A 67 year old obese female patient with a 7-year history of osteoarthritis and severe pain in both knees restricting activity was admitted for a right total knee replacement. Patient was not having any pre-existing co-morbid conditions. As per anaesthetic plan, an Epidural block was instituted with a mixture of Bupivacaine 100 mg. and Lignocaine 200 mg. in a volume of 24 ml. Patient was sedated with Inj.Diazepam 10 mg IV. The use of a tourniquet was not contraindicated as the popliteal and dorsalis pedis pulses were palpable. After exsanguinations, the tourniquet was inflated to 350 mm Hg and surgical site prepared and operation commenced. Upon incision, significant bleeding was noticed from the skin and subcutaneous planes. Suspecting tourniquet inadequacy, inflating pressure was increased to 425 mm Hg, which resulted in increased bleeding. Surgery was temporarily interrupted and the tourniquet cuff and inflator were changed, but with similar results. Suspecting bleeding due to incompressible artery and venous tourniquet effect, the tissues were closed and surgery deferred pending investigation and workup. Review of patient's radiological data revealed signs of femoral arterial calcification, confirmed by vascular Doppler study. Left total knee replacement was planned two weeks later with adequate blood components in reserve. Trial of tourniquet was decided. Under Epidural anaesthesia, after exsanguination, tourniquet was inflated to 425 mm Hg and venous congestion of the leg was noticed. 10 minutes prior to tourniquet inflation, Inj. Tranexamic acid 10 mg/kg was given as a bolus i.v. On incision, excessive bleeding was encountered. Subsequently the tourniquet was deflated and the operation continued. Surgery was completed with intraoperative loss of 600 ml and transfusion of two units of packed red cells. Subsequent course in the hospital was uneventful.


A 57-year old female presented to us with constipation, post-prandial epigastric fullness, tiredness, nausea and belching. These symptoms were present for more than 15 years without any significant change in severity. She used to pass stool every 2 to 3 days and had feeling of incomplete evacuation. Stools were hard, lumpy, which were difficult to pass (Bristol type I, ). She denied evacuating manually or having rectal prolapse. Alarm features such as unintentional weight loss, bleeding per rectum, severe abdominal pain and anorexia were absent. There was no family history of colonic malignancy. She did not have vomiting. She had 4 children and underwent hysterectomy 20 years ago for symptomatic fibroid. She underwent surgical treatment for left para-sellar cavernous hemangioma 6 months ago. Laboratory findings revealed mild anemia (hemoglobin 10.0 g/dL), fasting blood sugar 88 mg/dL, serum creatinine 0.7 mg/dL, serum albumin 4.0 g/dL and normal thyroid stimulating hormone level (2.82 mIU/L). Colonoscopy showed a diminutive polyp in rectum, which was a hyperplastic polyp on histology (). Colonic transit time using a previously validated protocol (20 markers each at 0, 12 and 24 hour; abdominal radiographs at 36 and 60 hour) showed 54 of 60 markers retained at 60 hour (abnormal > 14 markers) suggestive of slow colonic transit (). Radionuclide gastric emptying study using technetium 99-m sulfur colloid labeled standard solid meal showed clearance half time of 61 minutes (normal 83 ± 13 minutes). Glucose hydrogen breath test using a standard protocol (Quin Tron Breathtracker™ Digital Microlyzer, QuinTron Inc, Milwaukee, WI, USA) revealed rise in breath hydrogen from 13 parts per million (ppm) in fasting state to 36 ppm after ingestion of 100 g glucose (abnormal > 12 ppm than basal) suggesting small intestinal bacterial overgrowth (Figure; sensitivity 44% and specificity 80%). She had high methane excretion in breath (50 ppm) both in fasting state and after ingestion of glucose suggesting presence of methane producing bacteria in the gut (). Since methane is known to reduce gut transit that may result in constipation, she was treated with rifaximin 1,200 mg per day in 3 divided doses for 14 days to reduce methane in her gut. Her symptoms improved with stool frequency of 1 per day of Bristol type IV without laxative and enema (). CTT was repeated to reassess colonic transit, which showed 30 of 60 markers retained after 60 hours (). Glucose hydrogen breath test repeated after 2 weeks did not show small intestinal bacterial overgrowth and methane excretion in breath was low (< 5 ppm, ). At this stage, she was prescribed with low-dose tegaserod, which is known to prevent recurrence of symptoms following treatment directed to small intestinal bacterial overgrowth. She remained well during 2-month follow-up.


Case 1: C.C, female, 62 years old, admitted to our department on January 2003 due to severe residual post/traumatic right knee pain since 10 months. The pain was aggravated by standing and short distant walk which leading to limitation of her usual daily activities. She was suffered of chronic psychic depression, on treatment with antidepressant drugs. Clinical examination reveals mildly swollen tender right knee with varus alignment and extension lag of 15°. Passive range of motion limited between 15-90 degrees. Clear static instability in medio-lateral stress test while is negative in antero-posterior stress tests. She can stand and walk with assistance of one cane. AKS score was 30.
Two views plain radiographs (AP and Lateral) (Fig. ), showed mal-union of depressed medial plateau fracture, metphyseal sequestrum fragment and displaced medial fragment with attached medial collateral ligament, laterally sublaxed tibiofemoral articulation, varus deviation of limb mechanical axis of 21°. Moderate osteo-arthritic changes of paltello-femoral joint and wide diaphyseo-metophyseal canal with thin cortices are also noted on both views.
The plain radiographs of both hips and lower extremities were looks normal. CT scan of right knee confirmed the same X-Ray configuration, pointing out the displacement of bone fragments and the deep cavity defect of medial plateau. According to Anderson Orthopedic Research Institute (AORI) classification, we consider this case as type 2A bone stock defect due to wide medial metaphyseal (28mm height) defect and absence of medial cortex.


Case 2: R.L, female, 65 years old, on October 2008 admitted to our department referring severe recurrent post-traumatic left knee pain since accidental fracture of medial plateau 6 months before. It's associated with static and functional instability that necessitate wearing of hinged knee brace fixed in 30 degree of flexion. She was suffered of chronic panic depression since the age of 34 years, on treatment with anti-depressive drugs. Also had history of multiple myeloma of lower dorsal vertebre treated with drugs and radiation therapy.
Clinical examination revealed moderately swollen left knee, excessive varus alignment in resting position, extension lag of about 15°, passive range of motion restricted between 15-70°, widely unstable tibia-femoral articulation in both antero-posterior and medio-lateral provocative stress. She can stand and walk only with assistance of axillary crutches. AKS score was 34.
Usual radiographs of left knee (Fig. ) shows displaced depressed non-union of medial plateau fracture with central fragment sequestrum and displaced medial cortical fragment with the intact insertion of MCL, laterally subluxed tibio-femoral articulation, thin trabecular metaphyseal bone stock with thin cortices and varus knee angulation of 18° from central mechanical axis. Early osteo-arthritic changes on lateral patello-femoral view. CT scan of left knee has confirmed the same finding of plain X-Ray. Tibial bone defect considered to be type 2A of AORI due to large medial segmental (27mm height) defect and absence of medial cortical wall.


Case 3: T.R, Male, 59 years old, admitted to our department on March 2009 complaining of sever residual right knee pain and limb gait since car accident occurred one year before, which causing fracture of medial tibial plateau treated surgically with screw fixation, above knee cast. Two weeks later he was candidate for femoro-popletial bypass grafting due to popletial artery contusion and thrombosis. Clinical examination revealed mildly swollen knee with medial tenderness, small antero-medial skin scars. The lower limb resting in severe -varus deviation, with extension lag of 5°, and passive range of motion restricted between 5°-90°; marked medio-lateral joint laxity by applying provocative stress were also present. AKS score was 51.
The plain radiographs (Fig. ) showed mal-union of large segment medial palteau depression fixed with one cancellous screw, lateral subluxation of tibio-femoral joint, good metaphyseal bone characters and cortical thickness, varus angulation of 21° from neutral mechanical limb axis and no patello-femoral arthritis. CT scan with 3D reconstruction scan (Fig. ) of right knee confirmed the severity of the defect considered as type 2A of AORI due to large medial metaphyseal segmental (33mm height) defect and no medial cortical wall seen.
So by discussing the clinical and radiographic situation with each one of the three patients, regarding sever pain in unstable sublaxed knee with medial proximal tibial bone loss type 2A of AORI and considering progressive disability even for usual daily activities in such patient age group. We prepared and planned for total knee replacement surgery with stemmed tibial component, posterior stabilizer liner and reconstruction of the medial tibial segmental bone defect with massive structural bone allograft.


A 90-year-old man was admitted to the intensive care unit (ICU) because of dyspnea and lethargy. The patient had been diagnosed with prostatic cancer ten years before but had not received chemotherapy in the last two years. He also had chronic congestive heart failure with atrial fibrillation, probably related to arterial hypertension. His current prescriptions included furosemide (40 mg once a day) and captopril (25 mg twice daily). He had been institutionalized in a hospice for the previous six months and had symptoms of dyspnea after performing light to moderate activities.
Upon admission, the patient was somnolent (hypoactive delirium) and exhibited no focal neurologic signs. He complained of shortness of breath and lower back pain. His extremities were cold and had delayed capillary refill (about 4 seconds). The patient's blood pressure was 74/46 mmHg, and his heart rate was 120 beats per minute (atrial fibrillation). His central venous pressure was clinically estimated to be 17 cm of water. His breathing pattern was periodic and presented as apnea interspersed with faster and deeper breaths, which we determined to be Cheyne-Stokes respiration (CSR). He received supplementary oxygen through a nasal catheter at two liters per minute with a peripheral arterial O2 saturation of 99%. Rales were detected throughout the length of the patient's hemithoraces.
Laboratory exams showed a moderate level of metabolic acidosis, with a pH of 7.20, a low bicarbonate level of 17.8 mEq/L and a standard base excess of -10 mmol/L. His carbon dioxide level was slightly elevated at 46.3 mmHg, his lactate level was 2 mmol/L, and there were no electrolyte disorders detected. Initial troponin and creatine kinase-MB were both within normal ranges. Echocardiography was not available at the time.
A diagnosis of cardiogenic shock was established. After meeting with the patient's family, it was decided to treat the patient without invasive mechanical ventilation, dialysis, central venous puncture or vasoactive amines. The patient also refused noninvasive mechanical ventilation. Because the patient had already received 1L of 0.9% NaCl before ICU admission, only a small fluid challenge of 250 mL of lactated Ringer's solution was administered. However, no improvement in the patients parameters was observed. A low dose of dobutamine was administered, but the patient developed several episodes of non-sustained ventricular tachycardia and hypotension, so dobutamine administration was stopped. Additionally, nitrates were not administered due to the patient's low arterial blood pressure. Nesiritide was not available at the time. Therefore, we decided to try hypertonic saline infusion as a final, non-invasive approach to improve myocardial contractility. We infused 100 mL of 10% NaCl over a period of 10 minutes.
Less than three minutes after the infusion, the patient's breathing pattern improved, with the progressive reduction in the time of peak ventilation, cycle length, apnea length and ventilation length. Finally, the patient's breathing pattern returned to normal (). The patient's blood pressure increased to 110/70 mmHg, and his heart rate decreased to 106 beats per minute. His capillary refill time also improved. One hour later, intravenous infusion of a low dose of nitroprusside was performed, which was well tolerated by the patient. The patient was discharged from the ICU after being treated with oral vasodilators for an additional few days.


