 23rd day after onset. The radiological findings showed no marked change of the lesion. The third MRI 33 days after the onset showed the same findings as those of the first and second MRI.\nShe underwent surgery on the 45th day after onset. Right fronto-temporal craniotomy and superficial temporal artery (STA)-MCA anastomosis were performed. Then, the lesion was trapped and resected. The lesion consisted of a dilated arterial trunk with a whitish, thickened wall and two protrusions with red walls on the main lesion []. There was no organized thrombus around the aneurysm. Pathological examination of the resected lesion showed a disrupted internal elastic lamina and intimal thickening of the main trunk []. The large protrusion on the main trunk had a dissected intima without smooth muscle cells stained with anti-α-smooth muscle actin antibody [Figure , ]. In contrast, the small protrusion had no intimal flap, and no internal elastic lamina was observed in the dome [Figure and ]. Postoperative angiography showed the disappearance of the aneurysm and patency of the bypass. Her postoperative course was uneventful. She was discharged without neurological deficit on the 62nd day after onset. |
A 70-year-old man was diagnosed with right TN eight years ago. He took carbamazepine 300 mg three times a day and gabapentin 300 mg three times a day to relieve his facial pain. However, the treatment was not sufficient enough. He then underwent Gamma Knife radiosurgery (GKS) for TN five years ago. The problem was initially relieved after GKS, but TN relapsed four months later. Afterward, he underwent two times percutaneous nerve block and two times radiofrequency ablation. Meanwhile, he had developed essential tremor twenty years ago, and he took clonazepam 0.25 mg once a day and propranolol 60 mg twice a day. Also, he took aspirin for an unclear purpose. For persistent and medically intractable TN, the patients eventually underwent MVD. He stopped taking aspirin two weeks before surgery and did not take it again after surgery. In the supine position, his head was turned to the left less than 90 degrees, and the neck was flexed, with vertex being parallel to the floor. During skin incision and craniotomy, the bleeding tendency was detected, but not to the extent that it could not be controlled surgically. His preoperative clotting profile was within the normal range. When the dura was opened, the usual amount of CSF was drained with the typical speed. Arachnoid dissection was performed around the trigeminal nerve and surrounding vessels in the usual manner. Due to the age-related atrophic brain, additional CSF drainage was not necessary to make working space. A superior cerebellar artery was adhered to the trigeminal nerve and compressed the nerve at the root entry zone and mid-cisternal segment. Large superior petrosal veins were crossing over the surgical approach field of view. During the dissection between the offending vessel and the trigeminal nerve, the superior petrosal vein was slightly stretched, resulting in venous bleeding. Bleeding was entirely controlled by compression and using hemostatic materials. The offending vessel was successfully transposed not to conflict with the trigeminal nerve, and Teflon was inserted between the nerve and the vessel. There was no unusual finding except a mild degree of pneumocephalus on immediate postoperative brain computed tomography (CT). The patient’s pain alleviated immediately after surgery. He stopped taking carbamazepine and gabapentin. However, due to the recent aggravation of his essential tremor, he started taking topiramate 50 mg once a day in addition to propranolol and clonazepam. On the 4th day after surgery brain CT, an extra-axial fluid collection with CSF density with 7 mm thickness along the right cerebral convexity was observed. Still, there was no mass effect, and the patient was discharged (). A month later, he presented with worsening dizziness and headache. He tended to veer to the left and reported left-sided weakness without a history of head trauma. Brain CT showed CSDH in the right cerebral convexity. He underwent burr hole trephination to evacuate the CSDH. Although CSDH was completely drained, the brain was not fully re-expanded on 2nd day after the surgery brain CT, and there was still an excessive subdural space filled with CSF (). A month later, his subsequent CT scan showed a larger amount of CSDH mixed with acute stage hematoma in the right convexity. He underwent a repeated burr hole trephination, and a subdural catheter was inserted. A small amount of acute staged hematoma remained in the subdural space on the day after surgery brain CT. We considered that the patient was at high risk of CSDH recurrence due to age-related atrophic brain and bleeding tendency in the first intraoperative findings. Thus, we performed additional MMA embolization. There was still excessive subdural space filled with subacute staged hemorrhage on CT scan a month later, but the subdural space extent did not increase. Brain CT 3 months after the second surgery showed complete CSDH resolution and full expansion of the brain parenchyma. After that, he did not suffer from a recurrence of CSDH (). |
A 56-year-old man presented with diplopia due to the right oculomotor nerve palsy. The magnetic resonance imaging (MRI) results revealed a large sphenoidal tumor []. The patient underwent tumor resection, and the pathological diagnosis revealed an atypical meningioma. Diplopia improved after tumor resection and only a slight adduction disturbance of the right eye persisted. During the next 7 years, he underwent local radiation therapy at a total dose of 50 Gy, second tumor resection, and Gamma Knife (GK) radiosurgery to control the tumor’s repetitive recurrence around the right anterior clinoid process. After the GK radiosurgery, residual tumor was controlled for the next 3 years [].\nThree months after his last visit, he started to suffer from intermittent diplopia. MRI revealed a rapid growth of the posterior part of the residual tumor [], and heavily T2-weighted imaging showed the involvement of the right oculomotor nerve within the tumor []. His symptom was intermittent involuntary diplopia, an involuntary inward deviation of his right eye, which resolved within 1 min [, ]. The symptom was mostly triggered by tight eye closing, which frequently occurred every day. The ophthalmological examination of the right eye showed restricted eye movement during upward and medial gaze []. The ophthalmologist diagnosed this intermittent involuntary symptom as ONM. The results of the ophthalmological examination also revealed worsening of the right vision and nasal hemianopia [].\nThe third tumor resection was performed through the right pterional approach to prevent further visual disturbance aggravation. After the removal of the tumor occupying the right retrocarotid space, the oculomotor nerve was found within the tumor and was preserved []. The oculomotor nerve was tightened by the surrounding tumor. We found no neurovascular contact between the oculomotor nerve and vascular structures. Most of the tumor was removed, except for the part that was tightly adhered to the right optic nerve and optic chiasm, achieving a sufficient neural decompression []. The tumor was pathologically diagnosed as an atypical meningioma with a MIB-1 labeling index of 12.4%.\nONM disappeared immediately after surgery. A transient worsening of the right oculomotor nerve palsy occurred, which gradually recovered in the preoperative level [, ]. The right visual disturbance and nasal hemianopia continued after surgery. The postoperative course was uneventful. The patient was discharged 15 days after surgery and he remained free from ONM until the last follow-up visit (9 months after surgery). |
A 42-year-old male fell during work on a fence resulting in a severe perineal impalement injury leading to life threatening bleeding and an obvious anal injury. A midline lower abdominal incision was made. No intraperitoneal injuries could be seen, and a defunctioning colostomy in the left lower abdomen was done. The perineum was debrided and the open anal mucosa sutured. At this stage no attempt was made to reconstruct the anal sphincter mechanism. The right internal iliac artery was ligated, but the bleeding could only be controlled by packing the small pelvis with large swabs. This was done through the open perineum. After patient stabilization, a helicopter transfer was organized to a tertiary trauma centre. The CT scan at that stage showed isolated extra peritoneal soft tissue injury from the perineum extending into the left small pelvis (). No active bleeding, bowel, or bladder injury could be determined. Clinical examination confirmed an open book fracture of the pelvis, total ventral full thickness disruption of the anal sphincter complex, and total disruption of the dorsal urinary sphincter complex. There was a big defect in the perineum, demonstrating the extent of the impalement injury (Figures and ). Both the anal and urethral sphincters were repaired using an approximation technique. Mattress sutures were used for the anal sphincter and simple sutures were used for the urethral sphincter. The corpora cavernosa that were ripped far apart were also approximated ventral to the sphincteric urethra. After sphincter repair, a vacuum device was placed until it was safe to perform secondary wound closure. The transurethral catheter was left in position for three weeks. A micturating cystourethrogram was done at the time of catheter removal and no extravasation was noted. At this time, the patient was still incontinent of urine and a transurethral catheter was placed for another 6 weeks until the patient was mobilized. The patient became continent after pelvic floor exercises accompanied by a pelvic floor-trained physiotherapist after six months. |
A 25-year-old woman visited Department of Internal Medicine, Kyungpook National University Medical Center due to multiple colon polyps that had been detected incidentally. She had been healthy and had no remarkable medical history. According to her family history, her mother had colon cancer, and her brother had undergone a prophylactic colectomy for the treatment of FAP. The results of physical examination and laboratory data, including complete blood count and serum carcinoembryonic antigen level, were all within normal limits. Esophagogastroduodenoscopy showed a normal mucosa finding. Colonoscopy examination revealed hundreds of polyps carpeting the entire colon and rectum, consistent with FAP (). Histologically, these polyps were all proven to be tubular adenomas. Because the standard therapy is a prophylactic total colectomy, we recommended that she undergo a prophylactic surgical treatment. However, the patient was fully aware of the unfavorable effect that a total colectomy had had on her brother. In addition, she was 25 and single. She had no large-sized polyps and no colon cancer. Her family agreed with our second proposal, which was not to perform surgical treatment until the subject was in her late twenties, if there was no apparent development of colonic cancer. Therefore, we delayed the prophylactic colectomy and decided on a treatment with oral sulindac, 100 mg twice a day.\nAfter the sulindac medication had been taken for 5 months, colonoscopy showed that the number and the size of polyps had been drastically decreased and that the polyps had almost completely disappeared (). Sulindac was found to have a sustained effect on the complete regression of colonic adenomas without the need for a polypectomy. Mild nausea was a side effect of the medication. However, after a few days, symptoms had disappeared without treatment. After the sulindac medication had been taken for 25 months, colonoscopy showed several sporadic, tiny polyps (). Thus, the effect of sulindac on the regression of the colonic adenomas had been maintained. We had been continuously treating her with oral sulindac, 100 mg twice a day. Esophagogastroduodenoscopy and colonoscopy were scheduled at regular intervals of one year. |
