An 87-year-old woman visited the emergency department for worsening severe dyspnea and chest discomfort. On admission, the blood pressure was 150/90 mmHg, the respiratory rate was 36 per minute, the pulse rate was 112 per minute, and the peripheral oxygen saturation was 80%. Fine crackles were auscultated in both lower lung fields and an apical grade 2/6 holosystolic murmur was present. A chest radiograph revealed cardiomegaly and pulmonary congestion (). The electrocardiogram showed voltage criteria consistent with LV hypertrophy, and T wave inversion in leads V5 and V6 was compatible with LV strain ().
The CK-MB and troponin-I levels were normal (1.87 and 0.022 ng/mL, respectively). The B-type natriuretic peptide was markedly elevated (1,850 pg/mL). Other laboratory parameters were unremarkable.
The patient had a history of an acute MI during a previous admission; the electrocardiogram showed ST segment elevation in leads III and aVF and Q waves in leads II, III, and aVF. Coronary angiography revealed severe coronary artery stenoses (total occlusion of the proximal right coronary artery, total occlusion of the proximal left circumflex artery, and a 40% stenotic lesion in the distal left main artery). Thus, primary percutaneous coronary revascularization of the right coronary artery was performed.
Two-dimensional echocardiography at the previous admission showed decreased LV systolic function (ejection fraction, 40%) and a mild pericardial effusion. The regional wall motion abnormalities with akinetic basal to the mid-inferior and posterolateral walls of the LV were observed. In a color Doppler study, mild mitral regurgitation was noted in systole. The continuity of the myocardium of the mid-posterior wall was disrupted and a small sac (22 × 11 mm) with a narrow neck was seen which was suspected to be a rupture of the free wall with a thrombotic plug (). A LV pseudoaneurysm was diagnosed and contrast echocardiography was performed to evaluate further blood leakage through the ruptured myocardium and sac. Contrast echocardiography revealed that the pseudoaneurysm on the LV posterior wall was clearly defined and did not communicate with the pericardial space (). Cardiac magnetic resonance imaging (MRI) also showed a small bulging sac-like lesion with a neck portion in the mid-posterior wall of the LV without definite myocardial tissue ().
The patient and her family declined to undergo surgery for the LV pseudoaneurysm. The patient was discharged after a few days of medical therapy and did not return for follow-up.
During the admission, two-dimensional echocardiography revealed an increase in the size of the LV and decreased LV systolic function (ejection fraction, 30%). A large cavity in the posterior area of the mid-posterior wall of the LV (> 80 × 55 mm) was noted which was diagnosed as a small LV pseudoaneurysm 1 year earlier (). Blood flow across the hole from the LV to the cavity in systole () and from the cavity to the LV in diastole was observed by color Doppler study (). Mild-to-moderate mitral regurgitation in systole and diastole was noted in a color Doppler study () that was more severe compared with the previous admission. A small LV pseudoaneurysm after an acute MI progressed to a huge pseudoaneurysm with significant mitral regurgitation in 1 year without any treatment. Surgical management was strongly recommended, but she declined again and was discharged when dyspnea had improved with medication for congestive heart failure.


A 6 month old boy, the first child of healthy parents, presented with a short history of progressive muscle weakness followed by life threatening apnoeic spells necessitating artificial ventilation. Neurological examination revealed generalized muscle weakness with a severe head lag. A complete diaphragmatic paralysis was detected on ultrasound. Spinal muscular atrophy (SMA) and SMA with respiratory distress (SMARD) were excluded by genetic testing. Muscle histology at the age of 7 months demonstrated unclassified myopathic abnormalities. Biochemical analysis of the mitochondrial respiratory chain in fresh muscle tissue gave inconclusive results. Metabolic studies revealed abnormal concentrations of short- and medium-chain plasma acylcarnitines and a urine organic acid profile was suggestive of MADD (Tables and ). Because of the possibility of riboflavin-responsiveness (Olsen et al. ), the patient was treated with high dose oral riboflavin (vitamin B2, 10 mg/kg body weight/day). The MADD associated metabolic abnormalities disappeared within days. Cessation of riboflavin supplementation resulted in a recurrence of the abnormal metabolic profile in spite of a normal dietary riboflavin intake, and riboflavin medication was restarted. Classical MADD was ruled out by sequence analysis of the ETFDH, ETFA and ETFB genes. The biochemical profile, absence of urine thiosulfate and sulfo(thio)cysteine (Duran et al. ) argued against an ethylmalonic syndrome (ETHE) (Tiranti et al. ). Therefore we hypothesized a decreased availability of flavin adenine dinucleotide (FAD), the cofactor of MADD, due to defective uptake, synthesis or transport, to be the underlying cause of the disease.
A tracheotomy was placed and the boy’s muscle tone slowly improved over the next month. He was able to walk independently at the age of 22 months. As the diaphragmatic paralysis persisted, he needed nightly ventilation until the age of 41 months. Recently, the clinical diagnosis of Fazio Londe Syndrome was made. He is currently 46 months old, his cognitive development is fully normal and he demonstrates no further cranial nerve palsy.


A now 7 year old girl, second child of healthy parents, demonstrated feeding problems and a slow motor development since birth. At the age of 5 months she had generalized weakness, more pronounced in distal than proximal muscle groups, and developed respiratory insufficiency necessitating artificial ventilation due to diaphragmatic paralysis. Furthermore, she was found to have a severe sensorineural hearing loss. SMA and SMARD were excluded by genetic testing. Muscle histology at the age of one year showed slight predominance of type 1 fibers without type grouping. Biochemical analysis of the mitochondrial respiratory chain in muscle tissue was inconclusive.
At the age of 6 months, metabolic studies revealed an acylcarnitine profile suggestive of MADD (Table ). A fat restricted diet and supplementation with carnitine (50 mg/kg / day), riboflavin (10 mg/kg /day) and glycine and 3-hydroxybutyrate according to the publication by Van Hove et al. () were started. Her muscle strength improved and from the age of 2 years artificial ventilation was only necessary during sleep. Cognitive development was normal.
Classical MADD was ruled out by enzymatic testing in fibroblasts and mutation analysis of the ETFDH, ETFA and ETFB genes. Following the normalization of the acylcarnitine profiles, the fat restriction was gradually discontinued and the glycine, 3-hydroxybutyrate and carnitine supplementations were stopped without problems. However, withdrawal of riboflavin at the age of 4 years resulted in a rapid clinical deterioration with vomiting, progressive fatigue, and elevations of lactate, liver enzymes and CK. The acylcarnitine profile became abnormal again. Reintroduction of riboflavin (50 mg b.i.d.) resulted in clinical improvement and normalization of the biochemical abnormalities. Sequence analysis of the mitochondrial FAD transporter (Spaan et al. ) was performed on suspicion of a possible mitochondrial riboflavin transporter defect, but showed no abnormalities.
After reintroduction of riboflavin supplementation the patient had a stable clinical course. She walked with a foot drop and attended a school for hearing impaired children. The parents gradually decreased the dose of riboflavin to 10 mg b.i.d. because they suspected gastrointestinal side effects of the high doses of riboflavin. However, after 5 years she developed palsies of the 7th and 12th cranial nerves, and became wheelchair bound. Furthermore, following a lower respiratory tract infection at the age of 6.5 years she became once again completely ventilator dependent, and her muscle strength slowly deteriorated. Only recently, the clinical diagnosis of Brown-Violetto-Van Laere syndrome was confirmed. The riboflavin dose was increased to 50 mg t.i.d., thus far without improvement of her symptoms.


A 3-year-old boy, weighing 18 kg, ASA physical status I, required surgery for a fracture of the lower third of the left humerus. After a thorough discussion of the risks and benefits with his parents, who then provided written informed consent, a USG supraclavicular brachial plexus block was undertaken. After inducing general anesthesia with 1 mg/kg of ketamine, a laryngeal mask airway (LMA) was inserted and ventilation was spontaneous.
A portable system with a linear array 5-10 MHz transducer (SonoSite® 180plus, Bothell, USA) was used for the USG supraclavicular brachial plexus block. The probe was placed in the coronal-oblique plane in the supraclavicular fossa and the puncture was in-plane (IP) from the lateral to medial (). The brachial plexus was identified as hypoechoic nodules surrounded by a hyperreflective fascial sheath, superior and lateral to the subclavian artery (). After infiltrating the adjacent skin and subcutaneous tissue, a 25-gauge 50-mm insulated block needle (UniPlex NanoLine, Pajunk®, Germany) was advanced along the long axis of the probe in plane with US beam guidance. When the brachial plexus bundle () was reached, the nerve stimulator (Stimuplex® HNS 12, B/Braun, Germany) was turned on, starting from 1.0 mA and decreasing by 0.5 mA to elicit a muscle twitch. After injecting 1ml of 0.5% ropivacaine, the muscle twitch disappeared and local anesthetic spread at the time of injection was observed in real time. Nine ml of 0.5% ropivacaine was injected after a negative aspiration test.
The patient underwent a closed reduction and percutaneous pinning of the distal humerus. Maintenance of anesthesia was carried out with sevoflurane 1.5 vol% in 50% oxygen/air until the end of the surgery. His vital signs did not change and no opioid supplementation was administered during surgery. The child did not require rescue analgesia in the postoperative period and no adverse effects were noted.