The patient was a 15-year-old boy with noncontributory medical history. He complained of lingering pain to cold. The dental examination revealed normal soft tissue appearance. The first right lower molar had a large carious lesion.
Diagnostic tests were performed; the involved tooth responded with mild pain to percussion and severe lingering pain to the cold thermal test. It was not responded within normal limits to the electric pulp test (EPT). A radiographic examination showed a large carious lesion associated with condensing apical periodontitis []. The adjacent and opposing teeth were asymptomatic and responded normally to all diagnostic tests.
Following clinical and radiographic diagnostic examination, pulpotomy of the first mandibular molar was decided as the treatment. A 0.2% chlorhexidine rinse was carried out. The tooth was anesthetized with 2% lidocaine with 1:80,000 epinephrine (Daroupakhsh, Tehran, Iran), isolated with a rubber damn and then caries was excavated; the exposed coronal pulp tissue was removed with a sterile round diamond bur with water spray. Hemorrhage was controlled with sterile cotton pellets; a ~2 mm layer of the CEM cement (BioniqueDent, Tehran, Iran) was placed without any pressure to cover the exposed pulp stump. The permanent restoration was then completed with amalgam.
The patient was re-examined clinically after 1 and 7 days; tooth mobility was within the normal physiological range, the percussion test was slight positive to negative, and patients were asymptomatic. Six months, one-year, and 2-years follow-ups revealed no clinical or radiographic pathological findings (i.e. inflammation, infection, calcification, and resorption) on the first mandibular molar; radiographs showed normal periodontium apparatus, an evidence of complete periapical healing [–].


A 25-year-old woman was evaluated for refractive surgery. She was noted to be susceptible to keloid scarring, as demonstrated by the presence of scars on both ears. Preoperative best-corrected visual acuity (BCVA) was 20 / 16 in both eyes. Preoperative spherical equivalent (SE) was -6.5 diopters (D) in the right eye (OD) and -6.25 D in the left eye (OS). Ophthalmic examination was unremarkable except for moderate myopia. Preoperative characteristics and demographics are shown in .
The uncorrected visual acuity (UCVA) was 20 / 32 (OD) and 20 / 20 (OS) one day postoperatively. At 3 days, 1 week, 1 month, 3 months, and 6 months postoperatively, the UCVA was 20 / 100, 20 / 20, 20 / 20, 20 / 12.5 and 20 / 12.5 (OD) and 20 / 20, 20 / 20, 20 / 16, 20 / 12.5, and 20 / 12.5 (OS), respectively. The patient was invited to a re-examination 21 months after the surgery, when the SE was -0.125 D (OD) and -0.25 D (OS). No adverse events, including corneal haze, were observed in this patient, and her visual acuity remained at 20 / 12.5 in both eyes.


A 28-year-old woman was examined for refractive surgery. Her general history revealed a tendency to form keloids, as demonstrated by a scar on her left shoulder (). Preoperative BCVA was 20/16 in both eyes, and preoperative SE was -5.25 D (OD) and -6.00 D (OS). Ophthalmic examination was normal except for moderate myopia. Preoperative characteristics and demographics are shown in .
One day postoperatively, the UCVA was 20 / 25 in both eyes. At 3 days, 1 week, 1 month, 3 months, and 6 months postoperatively, the UCVA was 20 / 20, 20 / 20, 20 / 16, 20 / 12.5, and 20 / 12.5 (OD) and 20 / 25, 20 / 20, 20 / 16, 20 / 12.5, and 20 / 12.5 (OS), respectively. At her final examination, the SE was -0.50 D (OD) and -0.25 D (OS). There were no postoperative complications such as corneal haze ().


A 3-yr-old boy presented with abdominal pain was referred to our institution for the evaluation of a progressive bulge in his right abdomen that his mother had first noticed seven days previous on February 12, 2009. A biochemical evaluation revealed no significant abnormalities. Serum levels of tumoral markers (AFP, CA19-9, CA125 and CEA) were within reference limits. A physical exam revealed a mildly tender mass in the right hypochondrium extending to the epigastrium; however, two lesions were found after a dynamic computed tomography (). The primary lesion (8.5 × 4.3 cm) was located in the head of the pancreas and partly herniated into the abdominal wall. While the secondary lesion (1.8 × 2.1 cm) was in the left abdomen near the spleen and the tail of pancreas, and well demarcated from the surrounding structures, and no calcifications were evident within them (). They all showed well-defined margins and inhomogeneous attenuation due to the presence of cystic and solid areas, in the venous phase () and the delayed phase (), a continuous enhancement was observed with a parenchyma density that was higher than that of the arterial phase. In addition, a large and distorted vascular shadow () inside of the tumors in the arterial phase in the primary lesion. There was no radiological evidence of nodal or distant metastases; however, there was a local infiltration of the adjacent abdominal muscle that required a laparotomy.
Surgery revealed the primary lesion was firmly adhered to the adjacent omental and abdominal muscle; in addition, a local infiltration of the liver and stomach were also noted. The head of the pancreas was compressed and could be separated from the tumor due to well-defined margins. The bile duct, portal vein, and the inferior vena cava were also compressed but patent. The gross specimen showed a resected mass measuring 8 × 4 × 4 cm that was well circumscribed and solid with areas of necrosis and hemorrhaging. No calcifications were evident within the mass. The secondary lesion was also adhered to the adjacent omentum and spleen; however, it was well demarcated from the surrounding structures with no evidence of originating from the pancreas. A histopathological analysis revealed a resected mass measuring 3 × 4 × 3 cm, which was well encapsulated by omentum with no calcifications within the mass. The surgical resection was difficult due to the extensive nature of the tumor, but the two lesions were completely resected without the pancreas being damaged. In the final pathology report following surgery, the diagnosis of pancreatoblastoma was confirmed with histopathological results, and the secondary lesion histology and immunohistochemistry was consistent with the findings of the primary lesion ().
After surgery, the patient was treated with adjuvant chemotherapy (vincristine, cyclophosphamide, and epirubicin) regularly. At the time of this report, 18 months after complete tumor resection, the patient is currently in good condition and without radiological evidence of recurrent tumor or metastases () with normal levels of CA 125, CA 19-9 and AFP to date.


A 26-year-old male, a native of Rajasthan, presented to the dermatology OPD with complaints of swelling over his upper lip of 6 month's duration. The patient had initially noticed a small red raised lesion on the middle part of the upper lip which was not painful, not itchy, and not fluid-filled. The swelling progressively increased in size over a few weeks and then spontaneously ruptured in the center, forming an ulcer. There was crusting and bleeding from the ulcer. There was no history of fever, loss of weight, cough, or other respiratory complaints. There was no history of travel to any country endemic for leishmaniasis. Treatment with multiple drugs, including antibiotics, by various medical practitioners had not caused regression of the lesion.
On examination, the vital parameters were normal. There was no hepatosplenomegaly. All other systems were also normal. Local examination of the face revealed a 4 × 3 cm nodular, indurated, swelling with well-defined edges over the upper lip and extending into the labial mucosa []. The center of the nodule was ulcerated and tender, with overlying crusting and pus discharge. There was a solitary 1 × 1 cm enlarged, nontender, submental lymph node on the left side. A clinical diagnosis of cutaneous leishmaniasis was made, with insect bite reaction and cutaneous tuberculosis kept in mind as remote possibilities.
Investigations revealed a total leukocyte count of 7800/mm3and absolute eosinophilic count of 1050/mm.[] All other hematological, biochemical, and radiological investigations were normal. VDRL and ELISA for HIV were negative. Slit-skin smear from the lesion showed lymphocytes with plasma cells, but no diagnostic clue was obtained. Skin biopsy from the edge of the lesion showed well-defined lymphocyte granulomas, with few plasma cells []; however no definite diagnosis could be made. As we were not able to confirm the diagnosis with these usual tests, we carried out fine needle aspiration cytology (FNAC) from the draining lymph node. Slides from the aspirate showed macrophages filled with Leishman-Donovan (LD) bodies []. A few LD bodies were seen outside the cells also. The diagnosis of cutaneous leishmaniasis was thus confirmed.
We treated the patient with intralesional sodium stibogluconate once a week for 4 weeks and tablet ketoconazole 400 mg once daily for the same duration. Two weeks after the start of treatment, the lesion had dried up and had started regressing in size []. The lesion completely regressed, without any residual scarring or pigmentation, over 4 weeks. The patient has now been followed up for 1 year and shows no signs of relapse.


A 46-year-old man was admitted in the department of cardiology in Sfax Tunisia in January 2009 for coronary artery disease and underwent coronary artery stenting. Clopidogrel (150 mg/day) was initiated on January 8, 2009, with lysine acetylsalicylate (one bag/day) and molsidomine (60 mg/day). Four weeks later, simvastatin (20 mg/day) was started. After 11 days, the patient developed ageusia with decreased appetite. However, the sense of smell was not affected. The ear nose throat (ENT) examination ruled out abnormality (dryness, atrophy, or infection) of buccal mucosa and cranial nerve lesion. It was considered that simvastatin could be responsible for this adverse effect and it was then stopped. However, the inability to taste persisted. Hence, the dose of clopidogrel was reduced to one tablet per day. Five months later, ageusia partially reduced.