We evaluated a 41-year-old male presented in September 2004 with an episode of acute gastrointestinal bleeding, which was associated with non-Hodgkin's lymphoma with a nonspecific subtype. The patient lived in a remote rural area in Northern Brazil and was promptly transferred to a larger center to receive chemotherapy to avoid a surgical procedure.\nHe was treated with three cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), without clinical response. He was then submitted to a surgical procedure and the anatomopathological study of retroperitoneal lymph nodes was consistent with an infiltrative carcinoma. The immunohistochemical analysis revealed chromogranin and synaptophysin expression and also a Ki 67 < 3%. At this moment carcinoid related markers (glucagon, gastrine, prolactin, PTH, 5HIAA, and chromogranin A) were normal. Because of the discordance between the two biopsies regarding the histological diagnoses, a review was performed in both specimens and there were no histological findings suggesting lymphoma and the hypothesis of a low grade neuroendocrine tumor was confirmed. The patient was then treated with octreotide acetate until clinical recovery and then, without any clinical or radiological evidence of disease, treatment was withheld.\nOn December 2010, he presented with an abdominal obstructive syndrome and an exploratory laparotomy was necessary. The anatomopathological diagnosis of mesenteric lymph nodes resected was consistent with areas containing marginal zone B-cell lymphoma/chronic lymphocytic leukemia and neuroendocrine tumor. Bone marrow was not infiltrated and FDG-PET showed exclusively abdominal nodal disease which was compatible with lymphoma involvement. At this time he was treated with 6 cycles of CHOP and octreotide was started again. CT scan obtained at the end of the treatment showed complete response.\nThe patient's family history was revealed to be very unusual. He referred to the fact that his father had a prostate cancer and the majority (if not all) of the 9 father brothers presented lymphoma or leukemia. These data could not be double checked since these individuals have died many years before in very distant places, but the fact that the patient was able to describe a very unique type of cancer in his relatives, many of them with low level of literacy, suggested that the presence of lymphoproliferative disorders is quite common in the paternal side of the family.\nAt clinical examination the patient lacked any cutaneous stigma of neurofibromatosis, but one of the CT scans revealed the presence of various neurofibromas in the thorax and abdomen (). He was tested for NF1 gene mutations and chromosomal abnormalities encompassing part of the long arm of chromosome 17 containing the NF1 gene.\nThe coding region of the NF-1 gene was analyzed by real-time polymerase chain reaction (RT-PCR) and direct sequencing. Multiplex ligation-dependent probe amplification (MLPA) was also performed to detect the number of mutant copies (deletions and duplications).\nThe following alterations were met: duplication of NF1, TRAF4, and MYO1D. Additionally, fluorescence in situ hybridization (FISH) using probes (RP5-1002G3 and RP5-92689) flanking the NF1 gene in 17q11.2 and CEP17 for 17q11.11.1 was performed. There were three signals (RP5-1002G3conRP5-92689) in the interphases analyzed and two signals (RP5-1002G3conRP5-92689) in 93% of cells. Those findings suggest that the patient had a tandem duplication of 17q11.2.\nOn June 2013, the patient was submitted to routine laboratorial tests and a blood workout displayed a lymphocytosis (7500 lymphocytes/dL). The immunophenotype of the lymphocyte population was mostly composed of a clonal mature B lymphocytes positive for CD19, CD5, CD20, and CD23. These findings were consistent with CLL. FISH analysis displayed a del 17p13.1 ().\nThe patient has been in treatment with octreotide acetate, without signals of disease progression. CLL is under clinical surveillance and the patient is out of treatment in the moment. |
In a 68-year-old man, a large abdominal tumoral mass was discovered incidentally during checkup for vascular insufficiency of the lower limbs. The patient complained of vascular insufficiency and had no symptoms of abdominal pain. Physical examination revealed a palpable mass in the right upper quadrant of the abdomen. Results of blood samples were all within normal values.\nUltrasonography showed a well-defined large cystic mass with several membranous septa with a diameter of approximately 12 cm in the right hypochondrium (). Subsequent computed tomography (CT) scan demonstrated a large cystic lesion adjacent to the right liver lobe, the stomach, and the pancreatic head (). On MRI imaging, markedly high intensity, compatible with cystic nature, was revealed in the tumor. The tumor was lying in contact with the right liver lobe, the stomach, and the pancreatic head ().\nHowever, no clear connection with any of these organs was identified. The imaging findings demonstrated no vascularity of the lesion. Malignancy could not be excluded, and surgery was indicated.\nAt laparotomy a large exophytic mobile mass was found to be attached by a narrow stalk to the larger curvature of the stomach at the level of the antrum. No infiltration into the surrounding tissues was observed.\nSurgical resection of the mass along with a wedge resection of the stomach, at the larger curvature, adjoining the stalk and the adherent omentum was performed.\nThe resected tumor measured almost 12 cm in diameter. Grossly, it was a unilocular cystic tumor filled with serous fluid, with a smooth outer surface and several membranous septa projecting in the lumen (). The cyst had a thin wall. The stalk was connecting the cystic tumor with the stomach. The mitotic activity was less than 5 mitoses per 50 HPF. The tumor cells showed positivity for CD117 (KIT) and CD34. The histological features together with the immunohistiochemical profile of the tumor cells were consistent with a gastrointestinal stromal tumor (GIST) with a high risk of progressive disease according to the Fletcher classification. Molecular analysis of this tumor showed a mutation in exon 14 of the PDGFRA gene (substitution exon 14, N659 K). Since PDGFRA exon 14 mutant GIST is sensitive to imatinib mesylate (Glivec), this oral treatment was started postoperatively because the high risk of recurrence in this patient (high risk of progressive disease according to the Fletcher classification). Untill now the patient is still free of recurrence. |
A 28-year-old African black male was referred to a hospital in Tanzania for an intraoral radiography. His major complaint was an acute, diffuse-patterned oral pain in the left mandibular area. The patient had no significant previous medical history. A clinical examination confirmed satisfactory dental hygiene without any apparently relevant findings. The patient explained that his mandibular left third molar had been extracted six month previously and that the surgery had been quite laborious and lengthy. Although the healing process had been satisfactory, considerable pain had recently begun in this area. The dental X-ray showed an image consistent with a piece of metal embedded in a subgingival distal caries at the mandibular left second molar (37), which was probably related to a previous tooth impaction process of the mandibular left third molar (38). Two periapical lesions detected at the mandibular left second molar roots might have justified his acute tooth ache due to acute exacerbation (Figure ).\nInitially, the differential diagnosis of this radiographic finding was that of a possibly loosened or released piece of amalgam. Nevertheless, the patient had never previously undergone any dental fillings. After repeating the X-ray twice in order to rule out a possible image artefact, it was concluded that during the third molar extraction a curved blade elevator tip had fractured and impacted into the subgingival caries cavity. As the patient had only been referred for a dental X-ray he was given a written note explaining the finding. His dentist was informed about the need to perform an occlusal access cavity in order to initiate a root canal treatment and remove the foreign body from the second molar (37).\nThe breakage of some instruments, such as endodontic files and dental burs, due to a number of factors including defective manufacturing, stress, fatigue, rust, and poor handling is not unknown in dentistry []. Few papers, however, in the literature have dealt with the breakage of instruments used for exodontias. Yasuhara et al., registered various medical accidents caused by defective surgical instruments over two years. In the maxillofacial speciality the authors reported 7 incidences out of 548 operations []. According to Kluess et al., it is important that any incident with orthopaedic surgical instruments should be reported to the manufacturer and the health authorities for sufficient processing and risk assessment of the accident []. In the case of some reusable metal instruments both titanium alloys and stainless steel are in the high performance range. The latter is the most widely used material for instruments and, according to surgical requirements, its alloys vary: the most frequent being martensitic and austenitic stainless steels. Biomedical cutting instruments are often made of martensitic stainless steel due to its pronounced durability coupled with acceptable corrosion resistance. Surgical instruments that may be subject to high pressure forces, such as a dental elevator, are composed of austenitic stainless steel as it is less brittle []. A safe and effective elevator should have extreme values for torque, and high stress values [].\nSurgical instruments manufactures should carry out strict quality controls and have their instruments bear a visible mark as a sign of guarantee. Various authors have suggested that the inferior quality of some surgical instruments may be a reflection of poor working conditions and low standards, particularly in the developing world. Responsibility lies with the suppliers from developed countries manufacturing in the developing world who behave in an unethical manner, maximising profits and minimising the remuneration of the people who actually produce the goods [-].\nThe location and retrieval of broken fragments during a tooth extraction procedure should not be a serious problem, in most cases the fragment is immediately identified. Any instrument breakage implies the obligation to search for the fractured fragment and remove it in order to avoid possible infection and prevent complications due to swallowing or aspiration of the fragment []. Some of the metallic pieces of the surgical instrument could end up in a fibrous issue capsule and gain access to the adjacent spaces []. The case we report here is unusual in that the elevator tip was embedded in a subgingival caries and remained there asymptomatically for approximately six months.\nAt present there are a number of radiological explorations to identify metallic foreign objects. Cone beam computerized tomography (CBCT) is an excellent tool to locate metallic foreign objects []. However, a single periapical radiograph or using more than one radiograph to apply a tube-shift technique, may be sufficient. Whenever possible, simpler techniques should be first applied. In addition, occlusal radiography can also be employed as necessary. If an occlusal film is not available a periapical radiograph can be put on the surface of the tooth, or on the edentulous crest, and this may reveal an embedded foreign object. If with using these techniques, the foreign object is not detected, then other more sophisticated techniques such as CBCT should be applied. In our case, only conventional intraoral periapical radiographic imaging was available, due to limited hospital facilities, to inform the referral doctor about our findings.\nThe care of medical and surgical instruments has a decisive effect on their efficiency and durability. Damage due to breakage and scratches is usually caused by their being incorrectly stored and secured during sterilization, or being carelessly positioned in the treatment area thus favouring falls. Additionally, metal instruments used in clinical practice may be subjected to fatigue due to the effects of sterilization processes. Manufacturers recommend that all instruments made of metal should be checked regularly before packaging in order to diminish the risk of possible incidences [].\nThe concept of honest and correct communication between physician and patient is a crucial issue. It is linked to the disclosure of adverse events and errors, a complex topic covering medical, psychological, legal, and ethical aspects [-]. Current socio-cultural trends, which condition the medical profession in a variety of ways, and a greater focus on the dignity and rights of the patient, have led to a growing tendency to fully inform patients with regard to their illness, progress, and therapy. Nevertheless, it is still common for doctors to choose to remain silent or manipulate the truth in some way, especially when the prognosis or accident is serious or negative and the patient seems to be having real difficulties accepting it []. As clinicians, this reported case should be helpful by reminding us of the importance of our ethical code with respect to the patients, especially when an adverse eventuality appears during a surgical procedure. |
A 93-year-old female patient sought treatment in the emergency room of the University Hospital of the University of São Paulo, presenting with acute upper gastrointestinal bleeding. She had a clinical history of hypertension and cardiomyopathy with chronic atrial fibrillation. She reported dyspnea on mild exertion, together with chronic leg edema. She had been under treatment with carvedilol, captopril, furosemide, and spironolactone for the last 2 years. She complained of epigastric and substernal pain followed by evacuation resembling melena for the last few days before admission. Her first upper gastrointestinal bleeding episode was characterized by the emission of a small amount of blood mixed with saliva from her mouth while sleeping, as noted by her family. Because the bleeding was minimal, she was taken to a primary care facility, where she remained under clinical observation after being started on vitamin K, omeprazole, and intravenous saline infusion. On the next day, she began to bleed again and was referred to our hospital for endoscopic examination. At admission, she was conscious and lucid, although emaciated and pale. There were traces of blood in the oral cavity. Her blood pressure was 95/69 mmHg, and her pulse was 100 bpm. Pulmonary examination revealed decreased breath sounds at the right lung base. The abdomen was slightly painful on gentle palpation of the epigastrium. Rectal examination failed to reveal melena. The laboratory test results are shown in .\nUpper gastrointestinal endoscopy revealed pulsatile extrinsic compression of the esophagus at 25 cm from the superior dental arch (). At the most prominent area of the mass, we observed a small ulceration of the mucosa with whitish material, interpreted as probable atheroma, emerging into the esophageal lumen (). Because of these findings, the examination was promptly interrupted.\nThe patient was submitted to multidetector computed tomography (CT) of the chest. The scan showed an elongated, tortuous, atheromatous aorta, with wall thickening and calcified plaques. The descending aorta was irregular and fusiform, with an average diameter of 46 mm. Multiple parietal ulcers were seen on the descending aorta. A fistula, measuring 15 mm in diameter, was observed in the proximal descending aorta, with active contrast extravasation generating a hematoma of 60 × 46 mm, which compressed the esophagus ().\nA massive thrombus was observed on the descending thoracic aorta extending inferiorly to the thoracoabdominal junction (, and ).\nConsidering the severity of the illness, the advanced age of the patient, the presence of multiple comorbidities, and the relative hemodynamic stability, she was immediately referred to a tertiary hospital for surgical treatment. However, extensive rebleeding and cardiac arrest resulted in her death prior to surgery. |