An 11-year-old boy weighing 46 kg, ASA physical status I, required surgery for a fracture of the upper end of the left humerus. After a thorough discussion of the risks and benefits with the patient and parents, who then provided written, informed consent, a USG supraclavicular brachial plexus block was performed.
Immediately before placing the block, midazolam 2 mg and sufentanil 2.5 µg were administered intravenously for patient comfort. A portable system with a linear array 6-13 MHz transducer (SonoSite® M-turbo, Bothell, USA) was used for the USG supraclavicular brachial plexus block. The probe was placed in the coronal-oblique plane in the supraclavicular fossa and the puncture was IP from the lateral to medial. Neurostimulation was not used to confirm the correct position. The brachial plexus was identified as hypoechoic nodules surrounded by a hyper-reflective fascial sheath, superior and lateral to the subclavian artery (). After infiltrating the adjacent skin and subcutaneous tissue, a 22-gauge epidural needle (Tuohy Epidural Needle®, B/Braun, Germany) was advanced along the long axis of the probe in plane with the US beam. Upon reaching the brachial plexus bundle (), 1 ml of 0.5% ropivacaine was injected and the local anesthetic spread at the time of injection was observed in real time. Twenty four ml of 0.5% ropivacaine was injected after a negative aspiration test. Successful motor and sensory block of the upper limb occurred within 30 min of the injection.
The patient underwent a closed reduction and percutaneous pinning of the proximal humerus. Surgery proceeded uneventfully with no adverse effects noted.


A 12-year-old boy weighing 45 kg, ASA physical status I, was scheduled to undergo an open reduction and internal fixation of the radius and ulna fracture of the right forearm. After a thorough discussion of the risks and benefits with the patient and parents, who then provided written, informed consent, a USG supraclavicular brachial plexus block with perineural infusion was undertaken.
Immediately before placing the block, intravenous midazolam 2 mg and sufentanil 2.5 µg were administered. After sterile skin preparation and local anesthetic skin infiltration with 2% lidocaine, a 18 gauge Touhy-tip needle (Perican®, B/Braun, Germany) was guided IP using a linear array 5-10 MHz transducer (SonoSite® 180plus, Bothell, USA) to deposit 25 ml of 0.5% ropivacaine around the brachial plexus, which was cephalad and lateral to the subclavian artery (). Neurostimulation was not used to confirm the correct position. After injecting the local anesthetics, a flexible 20 gauge perineural catheter (Contiplex A®, B/Braun, Germany) was then inserted 2 cm past the tip of the needle lateral to the subclavian artery. The injection port was attached to the end of the catheter, and the catheter tip position was confirmed by injecting 1 ml of saline via the catheter under US visualization. The successful motor and sensory block of the upper limb occurred within 30 min of the injection.
The patient underwent an open reduction and internal fixation with a dynamic compression plate of the proximal ulna. The operation and recovery room stay were uneventful and without complications. The patient was pain free and comfortable. A 0.1% ropivacaine perineural infusion was initiated with a basal rate of 3 ml/h, a patient-controlled bolus dose of 2 ml, and a lockout interval of 20 min via a small, light, portable electronic infusion pump (Accumate® 1000, Wooyoung Medical, Korea).
The patient reported excellent postoperative analgesia without analgesic requirements during the infusion and no adverse events were encountered. The catheter was removed on the second postoperative day.


A 12-year-old girl weighing 47 kg, ASA physical status I, was scheduled to undergo an open reduction and internal fixation with a plate for a Monteggia fracture of the right forearm. After a thorough discussion of the risks and benefits with the patient and parents, who then provided written, informed consent, a USG supraclavicular brachial plexus block with perineural infusion was performed.
Immediately before block placement, midazolam 2 mg and sufentanil 2.5 µg were administered intravenously. After sterile skin preparation and local anesthetic skin infiltration with 2% lidocaine, a 18 gauge Touhy-tip needle (Perican®, B/Braun, Germany) was guided IP using a linear array 5-10 MHz transducer (SonoSite® 180plus, Bothell, USA) to deposit 25 ml of 0.5% ropivacaine around the brachial plexus, which was cephalad and lateral to the subclavian artery. Neurostimulation was not used to confirm the correct position. After injecting local anesthetics, a flexible 20 gauge perineural catheter (Contiplex A®, B/Braun, Germany) was then inserted 2 cm past the tip of the needle lateral to the subclavian artery. The injection port was attached to the end of the catheter, and the catheter tip position was confirmed by injecting 1 ml of saline through the catheter under US visualization. Successful motor and sensory block of the upper limb occurred within 30 min of the injection.
The patient underwent an open reduction and internal fixation of the proximal ulna with a dynamic compression plate. After surgery, the patient was transported to the recovery room. The patient was pain free and comfortable. A 0.1% ropivacaine perineural infusion was initiated with a basal rate of 3 ml/h, a patient-controlled bolus dose of 2 ml, and lockout interval of 20 min via a small, light, portable electronic infusion pump (Accumate® 1000, Wooyoung Medical, Korea).
The patient only experienced pain 12 h after the initial injection of local anesthesia. The pain was controlled using IM diclofenac 30 mg. On the second postoperative day, she complained of an insensate extremity, which resolved spontaneously after discontinuing the local anesthetic for 2 hours. After discontinuing the infusion, she complained of pain, and a decision was made to continue the infusion. She did not complain of any pain or insensate limbs. The catheter was removed on the third postoperative day.


On October 1st, 2009, a 32-yr-old man visited the outpatient clinic with a 1-month history of progressively worsening blood-tinged sputum and chest pain. He had no relevant previous medical history. The patient had worked in a small educational institute for three years, and was currently working as a company employee. However, he denied exposure to asbestos. On physical examination, hepatosplenomegaly and peripheral lymphadenopathy were absent, but a chest examination revealed retraction and tenderness of the left chest wall. Laboratory testing revealed hemoglobin 12.3 mg/dL, white cell count 11,100/µL, and platelet count 519 × 103/µL. Blood chemistry findings revealed an alanine aminotransferase 42 IU/L, aspartate aminotransferase 94 IU/L, and lactate dehydrogenase 451 IU/L. Computed tomography (CT) of the chest showed irregular pleural thickening and pleural effusion in the left lung (). An incisional biopsy of pleura was performed, and histologic findings suggested a malignant undifferentiated tumor. Microscopically, the tumor showed an infiltrative growth pattern with a desmoplastic stroma. The tumor cells had oval to spindled nuclei, and indented nuclei were frequently observed. The chromatin was often vesicular with inconspicuous nucleoli. The cytoplasm of the tumor cells was abundant and slightly eosinophilic with an indistinct border (). In addition, immunohistochemistry was performed on the Benchmark automated immunostaining system (Ventana Medical System, Tuscon, AZ, USA). The monoclonal antibodies used were the following: S100 (1:800; Dako, Glostrup, Demark), vimentin (1:200; Dako), CD45 (1:100; Leica, Newcastle-upon-Tyne, UK), CK (1:600; Leica), myeloperoxidase (1:200; Leica), HMB45 (1:150; Leica), CD1a (1:20; Leica), CD20 (1:200; Leica), CD21 (1:60; Leica), CD23 (1:100; Leica), CD31 (1:300; Leica), CD34 (1:20; Leica), CD56 (1:150; Leica), CK5 (1:300; Leica), WT-1 (1:40; Leica), calretrenin (1:100; Leica), CD68 (1:600; DiNonA, Iksan, Korea), CD3 (1:300; Neo, Fremont, USA), CD35 (1:50; Cell marque, Rocklin, CA, USA), and CK6 (1:50; Thermo, Fremont, CA, USA). Immunohistochemistry showed that the tumor cells were positive for S100, CD45 (), vimentin, and CD68, but negative for cytokeratin (epithelial cell marker), myeloperoxidase (myeloid cell marker), HMB45 (melanoma marker), CD1a (LCS marker), CD3 (T-cell marker), CD20 (B-cell marker), CD21, CD23, CD35 (FDCS marker), CD31 (vascular endothelial cell marker), CD34 (myeloid stem cell marker), CD56 (neuroendocrine cell marker), CK5, CK6, WT-1, and calretrenin (mesothelioma marker). The proliferation index (Ki 67) of the neoplastic cells was approximately 25%. A diagnosis of IDCS was made based on histological and immunohistochemical findings. The patient underwent a positron emission tomography (PET)-CT scan, which revealed 18-fluoro-deoxyglucose (FDG) uptake in the thickened pleura and whole axial skeleton (standardized uptake value (SUV); 10.5 and 9.8) compatible with malignant tissue (). Subsequently, the patient was treated with two courses of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) and one course of IMEP (ifosfamide, methotrexate, etoposide, prednisolone). However, the pleura-based masses were aggravated, and he died of progressive disease 3 months after diagnosis.