A 66-year-old man with an acute onset of headache and a brief period of loss of consciousness was diagnosed with acute subarachnoid hemorrhage and his cerebral angiogram revealed a small ACoA aneurysm []. His past medical history was remarkable for myocardial infarction, hypertension, and chronic obstructive pulmonary disease. On admission, his Glasgow coma scale score was 14. Subsequent attempts to occlude this ACoA aneurysm through endovascular routes were unsuccessful because of coil herniation into the ipsilateral A2 branch, which was retrieved successfully without any obvious ill effect. Surgical clipping of the aneurysm was subsequently performed through a standard right frontotemporal craniotomy on the first day of admission. Intraoperatively, following temporary occlusion of the A1 segment, a 7-mm Yasargil clip (Aesculap, Tuttlingen, Germany) was placed across the neck of the aneurysm and both aneurysm lobes were clipped across their common neck. There was no identified intraoperative injury to the aneurysm neck or ACoA during microdissection, including clip application. The wall of the ACoA complex was not obviously dysplastic. The aneurysm did not rupture intraoperatively. Intraoperative angiogram revealed no evidence of residual aneurysm.
The patient's neurological status remained stable postoperatively. A CT angiogram done 3 days later, as part of the work-up to assess for worsening confusion, revealed mild anterior circulation vasospasm without any evidence of residual or recurrent aneurysm []. A repeat four-vessel cerebral angiogram without 3D reconstructions 9 days later revealed mild right A1 and A2 vasospasm without any evidence of residual or recurrent aneurysm []. Eighteen days after his first surgery, the patient developed a sudden onset of altered mental status. A head CT demonstrated a small amount of increased basal subarachnoid hemorrhage. A subsequent cerebral angiogram revealed a relatively large pseudoaneurysm originating from the ACoA adjacent to the neck of the previously clipped aneurysm and projecting superiorly []. In retrospective review, this pseudoaneurysm may have been present on the angiogram completed on day 9 []. The pseudoaneurysm projected superiorly between the two A2 segments.
Endovascular attempts at aneurysm occlusion were again unsuccessful. A repeat right frontotemporal craniotomy was performed on the same day, and we clipped the newly diagnosed aneurysm by placing a 6-mm fenestrated clip across the ACoA and included the proximal A2 in the clip's fenestration. The aneurysm was found to barely contain a wall; its neck was located about 2 mm posterior to the neck of the original aneurysm and originated from the superior aspect of the ACoA. This aneurysm was not apparent during the first surgery despite a thorough inspection of the region. The clip from the first surgery was not manipulated and was noted to completely occlude the neck of the original aneurysm. Clip application during the second surgery was challenging due to the presence of the clip from the first surgery.
An intraoperative angiogram revealed reasonable occlusion of the aneurysm with its minimal faint filling and contrast stasis. A portion of the ACoA was noted to be significantly stenosed by the clip. Since other attempts at clip repositioning to keep the ACoA patent led to further filling of the aneurysm, we determined that sacrificing a portion of the ACoA, which did not harbor any hypothalamic perforator, was acceptable.
Two postoperative follow-up CT angiograms, performed 3 days apart, revealed no further growth of the pseudoaneurysm. Two weeks after the second surgery, a repeat cerebral angiogram revealed no significant residual aneurysm []. The patient made a good recovery and suffered from some memory difficulty, but was able to conduct his average daily activities independently. A repeat CT angiogram 3 months after the second surgery revealed no obvious further vascular abnormality.


A 67-year-old male underwent transurethral resection of prostate (TURP) four years ago for benign prostatic hyperplasia. Six months later he developed 1.5cm long 6Fr caliber proximal bulbar urethral stricture and underwent endoscopic internal urethrotomy (EIU). He had recurrence of stricture at the same site six months later. As he had multiple comorbid illnesses precluding a urethroplasty and he was not willing for the same, a 35mm Microvasive Ultraflex 42Fr permanent metallic urethral stent was placed after internal urethrotomy. He was asymptomatic for three years and later on developed voiding urinary tract symptoms associated with serosanguinous discharge per urethra for three days. He presented to the Emergency Department with acute urinary retention. An attempted urethral catheterization failed. Suprapubic cystostomy (SPC) was done and a 14Fr foley's catheter placed. Plain X-rays of the pelvis revealed a 2cm long calculus impacted within the urethral stent [Figures and ]. Urethrolithotripsy using 8.9 Fr / 13 Fr Wolf semi-rigid ureteroscope was done due to firm stone impaction. There was no obstruction intraluminally across the urethral stent and the stent was well covered. After urethral catheter removal, he voided well with good flow. Post operatively he is voiding well.


A 57-year-old female was admitted to our coronary care unit with the complaints of severe chest pain radiating to left arm and diaphoresis. She was a known hypertensive and on oral metoprolol 100 mg/day for the last 7 years. On examination she was found to be tachypnoeic; with a pulse rate 110/minute, regular, normal volume; and blood pressure 165/95 mmHg. Examination of other systems revealed no abnormalities. The biochemical parameters were within normal limits. Troponin-T value was strongly positive. After initial stabilization, she underwent coronary angiography and detected to have triple vessel disease with >80% blockade of all the major coronary vessels. Surgical correction was planned a week later.
She was premedicated with diazepam 10 mg night before, 5 mg in the morning PO and morphine sulfate 0.2 mg/kg, and phenergan 25 mg IM 1 hour before surgery. Metoprolol 50 mg PO was continued till the morning of surgery. The anesthesia induction and maintenance regime included thiopentone sodium, midazolam, fentanyl, rocuronium, and pancuronium. Coronary artery bypass grafting was done under moderate hypothermia and cold blood cardioplegia. The patient was weaned off successfully from cardiopulmonary bypass under the cover of nitroglycerine infusion and shifted to postoperative cardiac surgical intensive care unit. The trachea was extubated 6 hours after surgery. She was found to be comfortable, breathing normally, with blood pressure of 118/68 mmHg, pulse rate 67/minute, and SaO2 97% in room air. Two hours later she was found to be agitated, had a sudden rise in blood pressure to 200/100 mmHg and diaphoresis. As the last dose of fentanyl was administered 3 hours before extubation, pain was considered the cause and she was administered 1 μ/kg body weight fentanyl to get a quick relief from symptoms. However, instead of relief, she developed myoclonus, headache, nausea, and vomiting. An infusion of nitroglycerine 1 μg/kg/min was started to reduce the blood pressure. A possibility of neurological complication related to cardiopulmonary bypass was thought and an emergency CT scan of the head was performed which revealed no abnormality. The family was informed of the condition and possibility of an adverse outcome. Her husband revealed that she was under fluoxetine 60 mg/day therapy since the last 18 weeks. The possibility of serotonin syndrome was considered. Diazepam 5 mg was administered IV and cyproheptadine 20 mg PO to reduce the symptoms. The improvement in symptoms was noticed within 4 hours and complete resolution was achieved after 30 hours. Fluoxetine therapy was resumed after 36 hours in a reduced dose of 40 mg/day after a psychiatric consultation. The balance postoperative course was uneventful and she was discharged from hospital on 8th postoperative day.


A 38-year-old woman presented with a two-year history of swelling at the back of the tongue which had been progressively increasing in size. She complained of fastidious presence, difficulty in swallowing, and change in the quality of the voice. No other positive history was noted. Examination revealed a firm, nontender, globular elevated red mass of about 3 × 4 cm in size arising from the base of the tongue and obscuring the posterior pharyngeal wall. It became more prominent and popped further anterior with the protrusion and depression of the tongue by the patient [].
Indirect laryngoscopy could not be accomplished because of the presence of mass. Examination of head and neck was otherwise normal. Laboratory investigations were unremarkable. The contrast-enhanced computed tomography revealed a well-defined round homogenous mass lesion measuring 3.2 × 3.2 × 4.2 cm with smooth margins in the oropharynx extending from the uvula superiorly and up to the level of epiglottis inferiorly. It abutted the base of tongue anteriorly and the posterior pharyngeal wall posteriorly. On either side, it extended to the lateral pharyngeal walls []. There was no evidence of any calcification within the mass.
Fine needle aspiration cytology from the swelling revealed few epithelial cells. Excision biopsy of the swelling was planned and the patient was referred for preanesthetic check up.
General physical examination was unremarkable. Patient's airway assessment revealed Mallampati score 4 with a normal mouth opening and neck movements. The thyromental and sternomental distances were 7 and 13 cm, respectively. Bilateral nares were patent and there was no deviation of nasal septum. In view of the above mentioned findings, we planned to perform fiberoptic bronchoscope (FOB)-guided awake nasotracheal intubation followed by general anesthesia.
The procedure was explained to the patient and informed consent including consent for tracheostomy, in case of emergency, was taken. Standard preoperative protocol was followed. Injection glycopyrrolate 0.2 mg was administered intramuscularly 45 minutes prior to fiberoptic bronchoscopy. Oxymetazoline nasal drops were instilled in both nostrils. Difficult airway cart was prepared and surgeons were informed to be standby for emergency tracheostomy in case of failure of intubation. Routine monitoring was instituted. Patient was administered 1 mg midazolam and 20 μg fentanyl. After achieving airway anesthesia with nebulization of 4% lignocaine and preoxygenating the patient, a check direct laryngoscopy was attempted to assess the feasibility of passing FOB behind the swelling. The mass was seen to be attached to the right side of the tongue base and some space was there on the left side, adequate for the FOB to be passed. However, we were unable to lift the swelling by the laryngoscope blade. A size 7 cuffed Portex endotracheal tube was loaded on to a flexible FOB, which was then introduced through the right nostril and after its tip was in the oropharynx, the patient was asked to protrude and depress the tongue. The mass then popped out, thus further creating some more space in oropharynx, and then FOB was negotiated from left side of the tumor and guided into the trachea. The endotracheal tube was then passed over the FOB. Tube position was confirmed by bag ventilation and capnography. General anesthesia was then induced with propofol 80 mg and fentanyl 80 μg intravenously and muscle relaxation was achieved with intravenous vecuronium 4 mg. Anesthesia was maintained with isoflurane in oxygen and nitrous oxide. The mass was well encapsulated, arising from the base of the tongue and was extending to the right tonsil. It was not adherent to surrounding structures and good cleavage plane was found, and the complete mass was removed. After the completion of surgery, trachea was extubated uneventfully. The histopathology including immunochemistry of the resected specimen indicated that it was a Schwannoma.