This is a case of a 3-year-old male patient, with no medical or dental history of interest, suffered a dental trauma after a domestic accident and went as an emergency to the Pediatric Dentistry Service of the Hospital.\nChief complaint: his mother went to the emergency room with the fragment of her child's tooth root in her hand, explaining that she had found this “loose canine in the child's mouth” after a dental trauma. In the physical examination, the following was found: a bruise, abrasion, and laceration of the maxillary buccal mucosa and gingiva of the primary maxillary central incisors zone. There was mobility in the primary maxillary right central incisor and light mobility without displacement in the primary maxillary left central incisor. The clinical and radiological exploration of the lateral incisors was normal, without any pathology. The patient presented with anterior open bite because of pacifier habit persistence.\nPeriapical radiographic examination revealed the near complete absence of the primary maxillary right central incisor root. This exploration provided evidence that the patient had suffered a root fracture of the primary maxillary right central incisor (fracture located to cervical part of the root) accompanied by transalveolar and transmuco-gingival avulsion of the tooth root fragment (). The dental crypts of the maxillary alveolar process of the developing permanent teeth were found without any radiological alteration (the dental follicle of the permanent maxillary right central incisor was in 5 Nolla stage).\nRoot avulsion of the primary maxillary right central incisor, subluxation of the primary maxillary left central incisor, and fracture of the alveolar process were diagnosed.\nAn antibiotic regimen of amoxicillin-clavulanic acid was started as a treatment for one week. Soft diet was recommended and oral hygiene instructions were explained (brush with a soft brush after every meal). It was also recommended to apply chlorhexidine 0.1% topically to the affected area with cotton swabs twice a day for one week and to restrict the use of a pacifier, as well as to avoid putting pressure on the traumatized area.\nSeveral posttrauma follow-up appointments were performed, exactly 15 days, one month, and three months after the trauma.\nDuring the first examination, 15 days after the trauma, the clinical intraoral examination showed that the buccal mucosa of the primary maxillary right central incisor was healing, and the periapical radiography showed absence of the primary maxillary right central incisor root, but without any alteration or pathological image. The crown of the primary right central incisor did not present mobility (it was in its initial position in the dental arch) and there was not any risk of aspiration. In addition to this, the child had already given up the pacifier habit, as it was recommended in the posttrauma instructions.\nAnother examination was performed one month after the trauma. The clinical intraoral examination showed normal buccal mucosa and the primary maxillary right central incisor crown did not show mobility. A periapical radiography was also performed.\nAnother examination was performed 3 months after the trauma (). The clinical intraoral examination showed a perfectly healed vestibular mucosa with normal appearance and mobility of the primary maxillary right central incisor crown. A periapical radiography was also performed.\nAfter the last examination, the patient went to the emergency room three more times because he suffered different traumas on the primary maxillary right central incisor (playing football, hitting himself with a chair, and running). A lacerated contused wound in the lower lip was observed during the last physical trauma examination, as well as an abrasion injury on the chin ().\nFour months after the trauma, the primary maxillary right central crown remained in its position in the dental arch.\nAs there was a lack of occlusal contact with the opposing tooth, which would have caused the crown extrusion, the tooth remained in his mouth. This absence of occlusal contact was due to the anterior open bite caused by the former pacifier habit.\nSix months after the trauma, the patient's mother called to tell us that the primary maxillary right central incisor crown had been exfoliated and we appointed another examination to assess the state of the crown and alveolar bone ().\nAfter the exfoliation of the primary maxillary right central incisor crown, the growth and development of the patient had to be monitored in order to check the eruption of the permanent maxillary incisors. After an 8-year follow-up, the permanent maxillary central incisor had normally erupted (). |
A 57-year-old previously homeless male with a history of trauma from a bicycle crash originally presented to our level 1 trauma center for the evaluation of back and flank pain and a “click” in his chest. He was found to have an acute fracture of the left seventh rib and chronic nonunion of left eighth through twelfth ribs [ and ]. He underwent elective rib plating for symptom management in addition to evacuation of hemothorax and pulmonary decortication for fibrothorax and trapped lung. The seventh through tenth ribs were plated with a bicortical locking rib fixation system, and a 28 Blake chest tube was placed []. At least three bicortical locking screws were placed proximal and distal to the fracture locations. He tolerated the procedure well and had significant improvement in his symptoms on follow-up. He denied any residual pain or clicking of the ribs and could finally get a good night's sleep. An improvement in his symptoms greatly improved his quality of life so that he was able to obtain a job, was able to rent an apartment, and was no longer homeless.\nThree months later, he was riding his bicycle when he was again hit by a motor vehicle. In addition to numerous devastating injuries including a significant traumatic brain injury (TBI) prompting admission to our ICU, he was found to have flail chest with lateral and posterolateral rib fractures of the first through tenth ribs and periprosthetic fractures of the seventh through tenth ribs [ and ]. The rib plating hardware was completely intact, except for a single displaced screw from the seventh rib plate []. He was also found to have a hemothorax, for which a chest tube was placed. Notably, there was increased difficulty of the chest tube placement due to the prior pulmonary decortication and rib plating. His new rib fractures were treated nonoperatively due to his poor clinical status from his TBI. Unfortunately, he expired from his injuries. |
A 21-year-old female patient presented with instability of the left knee joint. She had no history of trauma but a continued feeling of instability during ambulation and experienced a feeling of giving way about two to three times a month. A physical examination showed no tenderness or swelling, and the range of motion was normal. The patient displayed some positive signs, grade 3 on Lachman's test and the anterior drawer test and grade 2 on the pivot shift test. The side to side difference in the anterior drawer test using the KT-2000 arthrometer was 15.3 mm.\nThe distal medial condyle of the left femur appeared hypoplastic on conventional radiographs. On scanogram, the left femur (37.8 cm) was shorter than the right femur (39.4 cm), while the left and right tibiae were the same length. Anterior and posterior simple radiographs showed hypoplasia of the left lateral tibial spine and a lateral view showed a decreased posterior tibial slope compared to that of the right side. On the Knee Lachman stress view, the side to side difference between each anterior drawer was determined to be 16.7 mm ().\nThe ACL was not visible on magnetic resonance imaging (MRI) scans and the posterior cruciate ligament (PCL) also seemed hypoplastic. The medial femoral condyle appeared comparatively smaller, the intercondylar notch was decreased in height and width, and the cartilaginous covering of the lateral intercondylar notch appeared broader compared to normal specimens ().\nReconstructive surgery of the cruciate ligament was planned and arthroscopy was performed. The arthroscopy showed that the ACL was absent, the PCL was maintained, and the lateral femoral intercondylar notch was broadly covered with cartilage as seen on the preoperative MRI. An incomplete longitudinal tear, about 5 mm in size, was observed at the posterior horn of the medial meniscus. It was left in situ because it did not appear to be pathological. Other defects or abnormal signs were not observed. Notchplasty was extensively performed to widen the abnormally narrow femoral intercondylar notch, after which transtibial single-bundle ACL reconstruction was performed using an allograft of the anterior tibialis tendon (). A tibial footprint of 10 mm in diameter was created on the tibial footprint of the ACL. Then, a femoral socket 10 mm in diameter was created at the 1 o'clock position using reamers and dilators with the knee at 90° of flexion. The transplanted tendon was fixed to the proximal tibia using an interference screw and to the distal femur using two Rigidfix Cross Pin devices (DePuy Mitek, Raynham, MA, USA). The reconstruction was completed after normal range of motion and stability of the knee joint were confirmed.\nThe patient was followed up for 6 months after the knee reconstruction. Examination of the knee range of motion revealed no limitation. The side to side difference in the anterior drawer test using the KT-2000 arthrometer was 3.8 mm and the pivot shift test was negative. |
A 34-year-old male patient with a past medical history of asthma presented as a transfer for new onset hypoxemic respiratory failure and management of pericardial effusion. The patient recently developed weakness, fatigue, and shortness of breath and presented to his primary care physician's office for evaluation. The patient tested positive for H1N1 influenza A and was treated with oseltamivir.\nHistory was also significant for contact with a child who had streptococcal sore throat 3 weeks prior to presentation as the patient worked as a school teacher. At the outside hospital, the patient was found to have significant leukocytosis of 30,000, transaminitis (AST and ALT in the 2000s), and elevated creatinine. Initial chest X-ray revealed an enlarged cardiac silhouette but no infiltrates. A CT scan was performed, revealing a pericardial effusion and trace pleural effusion (Figures –).\nOn arrival to our facility, muffled heart sounds and a soft diastolic murmur in the left lower sternal border were found on initial physical exam. A transthoracic echocardiogram revealed pericardial effusion and moderate mitral and tricuspid regurgitation. It also demonstrated vegetation on mitral valve.\nVancomycin, rifampin, and gentamicin were initially started as empirical endocarditis therapy but then changed to vancomycin and ceftriaxone after urine antigens and blood culture were positive for Streptococcus pneumoniae.\nA formal transesophageal echocardiogram for better valvular evaluation was performed and confirmed mitral valve regurgitation with vegetation and tricuspid regurgitation with a large echo density. The most impressive aspect of the imaging was the presence of a fistula formation between the aorta and the right atrium. Cardiothoracic surgery was consulted and the patient underwent an emergent two-stage cardiac procedure. Initial surgery was performed three days after hospital admission and consisted in a median sternotomy with 500 cc to 750 cc of purulent pericardial drainage. Due to these findings, initial surgical plans of fistula and valvular repair and replacement were postponed and first surgical intervention became a primary washout without chest closure.\nPostoperatively, the patient returned to the ICU and continued on vasopressors, extracorporeal membrane oxygenator (ECMO), and Intra-aortic Balloon Pump (IABP).\nTwo days later, the patient underwent a complex surgical repair with closure of the fistula, mitral, tricuspid valve repair, aortic replacement, and root enlargement. ECMO was continued postoperatively.\nDespite the fact that all these measures were performed within five days of arrival, the patient continued to clinically deteriorate and died within a few days from persistent multiorgan failure. |