This 72-year-old man presented with left shoulder and AC joint pain and swelling, which progressively got worse over the past several months. He also had difficulty in raising his arm above his head. Left shoulder pain over the past several months was associated with a palpable mass along the medial aspect of the clavicle. He was a patient with known primary hemochromatosis and was homozygous for the C282Y mutation. This genotype is consistent with hereditary hemochromatosis.
He had a past history of total hip replacement 13 years ago. Histologic sections of the articular surface of the femoral head revealed varying degrees of articular cartilage damage ranging from fibrillation to focal ulceration and changes in the subchondral bone, containing focal thinning and hemorrhagic changes in the base of the articular head. Mild intertrabecular fibrosis was also noted. Several areas showed a split in the articular cartilage at the border of the calcified and uncalcified cartilage. However, no refractile material (iron) was seen in the cartilage or in the subchondral bone.
On physical examination, he had swelling and pain in the medial aspect of the left clavicle and left AC and SC joint with associated neck stiffness.
Radiographs showed a focal erosion and linear chondrocalcinosis at the left humeral head and rotator cuff calcific tendinopathy Additional findings included degenerative changes at the AC joint and an old Hill–Sachs deformity.
MRI showed a 1-cm erosion at the humeral head, which enhanced with contrast. Additionally, there were subscapularis tendinosis, enlargement of anterior glenoid labrum, iron deposit causing blooming artifact at the tendon, joint and labrum, and formation of glenoid labral ovoid mass (GLOM). He also had erosion and inflammation with effusion at the AC joint. Synovial proliferation, inflammation and soft tissue prominence were also seen at the SC joint. Post-gadolinium images showed marked enhancement of the AC and SC joints, as well as the subacromion/subdeltoid bursa due to bursitis.


A boy aged 4 years and 7 months was admitted to the emergency room with a chief complaint of unsteady walking that had started 10 h earlier. He felt drowsy and dizzy and was hypoactive. Following the neurological symptoms, he had a large volume of watery diarrhea. Before that, he was in good health, with no history of neurological or metabolic disease, fever or abdominal pain.
On admission, the child was dehydrated, but had normal temperature and blood pressure. His weight was 17 kg (25th percentile), and his height was 108 cm (50th percentile). The physical examination showed moderate muscle hypotrophy, scarce adiposity, abdominal distention and no visceromegaly. The neurological examination showed unsteady walking, dysarthria, hand dysmetria and isochoric light reagent pupils. He had irritability and self-aggressive behavior. Glasgow coma scale was 15. Drug screening tests were negative.
The child was born at term; birth weight was normal, and he was breastfed until 5 months of age. From the age of 2, he had sporadic diarrhea controlled with a low-fiber diet and lactose-free milk, and a diagnosis of irritable bowel syndrome was made. Enteropathogenic agents and blood in the stools were absent on all occasions. Serology tests for antigliadin (AGA) and antiendomysium antibodies (EMA) IgA and IgG were negative for CD at ages 2 and 4.
Laboratory test results showed hyponatremia (133 mEq/L), hypokalemia (3.1 mEq/L), hyperchloremic metabolic acidosis (pH 7.2; bicarbonate: 7 mmol/L; BE: –19.9). Serum chloride was 122 mEq/L, and lactate (<2 mmol/L) and glucose (85 mg/dL) levels were normal. Blood tests were normal. Liver and kidney functions were normal (TGO: 46 U/mL; TGP: 35 U/mL; urea: 10 mg/dL; creatinine: 0.3 mg/dL). Levels of serum iron, ferritin, cholesterol and albumin were extremely low. Stool analyses revealed no parasites, rare leukocytes and erythrocytes; cultures were negative and no Clostridium difficile toxins were detected. Radiographs showed global abdominal distension; cranial magnetic resonance imaging (MRI), computed tomography (CT) scans and cerebrospinal fluid tests were normal. Both sleep and waking electroencephalograms were normal.
Similar episodes were observed four consecutive times at 1-week intervals. Before each admission, the child ingested large amounts of bread, cakes and pizza. In the first three hospital admissions, his neurological symptoms faded within 2 days, but the hyperchloremic metabolic acidosis persisted without any evident cause. He had received intravenous hydration and was placed on a low-fiber and hypoallergenic diet without gluten restriction. The need for bicarbonate and potassium chlorate was high, so it was suggested that the loss of potassium and bicarbonate was probably associated with fecal loss because his kidney function was normal. During his fourth admission, a complete investigation for malabsorption was carried out.
Fecal analysis revealed an increased loss of fats. Serum IgA EMA and IgA anti-tissue transglutaminase antibody (anti-tTG) was reagent, over 100 U/mL. CD was suspected. The mucosa magnified by endoscope showed microhemorrhage, reduction in duodenal folds and multiple erosions (). Histological examination showed partial and total villous atrophy, intraepithelial lymphocytes and increased length crypts (Marsh 3C) ().
After the introduction of a gluten-free diet, the child improved progressively, and a supplement of folic acid, iron and calcium was introduced.


A 51-year-old female patient in good health presented with nodular swellings over both her nasolabial folds. She reported a history of injection of the cosmetic filler ‘Dermalive’ (Dermatech, Paris, France) at the site of the swelling, 4 years ago at a clinic in Australia. The swellings had appeared 2 years following the filler injection []. She then visited another dermatologist in South-East Asia who suggested that a reinjection of more Dermalive would improve the swelling. The patient then received a reinjection with the same filler at the same site 2 years after the original filler implantation. The patient was not treated with any other filler material. The slightly erythematous palpable indurations had appeared insidiously, and had gradually increased in size and thickness. The swellings worsened in appearance following the second injection. There was no relevant medical history and the patient did not have any autoimmune or allergic diseases. On palpation, there were firm erythematous longitudinal swellings measuring 5 cm × 2 cm along the lines of filler implantation at the nasolabial folds []. The nodules did not become prominent on projection from inside the oral cavity, and did not show transilluminance on throwing light from within the oral cavity using a pen torch. The patient was emotionally devastated about these reactions, and was constantly applying camouflage make-up []. We treated the patient with 3 injections of Triamcinolone (40 mg/ml), followed by 3 more injections of 10 mg/ml Triamcinolone at monthly intervals, with good reduction of the swelling on the left nasolabial fold, but only minimal reduction in the size of the swelling on the right side [].
As the patient was willing to accept cosmetic surgery, a surgical excision was carried out by the plastic surgeons and the tissue was sent for a histopathological evaluation. Operative findings indicated well encapsulated, grayish white, multilobulated dermal nodules. The wound was thoroughly cleaned and sutured in layers after achieving complete haemostasis. The subcutaneous tissues were approximated with 5-0 Monocryl interrupted sutures (Ethicon, Inc). The skin closure was done with 8-0 Ethilon (Ethicon, Inc) []. Operation site healed well and suture removal was done on the 10th day.
The excision biopsy showed a dermal and subcutaneous adipose tissue infiltrate of foreign body giant cells and numerous irregular crystalline structures [Figures and ]. The surgical site showed good healing and our patient was satisfied with the result [].
The further treatment plan is to inject autologous fat as a filler to enhance the appearance following surgical excision. Autologous fat transplant is preferred both because of the hesitance on the part of the patient to accept another filler and also to prevent formation of new granulomas and foreign body reactions, which are known to occur even with temporary fillers like hyaluronic acid.


A 45-year-old female came to the hospital with chief complaints of recurrent swelling in the epigastric region since one year. The swelling was initially small in size, nodular gradually increasing in size and had currently achieved the dimensions of about 5 × 5 cm [].
The swelling, which was earlier painless, had now become painful after it ruptured with a bloody discharge. The skin around the swelling was retracted and shriveled in appearance, which was consistent with past history of wide excision with split thickness skin grafting seven years back. Clinical parameters were within normal limits. Chest roentgenogram was normal. With the provisional diagnosis of recurrent DFSP the patient was taken up for surgery. The patient underwent a wide local excision of the mass. The tumour involved rectus sheath, rectus sheath and part of rectus muscle with margin of 2.5 cm was removed. Same margin of 2.5 cm was taken in all directions []. The defect was covered with split thickness skin grafting. No intra-abdominal extension of the tumour was found.
Microscopic examination revealed elongated spindle-shaped cells arranged in sheets, tiny nodules, storiform pattern with pleomorphic, hyperchromatic or vesicular nuclei with 2-4 prominent nucleoli and moderate amount of eosinophilic cytoplasm mixed with histiocytes . The pathological diagnosis was DFSP, which was confirmed by immuno-histochemical examination. Immunohistochemistry showed that tumour cells were positive for CD34 and XIIIa.
Recovery was uneventful and patient was discharged on the fifth post-operative day. Patient is asymptomatic one year after surgery and is coming for regular follow-up.