An 18-year-old female patient with body weight 145 kg, height 164 cm, and body -mass index (BMI) 53.9 was posted for LSG. Preoperatively, her blood pressure was 118/68 mmHg, pulse 70/min, temperature 36.8°C, and she had a history of occasional attacks of bronchial asthma (seasonal). Her preoperative SaO2 % on room air was 99%, and there was no history suggestive of obstructive sleep apnea. Her pre-operative investigations did not reveal any abnormality except a large fatty liver. She was pre-medicated with omeprazole 40 mg IV and metoclopramide 10 mg IV 2h before surgery and midazolam 2 mg IV was administered in the receiving area of the operation theatre. She had received 3500 IU of tinaparin 2 h earlier. Graduated compression stockings were applied before the surgery. Ceftriaxone1000 mg was administered intravenously an hour prior to the surgery.
On arrival to the operation room, the monitors were attached and she was pre-oxygenated for 2 min. Anaesthesia was induced, with the patient in the 30° anti-trendelenberg position, with IV fentanyl 100 μg, IV lignocaine 60mg, IV propofol 200mg, and cisatracurium 18mg. Her trachea was intubated with a 7 mm ID oro-tracheal cuffed Portex tube. Anaesthesia was maintained with 80% oxygen, sevoflurane (end-tidal 2%), alfentanil infusion, and intermittent doses of cisatracurium, as required. The lungs were ventilated with tidal volume of 500 ml, at the rate of 14-18/min and PEEP of 7 cm of H2O using a low flow technique. ECG, NIBP, SaO2 %, end-tidal CO2, nasopharyngeal temperature, and TOF were monitored. She received 2000 ml of lactated Ringer's solution and 500 ml of 6% hydroxyl-ethyl starch (Haesteril 6%, Fresenius Kabi); during anaesthesia which lasted for 120 min.
Almost 20 min after induction of anaesthesia, when the patient was positioned for surgery, fentanyl 50 μg IV bolus was administered while the surgeon introduced the Veress needle through the upper left quadrant of the abdomen in the supine position. The intra-peritoneal insufflation using CO2 was started with high flows to achieve intra-abdominal pressure of 20 mmHg. Within less than 1 min, the end-tidal CO2 dropped from 35 to 9 mmHg, the patient was cyanosed and SaO2 % dropped to 67%. Her heart rate increased to 130/min from 78/min. However, her arterial blood pressure was maintained at 128/78 mmHg. The airway pressure was between 32 and 35 cm of H2O.
Gas embolism was suspected and the surgeon was informed immediately. FiO2 was increased to 1, fresh gas flow increased to 8L/min, sevoflurane stopped, and manual bag ventilation was carried out. The surgeon stopped the insufflation. Tredelenburg position was given immediately and intravenous 6% hydroxyl-ethyl starch was infused fast to raise the venous pressure to prevent further entrainment of the gas. Within 5 min SaO2 % increased to 100%, and end-tidal CO2 increased to 25 mmHg and later to 42 mmHg. The auscultation of chest revealed bilateral good and equal air entry, which excluded pneumothorax, and no mill-wheel murmur was detected. The pupils were bilaterally equal, small and reacting well to light. When heart rate dropped to 110/min, sevoflurane and alfentanil infusion 500 μg/h were resumed. Arterial blood gas analysis 15 min after the insult showed respiratory acidosis: pH 7.24, PCO2 67mmHg, PO2 220 mmHg, HCO3 28.5 mmol/L. FiO2 was decreased to 0.7 at this stage.
After almost 30 min, after the patient was stabilized, the surgeon directed the Veress needle more caudad and insufflated at lower flows to achieve the pressure of 10 mmHg under vision. A small hole on the anterior surface of the left lobe of the liver could be seen from where the gas was being entrained [Figures and ]. The surgeon covered this bleeding hole with a wet gauze [Figures and ] and applied pressure laparoscopically and later sealed it with surgicel. Since there was no further entrainment of the gas, he carried out the surgery uneventfully.
The arterial blood gas analysis after 1 h from the insult showed complete recovery: pH 7.36, PCO2 42 mmHg, PO2 147 mmHg, HCO3 23.2 mmol/L; though the airway pressure maintained between 32 and 35 cm of H2O and the heart rate was consistently around 110/min throughout the surgery.
At the end of the operation the neuromuscular blockade was reversed with atropine 1mg and neostigmine 2.5mg. The trachea was extubated when the T1:T4 ratio reached 87% and she was completely awake.
The patient stayed in the PACU (post anaesthesia care unit) for 2 h under complete monitoring and observation. She was vitally stable. Blood pressure was 140/80 mmHg, SaO2 % was 100% on O2 6 L/min through Hudson's mask, and 96% on room air. The pulse rate was 90-110/min and the respiratory rate was 16-18/min. Pain was relieved by IV infusion of paracetamol (Perfalgan, Bristol-Myers Squibb Pharmaceuticals Ltd) 1G and small boluses of IV pethidine.
The patient was shifted to the ward in stable condition under observation of vital signs and SaO2 % in the ward. O2 6 L/min through Hudson's facemask was given for 24 h. Incentive spirometry and chest physiotherapy were begun within 4 h after the surgery. The patient was discharged home after 2 days without any complications.


Mrs. R, an 84 year-old-lady, with no significant past medical history, presented to the emergency department with a fracture of the left femoral neck following a fall. Hemiarthroplasty was performed the following day. Post-procedure, the patient failed to regain her sensorium for more than 10 h. Examination revealed a febrile patient with a pulse rate of 112/min and a blood pressure of 110/80. General physical exam and cardiovascular exams were normal; she was intermittently tachypneic with a respiratory rate of 40/min and clear lungs. She was stuporous with minimal wincing to pain, but no verbal or motor response, deep tendon reflexes were sluggish and plantars were extensor. Fundoscopic examination was normal. The laboratory parameters are shown in . The renal, liver function tests, and the electrolytes were unremarkable. Arterial blood gas analysis did not show hypoxemia, paO2 was 102 mm Hg; and pulse oximetry showed an O2 saturation of 99%. In view of deteriorating sensorium, she was intubated and ventilated. CT scan of the brain was unremarkable. CT scan of her chest did not show evidence of pulmonary embolism, venous doppler of the lower limbs was normal. A transthoracic ECHO did not reveal a patent foramen ovale, elevated pulmonary artery pressures, or any other abnormality. Blood cultures showed no growth.
MRI brain done 2 days later showed a shower of emboli with multiple scattered tiny hyperintense DWI (diffusion-weighted imaging) [] lesions in bilateral cortical areas, deep watershed regions, basal ganglia, and the posterior fossa. Some of these lesions were hypointense on apparent diffusion coefficient (ADC) [] map and a few of them were isointense. Gradient Echo (GRE) showed hypointense lesions suggestive of petechial hemorrhages []. There was no vascular occlusion or stenosis on MR angiography.
Transcranial Doppler performed for a duration of 30 min detected emboli in bilateral middle cerebral arteries (MCAs). PMD (Spencer Technologies, Inc; PMD 100 mol/L) was used along with a 2-MHz pulsed-wave transducer to generate simultaneous M-mode and spectral TCD displays. Probe fixation using a head frame (Marc 500, Spencer Technologies) was used for monitoring. The insonation depth for spectrogram recording was between 45 and 65 mm for the ipsilateral MCA.
Microembolic signals (MES) were defined as high-amplitude, unidirectional, transient signals lasting less than 300 ms [] and associated with an acoustic resemblance to a characteristic chirp or snap. Ten MESs each were detected in either MCAs. The intensity of solid MESs ranged from 7 to 9 dB, and the duration ranged from 40 to 60 ms. These emboli had an Relative Energy Index of MES (REIM) of 0.40. The relative energy index of MES (REIM) was obtained by multiplication of maximum duration of MES in a 3-mm gate (MaxD) with MES maximum intensity adjusted for the intensity of background blood flow (MaxI) on Power M-mode display.[] A final diagnosis of FES was made. She received conservative management in the form of antiplatelet agents, intravenous fluids, deep vein thrombosis prophylaxis, and ventilatory support. Unfortunately, she progressively deteriorated and died after 5 days.


A 22-year-old woman presented with a 3-day history of progressive abdominal pain associated with one episode of vomiting. She had no other concurrent disease and no significant past medical or surgical history. On examination, there was mild tenderness in the periumbilical and right lumbar region. Routine investigations were normal apart from leukocytosis (total count: 20,400/cu mm). The abdominal radiograph revealed a large gas-ﬁlled loop of the bowel in the right mid and lower quadrants []. USG revealed dilated fluid-filled small bowel loops and free fluid in pelvis. A peritoneal tap revealed the fluid to be hemorrhagic.
Contrast-enhanced CT scan of the abdomen revealed a markedly dilated loop of sigmoid colon with loss of haustral pattern, and a thin wall in the right lower abdomen; the “whirl sign” was present, created by the twisted mesentery and bowel, and the afferent and efferent limbs of the sigmoid colon had the appearance of a beak [Figure –]. Ileal loops were also wrapped around the central whirl, and these too had thin, nonenhancing walls as well as afferent and efferent limbs with the appearance of a beak [Figure –]. The cecum and the distal descending colon were deviated medially, with their medial borders having a pointed appearance [Figure , ]. Stretched superior mesenteric vessels converged toward the whirl. In view of the history and the CT scan findings, a diagnosis of ileosigmoid knot with gangrene of the involved sigmoid colon and ileal loops was made.
The patient was immediately operated. Laparotomy showed a gangrenous sigmoid volvulus with gangrenous distal ileal loops twisted around it [Figures and ] and approximately 300 ml of hemorrhagic fluid in the peritoneal cavity. The gangrenous loops were resected and ileo-ascending colon and colo-colic anastomoses were done, along with a proximal defunctioning ileostomy. The postoperative course was uneventful and the patient was discharged a week after the surgery. Subsequent follow-up was uneventful.


Thirty-five year old unmarried male, known positive for HIV infection and on highly active antiretroviral therapy (HAART) since 2001, presented with genital ulcers for a prolonged duration of 16 months. He gave past history of recurrent genital ulcers which were responded to acyclovir. He had undergone circumcision because of recurrent herpes ulcers in 2005. He was initiated on NNRTI containing ART (AZT + 3TC+NVP) in 2001. His CD4 cell count improved in the years 2002–2006 and subsequently in late 2006 he had a decline in CD4 to 140. He underwent genotyping and found to have NRTI and NNRTI mutations. He was switched to boosted indinavir-containing second line HAART regimen in late 2006. His CD4 count improved to 290, 354, 405 and 398 cells/microliter in the follow-up visits. In spite of his improvement in CD4 he developed genital ulcers in 2007 [], which were not responding to the usual line of management with acyclovir and antibiotics. The imprint cytology and scrapings from the lesions showed degenerated and mature squamous cells in an inflammatory background and the patient did not give consent for biopsy. Since the patient was known to have recurrent herpes genitalis, and was responding to acyclovir in the past, we diagnosed it as HSV and his VDRL was non-reactive on four repeated attempts. Dark-field examination for Treponema pallidum was negative on repeated attempts. He was on acyclovir and antibacterial for a prolonged period with some improvement on the genital lesions but genital lesions did not heal fully. In July 2008, to avoid indinavir toxicity he was substituted with boosted atazanavir containing PI regimen. His genital lesions healed completely [] in 15 days with no recurrence for more than two months and his current CD4 count is 437 cells/microliter.


We describe a 33-year-old man who presented a painless massive amount of bright reddish and burgundy rectal bleeding for 3 consecutive days. The patient had neither fever nor abdominal pain. He denied previous digestive tract related medical history as well as taking any of current medicine history, like nonsteroid anti-inflammatory drug. On admission the patient had an acute ill looking appearance and no altered mental status. Resting tachycardia was observed, but his blood pressure was in a normal range. In the second day of admission, he was referred to the endoscopic center where we gauzed the systolic blood pressure just less than 100 mm Hg and he complained dizziness. There were no other specific abnormalities, such as abnormal bowel sound, tender abdomen, or hepatosplenomegaly. The initial hemoglobin level was 12.3 g/dL which subsequently dropped to 9.9 g/dL the next day. Other laboratory exams, like hematologic profiles, were within normal range. The patient was first resuscitated with intravenous saline solution and followed by digestive endoscopies accordingly. An emergency esophagogastroduodenoscopy did not claim any specific lesion, but the colonoscopy disclosed fresh blood at the appendiceal orifice (). Interestingly, after we performed a large amount of water irrigation and removed blood contained liquids, we could not see any fresh blood, implying that the bleeding source did not seem to be around the cecum. Further, following ileoscopy illustrated that the terminal ileum was not evident for any current bleeding episode from the upper level of the tract. However, getting back to the cecum, as the scope entered the appendiceal orifice and after irrigating a small amount of water, it allowed us to corroborate appendiceal intraluminal bleeding in real-time image (). An emergency appendectomy eliminated the bleeding source successfully. On surgery, the appendix, about 8 cm long, had grossly normal appearance except much blood filled in. An eroded focal mucosal lesion was observed at the distal portion of inner part of the vermiform appendix. The histopathological examination revealed a case of appendiceal mucosal erosion (). Although relatively rare, benign appendiceal lesions, such as ulcer or erosion, should be considered a possible cause in patient who present with LGIB. Colonoscopy could reveal the location as well as the characteristics of the affected lesions, whether it provides clinicians with any suspicious source that bleeding takes place.