A 43-year-old male patient presented with a chief complaint of deviation of the jaw toward the right side and progressive facial asymmetry for the past 1 year []. There was no history of trauma or infection in and around the temporomandibular joint (TMJ) region or ear. The patient visited a local practitioner for the same problem and was suggested to have left TMJ ankylosis. For second opinion, he reported to the Department of Oral and Maxillofacial Surgery at Punjab Government Dental College and Hospital, Amritsar. The main concern of the patient was remarkable facial asymmetry with mandibular deviation toward the right side. Further, fullness of the cheek was noticed on the left side with flatness on the right side of the patient. In addition, the right side of the patient's lip line appeared to have an obvious downward slant. On palpation of the preauricular region, condylar movements on both the left and right sides were palpable with no associated tenderness or clicking. Lateral jaw movements were also found to be restricted. Further, inspection revealed that mouth opening (approximately 43 mm) was in the normal range. On biting teeth into occlusion, the midline was found to be deviated by 13 mm to the right of the maxillary dental arch midline, reflecting an asymmetric prognathism with ipsilateral open bite and contralateral crossbite []. The occlusal plane of maxillary arch was normal with no apparent cant. On radiographic examination, axial and coronal computed tomography (CT) scans revealed an opaque mass that was located on the medial aspect of the left mandibular condyle extending medially and superiorly. There was no evidence of any fusion of the condylar head with articulating surface. The upper joint space and the joint disk were identifiable on radiograph [Figures , ]. A clinical differential diagnosis was made which included condylar hyperplasia, osteoma, osteochondroma, and malignant tumors such as chondrosarcoma and osteosarcoma. Angiography of the patient revealed the availability of safe zone to operate within the area without damaging the nearby vessels []. Under general anesthesia, Al-Kayat and Bramley's incision was taken on the left side and a flap was reflected. The tumor mass was attached on the medial aspect of the left condylar head extending medially and superiorly. Osteotomy cut was given at the upper end of neck of condyle. Then, the tumor mass was excised and taken out in one piece [Figures -]. The articulating disk had no fusion with tumor mass and appeared normal on exploration. Postsurgery CT scan after 2 year revealed no reoccurrence of lesion, and the patient's facial profile was significantly improved []. The teeth were brought into normal occlusion by applying traction forces on maxillomandibular fixation for 3 weeks. Active jaw exercises, including vertical jaw opening, lateral excursions, and protrusion, were performed 4–6 times/day, following release of intermaxillary ligation for the next 3 weeks. On the basis of the patient's history, clinical features, CT scan report, and histopathological examination, a diagnosis of osteochondroma was confirmed. |
A 76-year-old female had experienced unstable gait for 3 months. Because her disability was gradually progressing, she consulted a local hospital. When she came to our hospital for further examination 1 week later, she could not move without a wheel chair. Neurological examination revealed cerebellar truncal ataxia without Romberg's sign. Magnetic resonance imaging (MRI) of the head showed a cystic lesion in the foramen of Magendie, which was compressing the inferior part of the vermis and the medial part of the cerebellar hemisphere without causing hydrocephalus [].\nShe had previously consulted her local hospital with a complaint of headache 3 years ago. MRI performed at that time revealed the same cystic lesion to be present. The intensity of the cyst was similar to that of the cerebrospinal fluid (CSF), and the cyst was divided into two portions, suggesting the presence of a multilobular cyst. Because the patient's symptoms were believed to attribute to the presence of this cystic lesion, surgical decompression was planned.\nMedian suboccipital craniectomy was performed such that after the dural incision, the cyst was exposed. The retromedullary cistern was open, but the cyst had not shrunk, and the cyst was found not to communicate with the surrounding cistern []. Most of the cyst wall was successfully removed. A small part of the cyst that was still attached to the choroid plexus at the foramen of the Magendie was allowed to remain [], and the fourth ventricle was not opened via the obex.\nPathohistological examination demonstrated that the cyst wall was composed of a collagenous membrane lined by multiple layers of arachnoid cells []. Chronic inflammation and hemosiderin pigmentation were absent on the wall. This result confirmed the diagnosis of the cyst as an AC.\nThe patient's postoperative clinical course was excellent and her neurological recovery was remarkable. She regained her mobility and could walk without a cane. MRI performed 2 weeks after the operation revealed that the cyst had diminished and clearly demonstrated a flow artifact entering into the foramen of Magendie []. Recurrence of the cyst has not been detected on follow-up MRI for 4 years. |
We present a 23-year-old male patient who recently enlisted in the army. He presented to the Accident and Emergency department following a twisting injury while exercising.\nThe patient was oriented to person, place, and time. Analgesia was administered in the form of tramadol. The patient complained of severe pain at the level of and superior to his left knee and an inability to actively move his knee. This started immediately following an injury sustained while walking backward and turning left. He denied any falls, previous fractures, or previous medical and surgical history.\nOn examination, the orthopaedics team on call found marked swelling around the left knee and there was an obvious deformity. Upon palpation, the thigh was found to be tender and painful proximal to the knee. Neurovasculature was all revealed to be in tact.\nAP and lateral plain film radiographs of the left pelvis, hip, femur, and knee demonstrated a distal oblique fracture of the left femur, which was partially displaced posteriorly and medially. A decision was made to admit the patient for open reduction and internal fixation to be performed the following day. In the meantime, a backslab was applied and plain film radiographs were repeated. Once on the ward, consent for surgery and anesthetic was obtained and the patient was maintained on nil per os.\nThe patient was then moved to the operating theater where an open reduction and internal fixation was performed. Anatomical reduction was achieved using an AO Synthes locking plate and screw construct through a direct lateral vastus sparing approach. The decision to plate the fracture was taken because of the patient's extremely narrow medulla in the femur.\nThe patient had an uneventful postoperative recovery and was mobilized nonweight bearing on crutches on day one. Postoperative radiographs confirmed anatomical reduction and the patient was allowed passive range of movement on day three.\nAt latest follow-up, the patient achieved a range of movement of 0° to 100° and the fracture was uniting radiologically.\nThe narrow medulla, as well as the fracture both pre and postoperatively, can be well visualized in Figure . |
A 9-year-old male patient presented to the Department of Pedodontics and Preventive Dentistry, with a chief complaint of pain on the lower jaw. The parents gave a history of fall from the gate while playing in the school. On external examination there was an obvious deviation of mandible to the right side. Intraoral examination revealed laceration of the soft tissue and an obvious fracture between permanent mandibular right and left central incisors (). The occlusion was also deranged and the child was in mixed dentition period. Patient was otherwise healthy, conscious, cooperative, well-oriented to time, place and person. There was no history of convulsions or vomiting.\nOn palpation, fractured fragments were mobile and tender. The teeth along the fractured segments were not mobile.\nThe orthopantomogram () revealed radiolucent line extending from the superior border of symphysis between permanent mandibular right and left central incisors, to the base of the mandible. There was no overlap of the fractured fragments. Outer cortical boundary was irregular and had a step defect suggesting for an open reduction thus, diagnosing complete displaced symphysis fracture of the mandible. The presence of other concomitant fractures in the mandible however was ruled out.\nThe entire treatment plan was explained to the parents and the written informed consent was obtained.The child was admitted 1 day prior to the planned surgical procedure. Following the NPO guidelines, under general anesthesia, vestibular incision was made and the periosteum was exposed. Following eyelet wiring () which was done for any need of doing an intermaxillary fixation later the fractured fragments were reduced by digital manipulation (). The monocortical plate was passively adapted along the contour of the external cortex without any gap between the plate and bone, at the inferior border of the mandible. Holes were drilled through the plate into the bone and two monocortical screws were fixed on either side of the fracture line to secure the fractured fragments together ( and ).\nOcclusion was rechecked and was found to be satisfactory as a result intermaxillary fixation was not done. Flap was repositioned and sutures were placed (). Patient was shifted to the postoperative ward and recovery from general anesthesia was uneventful. Patient was discharged at the end of second postoperative day with instructions of soft diet and maintenance of good oral hygiene. As the patient was prescribed antibiotics and analgesics prior to the surgical procedure same was continued 3 days after the procedure. The child was recalled after 1 week for a checkup and was followed-up every fortnightly till the plate was removed.\nPlate removal was carried out at the end of 3 months. The consolidation of the fracture was confirmed clinically () and radiographically (). Satisfactory healing and occlusion was observed. |
A 52-year-old male patient presented with the complaint of sudden vision loss in his left eye 3 days earlier. Past medical history was significant for chronic kidney disease, secondary hypertension, chronic hepatitis C virus infection and arrhythmia. Ophthalmologic examination revealed best corrected visual acuity of 10/10 in the right eye and 4/10 in the left eye from the temporal field. Confrontation test revealed inferonasal visual field loss in the left eye. Direct and indirect light reflexes were normal in both eyes and there was no relative afferent pupillary defect. Anterior segment examination was normal and intraocular pressure was 13 mmHg in both eyes. Dilated fundus exam demonstrated soft exudates consistent with hypertensive retinopathy in the right eye. Fundoscopy of the left eye revealed an area of pallor in the superotemporal quadrant and the macula with macular cherry red spot, which were consistent with occlusion of the superotemporal branch of the left retinal artery (). On OCT, peripapillary retinal nerve fiber layer (RNFL) thickness was within normal limits (). In the patient’s visual field, there was an inferonasal defect in the left eye corresponding to the occluded region (). The patient was treated with a single dose of 500 cc intravenous dextran-40 and 200 mg intravenous pentoxifylline. In etiologic studies, Doppler ultrasonography revealed an atherosclerotic stenosis in the right and left main carotid arteries and a calcified plaque causing luminal narrowing in the left internal carotid artery. Transthoracic echocardiography revealed second- to third-degree aortic valve regurgitation and first-degree tricuspid valve regurgitation. There was no improvement in visual acuity or visual field despite treatment. At follow-up 7 months later, OCT showed thinning of the superior, inferior and temporal peripapillary RNFL (). On the thickness map, ganglion cell layer was thinner in the superior and temporal areas (). Decreased vascular density in the superficial and deep capillary plexus consistent with ischemia in the regions supplied by the superotemporal branch of the retinal artery was observed in a 6x6 mm macular field on OCTA (). The borders of the ischemic area were more clearly seen in en face images (). In optic disc OCTA, capillary density was reduced in the superotemporal region and collateral vessels were present in the area (). When compared to the fellow eye, there was a decrease in the macular deep and superficial capillary density in the superior and temporal quadrants () and a decrease in peripapillary capillary density in the superior quadrant (). Visual field loss persisted in post-treatment threshold perimetry (). |
A 9-year-old boy was brought to our Oral Surgery Clinic by his mother with the chief complaint of swelling on the ventral aspect of the tongue of 1 month duration. The swelling had gradually increased in size and interfered with speech and mastication. There was no history of trauma to the area, and the medical history was not significant.\nOn clinical examination, a bluish, nonulcerated, oval shaped mass measuring about 25 × 15 mm in size was noticed on the left ventral aspect of the tongue. The swelling was painless, soft and mucosa over the lesion was tensed (). On aspiration, the swelling recovered mucos. The swelling was clinically diagnosed as a mucocele of glands of Blandin-Nuhn based on the peculiar location. Blood investigation revealed the parameters within the normal limits.\nSurgical removal of the lesion was planned under local anesthesia with 2% lignocaine. A vertical incision was made on the ventrum of the tongue and excision of the lesion was attempted. In course of dissecting the lesion from the underlying tissue, the cystic lining ruptured leading to an immediate extravasation of mucos (). This made the lesion to collapse causing difficulty in further surgical exploration.\nTo improve the visual access of the lesion, a thin mix of alginate impression material was injected into the lesion through the ruptured wall using a 19 gauge needle. This technique helped in clearly demarcating the boundaries of the lesion (). The lesion was excised by dissecting down to the muscle layer and removed in toto together with the associated minor salivary glands () to avoid recurrence. The wound was approximated with 4-0 sutures. The postoperative course was uneventful. The excised mass was sent for histopathological examination.\nHistopathologic report revealed a Hematoxylin and Eosin stained section showing a well-delineated cavity containing eosinophilic mucinous material (). The cystic lining was composed of granulation tissue with fibroblasts, proliferating small caliber blood vessels and a mixed, acute and chronic inflammatory cell infiltration. At the periphery, few salivary glands were also evident (). The overall histopathological features were consistent with the clinical diagnosis of mucous extravasation phenomenon. |