An 8-year-old boy was admitted with pain in the left upper abdomen for 6 months. The pain was dull aching and he had low-grade fever off and on. There was no history of trauma, significant medical illness or surgical intervention in the past. He was pale and the abdominal examination revealed mild tenderness in the left hypochondriac region and splenomegaly. His hemoglobin was 10.5 g%. Other blood investigations were normal.
An ultrasonography of the abdomen was suggestive of a well-defined, encapsulated, mixed echogenic lesion in the posterosuperior surface of the spleen of 250-300 cc in volume. Contrast-enhanced computed tomogram (CECT) of the abdomen showed a space-occupying lesion of the spleen. He had undergone percutaneous aspiration of the cyst, which revealed 250 ml of greenish-colored fluid with no growth of microorganisms. Polyvalent pneumococcal vaccine was administered.
At laparotomy, the spleen was enlarged and densely adherent to the stomach on its anterior and medial sides. There were adhesions with the pancreas and also dense, inseperable adhesions with the diaphragm. There was a cystic area with surface discoloration on the posterosuperior surface of spleen. Intraoperative aspiration of the cystic area resulted in 200 ml of greenish-colored fluid. Total splenectomy was performed in view of the dense adhesions to the adjacent structures, large size of the cyst even after aspiration and the inability to leave behind adequate viable splenic parenchyma after cyst dissection []. Pus culture did not show any growth of organisms. The histopathology report of the specimen of spleen showed cysts lined by cuboidal epithelium in continuation with a stratified squamous lining, suggestive of metaplasia []. The diagnosis on histopathology was epithelial (primary) splenic cyst.
The post-operative course was uneventful and he was asymptomatic at the 3 months follow-up.


A 59-day-old, 5.3 kg, male patient presented for general anesthesia for undergoing craniotomy and vertical parasagittal right hemispherotomy. He was normally delivered at 39 weeks of gestation with a birth weight of 3,180 g without any complications or abnormal findings. One week after birth, his seizures started with repetitive clustered tonic spasms for 30 min, at a frequency of 10 clusters a day. He was admitted at an outside hospital, and vigabatrin 250 mg and phenobarbital 20 mg were administered. Yet his seizures were refractory to anticonvulsants, so he was transferred to our hospital at the age of 30 days.
There were no abnormal physical findings and laboratory investigations. The electrocardiogram and chest X-ray were also normal. After admission, he had generalized tonic seizure with right-ward eyeball deviation, blinking and nystagmus at a frequency of 1-3 a day. Flexor spasms in clusters were also observed for 10 minutes, at a frequency of 7 clusters a day. His magnetic resonance imaging (MRI) showed cortical dysplasia at the right temporal and parietal lobes (). On the positron emission tomography (PET) study, abnormal high uptake for that age was discovered at the right cerebral hemisphere. His video EEG for 2 days demonstrated excessively asynchronous and discontinuous rhythms, the so called suppression-burst patterns, and this was consistent with OS during both the awake and asleep states (). He was diagnosed with OS and right hemispherotomy was planned for treatment.
He was transported to the operating room and the immediate vital signs revealed a blood pressure, pulse rate and oxygen saturation of 83/42 mmHg, 152 beats/min and 100%, respectively. Before induction, 0.02 mg of glycopyrrolate was injected intravenously. The patient was preoxygenated and general anesthesia was induced with thiopental sodium 25 mg and atracurium 3 mg. Mask ventilation was begun with 100% oxygen and there was no difficulty in ventilation. After confirming full relaxation, the patient's trachea was intubated with a 3.5-mm ID endotracheal tube and volume controlled ventilation was provided with a tidal volume of 45 ml. The respiratory rate was adjusted to maintain the end-tidal carbon dioxide (EtCO2) at 35-40 mmHg. Anesthesia was maintained with sevoflurane 2.5 vol%, FiO2 0.5 with air and 2 L/min of inspiratory fresh gas flow. A 24-G catheter was placed in the left radial artery for continuous BP monitoring and arterial sampling, and 20-G catheter was inserted into the right femoral vein.
During surgery, the blood pressure, pulse rate and oxygen saturation were stable at 62-74/38-51 mmHg, 127-151 beats/min and 99-100%, respectively. The intra-operative arterial blood gas analysis was in the normal range, and the pH, partial pressure of arterial oxygen (PaO2) and partial pressure of arterial carbon dioxide (PaCO2) was 7.43, 173.2 mmHg and 33.1 mmHg, respectively. On completion of surgery, spontaneous respiration was fully restored by manual ventilation. Atropine 0.05 mg and neostigmine 0.1 mg were administrated to reverse the residual muscle relaxation. The endotracheal tube was removed after confirming that the patient was awake and the muscle function had been fully restored. The operation was uneventful. The patient was transported to the post-anesthetic care unit (PACU). The operation time was 6 h 45 min, and the duration of anesthesia was 7 h 55 min. The total amount of fluids administered was 400 ml of 1 : 4 dextrose solution and 500 ml of normal saline, and 200 ml of packed RBCs were also transfused. The total blood loss was 170 ml and the urine output was checked to be 280 ml. The preoperative level of hemoglobin was 9.0 g/dl, and the postoperative level was 10.5 g/dl.
In the PACU, the patient showed stable vital signs and a regular breathing pattern. His mentality was alert and there was no newly developed neurologic deficit. There were no complications, including hematoma or loss of distal pulsation on the sites of the radial arterial and femoral venous cannulation. He stayed for 50 min in the PACU and then he was moved to the neurologic intensive care unit. He had no seizure in the first 7 days after surgery and then he was discharged. During the 2 months after surgery, he had no seizure and he has been followed up at our hospital.


A 66-year-old woman visited our hospital with a painless lump in the left breast. The patient had a history of trauma in the left breast three years earlier. The patient had fallen and damaged her left breast. At that time, the patient had received an oriental medicine, traditional alternative treatment, called "Bu-Hwang" for the bruise and swelling in the left breast. After several months, the patient felt a painless lump in the left breast, and thereafter, the breast lump gradually increased in size for the next three years. The patient visited our hospital for evaluation of a palpable lump in the breast. At that time, a breast surgeon noticed no abnormalities on the chest wall, except for the breast lesion. Routine mammography was performed and it showed the presence of a relatively well-demarcated bizarre shaped lesion with brighter density as compared to calcification in the upper medial portion of the left breast. The maximum diameter of the lesion, as depicted on mammography, was approximately 3.5 cm (). The mass was excised at a breast surgery clinic at the request of the patient for cosmetic reasons and discomfort. Unfortunately, the mass was excised without an ultrasound examination. The mass was surgically excised and a histopathological examination showed a metaplastic focus of bone formation, multiple stromal fat vacuoles, scattered lymphocytes and foreign body type multinucleated giant cells forming a granuloma (). The pathologist confirmed that there were osteocytes in the newly-formed bony trabeculae (), and also verified epithelioid cells within the granulomatous changes, which support the diagnosis of a lipogranuloma with osseous metaplasia rather than fat necrosis. The pathologist also found that breast gland tissue was continuous with the lesion, suggesting that this lesion arose in the breast tissue. These findings were consistent with a lipogranuloma with osseous metaplasia in the breast.


A 28-year-old male presented with a 2-day history of acute and progressive back–neck pain, and numbness and weakness of both lower extremities concurrent with feces and urine retention secondary to an influenza 1 week before onset. He responded markedly to intravenous administration of glucocorticoid for 1 week after being initially misevaluated as “acute myelitis” in a poor countryside clinic, who was misdiagnosed by the barefoot doctor only according to his natural history without detailed examination by CT or MRI. On our body examination, muscle strength of lower limbs was grade 5−, with increase of their muscle tone and accentuation of deep tendon reflex. Banbinski sign and meningeal stimulation response were positive. Superficial and deep hypoesthesia below T9 were found.
Routine examinations, including cerebrospinal fluid, showed no abnormality except that abdominal ultrasound demonstrated renal lesions. Magnetic resonance brain, spinal cord, and kidney images showed multiple lesions of different size, mostly with clear boundaries and marked contrast enhancement (Fig. a–d). Spinal canal explorations revealed two red nodular subdural lesions at T10 and T12 that accorded with Fig. a. Pathological analysis indicated many small blood vessels with a small amount of foam cells, fat-like cells and fibroblasts, which tested positive for GFAP, F8, and Lys, though negative for CD68, CK-pan, and EMA by immunohistochemistry assay (Fig. e, f). The mutation analysis was completed yet did not find the variation after polymerase chain reaction (PCR) and sequencing of three exons of VHL gene. Systematic enquiry and examinations indicated his father as an asymptomatic affected individual with hemangioblastomas involved in brain and kidney. The patient improved further on his neurological manifestation 2 weeks after surgery on the two nodules. One year later, a follow-up study indicated that he was completely rehabilitated.