A 10-year-old boy had been admitted at a nephrology clinic for generalized edema, hypertension, and fatigue that appeared 3 weeks before. His medical past was unremarkable and immunization status was complete. His mother reported baths in stagnant water 5 years ago in their village. But, there was neither personal nor familial history of hematuria or edema. Physical examination showed height 130 cm, weight 30 kg, temperature 37°C, high blood pressure (150/90 mmHg), tachycardia (100 beats/min), and edema over face, back, limbs, and mild ascites. Cardiac and pulmonary auscultation was normal and there was neither arthritis, nor skin abnormality.
Laboratory findings are represented in . Serologies for hepatitis B, C, and HIV were negative and serum complement (CH50) was slightly low. Cryoglobulins, and anti-DNA and anti-streptococcal antibodies were not found. Ultrasound examination of kidneys and the lower urinary tract was strictly normal.
Diagnosis of nephrotic syndrome was made and the patient was treated with oral prednisolone (2 mg/kg/day) combined with captopril (50 mg/day), salt-free diet, furosemide (60 mg/day), and oral supplementation with potassium, calcium, and vitamin D3. Four weeks later, edema decreased slightly and urine albumin excretion slowed down at 187 mg/kg/day. Urine sediment was still unremarkable. A second 4-week period of oral prednisolone was started after three daily IV pulses of methylprednisolone (10 mg/kg/day). However, clinical and biological findings did not improve at the end of this cure. Then, renal biopsy was performed and showed type 1 membranoproliferative glomerulonephritis (MPGN) [] with mild tubulointerstitial fibrosis and a granuloma made of histiocytes and giant cells surrounding an egg of S. hematobium []. Congo red staining was negative and immunofluorescence did not find any deposition. Circulating IgG antibodies against schistosomal antigens were high (1/250). A final diagnosis of type 1 MPGN secondary to S. hematobium was made. The histological findings corresponded to class III–IV of the African Association of Nephrology (AFRAN) classification.[] Despite the absence of adult worms in urine and renal parenchyma, treatment with praziquantel was given (single 40 mg/kg dose per os) to ensure complete cure. After 1 month, there was no improvement in renal function. Then, oral cyclophosphamide (2 mg/kg/day) was added to corticosteroids with supportive therapy (salt-free diet, captopril, furosemide, calcium, and vitamin D3) for 6 months. Nevertheless, the patient reached end-stage renal disease (ESRD) 1 year later and had to start hemodialysis.


A 22-year-old male patient presented with gradually progressive swelling over the right parotid region for last 7 years. He had multiple bilateral enlarged cervical lymph nodes. History and physical examination did not reveal any other illness except mild skin eczema in legs. Airway examination revealed mouth opening 4 cm, modified Mallampatti class II with normal thyromental distance and neck movements. The swelling was 6×5 cm in size, nontender, nonfluctuant, firm in consistency, palpable in the left anterior triangle of the neck, extending from the angle of the jaw to the left preauricular area. No warmth or redness was noted on the overlying skin. Histo-pathology of the mass revealed lymphoid follicles formation with prominent germinal centers, infiltration of eosinophils, and increased postcapillary venules and vascular proliferation suggestive of KD. Cervical lymph node biopsy, done to rule out malignancy, also revealed similar finding.
On further investigation, hemoglobin was 12.8 mg/dl, total leucocyte count 8100 with peripheral eosinophilia (eosinophil 18%, neutrophil 52%, basophil 1%, lymphocyte 25% and monocyte 4%), platelet count 2,43,000 and erythrocyte sedimentation rate was 18 mm fall in first hour. Liver function test showed bilirubin 0.8 mg/dl, total protein 6.9 g/dl, albumin 4.2 g/dl, and normal liver enzymes. Renal function test revealed elevated blood urea (60 mg/dl) and serum creatinine (1.1 mg/dl). Proteinuria 2+ was present in the urine dip-stick test. Serum electrolytes were Na 137 mEq/l and K 4.2 mEq/l. Serum IgE level was markedly elevated (559 IU/ml; normal 0-14 IU/ml). Ultrasound of the abdomen showed that both the kidneys were of normal size and echo-texture. The chest X-ray was normal.
The patient was administered oral alprazolam 0.25 mg and ranitidine 150 mg the night before and on the morning of surgery. In the operating room, intravenous access was obtained and anesthesia was induced with fentanyl 120 μg and propofol 60 mg intravenous. Oro-tracheal intubation was facilitated with atracurium 30 mg intravenous and mechanical ventilation was initiated. Monitoring included pulse oximetry, continuous electrocardiography, noninvasive blood pressure, end-tidal CO2, temperature and neuro-muscular blockade depth by train-of-four (TOF). Anesthesia was maintained with oxygen nitrous oxide, and isoflurane (end-tidal concentration 0.6-0.8%). Analgesia was provided with fentanyl infusion 50-70 μg/h and intermittent boluses of atracurium maintain a TOF count of 1 to 2.
The patient underwent a selective neck dissection to excise the right buccal space mass, including right superficial parotidectomy, and excision of multiple lymph nodes involving the peri-parotid tissues. The facial nerve was preserved by surgeons without resorting to facial nerve stimulation, as the nerve was quite thick and easily identified. Duration of surgery was 4 h. The glands were quite vascular; intra-operative blood loss was 750 ml which was adequately replaced by crystalloids. The patient made 500 ml urine.
Intra-operatively, heart rate was 70-85/min, blood pressure 94/60-128/80 mmHg, oxygen saturation 98-100% and EtCO2 35-38 mmHg. At the end of surgery, fentanyl infusion was stopped, neuro-muscular blockade was reversed and trachea extubated. He was awake and comfortable in the post-anesthesia care unit. Intravenous tramadol 50 mg 8 hourly and intravenous paracetamol 1 gm 8 hourly were prescribed for post-operative analgesia. The post-operative course was uneventful and patient was discharged home after 6 days.


A 22-year-old (weight 60 kg, height 157 cm) American Society of Anesthesiologists (ASA) physical status 1, male patient presented for incision and drainage of an abscess over his left elbow. There was no history of addiction or self-medication with any drugs. Past surgical interventions included external fixation for fracture of both bones of the left forearm along with left brachial artery repair under general anesthesia. Previous surgery and postoperative recovery had been uneventful. General physical examination was unremarkable, except for the abscess over his left elbow.
The patient was premedicated with Diazepam 10 mg orally two hours prior to the surgery. Preoperatively, he had a heart rate (HR) of 72 beats per minute, respiratory rate (RR) of 14 breaths per minute, and BP 110/70 mm Hg. Anesthesia was induced with glycopyrrolate 0.2 mg, ketamine 100 mg, and propofol 100 mg intravenously. Anesthesia was maintained with a mixture of oxygen: Nitrous oxide (40:60) and 1.5% isoflurane, on spontaneous ventilation using a face mask. Duration of the surgery was 15 minutes. He was transferred to the post anesthesia care unit (PACU) after he regained consciousness and had stable vital signs.
Within half an hour of admission to PACU, the patient complained of pain, and became restless and tachypneic (RR = 34/min). He complained of a tingling sensation all over the body and painful spasms. He was given oxygen by face mask. Electrocardiography (ECG) and pulse oximetry (SpO2) monitoring was initiated. His restlessness and tingling sensations continued to worsen. He complained of inability to move his right hand and soon developed carpal spasm of the right hand. Blood samples were collected immediately for arterial blood gas (ABG) analysis and to determine the serum electrolytes, calcium, albumin, and magnesium (Mg) levels. A 12-lead ECG was also taken.
The patient was given 20 ml of calcium gluconate 10% as a slow intravenous infusion over 15 minutes. He showed dramatic improvement in symptoms after the infusion and his vital signs returned to the preoperative values. He was admitted to the PACU for overnight observation. The ABG revealed alkalosis with a pH 7.49, pCO2 22 mm Hg, HCO3 20 meq/L and low serum ionized calcium of 0.70 mmol/L. The serum calcium level was reported as 8 mg%, with an albumin of 2 mg % (corrected calcium of 9.6 mg %). The serum electrolytes and magnesium levels were within the normal range. The patient was discharged the next day from the hospital and was prescribed oral calcium supplements. He was referred to the endocrinology department for further workup of the episode of tetany.


A 14 year-old boy fell on the ground while landing from a high jump during a basketball game. He felt immediate bilateral knee pain and was unable to stand. The physical examination, performed in the Emergency Room of our Hospital, showed bilateral anterior knee swelling, with a marked restriction of the range of motion of both knees. No neurovascular deficits were present. The patient was a well-developed teen-ager, who weighed 80 kg and was 180 cm tall; no previous history of trauma was reported. Antero-posterior and lateral radiographic examinations of both knees showed bilateral fractures of the proximal tibial physis, classified as flexion-type Salter-Harris II, non-displaced on the right side and displaced on the left with an anterior apex.
The day after admission to our Hospital, the patient was treated in the operating room under general anaesthesia. The displaced fracture was reduced, under fluoroscopic control, by closed manipulation in hyperextension but, since it was unstable, it was fixed percutaneously by two 5 mm cannulated screws, introduced from the anterior tibial tubercle to the posterior metaphyseal cortex. A long-leg cast extending above the knee was applied in extension position. The same cast was applied to stabilize the non-displaced fracture, without any fixation. Both casts were left in place for six weeks without weight-bearing. X-rays of both knees, performed one week after the operation, did not show any secondary displacement of the fractures. Six weeks after surgery the casts were removed, and x-rays showed complete healing of both fractures. The patient started physical therapy and weight-bearing was allowed. At the final follow-up, one year after the operation, the patient had resumed playing basketball without restrictions, the range of motion of both knees was complete, and no deformity or leg-length discrepancy was present. The radiographic examination showed almost complete fusion of the proximal tibial epiphyses of both knees (Fig. ).