In November 2009, a 52-year-old Korean woman who had never smoked presented with a history of isolated coughing. Chest computed tomography (CT) scan revealed a 4×3 cm sized mass on the upper lobe of the left lung (). Percutaneous needle biopsy of the lesion revealed adenocarcinoma. There was no evidence of metastases in bone scan, brain magnetic resonance imaging, or positron emission tomography (PET)-CT scan. Finally her disease was clinically staged as T2aN0M0. Polymerase chain reaction-based DNA sequencing of the EGFR gene revealed an activating mutation (Leu858Arg) in exon 21 of the EGFR gene. She underwent lobectomy of the upper lobe of the right lung. Tumor was a 4.0×4.0×3.5 cm in size and directly extended to visceral pleura on pathologic examination. She subsequently received three cycles of adjuvant chemotherapy with paclitaxel and cisplatin from December 2009. Following adjuvant chemotherapy, evaluation with chest CT scan, PET-CT scan and bone scan every three months revealed no evidence of recurrence until March 2011 when multiple small nodules were developed on both lung fields. Wedge resection of the upper lobe of the left lung was performed and metastatic adenocarcinoma was pathologically confirmed in the specimen. The DNA sequencing of the EGFR gene of resected specimen did not revealed any activating mutations of the EGFR gene. She received four cycles of chemotherapy with irinotecan and cisplatin and her disease was shown to be stable. In December 2011, pulmonary nodules were newly noted in the upper lobe of the left lung (). Then treatment was switched to the erlotinib monotherapy, 150 mg once daily. Her disease was shown to be partial response to the treatment by criteria of Response Evaluation Criteria In Solid Tumors version 1.1 in February 2012 and has been stable until June 2012. During the treatment, she experienced skin rashes on her face, chest, and scalp, which were adequately controlled with topical therapy. In addition she was complained of excessive elongated irregular growth of both eyelashes which irritated eyeball in two months after initiation of erlotinib (). She underwent monthly eyelash trimmings with scissors for relieving local symptom and cosmetic purpose. |
A previously asymptomatic, 55-year-old diabetic female, came to our emergency with sudden onset of inability to maintain posture and impaired co-ordination in body movements for 8 h. She had difficulty in sitting upright from lying down posture and was unable to walk without support. Patient also faced difficulty in reaching objects around her. These complaints were worst at the time of onset and had slightly improved upon presentation. Patient was a known diabetic for 10 years on glibenclamide therapy at a dose of 5 mg once daily. Her glycemic status was unknown at presentation. She was not previously diagnosed or treated for hypertension, coronary artery disease, and valvular heart disease. She had no history of smoking, alcohol, or any substance abuse. On examination, the patient was conscious, alert, and afebrile with pulse rate of 108/min without any irregularity or asymmetry. There was no carotid bruit. Her blood pressure was 110/70 mmHg without any postural drop. Respiratory rate was 16/min. Patients cardiac, respiratory, and abdominal examinations were within normal limits. Her Glasgow coma scale score was 15/15. Her higher mental functions were normal with no meningeal signs. Her pupils were equal and reactive without any nystagmus. Cranial nerve, motor, and sensory examinations were normal. Patient demonstrated dysmetria on the left side in the form of past pointing on finger-nose testing and abnormal heel knee-shin test. Her gait was slow, broad based and she was able to take few steps only without support. Patient had abnormal rapid alternating movements when performed by using left hand. A provisional diagnosis of cerebellar stroke was made and a MRI of brain was requested. At this stage, bedside blood glucose was measured and it was 41 mg/dl. She was given 50 ml of 50% dextrose followed by an infusion of 10% dextrose before being shifted for imaging. On re-examination after 20 min of dextrose administration, there was complete resolution of cerebellar signs previously noted at presentation. Her repeat blood glucose was 186 mg/dl. Considering the risk of recurrent hypoglycemia due to long acting sulfonylurea, glibenclamide, the patient was observed clinically in emergency for 24 h with frequent blood glucose testing. Neurologic examination of the patient remained normal during her hospital stay and the blood glucose did not fall below 120 mg/dl. A diagnosis of hypoglycemia-induced cerebellar dysfunction was made but a possibility of a transient ischemic attack was also kept. MRI showed subtle but diffuse cerebral and cerebellar atrophic changes with no areas of infarction, hemorrhage, or demyelination [Figures and ]. Doppler study revealed no atherosclerotic plaques or luminal narrowing in vertebrobasilar or carotid arteries refuting transient ischemic attack for her cerebellar symptoms. The patient was discharged after 24 h and followed up at day 30 with no recurrence of cerebellar complaints subsequently. |
We report a case of a 75 year old woman addressed to Allergology Department of our hospital in January 2014 for recurrent episodes of angioedema since the age of 66, with progressively increased severity and frequency. It was first considered to be induced by treatment with angiotensin-converting-enzyme inhibitors (ACEI) for mild hypertension, but she continued to have angioedema attacks for the next 6 years after discontinuation of ACE, with progressive aggravation during the last year. The previous multiple evaluations by many specialists in other hospitals did not succeed to give a clear diagnosis and treatment.\nThe patient had no relevant medical history and took no medication, except ACEI that was stopped some months after angioedema onset. No relation with possible allergic stimuli could be identified and she had no clinical manifestations between attacks. Angioedema was painful, not accompanied by urticaria or abdominal symptoms, located variably to neck, arms or buttocks, without facial involvement during 6 years. The attacks occurred at weeks or months intervals and usually lasted between 48 and 72 h, irrespective of corticosteroids and antihistamines treatment usually administered. The frequency of attacks had progressively increased from one at 2–3 months intervals to almost weekly during the last year before presentation. The last angioedema attack, determining emergency hospitalization in September 2014, was more severe and prolonged, accompanied for the first time by laryngeal edema and respiratory symptoms. The extended medical evaluation, including complete blood tests for inflammation, allergy, autoimmunity and cancer, were all negative (Table ). Full body CT scan and bone marrow examination were normal. No criteria for lymphoproliferative, mieloproliferative or autoimmune diseases could be found. Measurement of C1 inhibitor (C1INH) in plasma showed significantly decreased level at all measurements, with low activity ranging from 58 to 4% and constantly low C4 (Fig. ). Complement fraction C1q plasma level was measured twice and had normal value. Genetic tests were not performed, given the patient advanced age and lack of family history of angioedema, which are against HAE. A spontaneous mutation in SERPING 1 gene is noticed in up to 25% cases of HAE without family history, but we considered this probability very low in our case, due to late onset of angioedema.\nTreatment of angioedema attacks before hospitalization consisted of antihistamines and systemic corticosteroids, which proved to be ineffective. Since no pathogenic therapy with C1INH concentrate, antagonists of bradikinin receptors (icatibant) or selective inhibitor of plasma kallikrein (ecallantide) was available in 2014, we first initiated daily prophylactic treatment with tranexamic acid for 3 months, with no amelioration. During the more severe attack with laryngeal edema, in September 2014, we have switched to attenuated androgen danazol, given 400 mg the initial dose, reduced to 200 mg daily after 1 week and then to 100 mg daily. The clinical evolution was very good, no angioedema attack occurred since the introduction of danazol. C1INH and C4 plasma levels increased after 2 weeks of treatment and became normal after 1 month. After some months, the patient decided herself to discontinue danazol for short time, in order to check effects and to taper the minimal dose. Serum C1-INH and C4 plasma levels were measured after 2 and 4 weeks and showed significant lower levels, but no angioedema attack occurred during this period of time. She therefore restarted danazol prophylactic therapy at a minimum of 50 mg dose daily, ongoing after 3 years. The patient was closely monitored during the next 3 years, with complete clinical and laboratory control twice a year. We took into consideration the possible side effects of danazol, mainly dislipidemia, haematological and liver malignancies and any other complications or concomitant diseases. The clinical evaluation was very good, except two episodes of pulmonary cysts infection, remitted with broad spectrum antibiotherapy, which could not be related to danazol treatment. The clinical outcome of angioedema after 3 years of danazol treatment is very good, with no attacks or other related symptoms. No clinical or laboratory sign of any disease that could induce C1 INH deficiency occurred. No relevant side effects of danazol were noticed. The patient has an improved quality of life due to therapeutic compliance and general management plan. |
A one month old baby girl was brought by her mother to the lactation clinic for further evaluation due to the difficulty of breastfeeding. The mother complained of pain in the nipple which was present throughout the entire breastfeeding session and usually recurred with each episode of breastfeeding. Two weeks after delivery, the mother noticed a tooth on the baby’s lower left gum. A week later, she noticed an ulcer under her baby’s tongue, which occasionally bled.\nIn light of the continuous pain during breastfeeding, the mother became reluctant to continue and infant formula milk was given as a substitute. Self-examination by the mother did not reveal any nipple crack or breast injury. Her antenatal history was uneventful. There was strong family history of natal teeth, and this baby’s siblings had similar problems.\nOn examination, the mother’s breast appeared to be normal and consistent with a lactating breast. There was no inflammation or engorgement. Some mild eczema was noted around the nipple, but there was no infection. Cervical and axillary lymph nodes were not palpable.\nExamination of the oral cavity of the baby revealed a neonatal tooth over the left anterior region of the mandibular ridge. The tooth measured 2 mm × 1 mm in size, was whitish opaque, and had Grade II mobility (Fig. ). There was also a whitish ulcer over the ventral aspect of the tongue measuring 1 mm × 1 mm (Fig. ). The parents declined radiological investigations and tongue biopsy for the baby. Hence, the clinical diagnosis was neonatal tooth associated with Riga-Fede disease causing difficulty in breastfeeding.\nThe baby was then referred to the pediatric dental surgery department. After a thorough discussion with the parents, the neonatal tooth was extracted under local anesthesia. The tooth had a crown but lacked a root. Following extraction, the baby did not have any complication such as bleeding and infection. The wound healed well within 2 days and she successfully resumed taking breast feeds.\nCurrently, at 10 months post-extraction, the child is growing well and still breastfeeding. Examination of the oral cavity revealed that only one central lower incisor tooth was present (Fig. ). |