A 49-year-old male patient was admitted to our emergency service with a one-week history of abdominal pain. Despite using analgesics, abdominal discomfort progressed to generalized abdominal pain, nausea and vomiting. Upon physical examination, generalized abdominal tenderness and rebound tenderness were detected. Laboratory parameters revealed leukocytosis [14.3 × 103/µl (range 4.5-11)] and a high C-reactive protein level of 247.12 mg/l (range <5). No disorder was determined from other parameters. Plain x-ray abdominal radiography was unremarkable. Abdominal ultrasonography (US) and computerized tomography (CT) revealed dilated small intestines in the right lower abdominal quadrant with pericecal free fluid that was compatible with a possible perforated or plastron appendicitis (Fig. 1). Urgent surgery was planned after obtaining the patient's consent. On exploratory laparotomy, a complicated mass formation composed of the appendix, cecum and sigmoid colon was identified in the right lower abdominal quadrant. After separation of the sigmoid colon, a perforated cecal area of necrosis and the appendix were identified. A right hemicolectomy and a hand-sewn ileotransversostomy were performed. On the third postoperative day, the patient's vital signs began to deteriorate and an enteric fistula was observed at the drainage tube. Therefore, a second-look laparotomy was performed. On exploration, an anastomotic leakage associated with a segment of the tapeworm was identified (Fig. 2). The anastomosis was ligated, and an end ileostomy was performed due to the generalized fecal peritonitis. The postoperative course was uneventful. The patient received a single dose of niclosamide (4×500 mg) 10 days after surgery. The histopathologic examination of the specimen was read as infectious colitis, and the parasitological examination revealed T. saginata.


The patient was a 37-year-old woman who had been diagnosed 9 years prior with OA caused by inhalation of cefteram powder. Her skin-prick test at her initial visit revealed negative responses to common inhalant allergens, except for a positive response to cefteram powder (10 mg/mL). She left her workplace and had been well for 9 subsequent years; her asthma was controlled with a medium dose of inhaled corticosteroid/long-acting β-2 agonist and leukotriene receptor antagonist based on regular follow-up.
She experienced aggravation of her rhinitis and asthmatic symptoms again several months prior to presentation. At presentation, her blood differential count, serum biochemistry, and chest and paranasal sinus radiographs showed no abnormality, with normal lung function. However, her skin prick test results had converted to strongly positive responses to two types of house dust mites: Dermatophagoides pteronyssinus (18×14/50×44 mm) and Dermatophagoides farinae (18×9/46×46 mm). The total immunoglobulin (Ig)E level was elevated to 119 kU/L (normal: 0-114 kU/L), with high serum specific IgE levels to D. pteronyssinus and D. farinae of 4.35 and 7.63 kU/L, respectively (normal: <0.35), which had previously been undetectable. The eosinophil cationic protein (ECP) level was elevated to 23.2 µg/L (normal: 0-13.50 µg/L), with a normal peripheral total eosinophil count (377.3/µL), as shown in .
We speculated that she was newly sensitized to house dust mites, which aggravated her asthma symptoms. An inhalation challenge test with D. farinae was performed, and significant bronchoconstriction (21.1% reduction in the forced expiratory volume in the first second [FEV1]) with asthma symptoms was observed at 10 minutes, with no late asthmatic reaction.


A 52 years old Pakistani right handed woman presented to Emergency Department with polyuria and polydepsia for four days. Also she had recurrent abnormal movements in the left hand with progressive involvement of the upper limb that started abruptly three days earlier. There was no significant past medical history. She was not taking any medication. The blood sugar was found to be more than 24 mmol/l when tested by glucometer in the emergency department. The vital signs, heart, lung and abdominal examination were normal apart from sinus tachycardia. However, her head was intermittently turning to the left side with abduction and external rotation of the left arm. Glasgow Coma Scale was 10/15.
Laboratory results showed that blood sugar of 96 mmol/L (1728 mg), serum sodium of 123 meq/L, potassium level of l 5.6 meq/L and serum osmolarity of 375 mOsm/L. There were negative ketons in blood and urine with bicarbonate level of 24 mmol/L. Blood gases showed normal Ph 7.39. Cerebrospinal fluid analysis showed normal cells, protein and high glucose level (22 mmol/L). Computerized tomography (CT) and Magnetic resonance imaging and (MRI) of brain were normal. Electroencephalogram (EEG) showed frequent epileptiform discharges involving the right frontocentral area which is consistent with EPC (). Carbamazepine 400 mg daily was started. Correction of metabolic disorder (Nonketotic hyperglycemia) with proper fluid replacement and insulin intravenous drip resulted in a gradual reduction in the number of seizures until they stopped three days later. The patient stabilized and was transferred to the medical floor. She was seen by a diabetic educator and discharged home in good condition. Carbamazepine was discontinued one month after discharge. No seizures were reported during nine months of follow up.


A 20-year old man was referred to the maxillofacial department following a single alleged punch to the left side of the face. He complained of swelling and parasthesia of his left cheek, left lateral nose and upper left gingivae.
On examination he had gross swelling of the left side of the face, but there was no bony deformity or bony tenderness. A fracture of the left lateral wall of the maxillary sinus was diagnosed on the basis of the occipito-mental (OM) radiographs () and he was treated conservatively with oral antibiotics. One week after his injury, a small haematoma was noted on the left cheek. He was advised to massage the haematoma to decrease its size and to return to the outpatient clinic in one week. Two days later he presented with increased oedema. On examination he was found to have a 3-cm×3-cm fluctuant erythematous swelling of the external left cheek. He underwent incision and drainage of the suspected infected haematoma under general anaesthesia. More than 50 ml of purulent fluid was drained and the patient was discharged two days later.
An orthopantomograph (OPG) was taken to rule out dental abscess. The OPG () showed radiolucency in the left side of the maxilla extending from the upper right central incisor to the upper left first molar, which had not been clear on the previous occipito-mental views.
A computed tomography (CT) scan showed an extremely large lobulated lesion with a relatively well defined border extending from the upper right lateral incisor to the upper left first molar (). The lateral wall of the antrum was perforated. Due to the massive size of the lesion and its multi-lobulated nature on the CT scan, the diagnosis of an odontogenic keratocyst was suspected, but ameloblastoma could not be ruled out.
Following the OPG and CT results, a biopsy was performed at two sites under local anaesthesia. Histopathology reports indicated this was a cystic cavity lined with stratified squamous epithelium showing some hyperplasia. The wall of the cyst consisted of dense fibrous connective tissue with abundant chronic inflammatory cells, few mast cells, and eosinophils. The condition was diagnosed as a radicular cyst.
The cyst was enucleated by a Caldwell-Luc approach and was found to extend through the entire maxilla on the left side into the antrum, and distally to the second molar tooth. The postoperative course was uneventful. The patient required long-term follow-up to check for recurrence. He was referred to his GDP for thorough investigation and treatment of non-vital teeth.


A 19-year-old man, with no past and familial history of heart disease, was brought to the emergency department following a motorcycle accident in which his chest hit the steering wheel with considerable force. He presented left knee, chest and abdominal pain. The patient's vital signs were temperature 36.5℃, heart rate 105 beats per minute, respiration rate 20 per min, blood pressure 100/60 mmHg, and pulse oximetry 98% on room air. On the initial physical examination, a pansystolic murmur was heard along the lower left sternal border with inspiratory accentuation, but signs of right heart failure were not detected. Chest radiograph was normal. Electrocardiogram showed complete right bundle branch block with left posterior fascicular block (). Results of laboratory tests were notable for elevated cardiac biomarker, creatine kinase myocardial band isoenzyme was 144.1 ng/mL, troponin I was 13.88 ng/mL. Echocardiography was performed for evaluation of chest pain and elevated cardiac biomarker. A flail of the septal and anterior tricuspid leaflet was present and rupture of the papillary muscle was suspected on transthoracic echocardiography (). Although all cardiac structures were identified on transthoracic echocardiography, we performed transesophageal echocardiography to delineate exact anatomy of subvalvular structure. It confirmed prolapse of the septal and anterior tricuspid valve leaflet with large portions of the valve and the subvalvular apparatus protruding into the right atrium indicating rupture of both anterior and posterior papillary muscles (). Color-flow Doppler echocardiography shows severe tricuspid valve regurgitation (). Peak velocity of tricuspid valve was 1.62 m/sec and estimated right ventricular systolic pressure was 20.5 mmHg (). We decided to repair tricuspid valve and referred to thoracic surgery department. Intraoperative findings confirmed the echocardiographic diagnosis of papillary muscle rupture. Tricuspid valve repair was performed with reimplantation of the ruptured papillary muscle and a ring annuloplasty. Postoperative echocardiography showed satisfactory leaflet coaptation () and repaired papillary muscle (). Only mild tricuspid regurgitation remained. The patient discharged from hospital and continues to be asymptomatic after 1 month.