A 7-year-old girl presented to the emergency room at the rural health center with history of nasal stuffiness for 4 weeks along with history of persistent bleeding from the left nostril for 3 days which frequently soaked her handkerchief in the form of small drops. She gave history of marked irritation in the left nostril, associated with difficulty in breathing when right nostril was occluded. She complained of having a sensation of a moving object in the left nostril and was repeatedly picking her left nostril. She had been prescribed xylometazoline nasal drops and oral pseudoephedrine; however, these did not result in improvement. She did not give history of inserting any foreign body in the nostril, fever, runny nose or similar episodes of epistaxis in the past. Physical examination revealed that the child was in agony and was crying with pain. Her pulse rate was 96/min and blood pressure was 126/74 mm Hg. She had pallor, and fresh blood was persistently oozing from the left nostril. Local pressure did not help in controlling the epistaxis.
Examination of the nasal cavity revealed a soft fleshy material bulging into the left nostril close to the inferior turbinate. Attempt was made to pull it out with the help of an artery forceps; however, being a slimy structure, it repeatedly slipped from the grip of artery forceps and appeared to be tightly stuck to the inferior turbinate. After making a few attempts, the lower part of this fleshy material could be disentangled, and it was found to be moving. At this stage, it was identified as leech infesting the left nostril. Since repeated attempts to dislodge the leech by artery forceps failed, irrigation of the left nostril with normal saline was done and it was decided to wait and watch. After a few minutes, it could be easily dislodged with the help of artery forceps. The leech was still alive and was 8 cm long []. After a few minutes, the bleeding from the left nostril also decreased. Left nostril was packed with gauze piece. The child was given oral decongestant tablets, oral amoxicillin and intramuscular tetanus toxoid. Hematological investigations revealed hemoglobin to be 7.6 g/dL; and total leukocyte count, 9,800/mm3. Examination of external auditory canal did not reveal any evidence of leech infestation. After the procedure, she informed that she belonged to a very low socioeconomic stratum of society and had no access to safe drinking water and had to drink polluted water from an open pond and also take bath in it. Nasal packing was removed the next day, and the child became completely asymptomatic. She was advised not to take bath in polluted stagnant ponds or drink water from the open ponds. She was also advised to report immediately to the emergency department if similar complaints arose.


A 32-year-old HIV-negative woman was referred to the neuro-ophthalmology department with sudden visual loss and retro-ocular pain in left eye that increased with eye movement. She had a history of intractable headache and vertigo. Best corrected visual acuity (BCVA) was 20/40 in the right eye and 20/200 in the left eye. Color vision was reduced in both eyes. Anterior segment examination was normal in the right eye but revealed a mild inflammation in the left. Intraocular pressure was within the normal limits bilaterally. Both fundii had hyperemic optic disc edema, swelling, associated peripapillary hemorrhages, and bilateral neuritis. The left fundus demonstrated an old scar from toxoplasma retinochoroiditis with a new whitish retinochoroidal lesion at the upper end of the optic nerve head []. Laboratory workup results included an erythrocyte sedimentation rate of 6 mm in the first hour (reference range 1–12), and normal white blood cell count. Lumbar puncture (LP) was performed. Serological testing for toxoplasmosis was negative for IgM but IgG titres were 60 IU/ml (reference range >3). Serology tests for Treponema pallidum, and HIV were normal. Fluorescein angiography showed early optic disc diffuse hyperfluorescence in both eyes and branch retinal artery and vein occlusion with late leakage overlying and adjacent to the disc in the left eye. Automated perimetry demonstrated right optic disc enlargement and depression in sensitivity. Automated perimetry of the left eye demonstrated a diffuse depression in sensitivity with a cecocentral scotoma and inferior arcuate defect. T1-weighted axial magnetic resonance imaging (MRI) scan and fluid-attenuated inversion-recovery (FLAIR) MRI of the brain, demonstrated a few tiny non-specific high signal foci in the hemispheric white matter . The patient was treated with sulphadiazine (4 × 1 g/day), pyrimethamine (2 × 25 mg/day) and folinic acid over 6 weeks. Topical steroids and mydriatic drops were also prescribed for inflammation of the left eye. Prednisone (1 mg/kg) was introduced, at tapering doses, during the treatment. After 3 months, visual acuity returned to 20/20 without correction in both eyes. Anterior segment examination was normal. Examination of the posterior segment of the left eye showed regression of the hemorrhages and a white chorioretinal scar.


A 70-year-old man was admitted to our emergency department with weakness, palpitation, nausea and vomiting that arose from eating a few teaspoons of mad honey. He did not have any cardiovascular or systemic disease and was not taking any medication. On physical examination, he was confused, his blood pressure was 60/20 mmHg, and his heart rate was 105 beats/min. The neurological examination was normal. Upon admission, his electrocardiography revealed paroxysmal atrial fibrillation and intermittent left bundle branch block (). There was no structural cardiac abnormality found from transthoracic echocardiography. His hypotension improved rapidly after saline infusion. During observation, his rhythm returned to a normal sinus rhythm six hours after admission (), and he was discharged on the second day of his hospital stay.


A 25-year-old Nigerian man presented to our emergency department with complaints of tiredness, night sweats, and dizziness on exertion. On clinical examination the patient had facial swelling and venous distension in the neck. Blood pressure was 135/77 mmHg with pulsus paradoxus. Cardiac auscultation was normal. The liver was enlarged. There was no peripheral oedema. Electrocardiography showed sinus tachycardia of 110 beats/min, a vertical axis, QS pattern in V1 and a Q wave in V2 and low voltages in the limb leads. Laboratory testing showed lymphocytosis, increased creatinine (120 mmol/l) and abnormal liver enzymes (total bilirubin 47 μmol/l, aspartate aminotransferase 124 U/l, alanine aminotransferase 200 U/l and γ-glutamyl transferase 268 U/l). On chest X-ray, right pleural effusion and mediastinal widening were seen. With abdominal ultrasound the liver diameter was 17.5 cm, with ascites, pleural and pericardial effusion and hepatopetal flow in the portal vein. Transthoracic echocardiography revealed a mass almost completely occupying the right atrium and ventricle with a distended inferior vena cava (Fig. ). The pressure gradient over the tricuspid valve was 25 mmHg (PAP 25 + 15 mmHg). Pericardial effusion was present. Computed tomography (CT) of the thorax with contrast injected into the right antecubital vein revealed a mass in the anterior mediastinum with no clear distinction from the surrounding structures. There was a lack of contrast in the innominate and superior caval veins, and contrast filled the right atrium through the inferior caval vein by way of collateral veins. A large right atrial filling defect was found with faint filling of the right ventricle (Fig. ).
Due to the important haemodynamic compromise, the induction of anaesthesia was possible only after initiation of cardiopulmonary bypass with cannulation of femoral vessels under local anaesthesia. A large mass was removed from the right atrium (7 × 5 × 5 cm), but tumour tissue extending into the superior vena cava was only partially removed. Histology was compatible with type B2 thymoma. Moreover, within the tumour, immature T cells were found, with positive CD4, CD8, CD5, TdT and cytoplasmatic CD3 immunostaining. After an uneventful recovery, the patient was referred for palliative chemotherapy with cisplatinum, adriamycin and cyclophosphamide. Six months later, after four cycles of chemotherapy, regression of the residual tumour was seen and additive radiotherapy was planned.


A 27-year-old parturient (G4P1110) was referred to the obstetric anesthesia service at 34 weeks gestation for assessment before elective Cesarean delivery. Her third pregnancy resulted in a full-term normal vaginal delivery at home. However, the baby died of pneumonia at 4 months of age. She had not received any antenatal visit in her prior three pregnancies. She was diagnosed with TOF 1 year back, when she developed symptoms of dyspnea on exertion, palpitation and easy fatigability. She was advised propanolol 40 mg twice a day. Surgical correction of TOF was suggested which she refused to undergo. She was incidentally diagnosed to be HIV-positive at 12 weeks of current pregnancy and was started on zidovudine 300 mg twice a day.
On preanesthetic examination she was 58 kg in weight with height of 150 cm. Her pulse rate was 120/min with blood pressure of 110/84 mmHg and respiratory rate of 24/min. On auscultation normal S1 with ejection systolic murmur at pulmonary area was heard. Airway examination revealed short neck with small receding chin with a thyromental distance of 7 cm. Her mouth opening was 3 cm with protruding teeth. She was mallampati class III with full range of neck movements. Her complete blood count revealed hemoglobin of 13.5 gm% with total leukocyte count of 6600/mm3 and platelet count of 2 lac/mm3. Serum biochemistry was normal. Renal and liver functions tests were also normal. Her T-helper % of lymphocytes (CD3+CD4+/CD45+) was 9% with T-helper lymphocyte absolute count of 326. Her blood gases were pH-7.33, pO2-48, pCO2-29, cHCO3 – 14, BE- (-9) and SaO2–83%. Chest X-ray showed right ventricular hypertrophy (RVH) with oligemic lung field. Electrocardiogram showed RVH with right-axis deviation. Echocardiography revealed large ventricular septal defect (VSD) with overriding of aorta and right to left shunt. Severe valvular and subvalvular pulmonary stenosis with peak systolic gradient of 58 mmHg was evident.
In the operating room standard 5 leads ECG, NIBP and pulse oximetry were attached. Her blood pressure was 117/79 mmHg, heart rate was 134/min and SpO2 was 82%. Oxygen supplementation (FiO2 0.5) was administered via venturi mask. Invasive blood pressure (IBP) monitoring was established via left radial artery cannulation under local anesthesia. Central venous cannulation of the right internal jugular vein was performed. An 18-G epidural catheter was inserted at the L3-L4 level in the left lateral position. The subarachnoid space was located using a 26-G Quincke needle. A 2.5 mg (0.5 ml of 0.5%) of hyperbaric bupivacaine with 25 μg fentanyl was administered intrathecally. A prophylactic phenylephrine infusion was started at 20 μg/min. The patient was subsequently placed in the supine position with left lateral tilt and slightly head up position. A T10 sensory block to pin-prick was achieved. Epidural boluses of 3 mL and then 2 mL of plain 0.5% bupivacaine were given 10 and 20 min after the intrathecal injection. This was supplemented by fentanyl 100 μg, also given via the epidural catheter. After 35 min there was a bilateral sensory level to pin-prick, at T4. At this point, the patient was turned into the wedged supine position. About 60 min after the intrathecal injection, a further 5 mL of plain 0.25% bupivacaine with fentanyl 50 μg was given epidurally. Cardiovascular parameters remained stable during the development of the block and during surgery. Her pulse rate ranged from 82 to 120/min with mean blood pressure between 85 and 106 mmHg and saturation of 92-95%. Arterial blood gases obtained 1 hr after intrathecal injection revealed were pH-7.39, pO2-64, pCO2-26, cHCO3 – 15, BE- (-7), SO2–93%. She received a total of 1300 mL of Hartmann's solution, given as a slow intravenous infusion throughout the surgery. Her central venous pressure (CVP) was maintained between 12 and 15 cm H2O. Estimated blood loss was 300 mL. A low birth-weight baby boy of 1900 g was delivered. The APGAR scores were 8 and 9 at 1and 5 minutes respectively with cord pH of 7.22. The uterus contracted well so oxytocin was withheld. Immediately postoperatively the patient was transferred to the postanesthesia care unit where she received epidural morphine 3 mg for analgesia. She was nursed supine, with headup tilt until sympathetic tone returned. Her subsequent course in hospital was uneventful.