A 6-year-old girl presented with complaint of a solitary, raised skin lesion of size 15 × 12 cm over the back of her left knee since five months. Initially, she developed an erythematous swelling of size 1 × 1 cm over the site; the swelling was insidious in onset and gradually progressive in nature. She subsequently developed intermittent, moderate grade fever associated with chills and rigors that subsided upon taking medication. The swelling grew to 15 × 12 cm size within 3 months, with tenderness, but no discharge. It also led to difficulty in flexion of the left knee joint. She underwent incision and drainage of the swelling at a private clinic. However, the swelling continued to progress and within one month of surgery, the swelling progressed to 15 × 10 cm size, filled with pus and associated with tenderness. Magnetic resonance imaging of the swelling showed it to be cellulitis and fine-needle aspiration cytology suggested an abscess. She again underwent incision and drainage. At the time of presentation to our centre, she had an annular plaque of size 15 × 12 cm having an erythematous to violaceous, raised, indurated and tender periphery and a centrally atrophied plaque extending from the lower third of thigh, involving the knee joint along with the popliteal fossa and downward up to the upper one-third of left leg []. The center of the plaque showed three hypertrophic scar marks of the previous incision and drainage []. Movements of left knee joint were restricted. She had no history of trauma, insect bite, no atopic diathesis such as seasonal rhinitis or atopic dermatitis, contact dermatitis, morning stiffness of the joint, Raynaud's phenomenon, thyroid disorder, vitiligo, or alopecia areata.\nPast history was insignificant; there was no history of consanguinity and the patient was delivered as full term normal vaginal delivery. Her systemic examination was normal. She had leukocytosis with an erythrocyte sedimentation rate of 70 mm/h. Absolute eosinophilic counts along with serum IgE levels were within normal limits. Her thyroid profile showed raised serum total T3 level (225.5 ng/dl) along with raised free T3 level (4.6 pg/ml). Her pediatrician consultation was done, but no active intervention was required. Her blood sugar, antinuclear antibodies, renal function and liver function tests were within normal limits. Rheumatoid arthritis factor and C-reactive protein were negative. Routine and microscopic examination of stool and urine were normal. Serology for human immunodeficiency virus (HIV) I and II by Combi-AIDS kit was negative. Ultrasonography of the affected leg showed subcutaneous soft tissue swelling around the knee joint. Skin biopsy confirmed a diagnosis of eosinophillic panniculitis showing infiltration of eosinophils in subcutaneous fat []. In our case, no definitive causative factor for panniculitis was found. Patient was given oral antibiotic for 1 week before 1 month of reporting to us. Patient responded well to the oral prednisolone 30 mg given once a day. It was tapered after every 15 days by 10 mg, which got completed after 6 weeks and also we had put her on tablet dapsone 50 mg daily for a period of 1 month. Orthopedic referral was made, and she was advised physiotherapy for the left knee to which she showed good response. She responded well to treatment []. The treatment was then stopped; the patient was advised monthly follow up for 6 months that was uneventful. |
A 14-year-old female patient had come to the Department of Orthodontics, with the chief complaint of forwardly placed upper front teeth. Her medical history was found to be noncontributory.\nOn extraoral examination, facial asymmetry was evident for which patient was unaware. There was no history of trauma. The patient did not have any difficulty in opening of mouth. Inspection showed elongation of left side lower half of face as compared to the right side. The left side of the face appeared flattened as the prominence of the chin appeared shifted to the right side [].\nOn intraoral examination, her molar relationship was found to be Angles Class II on right side and end on the relationship on the left side. The lower midline was shifted 3 mm to the right side with respect to maxillary midline [].\nRadiographic investigations were also carried out. Panoramic view of girl revealed symmetrical condylar enlargement of the left side in comparison to the right side and condylar neck along with the elongation of the left side of ramus [].\nPA cephalogram of the girl showed a shift of dental midlines due to the asymmetry of the mandible [].\nHer mother who was 44 years old had accompanied her and asymmetry in her face was also noticed. She was unaware of it and gave consent to be examined. On examination, she did not have any difficulty in opening her mouth. The chin was deviated to the right side as left side of her face was elongated. The left side of face appeared flattened [].\nIntraorally, her oral mucosa was normal with permanent dentition and a total of 24 teeth. Her oral hygiene was poor and no previous history of trauma. Medical and family history was noncontributory. There were root stumps and carious teeth present. Maxillary right first molar had already been extracted. Clinically, she showed a lower midline shift to the right side, chin deviation and misalignment [].\nPA cephalogram of the mother showed a shift of dental midlines due to asymmetry of the mandible [].\nPanoramic view revealed symmetrical condylar enlargement of the left side and elongated condylar neck as compared to the right side []. |
A 21-year-old, unmarried Arab woman with no known comorbidities presented with a massive swelling on the right side of her face that had been slowly increasing in size for the past 3 years, as well as with difficulty in bending her left knee. The swelling was large enough to cause trismus, and the patient was unable to open her right eye fully. She had no associated pain, bleeding, or superficial ulceration of the mass. The history taking for her presenting illness revealed multiple admissions for recurrent fractures. She had experienced no associated headaches, psychiatric manifestations, renal stones, or abdominal pain. She had no history of weight loss, fever, or night sweats. She had a negative family history for any tumors or disorders of calcium homeostasis. She also displayed psychological distress and concern regarding the impact of the mass on her appearance. Her social functioning and activities of daily living were markedly affected.\nAn extraoral examination showed a nontender hard swelling on the right side of the mandible that was causing facial deformity. An intraoral examination revealed a nontender firm swelling that was expanding the buccal mucosa and lingual mandibular plates, obliterating the buccal sulcus, and extending from the right premolars to the ascending ramus. The patient had a displaced, partially impacted tooth 18, mobile teeth 36 and 37, and nontender swelling of the left maxillary region with obliteration of the upper left buccal sulcus. A panoramic x-ray of the patient’s teeth showed a right mandibular swelling and an impacted left lower wisdom tooth (Fig. ).\nThe patient’s thyroid examination result was normal. She had no associated lymphadenopathy or skeletal deformities. An examination of the lower extremities showed that the left knee had mild limitation of movement. The patient had no tenderness, erythema, or swelling of the knee joint or proximal leg on the left side. An examination of the patient’s right lower extremity was unremarkable.\nShe initially presented as an outpatient to the maxillofacial surgery department. The mass was suspected to be malignant, and a biopsy of the lesion was taken. The biopsy report showed a giant cell lesion and was referred to the endocrinology department to rule out metabolic bone disease. Her lesion was identified as a brown tumor of hyperparathyroidism, and laboratory investigations were done (Table ).\nThe initial impression was of secondary hyperparathyroidism due to severe vitamin D deficiency, although the patient’s PTH level was remarkably high. The other suspicion was of PHPT masked by vitamin D deficiency.\nMaxillofacial computed tomography (CT) with contrast enhancement showed multiple maxillofacial expansile lesions with ground-glass ossification. The largest lesion within the right mandible involved the body and ramus. It measured 6.4 × 4.1 cm. The largest lesion in the left maxilla involved the alveolar edge and measured 3.6 × 3.3 cm. No lymphadenopathy was seen (Fig. ).\nA positron emission tomography CT scan showed multiple hypermetabolic, expansile, bony lytic lesions in the right mandible, left maxillary sinus, left mandible, and manubrum sterni (Fig. ).\nMagnetic resonance imaging (MRI) of the left lower extremity showed a heterogeneous, solid, and soft tissue mass involving the proximal epimetaphysis of the tibia and extending to the subarticular cortex. The mass was approximately 4.8 × 4.2 cm in size (Fig. ).\nThe patient was started on vitamin D 50,000 U by mouth weekly for 6 weeks. Her PTH levels decreased after 4 months on vitamin D therapy, but her calcium levels rose significantly (Table ). The vitamin D dose was reduced because of fear of hypercalcemia, and the patient was kept on 5000 U weekly thereafter. An ultrasound scan of the parathyroid gland was done at the time, which showed a right lower lobe parathyroid solid nodule. A technetium Tc-99 m sestamibi parathyroid study showed increased activity in the soft tissue mass, approximately 13.0 mm in size and lying behind the lower part of right thyroid lobe, suggestive of parathyroid adenoma (Fig. ).\nShe was referred to surgical oncology and underwent right lower parathyroid adenoma excision. Her PTH levels improved significantly after surgery (Table ). Postoperatively, she had a brief episode of hypocalcemia (1.97 mmol/L), and she was started on calcium carbonate 1200-mg tablets twice daily for 2 weeks. She was received maxillofacial follow-up for intralesional steroid 60-mg methylprednisolone depot injection as well as endocrinological follow-up for vitamin D therapy. She was followed with frequent laboratory and radiological investigations for the next 3 years, through the reporting of this case, and the masses have shrunk in size.\nMaxillofacial contrast-enhanced CT performed 5 months after resection of the parathyroid adenoma showed lesions with more ossification, which could be a sign of healing. The largest right mandibular lesion was stable in size, but the left maxillary mass was reduced in size to 3 × 2.5 cm (compared with 3.6 × 3.3 cm previously) (Fig. ). CT performed 1.5 years postsurgery showed reduction in size of the right mandibular lesion to 5.5 × 4.0 cm (compared with 6.4 × 4.1 cm previously), and the maxillary lesion was further reduced to 2.3 × 2.2 cm (Fig. ).\nMRI of the left knee with contrast enhancement performed 6 months postsurgery showed a mild decrease in size since the previous study, with increased sclerosis of the solid component (Fig. ). MRI of the left knee performed 2.5 years postsurgery showed further reduction in the size of the lesion from the previous study (Fig. ).\nOverall, the patient showed serological, radiological, clinical, and psychological improvement from her initial condition. Although we were not able to retrieve an initial photograph for comparison, we took a picture of the mass after its marked reduction 1.5 years postoperatively. |
A 30-year-old female was diagnosed with SLE at 12 years of age. At around 27 years of age, she experienced several instances of seizures and began taking antiepileptic drugs. On brain magnetic resonance imaging (MRI), diffuse cortical atrophic changes and an old infarct lesion in the left superior parietal cortex were detected. Three years later, she had fever, clonic seizure and loss of consciousness. On T2-fluid attenuation inversion recovery (FLAIR) imaging, a high-intensity signal change was detected in the left temporal and frontal lobes, both occipital lobes, and the midbrain (). She was also diagnosed with antiphospholipid syndrome. After 15 days of treatment, which included intravenous methylprednisolone, immunoglobulin and anti-epileptic drugs, she regained consciousness and her motor weakness was improved. Follow-up brain MRI showed improvement of the previous lesions, which were suggestive of posterior reversible encephalopathy syndrome (PRES) (). As she had fever and a urinary tract infection (UTI), the infectious condition following immunosuppressant therapy with cyclosporine was thought to be the cause of PRES. At her first admission to the Department of Rehabilitation Medicine, consistently with the dramatic neurological recovery commonly observed in patients with PRES, she showed mild functional and cognitive impairment. One week after the start of rehabilitation, weakness of the left extremities and fever were noted. Brain MRI showed acute infarction in the right basal ganglia (). Acute cerebral infarction was considered related to SLE activation due to UTI. One month after treatment in the Department of Rheumatology, she was returned to the Department of Rehabilitation Medicine. We performed an electrodiagnostic study and whole spinal cord MRI to rule out other superimposed diseases, such as neuropathy, transverse myelitis, and myelopathy. The cross-sectional area of the spinal cord from the cervical through the conus medullaris was markedly thin on sagittal images, and no other significant abnormal findings were evident (). The electrodiagnostic study showed severe sensory peripheral polyneuropathy involving the lower extremities.\nWhen she was returned to the Department of Rehabilitation Medicine, we specified her clinical problems multiphasically using the ICF (). After addressing the full spectrum of problems that she had, we comprehensively and rapidly planned how to care for and provide a tailored rehabilitation program to the patient.\nRegarding the lower extremity weakness and poor functional level, physical therapy including tilt table standing, trunk muscle-strengthening training and sitting-balance training were administered. To investigate cognitive dysfunction, neurocognitive testing was performed using the Korean version of the Consortium to Establish a Registry for Alzheimer's Disease Assessment Packet (CERAD-K), and a computerized neuropsychological test was also performed. These showed remarkable impairment in the praxis recall, word list recall, and trail making tests (). She underwent a therapeutic program using a computer-aided cognitive rehabilitation training system (COMCOG; MaxMedica Inc., Seoul, Korea), which included memory function training and attention training components. As she experienced severe cerebral infarction after UTI, we aggressively controlled her condition to prevent development any further infections as confounding factors. To manage the tingling sensation and neuropathic pain in both of her lower extremities, we prescribed the anticonvulsant, pregabalin. She also experienced psychological problems, including depression and avolition, for which we prescribed anti-depressants and referred her to a psychologist and social work team.\nUsing the ICF code, our multidisciplinary rehabilitation team could determine the appropriate therapeutic approach to each problem. For example, several interventions were chosen to treat the target intervention "d530 toileting". A physician prescribed antibiotics to prevent UTI, a physical therapist instructed the patient and her parents about proper position during urination or defecation and nurses checked her water intake. This approach allowed us to rapidly achieve the goal. Thus, we believe that use of the ICF code facilitates precise and rapid communication among rehabilitation team members.\nAfter completing the comprehensive rehabilitation program, the patient showed favorable improvements in multiple respects (). |