A 40-year-old woman presented with pain in the right upper abdomen for last 6 months. Ultrasonography of upper abdomen failed to show suspected biliary pathology. But a possible adrenal mass was noted close to the upper pole of right kidney. The clinician suspected of an adrenal mass favoring adrenal adenoma. But the routine blood and urine examination along with estimation of 24 h urinary cortisol and vanillyl mandelic acid excretion did not show any abnormality. Computed tomography (CT) scan of the abdomen was advised, which showed a lesion of right adrenal gland (7 × 5.5 cm) containing fat and specs of calcification []. A possible radiologic diagnosis of adrenal myelolipoma was suggested and histopathologic examination was recommended. The tumor was dissected out. Grossly, it was soft, well circumscribed, 6.5 × 4.5 cm in size. Multiple sections from different parts of the tumor were processed and examined under microscope. The tumor was seen to be composed of mature adipose tissue and trilineage hematopoietic elements (erythroid, myeloid, and megakaryocytic) without any evidence of dysplasia []. A thin rim of normal adrenal tissue was also seen adjacent to the tumor []. A diagnosis of adrenal myelolipoma was confirmed. The patient was followed-up for 1 year, which was uneventful.


A 55-year-old female patient of neurocysticercosis, on phenytoin therapy for 3 years (plasma concentration of drug 10 μg/L), with otherwise normal clinical profile underwent laparoscopic cholecystectomy for cholelithiasis. General anesthesia (GA) was administered following routine pre-anesthetic medications comprising of inj. glycopyrrolate 0.2 mg, inj. ondansetron 4 mg, and inj. fentanyl 100 μg. Induction was done with 2.5% thiopentone sodium 250 mg and intubation with inj. succinylcholine 100 mg. GA was maintained with O2 , N2 O, 0.5% isoflurane and inj. vecuronium 0.1 mg. Vital parameters were monitored including blood pressure, heart rate and SPO2 by pulse oximetry till the end of surgery. The duration of action of vecuronium was markedly reduced to 5--6 min, resulting in recurrent acquisition of lighter plane of anesthesia, bronchospasm with rapid fall in SPO2 from 100% to 85%. Bronchospasm could not be adequately managed with standard bronchiodilators. Vecuronium drip was started at the rate of 2 μg/kg/min but hypoxia still could not be managed. Pneumoperitonium and reverse trendelenberg position of laparoscopic surgery could not be allowed and the procedure had to be converted to an open cholecystectomy. Thereafter, SPO2 was maintained within acceptable limits but resistance to vecuronium continued till the end of surgery.


A 78-year-old male patient presented with pain and swelling in the middle of left leg for the past 3 years. There was no history of fever, weight loss or other constitutional symptoms. Radiograph of the leg revealed a lytic, eccentrically located defect in the shaft of left tibia with sharply defined margins. There was no extension to the overlying soft tissue. Clinico-radiologically, a diagnosis of simple bone cyst was made. Considering the age of the patient, metastatic carcinoma was also kept in mind. However, routine investigations were within normal limits.
Fine needle aspiration cytology (FNAC) of the lesion was done using an 18-G needle and a provisional diagnosis of adamantinoma of tibia was made. En bloc excision was done and the patient was kept under follow-up. Till 1 year, there was no history of recurrence or metastasis.
FNAC smears were highly cellular and showed few dispersed, benign appearing, spindle-shaped cells admixed with cohesive clusters of small, uniform ovoid to round epithelial cells. The latter had oval to round nuclei, mostly uniform with finely granular chromatin and inconspicuous nucleoli []. Histopathology showed solid nests of basaloid cells with peripheral palisading and sometimes a stellate configuration in the center. Immunohistochemistry confirmed the epithelial nature of the cells as they were positive for CK14 and 19.


60 yrs old, 55 kg female with known case of von Recklinghausen neurofibromatosis () was scheduled for dynamic hip screw fixation of left femur and intramedullary interlocking nail for humerus. There was no history of previous surgeries and drug allergies. She was poorly built & systemic examination revealed a normal cardiovascular and respiratory systems. She had protruding upper incissors with adequate mouth opening, normal neck and temporo mandibular joint movements, Mallampati class-1 on oropharyngeal examination.
Pulmonary function tests revealed forced vital capacity (FVC of 1.5 lts/min), forced expiratory volume in first second (1.09lt/min) and FEV1/FVC of 91%. X-ray chest (PA), CT abdomen, CT scan of head & brain were normal and other blood investigations are within normal limits.
Regional anaesthesia was planned for both the surgical procedures. The anaesthetic procedures were explained to the patient and written informed consent obtained. She was advised oral Midazolam (10mg) at bed time on the night before and 5 mg on the morning of surgery following an overnight fast. In the operating room her heart rate was 88/ min & blood pressure was 140/80 mmHg, oxygen saturation was 99%. Intravenous access with 18G canula was obtained. The patient was positioned for subarachnoid block in a sitting position. After preparation of back of the patient, appreciation of the tips of the spinous processes and interspinous spaces, sub-arachnoid space was located in L2-L3 space with midline approach. After free flow of cerebro spinal fluid, 3 ml of 0.5% Bupivacaine heavy was given into the sub-arachnoid space. The patient was put in supine position and oxygen supplementation was given with Hudsons mask at a flow rate 3 lts/min. After 5 min, level of anaesthesia was checked and patient is shifted on to the fracture table. The surgery lasted for 1½ hour during which 1500ml of crystalloids were given. The urine out put during the intra operative period was 900 ml. After the completion of hip surgery patient was shifted to another table.
The patient was explained about the procedure before giving brachial plexus block (25ml 1% lignocaine and 1 in 200000 Adrenaline ) by supra clavicular route .After giving the block, the patient was monitored for 10 minutes and kept in supine position. The surgery lasted for 1 hr 20 min during which 500 ml of crystalloid & one unit of compatible blood was transfused. The urine output during the intra operative period was 600ml. Post operative period was uneventful and post-operative pain was managed with IV Tramadol hydrochloride (50 mg, 8 hourly)


A 40-year old female presented with a several month history of slow growing, painless nodule in anterior maxillary gingiva. The lesion was a well circumscribed, raised sessile nodule, the same color as the surrounding mucosa, in the area between teeth numbers 8 and 9 (Fig. ). There was no bleeding or exudates in the area. The patient’s medical and social history was non-contributory and she did not take any medications. Radiographic examination was unremarkable. The initial clinical differential diagnosis was pyogenic granuloma, fibroma, peripheral giant cell lesion and peripheral ossifying fibroma.
The lesion was excised and submitted to the Oral Pathology Department at the University of Maryland, Baltimore. Gross examination of the specimen revealed a tan soft tissue mass, measuring 1.5 x 1.1 x 0.7 cm. Microscopic examination of the soft tissue specimen, using hematoxylin and eosin stain, revealed a mixture predominately composed of inflammatory cells, with scant irregularly arranged fascicular spindle cells mixed with a prevalent plasma cell infiltrate (Fig. , ). The inflammatory infiltrate also contained varying numbers of neutrophils, lymphocytes and macrophages. The background stroma showed focal myxoid and sclerotic foci. Nuclear atypia was not seen. Immunohistochemical analysis was positive for the expression of muscle specific actin (Fig. ), anaplastic lymphoma kinase-1 (ALK-1) (Fig. ), and CD-68 (Fig. ).
A final diagnosis of IMT was made. There was no evidence of recurrence after four months of follow-up (Fig. ).


A 46-year-old female patient was admitted to our department due to a cystic tumor in pancreatic body and tail. Routine chemistry and tumor marker levels (CA 19-9, CEA, CA 125) were all within normal limits. Abdominal CT scan and EUS showed about 5×4 cm sized, slightly septated cystic tumor between the body and tail of the pancreas (). There was no mural nodule, no calcification, and no eccentrically located mass within the cystic area. The benign mucinous cystic tumor of the pancreas was the most favorable preoperative diagnosis. The operation sequence was similar to usual laparoscopic distal pancreatectomy except trocar placement (). The robotic camera arm and other three instrument arms were then connected to their respective ports. At this point, the surgeon sat at the surgical console located about 3 m from the operating table. The assistant surgeon was positioned on the patient's left side for temporary use of laparoscopic instruments through Port 4. In spite of our previous experiences of robot surgical system, the cystic tumor and the remaining splenic vessels were so closely adherent that bleeding was frequently encountered, and most operation time was spent for this process. The risk of cyst rupture was also considered when we tried to separate and divide the small splenic vessels from pancreatic cyst (). Therefore, concomitant segmental resection of splenic vessels was thought to be necessary for spleen preservation. Total operation took about 480 minutes, and about 800 mL of blood loss was noted without blood transfusion. The specimen was delivered through the umbilical port site with small vertical incision after putting into the plastic bag. The patient recovered without complication. She returned to an oral diet on the postoperative second day. After the drain was removed, she was discharged on the fifth day post-operation. The final pathologic examination revealed that the tumor was benign mucinous cystic neoplasm without any characteristics of malignant potential (). A color doppler ultrasound scan obtained 14 days after surgery showed no evidence of splenic infarction and well preserved blood flow within the spleen without definitive peripancreatic fluid collection ().