A 35-year-old female was admitted with complaints of left-sided headache and progressive loss of vision over a period of 18 months. She was conscious with a heart rate of 70 beats/min and blood pressure of 120/70 mmHg. Neurological examination revealed no other sensory or motor deficits. Other systemic evaluation was unremarkable. Computed tomographic of head showed a partially thrombosed aneurysm and digital subtraction cerebral angiography showed partially thrombosed anterior communicating artery aneurysm. Hence, endovascular coiling of the aneurysm under general anesthesia was planned. In the interventional neuroradiology suite, routine monitors such as SpO2, ECG, and non-invasive BP were connected to the patient. Anesthesia was induced with intravenous (IV) propofol 3 mg/kg and fentanyl 2 μg/ kg. Tracheal intubation was facilitated with rocuronium 1 mg/ kg. Maintenance of anesthesia was done with O2/N2O mixture (50:50), isoflurane along with intermittent boluses of fentanyl and rocuronium. The patient was subjected to controlled ventilation with an end tidal CO2 targeted at 35 ± 2 mmHg. Right femoral artery was cannulated using 8 Fr-macrocatheter and heparinization was done using 1 U/kg of heparin. Subsequently, the microcatheter was introduced into cerebral vasculature and the lumen of aneurysm was coiled with Guglielmi detachable coils (GDC, total 3 in number). During placement of the last coil, there occurred sudden bradycardia. The heart rate dropped down from 86 to 40 beats/min with irregular rhythm. There was associated fall in BP to about 60/46 mmHg for few seconds which was followed by an increase up to 220/126 mmHg. Ventricular ectopics appeared on ECG which was normalized with IV lignocaine. At the same time, extravasation of contrast media, suggestive of aneurysmal rupture, was observed []. The leak was sealed with the same coil. The effects of heparin were reversed with protamine so as to prevent further bleeding. However, the neuromuscular blockade was not reversed and the patient was shifted to intensive care unit (ICU) for elective ventilation in view of aneurysmal rupture. The patient was put on mechanical ventilation with SIMV mode (FiO2 0.4, tidal volume 500 ml, and respiratory rate 12/min). After 30 min, the patient developed hypotension 70/45 mmHg along with drop in arterial oxygen saturation (SpO2 85%). Auscultation of chest revealed bilateral basal crepitations. Simultaneously, an increase in airway pressure to 35 mmHg was observed. Endotracheal suction had copious pink, frothy secretions; and hence, pulmonary edema was suspected. Dopamine infusion (10 μg/kg/min) was started for hemodynamic stabilization; however, it was ineffective. Hence, infusion of dobutamine (5 μg/kg/min) and noradrenaline (0.1 μg/kg/min) were started. Central venous catheterization via subclavian vein was done, and a CVP of 15 mmHg was recorded. Arterial blood gas (ABG) analysis was done which showed metabolic acidosis (pH 7.12, PaO2 68 mmHg, PaCO2 22.5 mmHg, bicarbonate 12.7 meq/l, and lactate 6.6 mmol/l). Chest X-ray revealed diffuse bilateral pulmonary infiltrates []. Frusemide 20 mg and morphine 5 mg were given IV, every 8 h. The troponin T-test was negative and ECG documented nonspecific T-inversion in leads I, V1, V2, and V3 along with tachycardia. Two-dimensional echocardiography showed a normal left ventricular function with mild diastolic dysfunction. Thus, a diagnosis of neurogenic pulmonary edema was made. The patient required pressure support ventilation along with positive end-expiratory pressure (PEEP) 10 mmHg and a FiO2 of 0.9 to maintain adequate oxygenation, for 24 h. Inotropic support was tapered off gradually, and the trachea was extubated on the second day after coiling when the chest X-ray [] and ABG picture was improved. On fifth post-procedural day, the patient was discharged with an advice for follow-up.


A 64-year-old female patient was admitted at our hospital with progressive jaundice and right upper quadrant pain for two days. The patient had a history of laparoscopic cholecystectomy due to gall stones two months previously. One month ago, a percutaneous drainage catheter had been inserted due to ascites. The laboratory studies performed at admission showed elevated total bilirubin (13.2 mg/dL; normal range, 0.2-1.0 mg/dL) and direct bilirubin (10.4 mg/dL; normal range, 0-0.3 mg/dL). The hepatic functional enzymes were within the normal limits.
Abdominal CT had been performed on the 11th day before admission at an outside hospital. These abdominal CT images demonstrated mild hepatomegaly with decreased attenuation on the precontrast phase images (). The arterial phase images revealed heterogeneous enhancement of the liver with low-density lesions in the periportal areas (). Ascites was also observed. The portal phase scan images showed homogeneous enhancement of the liver with focal hypoattenuating areas (). These findings may be consistent with amyloidosis of the liver. Diffuse decreased splenic attenuation with contrast enhancement could be seen on the CT scans, but the size and shape of the spleen were within normal limits. The magnetic resonance cholangiopancreatographic images taken outside our hospital showed no evidence of biliary obstruction.
PET/CT using a Discovery STe scanner (GE Healthcare, Milwaukee, WI) was performed to rule out the possibility of a hidden malignancy. The patient fasted for six hours before scanning. Before injection, the patient's glucose levels were within the normal range. One hour after injection of 18F-FDG (5.5 MBq/kg), a non-contrast CT scan and subsequent emission scan (2.5 min/bed, 3D mode) were obtained from the mid femur to the base of the skull. The attenuation-corrected PET images were reconstructed with a 3D iterative ordered subsets expectation maximization algorithm (2 iterations, 20 subsets). In the final step, a 3D isotropic Gauss filter was applied for a final image resolution of 4.29 mm full width at half maximum.
The maximum intensity projection and transverse fusion PET/CT images revealed marked diffusely increased 18F-FDG uptake in the enlarged liver with a maximum standardized uptake value (SUVmax) of 7.2 (). The average hepatic SUV (SUVave) was measured on three contiguous transverse slices at the hepatic hilum using a cut-off SUV of 1.8 for boundary determination (derived from the blood pool activity of the aortic arch), which resulted in a value of 4.1. Other findings on the PET/CT images included high 18F-FDG uptake (SUVmax, right:left = 10.9:14.6) in the bilateral adrenal glands without a definite mass seen on the non-contrast CT images that would suggest benign lesion. However, the spleen and bone marrow did not show increased 18F-FDG uptake, and the size of spleen was within the normal limit.
On the 2nd admission day, a percutaneous ultrasound-guided biopsy targeting the right hepatic lobe was performed. The obtained liver specimen stained positive with Congo red. There were irregularly expanded portal triads infiltrated with an amorphous eosinophilic material, and diffuse amyloid (AL type) deposition had replaced most of the normal liver tissue, which is all consistent with amyloidosis (). On the 7th admission day, the patient's blood creatinine level increased (1.34 mg/dL) and she developed anuria. On the 8th day, bone marrow biopsy was performed due to detecting serum monoclonal gammopathy. This led to the definitive diagnosis of multiple myeloma through the demonstration of a 35% plasma cell population and the presence of kappa light chain-restricted monoclonal plasma cells. The patient then suffered gradual progression of the hepatic coma, and she subsequently underwent liver transplantation, followed by hepatic artery revision and a 2nd liver transplantation due to hepatic artery occlusion. The patient finally died of hepatic failure with sepsis on the 29th admission day.


A 45-year-old female was brought by her relative to emergency department (ED) with history of shortness of breath for 2 days. Patient was apparently asymptomatic 2 months back; when she gradually started noting malaise, lethargy, slow speech, edema of the face and extremities, and progressive weight gain. There was no history of medical illness and drug history in the past. Vital signs on admission were recorded (temperature, 36°C; blood pressure, 90/52 mmHg; heart rate, regular at 74 beats/min). Body mass index (BMI) was 32.6 kg/m2(obese). Patient was hypothermic with pulsus paradoxus (inspiratory fall in systolic blood pressure >10 mmHg). Physical examination found facial edema, coarse hair, dry skin, an engorged jugular vein, mild pallor, and non-pitting edema of the extremities. Oxygen saturation (SpO2) was 84%. The cardiac apical impulse was not visible. The apex beat was not felt. The heart sounds were soft and distant with bilateral basal rales in both lung fields. On per abdomen examination, there was mild hepatomegaly. Her deep reflexes showed delayed relaxation.
Chest radiogram showed cardiomegaly with globular enlargement of the cardiac silhouette with “water bottle” configuration []. Electrocardiogram showed heart rate of 74/min with low voltage pattern with electrical alternans. Bedside echocardiogram demonstrated small heart size with massive pericardial effusion and signs of early diastolic right ventricular (RV) collapse. There was swinging motion of heart within large effusion, prominent respiratory alteration of RV dimension with right atrial (RA) and RV collapse during diastole. Pericardial effusion was all around and all cardiac chambers were of small size with predominant diastolic heart failure []. Right ventricular diastolic collapse was echocardiographic hallmark of cardiac tamponade. Considering echocardiographic findings and clinical scenario, diagnosis of large pericardial effusion with cardiac tamponade was made.
Immediately, we shifted the patient to intensive care unit for pericardial fluid tapping procedure (pericardiocentesis). Written and informed consent was taken for pericardiocentesis. Pericardial tapping was done under all aseptic precautions. We tapped about 450 ml of pericardial fluid through standard xiphisternal route under cardiac monitoring. Evacuative pericardiocentesis was carried out using a spinal needle connected to an ECG line. Fluid was yellowish golden in color which was sent for cytological and biochemical investigation.
Immediately after pericardial fluid tapping, the patient showed significant improvement in the cardiopulmonary status. Tachycardia and tachypnea was significantly reduced. Oxygen saturation was increased to 94% from 84% without oxygen supplementation. After pericardiocentesis, chest radiogram showed significant reduction of cardiomegaly. Post pericardiocentesis, two-dimensional echocardiogram revealed minimal pericardial effusion with no evidence of tamponade.
Considering her history and findings, we did thyroid function test for this patient, which was suggestive of primary hypothyroidism with high thyroid-stimulating hormone (TSH) and very low T3 and T4 levels. The patient was treated with thyroxine 100 μg daily which was later increased gradually to 200 μg daily. The patient's condition improved and she was discharged in a good general condition after 2 weeks. Follow-up echocardiogram after a period of 3 months showed near total resolution of pericardial effusion. Within a few weeks, all her symptoms and signs disappeared and she became normal.
Hemoglobin (Hb), 11.5 g%; erythrocyte sedimentation rate (ESR), 24 at the end of 1 hour; total leukocyte count, 9850 mm3; platelet count, 2.1 lac; blood urea level (BUL), 24 mg%; serum creatinine level, 1.1 mg%; liver function test (LFT), within normal limits. Thyroid function test: serum TSH, 84 mU/ml (normal 0.35–5.5 mU/ml); T3, 26.2 ng/dl (normal 90–190 ng/dl); and T4, 0.56 μg/dl (normal 5–12 μg/dl). Lipid profile: total cholesterol, 317 mg/dl; high-density lipoprotein cholesterol (HDL-C), 52 mg/dl; triglyceride, 214 mg/dl. Serum sodium was 136 mEq/l and serum potassium was 4.2 mEq/l. HIV and HbSAg were negative.
Cardiomegaly with globular enlargement of the cardiac silhouette with “water bottle” configuration was seen.
Electrocardiogram showed heart rate of 74/min with low voltage pattern with electrical alternans (alternating size of QRS complex due to swinging of heart).
There was massive pericardial effusion (about 600–700 ml) and signs of early diastolic RV collapse. There was swinging motion (apical-4-chamber view) of heart within large effusion; prominent respiratory alteration of RV dimension with RA and RV collapse during diastole (parasternal long axis view) was seen. Pericardial effusion was all around and all cardiac chambers were of small size with predominant diastolic heart failure. Right ventricular diastolic collapse was an echocardiographic hallmark of cardiac tamponade. Left ventricular (LV) systolic function was normal with an overall LV ejection fraction of 60% []. Pericardial fluid analysis: appearance, golden yellow in color (“Gold Paint” effusion); lymphocytes, 8–10/mm3; proteins, 4.7 g%; sugar, 80 mg% and cholesterol, 192 mg%; no organisms were grown on culture.