A 74-year-old man with a history of metastatic colorectal cancer was admitted with a four week history of progressive dyspnoea and haemoptysis. He had undergone a right hemicolectomy three and a half years previously for a Duke’s A colorectal cancer. Three years post resection, a large single liver metastasis was identified with no other evidence of metastatic disease on PET scanning. CT examination of the chest at the time demonstrated only mild emphysematous changes ().\nThe patient was commenced on FOLFOX chemotherapy (oxaliplatin, 5-fluorouracil and leucovorin) and underwent six cycles from October to December 2008. A re-staging CT scan in January 2009 demonstrated progressive malignant disease with multiple new metastases within the liver. Chest imaging at this time revealed bilateral peripheral subpleural reticular changes and interlobular septal thickening without evidence of overt lung metastases (). No changes to treatment occurred and the patient was planned for second line systemic chemotherapy.\nThe patient subsequently presented in early February 2009 with four weeks of worsening dyspnoea and haemoptysis. His Creactive protein level was moderately elevated although white cell count and differential were normal. Arterial blood gases demonstrated severe type I respiratory failure. CT pulmonary angiogram excluded pulmonary embolism but demonstrated new patchy infiltrates with interlobular septal thickening (). Sputum samples were unable to be obtained and the patient was considered to be too high risk to undergo bronchoscopy.\nHe was commenced on empirical broad spectrum antibiotics and high dose intravenous corticosteroid therapy. His respiratory failure worsened and after discussion with both the patient and his family, mechanical ventilation was not administered. The patient died four days after admission from progressive respiratory failure.\nPost-mortem examination did not demonstrate evidence of an infective aetiology on staining and culture of fresh specimens. There was also no evidence of emboli, thrombus or malignant infiltration within the lungs. Histological examination demonstrated widespread generalised changes within both lungs, with oedema, intra-alveolar haemorrhage and fibrin deposition along with interstitial and intraluminal fibroblastic proliferation without mature fibrosis. These findings were consistent with diffuse alveolar damage. |
A 32-year-old white woman, who was undergoing transgender body modifications and no previous medical history, presented to our emergency department (ED) with a chief complaint of right forearm pain, associated with redness and blistering. The patient was a tattoo fanatic who underwent solar branding on her right forearm approximately 2 weeks before presentation. The patient first noticed the blistering of the branded area 3 days after her body modification. Over the next few days, the entire branded area became very warm, swollen, painful to touch, and developed cellulitis with weeping blisters. Given her dire situation, the patient finally sought medical care in the local urgent care center. The patient was diagnosed with a superficial infection of her right forearm full thickness burn and prescribed oral clindamycin. The patient continued to develop painful blisters for the next week and did not notice any improvements in her cellulitis despite the antibiotic treatment, prompting her to come to our ED for a second medical opinion.\nDuring her physical examination, the patient was noted to have painful blisters, redness, and swelling of the right forearm and hand (Figure ). She did have a full range of motion of the hand; however, there was mild pain noted on the flexion of the hand. There were no signs of sepsis, and her vital signs and her laboratory panel which included white blood cell count were all within normal limits. The rest of the physical examination was also unremarkable. The patient was admitted to the Burn Surgery Service for intravenous (IV) antibiotics and possible operative interventions.\nThe patient was started on IV clindamycin 600 mg every 8 hours, and once adequate pain control was achieved, a bedside debridement was performed in the Burn Unit. The following day, the patient was taken to the operating room for tangential excision of her wound and split-thickness skin autograft (STSG) placement onto her right forearm. The left anterolateral thigh was chosen as the donor site. The grafted site was dressed with xeroform, followed by a layer of bacitracin ointment and then wrapped with Kerlex and Coban dressings. A right forearm elbow splint was fitted and placed by the occupational therapy service for further graft protection. Postoperatively, the patient was transferred back to the floors without complications, and antibiotics were stopped. The right forearm dressing was taken down on postoperative day 4 to evaluate the STSG. The entire graft was viable and taken. Subsequently, the graft staples were removed on postoperative day 5.\nThe patient recovered exceptionally well and was discharged home a few days later. Two weeks later, the patient returned to the burn clinic for a follow-up appointment. The skin graft and the donor site healed remarkably well (Figure ). The patient reported no pain and had a full range of motion of the hand and wrist. The patient was discharged from the burn service and advised to use over-the-counter moisturizer or lotion as needed. |
An 11-year-old male patient presented with complaint of a painless facial swelling on the right side of the face for about 4 years. The swelling started as a small hard mass in the oral cavity on the roof of the mouth. It was a painless swelling which gradually but progressively increased in size with resultant severe facial deformity that almost totally blocked the right nostril. Apart from the facial disfigurement, the patient twice experienced fractures of the lower right limb at 3 years intervals.\nExamination revealed a healthy young boy who was well oriented to his surroundings. He had a short stature with bowing of right arm and right leg that forced him to walk with the aid of clutches. Generally, he had normal looking skin with irregular skin pigmentation on the neck. On local examination of the craniofacial region, the patient had facial disfigurement due to a swelling on the midfacial region. There was an increase in inter-canthal distance with deviation of the nose to the left. The swelling had normal temperature, non-tender, bony hard and fixed to the underlying structures (Fig. ). Intraorally, the lesion was on right side of the upper jaw, oval in shape, had normal overlying mucosa with more of palatal bone expansion compared to the buccal bone. The tumor caused some degree of displacement of teeth on the right side of the upper jaw without mobility. Based on these clinical findings a provisional diagnosis of McCune-Albright syndrome was made.\nThe work up done on the patient included, histopathological analysis, skeletal survey (CT scan of craniofacial region and conventional radiographs for rest of the body), complete blood count, calcium and phosphate levels in the blood, thyroid and parathyroid function tests, and echocardiogram. The histological features of the specimen taken intraorally from the anterior aspect of the maxilla were of moderate cellular fibrous stroma surrounding irregularly shaped bone trabecular with minimum osteoblastic rimming consistent with fibrous dysplasia. Skeletal survey showed bowing of upper and lower limbs. CT scan of head and neck region showed a ground glass appearing lesion that involved the maxilla, palate, zygomatic bone, frontal bone and base of the skull (Fig. a & b). Both maxillary sinuses were obliterated. The lesion was filling the oral cavity. The complete blood count, calcium and phosphate levels and the thyroid function tests were all within normal ranges. A series of surgical interventions to correct his disfigurement were planned. The first surgery (bone remodeling of the maxilla) was carried out in April 2018.The patient was discharged one-month post operatively (Fig. ) and he is doing fine. The patient was advised to report back on 3 months basis for follow up. |
A 28 years old female, Gravida 1 Para1was referred to Gynecology and Obstetrics Department of our hospital at 36 weeks of pregnancy with 4 days history of progressive weakness of limbs and difficulty in walking, which culminated in loss of ability to walk; the weakness progressed gradually to upper limbs, with a 1 day history of difficulty in swallowing and breathing.\nThe patient was in her usual state of health 4 days back when she noticed weakness in both lower limbs after she got up in the morning. The weakness worsened the following day and she was unable to walk. By the end of 3rd day, weakness was also noted in upper limbs and had difficulty in lifting her arms. On the 4th day of her illness, she had difficulty in swallowing and breathing and was referred to the hospital.\nOn examination, the patient was found acutely ill, pale, afebrile, acyanosed, anicteric, and hydration status was satisfactory. She was dyspneic with shallow breathing. Her breathing was vesicular with no added sounds. Cardiovascular examination showed no abnormality. On abdominal examination, she was almost in full — term pregnancy (36weeks) and was not in labor. On neurological examination, she was found to be conscious but restless, and apprehensive. She was aphonic and had weak cough reflex and gag reflex; all her cranial nerves were normal. She presented with all the features of flaccid quadriplegia with grade zero power in both lower limbs and grade three in upper limbs. Muscle tone was decreased and deep tendon reflexes were lost. There was no sensory impairment or bladder and bowel involvement. Nerve conduction tests and cerebrospinal fluid analysis suggested diagnosis of GBS.\nThe patient was taken up for Cesarean Section, surgery was conducted under general anesthesia, and a 2.5 kg healthy baby was delivered. The patient could not be extubated following surgery as she was not able to maintain spontaneous breathing. She was then shifted to ICU and kept on ventilator and the next day plasma exchange was started; this was the 5th day of illness. Therapeutic plasma exchange was performed as first line treatment and IVIG or steroids were not tried before, after, or simultaneously with plasma exchange. Plasma exchange was done on alternate days, with a total of five sessions and in each session; 2308.8 ± 182.73 ml (range: 2105-2562 ml) of plasma was removed. This was in accordance with the guidelines of removing 1-1.5 times plasma in each session. Five percent albumin was used as replacement fluid. No plasma was used as the patient's coagulation profile was within normal limits. Peripheral femoral access was used to carry out the procedure; proper catheter care was maintained and lines were flushed with heparin before and after the exchange. The patient had no complications during these sessions. Complete coagulation profile including: prothrombin time (PT), prothrombin index (PTI), activated partial thromboplastin time (APTT); complete blood count (CBC) including haemoglobin, platelet count; electrolytes (Na+, K+, Ca++); s. protein levels were checked before and after the procedure and remained within normal limits throughout the treatment period. After three sessions of plasma exchange, i.e., on the 9th day of illness, the patient was weaned off the ventilator and physiotherapy started. The patient was discharged on 14th day of illness. At the time of discharge, the power was 5/5 in upper limbs and 3/5 in the lower limbs. Follow-up of the patient after 2 months showed no residual weakness. |