An 8.5-yr-old girl was referred to an endocrine clinic because of vaginal bleeding and uncontrolled hypertension on January 15, 2004. At 7.5-yr-old, the girl presented with severe headaches and nausea and was diagnosed with hypertension. She was prescribed antihypertensive drugs (Unipril 10 mg and Norvasc 10 mg). However, the hypertension remained uncontrolled. Concurrently, she showed breast development, and 3 months later, frequent vaginal spotting was observed. At 8-yr-old, pubic hair was observed. The girl had undergone surgery at 15 months of age due to bilateral grade III vesicourethral reflux but was subsequently found to be healthy. Her grandfather had been treated with hypertension.
Upon presentation to our clinic, the patient's height was 149 cm (> 95 percentile) and her weight was 42 kg (> 95 percentile). Her arterial blood pressure was 140/90 mmHg. On physical examination, the Tanner stages of breast and pubic hair were at stage III. Neurological examination was normal. Neither androgenic nor glucocorticoid excess was detected.
Laboratory investigations revealed normal renal function and low serum potassium (2.7 mEq/L). Plasma renin (133.5 ng/mL/hr; normal: < 4.4 ng/mL/hr), angiotensin II (5,230 pg/mL; normal: 9-47 pg/mL) and aldosterone levels (824.1 pg/mL; normal: < 130 pg/mL) were extremely elevated. The remaining serum electrolyte and complete blood cell count were normal, but proteinuria was detected in the urine analysis. Thyroid function was normal, and there was very mild prolactin elevation (33.69 ng/mL; normal 0-29.5 ng/mL). There was no elevation in Luteinizing hormone (0.29 mIU/mL) or Follicle-stimulating hormone (< 0.1 mIU/mL); however, estradiol was elevated (290.38 pg/mL). Prepubertal response was observed in a Gonadotropin-releasing hormone (GnRH) stimulation test, and beta HCG was in the normal range.
On radiologic tests, the patient's bone age was advanced (12 yr old by the Greulich-Pyle method). Pelvic computed tomography confirmed the well-defined right solid ovarian tumor (5.1 × 4.0 cm, ). Right oophorectomy was performed and antihypertensive treatment was tapered off over several days.
On gross examination, the right ovary measured 5.0 × 4.0 cm and the capsule was intact. The cut surface showed a bright yellow solid tumor with lobulation. There was no hemorrhage or necrosis.
Histological examination revealed that the ovarian tumor had sheets of clear or eosinophilic cells surrounded with a delicate fibrous stroma. The tumor cells showed small, dark-stained single nuclei with occasional nucleoli. Cells undergoing mitosis were counted less than 2 per 10 high power fields. Immunohistochemistry revealed a diffuse positive reaction for renin, alphainhibin in tumor cells. Ovarian stromal cells were positive for progesterone receptor ().
In conjunction with the clinical history, these findings supported a diagnosis of renin-secreting ovarian steroid cell tumor, NOS.
The patient was progressing well 16 months after surgery. Her blood pressure was normal (100/60 mmHg) without medication, and proteinuria had disappeared. Plasma renin, aldosterone and potassium levels had returned to normal, a normal pubertal response was shown in a GnRH stimulation test, and regular menstruation was present.


A previously healthy 43-year-old woman complained of progressive visual field restriction, with a bitemporal hemianopsia and headache.
On admittance, the patient presented also extraocular movements within normal limits and double vision in the right gaze. The bitemporal hemianopsia was confirmed by visual field analysis.
Magnetic resonance imaging and magnetic resonance angiography revealed two spherical formations in the right and left carotid siphon.
The patient underwent to a cerebral angiography [Figures and ]. There were two aneurysms. A right supraclinoid one measuring 3 cm in maximum diameter and a left one, also supraclinoid and measuring a maximum diameter of 10 mm.
We decided to clip the right carotid aneurysm microsurgically. A right pterional route was performed and the right carotid aneurysm was approached during temporary endovascular occlusion of the right internal carotid artery (ICA). This allowed careful dissection of the aneurysm sac from the surrounding structures.
Preoperatively, a balloon occlusion test of right ICA was performed for 20 minutes; the test confirmed the counter lateral compensate by the anterior communicating artery.
During the procedure, however, the aneurysm ruptured at the neck and, as surgical clipping could not be achieved, the neck of the aneurysm was sutured and then reinforced with a Yasargil clip. The patient was then transferred to the Intensive Care Unit for postoperative monitoring.
Angiographic control demonstrated the complete exclusion of the right ICA giant aneurysm, but a right ICA occlusion was discovered []. Right intracerebral vascularization was compensated by the left circulation. The left carotid aneurysm remained unchanged []. At discharge, the patient presented a right third cranial nerve palsy and hypoesthesia in the right V1and V2nervous branches that recovered within 1 month. After 3 months, the patient returned to our institution to undergo treatment for the left ICA aneurysm for which an endovascular approach was selected.
The wide neck of the left ICA aneurysm suggested the possibility of a stent-assisted coiling, instead of a simple coiling. A right EC-IC bypass was proposed mainly due to the possible risk of a left ICA occlusion during the endovascular procedure. EC-IC bypass has been proposed by Donaghy[] and Yasargil[] to bypass an occlusive process in the arteries supplying the brain that is not accessible surgically in another way.
It was our opinion that a stent-assisted coiling procedure of the left ICA aneurysm could increase the risk of thrombotic complications. Therefore, a right ICA-MCA bypass was planned and a saphenous vein graft was chosen because radial artery was not available as a suitable vessel (Allen test negative).The bypass was performed under electroencephalographic confirmed burst suppression. A frontal branch of the MCA-M2 was chosen as a recipient vessel without perforators. The right ICA was isolated and sectioned 1 cm above the common carotid artery bifurcation and, after removal of the thrombus, a proximal end-to-end anastomosis of the saphenous vein graft to the right ICA and a distal end-to-side anastomosis of distal end of the graft to the MCA-M2 branch was performed. An interrupted 8-0 Prolene suture was utilized both at the proximal and distal end of the graft. The patency of the bypass was assessed intraoperatively by an intraoperative indocyanine green angiography and Doppler ultrasound.
The patient did well postoperatively and magnetic resonance angiography demonstrated the patency of the bypass despite a kinking at the level of the proximal anastomosis [].
Two months later, following an intracranial angiography, endovascular treatment for exclusion of the left carotid aneurysm was performed. Before undergoing the procedure, the patient was pharmacologically treated for 7 days with antiplatelet drugs (ASA 300 mg + ticlopidine 300 mg per QD). During the procedure, an intracranial carotid stent was placed to cover the aneurysm neck (Neuroform, Boston Scientific). A microcatheter was then passed through the stent and GDCs (Guglielmi Detachable Coils) were used to fill the aneurysm sac. The procedure was well-tolerated with no further neurological deficits or complications [].
Three months later, the patient underwent an angiographic study confirming a complete aneurysm occlusion, and demonstrating an area of segmental narrowing at the level of the previously revealed kinking of the proximal portion of the graft.The narrowing was graded 75% (NASCET method) []. The endovascular team then proceeded with an angioplasty and stenting of the graft []. The procedure was well-tolerated and followed by a regular clinical course and immediate mobilization.
Antiplatelet agents (ASA 300 mg + ticlopidine 300 mg per QD) were continued after the procedure.
One year after the first surgical procedure, the patient presented visual field loss improvement and no other neurological deficits. A follow-up angiogram performed 36 months later showed a widely patent graft and robust flow to the MCA territory []. The patient remained asymptomatic.


A 35-year-old female presented with multiple scaly pigmented plaques on the trunk and limbs of 2 years duration. The lesions initially started as scaly annular plaques on the lower limbs, which became ulcerated, crusted and healed with pigmentation and gradually spread to involve other parts of the body. There were no associated systemic complaints. There was no history of viral infection or drug intake prior to onset of lesions. On examination, multiple hyperpigmented and annular polycyclic plaques were present on the trunk and both limbs. Few erosive and crusted plaques were seen in the forearms and lower legs
[]. Oral cavity showed palatal erosions with hyperpigmentation []. Genitalia, palms, soles, and nails were normal.
Her complete blood count, liver and renal function tests were normal. ANA and Anti- Ro antibodies were positive. Lupus anticoagulant, anti cardiolipin antibody, VDRL, and HIV ELISA were negative. Histopathology revealed epidermis with hyperkeratosis, follicular plugging and basal vacuolar damage. Few civatte bodies were seen in the epidermis and superficial dermis []. The superficial dermis was edematous and showed mucin deposits with perivascular lymhocytic infiltrate and melanophages. Direct immunofluorescence showed linear deposits of fibrinogen at the basement membrane zone. With the history and investigations, a diagnosis of SCLE-LP overlap syndrome was made. She was administered oral steroids and hydroxychloroquine and the lesions subsided.