Figure a shows the pedigree of our patients. A 70-year-old woman (II-4) was admitted to our hospital due to a 6-year history of progressive gait disturbance. At the age of 45 years, she developed bilateral hearing deficits, and at 64 years, she underwent surgery to correct cataracts and glaucoma. Neurological examination of the patient revealed mild external ophthalmoparesis, nystagmus, sensory neural hearing loss, dysarthria, and severe cerebellar ataxia. There was mild proximal muscle weakness of the limbs. Tendon reflexes were reduced generally. Electroencephalogram (EEG) assessment showed diffuse paroxysmal slow activity (3–6 Hz), which was induced by photo-stimulation. No epileptic discharge was found. Needle electromyography showed low amplitude and short motor-unit potential in the proximal extremities. Brain magnetic resonance imaging (MRI) showed atrophy of frontal lobe and cerebellar cortex (Fig. ). There was no lactate peak on brain magnetic resonance spectroscopy in a voxel placed over a lateral ventricle. Laboratory test results revealed normal lactate levels and slightly increased pyruvic acid levels. Cerebrospinal fluid analysis was normal. The psychometric test showed impaired intelligence, memory, and frontal function.
The proband’s mother (I-2) suffered from hearing loss and glaucoma. Since the age of 80, she had slowly become progressively bedridden. She died at the age of 84.
The proband’s first daughter (III-2) is 41 years old and has had palindromic rheumatism since the age of 14 years. She has not experienced convulsion, myoclonic event or stroke-like episodes, but has had recurrent migraine attacks. Brain MRI showed multiple, small hyper-intense areas in subcortical white matter of the cerebrum on a T2-weighted sequence. Her neurological examination showed no abnormalities.
The proband’s second daughter (III-3) is 39 years old and has experienced photo-induced myoclonus since the age 36 years. At that time, atrophy of the cerebellum was noted on MRI. At the age of 38 years, she started taking anticonvulsant medication due to negative myoclonus and photo-induced myoclonus on EEG examination. Her neurological examination showed an absence of tendon reflexes in all limbs and mild truncal ataxia. Muscle strength and mental status were normal.
The proband’s first granddaughter (IV-1) is 17 years old. She was delivered with the aid of a vacuum extractor at 37 gestational weeks. At the age of 15 years, loss of consciousness lasting a few seconds and myoclonic jerks in her upper extremities and trunk appeared. Two years later, she experienced her first generalized seizure with an abnormal EEG record and started taking antiepileptic drugs. School records indicated below average academic performance. Her neurological examination showed an absence of tendon reflexes in all four limbs and slight cognitive decline. Coordination and muscle strength were normal.
Otherwise, there are no other family members known to have any endocrine or neurological disease.


A 51-year-old male patient with a 30 pack-year current smoking presented with a recent onset of exertion chest pain. He had a free medical history. The treadmill test provoked severe chest pain, without ST changes on the electrocardiography (ECG), at stage 2 of the modified Bruce protocol. The patient underwent elective coronary angiography via the left femoral approach, which revealed a discrete critical narrowing of the middle left anterior descending artery (LAD), just distal to the first diagonal branch (D1) (). A 7 Fr JL4 guide catheter (Cordis, Miami, FL, USA) was used to engage the left main coronary artery, and a 0.014" ATW™ guidewire (Cordis, Miami, FL, USA) was passed into the distal LAD, without difficulty. The proposed treatment was a cross-over stenting followed by provisional T stenting at that lesion site. Angioplasty was performed twice with the Ryujin™ Plus (TERUMO, Tokyo, Japan) 3.5×10 mm balloon at 6-atm (up to 3.5 mm) inflation. A Zotarolimus-Eluting stent (Endeavor Resolute, Medtronic, Minneapolis, MN, USA) 4.0×15 mm stent was deployed, with 12-atm (up to 4.08 mm) inflation. The repeat angiogram showed adequate deployment of the stent, but a jailed D1 (). An attempt was made to pass into D1 using a 0.014" Choice™PT guidewire (Boston Scientific, Miami, FL, USA) through the instent. After initially inflating a Sprinter (Medtronic, Minneapolis, MN, USA) 3.0×15 mm semi-compliant balloon, up to 3.18 mm at the D1 ostium, 'kissing' balloon angioplasty, using a Sprinter 3.5×15 mm for the LAD and a Sprinter 3.0×15 mm for D1, was finally performed (). The final angiographic result appeared good, without any complications (). During observation in the intensive care unit, the patient grumbled about some chest tightness, but was hemodynamically stable. Three hours after PCI, he complained of more severe chest pain, dyspnea, tachypnea and heavy sweating. Moreover, his systolic blood pressure dropped to below 60 mmHg. An emergent echocardiogram was taken at the patient's bedside, under the suspicion of a cardiac tamponade, due to delayed extravasation after PCI. It showed a pericardial effusion of less than 10 mm, as well as the collapse of the right atrium. Emergency pericardiocentesis was performed and over 100 mL of bloody pericardial fluid was initially drained. The patient's vital signs returned to normal. Pericardial fluids were drained continuously and blood was transfused through the peripheral vein, with the expectation that it will stop the extravasation spontaneously. Considering the drainage rate of the pericardial fluid at over 200 mL/hr, a large perforation of the coronary artery at the stenting site was suspected. However, an emergent angiogram showed massive extravasation (Ellis Grade 3 coronary perforation) at the far distal area of D1 (). An attempt was made to recross D1 and a Sprinter 3.0×15 mm semi-compliant coronary balloon with low pressure was inflated at the middle portion of D1. However, the perforation could not be sealed and continuous leakage occurred after the balloon's deflation, even after an inflation period of over 60 minutes. We attempted to occlude the D1 branch via a microcoil embolization to repair the perforation site completely. A MicroFerret® superselective catheter (Cook, Sandet, Denmark) was advanced superselectively into D1 at the level of the perforation. One 0.018-inch and 14.2-mm-long tapered microcoil (10×4 mm in diameter; Tornado; Cook, Bloomington, IN, USA) was quickly released, using the microcatheter's supporting guidewire (Transend® 300, Boston Scientific, Miami, FL, USA) as a pusher. A post-coil LAD arteriogram demonstrated the total occlusion of D1 and no further extravasation (). On the following day, the post procedure echocardiogram revealed an ejection fraction of 60%, with mild hypokinesia on the apical lateral and apical anterior walls, and no pericardial effusion. The peak infarct size, as measured using serum biomarkers, was a troponin of 2.65 ng/mL and a creatinine phosphokinase-MB of 100.8 ng/mL. The patient was uneventful and discharged 5 days later in a stable condition. Three months after the PCI, he took the treadmill test and the echocardiogram. The treadmill test provoked chest pain at stage 5, with a 1 mm ST depression on the ECG, and the echocardiogram showed no interval change. For 12 months after PCI, the patient had several episodes of chest pain and dyspnea on exertion. He then underwent repeated coronary angiography. Angiographic findings of D1 showed a normal flow without delay (). The treadmill test showed a negative finding.


A 10-year-old female patient presented with gradual weakness in both lower limbs and numbness from lower thorax to foot for 3 months. There was also history of bowel and bladder incontinence. There was no history of fever, rash, or trauma. The child was conscious with preserved higher functions. Neurological examination revealed spastic paraplegia (motor power 3/5), brisk B/L deep tendon reflexes with extensor plantar and loss of sensations to pinprick bilaterally below T5 dermatomes. Peripheral blood count was normal. Mantoux test was negative and radiographs of the spine and chest revealed no abnormality.
Contrast-enhanced MRI of the spine revealed an isolated, well-defined, intramedullary cystic lesion measuring 1.8 1.0 cm at the level of D2 vertebral body. On T1-weighted imaging, the lesion was hypointense with a central isointense mural nodule []. The lesion was hyperintense on T2-weighted image with an isointense mural nodule [Figure and ]. There was edema of the surrounding cord. Ring enhancement was seen on post-contrast T1-weighted image []. Based on these findings, a diagnosis of cysticercus cyst was suggested. Screening of MRI brain was normal. CSF examination done revealed elevated protein and normal glucose levels with increased lymphocytes, and few eosinophils and positive enzyme-linked immunosorbent assay (ELISA) for anti-T. solium IgG immunoglobulin.
Keeping the provisional diagnosis of cysticercosis in mind, empirical treatment with antihelminthic along with steroids was started. Marked clinical improvement was seen within a few days in the form of increase in limb power and return of tendon reflexes. Complete improvement was seen during the follow-up period of 3 months.


An 18-year-old man presented with poor vision in both eyes that had been present since birth. On presentation, his visual acuity was 60 / 200 in the right eye and light perception only in the left eye. The refractive error in the right eye was -11.0 diopters, and we were unable to measure the refractive error of the left eye because of sclerocornea. Intraocular pressure was 27 mmHg in the right eye and 21 mmHg in the left eye. Central corneal opacity and inferior peripheral sclerocornea with iridocorneal adhesion were observed in the anterior segment of the left eye (). The right eye had an iris coloboma that manifested as a small notch in the inferior pupillary margin and cataract (). Fundus examination of the right eye showed a large inferior chorioretinal coloboma involving the optic disc and macula (). Staphyloma was observed in the right eye by B-scan ultrasonography (). We did not detect any systemic malformations and there was no relevant family history.


A 23-year-old male with history of Type-IV Ehlers-Danlos Syndrome and hemorrhagic stroke presented with severe abdominal pain following an episode of vomiting that was precipitated by alcohol consumption. Like the case reported by Soonawalla et al. [] the patient was tachycardic on initial presentation suggesting hypovolemia, and an abdominal CT-scan revealed a large retroperitoneal hemorrhage (Fig. ). Angiographic evaluation using interventional radiology was considered for evaluation of this patient’s hemorrhage, but was decided against due to the high risk of dissection from arterial catheters in Type IV Ehlers-Danlos Syndrome. Furthermore, based upon the catastrophic consequences of exploratory laparotomy in the case reported by Soonawalla et al. [] we elected for a trial of conservative management. The patient’s initial Hgb was 15.9 that fell to 11.8 on his first hospital day, and then stabilized. For the first 48 hours, the patient was placed on strict bed-rest to prevent any further exacerbation of the hemorrhage, and administered anti-emetics liberally to prevent any further episodes of emesis. Throughout the rest of his hospital stay, the patient’s pain improved, Hgb remained stable, and he was discharged on his fourth hospital day. Currently, the patient continues to do well nearly one year later and has returned to his usual state of health. To our knowledge, this is the first patient with Type IV Ehlers-Danlos Syndrome to survive the development of a massive retroperitoneal hemorrhage.