A 65-year-old man with history of myocardial infarction, previous coronary artery by-pass grafting, chronic heart failure with severe left ventricle (LV) dysfunction, and left bundle branch block, received a first biventricular ICD in 2003 with a dual coil defibrillation lead, a unipolar CS lead and a bipolar atrial lead. The device was replaced in 2005 due to a recall from the manufacturer and in 2009 for battery depletion. In August 2010 the patient presented haemodynamic decompensation, widening of the QRS and modification of paced-QRS axis. A check on the device showed an increase in the impedance and loss of capture of the unipolar CS lead. The chest X-ray revealed a fracture of the conductor of the CS lead in the subcutaneous pouch. A new bipolar CS lead was reimplanted via the left subclavian vein. During the implantation we tested different pacing configurations: the LV pacing thresholds were good and no PNS was observed with an output of 10 V. The fractured catheter was enveloped in thick and tenacious scar tissue, thus it was capped and abandoned in the pouch (). In February 2011 the patient started to complain of PNS which could not be corrected by changes in LV pacing configuration and output. A surgical revision was planned in order to check the catheter position. The bipolar lead was still inside a CS lateral side-branch. After insertion of a heavy-weight guide-wire which extended beyond the tip, we gently moved the catheter trying to restore a satisfactory position, without success. Then the bipolar CS lead was removed, leaving the guide-wire in the atrium via the subclavian vein. The insertion of an adequate size introducer for CS lead reimplant failed due to subocclusive vein stenosis despite the use of dilators with increasing diameter over the guide-wire. Repeated punctures of the vein downstream and upstream of the stenosis were also unsuccessful. Consequently we decided to restore the abandoned unipolar CS lead which was disengaged from the surrounding scar tissue. The insulation was transected proximally to the fracture point and pulled back. The conductor was stretched out and exposed beyond the insulation for a 0.5 cm portion. A 1.5 cm piece of a standard stylet was inserted into the lumen of the distal part of the lead so as to have a portion of a few millimetres extending beyond the conductor. The stylet was gently compressed with a clamp and secured with a silk tie and silicone glue in order to make the lead terminal stiff. Then, the fitted catheter was connected to a lead splice unipolar adaptor (VKU 17, Osypka AG, Germany) (). It was filled with silicone glue and secured to the body of the lead with a silk tie. The electrical parameters of the repaired electrode were good, comparable with previous chronic values. The pacing threshold was 1 V at 0.5 msec in the pre-fracture phase and 1.5 V at 0.5 msec in the post-repair phase. No PNS was observed at 10 V in the available configurations. The endocardial LV electrogram was good and no noise was recorded while moving and stretching the adaptor. At twelve months follow-up the patients was clinically stable and the repaired electrode showed adequate electrical parameters with unchanged pacing and impedance thresholds (). |
A 2-month-old female child was brought to Orthopaedic OPD with stiffness of elbow and congenital deformities of right hand. She was second child, born out of a non-consanguineous marriage by full-term normal vaginal delivery. There was no family history of congenital disorders, and antenatal period was uneventful. Examination revealed shortening of right upper limb with slight flexion at elbow and radial deviation of wrist (Fig. ). The hand had seven well-formed digits arranged symmetrically as mirror images on either side of a presumptive sagittal axis (Figs. , ). Passive movements at shoulder were normal; elbow had fixed flexion deformity of 20° with further flexion of 30°. Supination and pronation was grossly restricted at forearm; wrist movement was from 15° to 45° of palmar flexion. Child was able to flex and extend the fingers and occasionally grasped object between the fingers (although exact finger function could not be assessed as the child was too small). Function of opposite limb was essentially normal. Radiographs of affected limb show two ulnae, one following the other, with broadened distal end and absent radius (Fig. ). The hand bears seven triphalangeal digits with seven corresponding metacarpals all in one plane (Fig. ). We planned gentle manipulation at wrist and elbow, followed by serial corrective cast application at weekly intervals to correct as much deformity of wrist and elbow as possible. Correction of deformity can be maintained by suitable orthosis until the child reaches 1 year of age. After 1 year of age, staged surgeries can be planned because by that time the limb size becomes reasonably large for corrective and reconstructive surgeries and also the child can be trained well in use of hand and muscle training of pollicised finger. Our aim was to obtain useful elbow flexion, forearm rotation and reconstruction of thumb from the functional point of view. In the first stage, soft tissue distraction would be achieved by application of radial distractor across the wrist to correct wrist flexion and radial deviation. Second-stage surgery would be planned after correction of wrist deformity to assess full excursion of long flexors of fingers. First, we would achieve maximum flexion of elbow by excising about 1 inch of bone from proximal part of the radially placed extra ulna, followed by reconstruction of the collateral ligament and soft tissues to the proximal part of the normally placed ulna. Then we would excise two radial-most fingers with preservation of the skin flap from the middle finger with release of the first web. Shortening osteotomy at the level of the metacarpal neck of the remaining radial digit would be done, and the metacarpal head rotated for about 80° of apposition to make it thumb and fixed by two K-wires. A cortical bone graft from the amputated metacarpal bone would be pressed between the second metacarpal bone and that of the pollicised digit to fix it in abduction, and the skin flap would be transposed to first web. Post-operatively the limb would be kept in long arm splint with elbow flexed, wrist extended and thumb abducted for 4–6 weeks. Gradual passive physiotherapy would be started after removal of K-wires and splint. |
A 30-year-old male patient presented to our hospital with pain in the left hip region, reduced leg mobility due to theleft hip PJI with two fistulas in the upper lateral and anterior thigh region. Collecting anamnesis, the patient had a left distal femoral metaphyseal fracture due to a fall at the age of 10. At that time, X-rays showed dysplastic bone lesions of the left femur and the patient was diagnosed with monostotic FD. The patient had distal femoral osteosynthesis with a dynamic compression plate. The fracture healed without any complications. The plate was evacuated 1 year later and the dysplastic bone lesions were sealed with a bone allotransplant. Ten years later, the patient had a pathological left femoral neck fracture without any trauma. Osteosynthesis with intramedullary femoral nail was performed in another hospital. Six weeks after the operation, the patient developed growing pain and swelling in the left hip and distal thigh region. Septic arthritis of the left hip joint was diagnosed. The intramedullary nail was removed, femoral head resected, and the patient received systemic antibiotic therapy for 6 weeks. Afterward, debridement of the distal femoral metaphysis was performed, and bony cavities were sealed with bone graft substitute made of calcium sulfate. After 8 weeks, there were no signs of active infection and the patient over went total left hip arthroplasty with anuncemented prosthesis. Three years later, the patient developed a PJI. Local debridement, removal of the prosthesis, and placement of cement spacer incorporating gentamicin () were performed in combination with systemic antibiotic therapy. Six weeks later, the spacer was removed and these conduncemented total hip arthroplasty was performed. As a complication, the patient developed lymphedema of the left leg. One year later, the patient had a repeated PJI which was treated with debridement, retention of the prosthesis, and systemic antibiotic therapy. Six months later, the patient developed deep vein thrombosis of the right axillary vein, which was treated with anticoagulant therapy. He was diagnosed with antiphospholipid syndrome and thoracic outlet syndrome. One year later, the patient developed posterior displacement of the femoral component in the left hip joint twice with a 1-month interval and both were reduced in a closed manner. One month later, he developed a repeated PJI which was treated with debridement, drainage, and systemic antibiotic therapy. Two fistulas opened in the left thigh region and the patient was transferred to our hospital. The patient had pain during motion in the left hip and moderate decrease in the range of motion. Routine analyses showed leukocytosis (15.90 × 109L) and increased C-reactive protein (67.1mg/L). Radiographs of the left hip showed osteolytic zones around the acetabular and femoral components with sclerotic deformation of the distal femoral metaepiphysis ( and ). Our plan was a two-stage exchange hip revision arthroplasty by performing removal of the prosthesis, implantation of an antibiotic-impregnated articulating cement spacer, and after 6 weeks of systemic antibiotic therapy– implantation of a distally-interlocked modular femoral reconstruction prosthesis due to the severe bone defects in the left femur. The operative approach was through the previous operation scar of anterolateral approach– the third fistula was found underneath the scar tissue which was connected to the greater trochanter region. Tissue samples were taken for microbiological examination. Debridement, resection of the three fistulas, removal of the femoral and acetabular componentswith the use of an osteotome,and irrigation using the pulse lavage system with physiological sodium chloride solution wereperformed.Due to the severestructural changes and a large medial wall defect in the proximal femur withimpairment of a large soft tissue zone, it was decided during operation not to put any other implants in the hip joint. All cultures from soft tissue samples collected from the hip joint region grew Staphylococcus aureus (resistant only to trimethoprim-sulfamethoxazole). The patient received antibiotic therapy with oxacillin for 16 days. On the 1stpost-operative day, the patient started physiotherapy– learned to sit in the bed and began passive exercises of the left hip joint. On the 2ndday,the patient began to stand and gradually learned to walk with crutch assistance. He was allowed touch-down weight-bearinguntil full wound healing. A soft left hip orthosis was applied to maintain muscle tone and decreaseedema. The surgical wound healed without any signs of infection and the skin clips were removed on the 14th post-operative day. The patient was discharged from the hospital and continuedantibiotic therapy with ciprofloxacin for 4 weeks. After 5 months, magnetic resonance imaging of the pelvic region showed fluid collections in the soft tissues of the left hip joint region (). The patient had an ultrasound-guidedleft hip joint fluid aspiration, but the acquired samples did not grow any microorganisms. After 1 year from the last surgery, the patient is relatively satisfied–he is walking with two crutches and can apply 10% of body weight on the left leg when standing. The patient has acceptable clinical results without antibiotic suppression therapy –edema in the left leg has decrease; there is no pain in the left hip region during movement and no active fistulas. C-reactive protein is 2.0mg/L. |
A 36-year-old male patient with a 6-year history of chronic kidney disease of unknown etiology was brought to the emergency department due to neurological impairment that started during the previous hour characterized by altered mental status while he was walking down the street. On examination he was stuporous, with poor response to external stimuli. The patient was admitted to the hospital and vital signs and capillary glucose were determined. Glucose levels were 20 mg/dl and increased to 42 mg/dl after a 50 ml infusion of 50% dextrose. During his stay his mental status recovered after glucose levels were returned to normal parameters, requiring high doses of intravenous glucose. After his stabilization he was transferred to the internal medicine department. We confirmed that the patient was not taking medications that would cause hypoglycemia. On physical examination he was somnolent and pale, with slight oedema in both legs. A new episode of symptomatic hypoglycemia was observed while he was receiving an infusion of 20% dextrose. The infusion rate at that moment was 10.416 ml/hr. The infusion was not being weaned off or was an acute disruption. It caught our attention that although the patient was on a 20% dextrose infusion, he continued with hypoglycemia. Blood samples were taken and the results were abnormal ().\nAlthough hypoglycemia could be explained by chronic kidney disease, the diagnosis of insulinoma was considered, so a computed axial tomography with double contrast was taken but unfortunately there were no abnormal findings. During the patient's hospital stay he received several treatments that failed to achieve proper glucose control. We used ascending doses of diazoxide up to 600 mg/day with a poor response. In order to reduce episodes of hypoglycemia, we started with low doses of octreotide and found a good response that created tolerance quickly, so we decided to increase the dose by 0.1 mcg/kg/h always observing the same phenomenon. We decided to suspend this treatment when we reached 0.4. mcg/kg/h. Finally, we decided to maintain a continuous infusion of 50% dextrose with which we achieved serum glucose levels between 120 and 160 mg/dl. Magnetic resonance imaging and an endoscopic ultrasonography were performed but no conclusive data on any structural pancreatic disorder were obtained. In order to locate the tumor, we performed selective intra-arterial pancreatic stimulation with hepatic venous sampling at 0, 20, 40, and 60 seconds. High insulin levels were obtained after a selective injection of 0.025mEq/Kg calcium gluconate in the proximal splenic and gastroduodenal arteries () (). With these results, the patient was scheduled for surgery. During the procedure, bimanual palpation of the pancreas was performed, as well as a pancreatic ultrasound in which no tumor could be identified. The body and tail of the pancreas were resected. For two days the patient had an adequate glycemic control but after a couple of days, he presented with hypoglycemia again. The macroscopic pathology report did not show any tumor compatible with insulinoma; however, on microscopic examination pancreatic islets with elongated cells and clear cytoplasm compatible with nesidioblastosis were seen (). Unfortunately, during his stay at the intensive care unit, the patient developed late-onset hospital-acquired pneumonia and, in spite of treatment, he developed sepsis followed by septic shock which ultimately caused his death. |
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