A 66-yr-old man was admitted to the hospital because of back pain and dyspnea on exertion. The patient had no history of medical illness, and he had good health until this hospital visit. Complete blood count analysis revealed that the hemoglobin level was 8.6 g/dL with microcytic and hypochromic anemia. The iron panel indicated that his anemia was caused by iron deficiency: the serum ferritin level was 5.62 ng/mL (normal range, 30-310 ng/mL) and transferrin saturation was 7.34% (20-55%). His serum chemical values were as follows: total protein, 7.9 g/dL (normal range, 6.4-8.3 g/dL); albumin, 2.7 g/dL (3.4-5.1 g/dL); lactate dehydrogenase level, 366 IU/L (240-480 IU/L); calcium level, 8.0 mg/dL (8.4-10.0 mg/dL); blood urea nitrogen, 12.7 mg/dL (6.0-20.0 mg/dL); and creatinine level, 0.8 mg/dL (0.5-1.2 mg/dL). Serum protein electrophoresis showed an abnormal zone of restriction in the gamma globin lane. The level of the M-peak was 2.1 g/dL. Serum immunofixation electrophoresis demonstrated monoclonal gammopathy of the IgM lambda type ().
Computed tomography (CT) was performed because the patient developed abrupt severe abdominal pain. The CT scan showed a circumferential huge mass in the ileal loop along with perforation and multiple lymphadenopathy. The patient underwent an emergency operation, and he was diagnosed with ENMZL of MALT with focal large cell transformation (diffuse large B-cell lymphoma) of the small bowel. Histologic examination revealed the MZ tumor cells infiltrating around the reactive follicles and disseminating to the small bowel mucosa (). The tumor cells were small to medium-sized, and they contained slightly irregular nuclei with moderately dispersed chromatin and inconspicuous nucleoli. The intestinal crypt epithelium was often invaded and destroyed by these tumor cells, leading to the formation of the lymphoepithelial lesion (). Plasmacytic differentiation was observed, which was verified by immunohistochemistry for CD38 (). Immunohistochemical analysis showed that the neoplastic lymphocytes were positive for CD20 and negative for CD5, CD10, CD23, and cyclin D1.
Bone marrow biopsy was performed, which showed bone marrow involvement of ENMZL. The bone marrow aspirate showed 20% cellularity and 2.1% of plasma cells (). Bone marrow biopsy revealed focal infiltration of the neoplastic lymphoid cells, which showed a morphologic spectrum, including small lymphocytes with inconspicuous nucleoli and plasmacytoid lymphocytes ().
A whole-body positron emission tomography (PET)-CT scan revealed a hypermetabolic mass (diameter, 7 cm) in the ileum and multiple abdominopelvic lymph node metastases. The patient was diagnosed with IgM monoclonal gammopathy and stage IV ENMZL of the small intestine involving the bone marrow and multiple abdominopelvic lymph nodes. The patient was administered a combination chemotherapy of cyclophosphamide (750 mg/m2 on day 1), adriamycin (50 mg/m2 on day 1), vincristine (1.4 mg/m2 on day 1), prednisolone (100 mg/day on days 1-5), and rituximab (375 mg/m2 on day 1) every 21 days. After the fourth cycle of chemotherapy, the patient recovered, and he is now being followed-up on an outpatient basis.


The proband (KUCG 797) was a 5-year-old boy born to healthy Japanese parents without any family history of muscle disease. He started to walk independently at 1 year and 4 months of age and his motor development was normal. At the age of 2 years, his serum creatine kinase level was found to be 2,567 IU l−1 (normal <169 IU l−1) in a routine blood examination conducted on admission to hospital. He was referred to Kobe University Hospital to have his dystrophin gene examined for mutations. The mild creatine kinase elevation persisted (1,331–4,740 IU l−1). No muscle weakness or abnormal gait was observed. At the age of 5 years, a muscle biopsy was performed. Informed consent for all study aspects including genetic testing was obtained from the parents, and these studies were approved by our ethics committees.


A 50-year-old postmenopausal woman presented with increased frequency of per vaginal bleeding since 6 months and distension of abdomen since 15 days. The patient's history revealed that she had attained menarche at the age of 14 years, had regular menstrual cycles of 4–5 days duration and moderate intensity at 28 days interval. She attained menopause 2 years back.
Gynecological examination revealed no abnormalities of the vulva, cylindrical vaginal portion of the cervix and no evident pathological change was detectable with clinical examination. Findings of ultrasonography examination suggested bulky uterus with thickened endometrium of 6 mm and hyperechoic mass suggestive of myoma of posterior wall of uterus, measuring 3.5 cm in diameter. In addition, transvaginal sonography revealed hyperechoic lesion of 3.5 × 3.5 cm in the posterofundal region. Also, there were two small subserosal leiomyomas of 0.5 cm diameter each. Both the ovaries showed follicular cysts measuring 3.3 × 3.3 cm each and tubes were normal in appearance.
All the standard serological and hematological parameters were within normal range. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy because of multiple leiomyomas.
On gross examination of the specimen, the uterus measured 8.5 × 6.1 × 5 cm and had three intramural and subserosal well-circumscribed round masses. The biggest nodule which was measuring 3.5 cm in diameter differed from a typical appearance of uterine leiomyoma by being pale yellow and having a somewhat softer consistency on its cut surface
[]. The other two leiomyomas, each of 0.5 cm diameter, showed a coarsely whorled pattern with grayish white appearance on their cut surface. The serosal surfaces of the uterus were normal. Cut section of ovaries showed follicular cysts of 3.5 cm diameter each. The fallopian tubes appeared grossly normal.
Histological examination of the biggest nodule showed a mixture of bland, spindle-shaped smooth muscle cells without nuclear atypia in a whorled pattern with admixed mature adipocytes. The nuclei of the smooth muscles were elongated and had finely dispersed chromatin and small nucleoli. Between these muscle cells, a significant amount of fat cells were visible. The adipose component was entirely mature without any lipoblasts [Figures –]. Based on the above findings, the tumor was diagnosed as a benign lipoleiomyoma. Sections from the other fibroids showed classical histomorphology of conventional uterine leiomyomata. The endometrium showed changes of simple hyperplasia without atypia. Sections from both the ovaries showed follicular cysts with the tubes being unremarkable histologically.


A 3-month-old male infant had visited our pediatric clinic for lethargy, floppy muscle tone, poor oral intake and partial seizures on May 9, 2007. He was born at 38 weeks gestation by vaginal delivery. His birth weight was 3,180 g. He was healthy at birth and a neonatal metabolic screening test was negative. His hair was kinky, brown colored and fragile (). Symptoms of seizure were characterized by left facial twitching and left arm clonic movements. The initial EEG showed one episode of 2 Hz rhythmic spike and wave activity starting from the right central area evolving to the generalized slowings lasting about 100 seconds without clinical seizures, which was consistent with electrical partial seizures (). There was no significant acidosis and ammonia was not increased. Serum lactate, tandem mass screening, serum amino acid and urine organic acids were all within the normal range. Pyruvate level (0.263 mM/L, reference range: 0.03-0.08 mM/L) was high. Partial seizures became more frequent and intractable to antiepileptic medications. About 1 month later, generalized flexor spasms developed. At this time, the EEG had changed to irregular high amplitude delta slowings on the background activities and frequent spikes from the right or left frontal or occipital areas that were consistent with hypsarrhythmia (). Vascular tortuosity and diffuse brain atrophy with callosal thinning were detected in an MRI scan () at 3.5 months. Biochemical markers showed low serum copper (9.0 µg/dL, reference range: 70-130 µg/dL) and ceruloplasmin (5.6 mg/dL, reference range: 16-31.5 mg/dL) levels. From genetic analysis, a c.2743C>T (p.Gln915X) missense mutation () in exon 13 of the ATP7A gene was detected, and the infant was diagnosed with Menkes disease (MD). The mutation was a novel one that has not been previously reported as a cause of MD. He died at 13 months of life.


A 2-month-old 4.3 kg male child was diagnosed as extrahepatic biliary atresia and was scheduled for laparotomy. The child was brought to the pediatrics clinics 2 weeks earlier with complaints of rapid breathing since 12 days, vomiting (non bilious) since 8 days, and decreased oral intake since 1 day. The child was passing clay colored stools and dark colored urine. There was yellow discoloration of skin since 2 weeks, which was progressively increasing. The mother revealed the presence of recurrent rhinorrhoea from the first day of life.
Examination revealed icterus. Abdomen examination showed hepatospenomegaly (liver on left side and spleen on right side) and umbilical hernia. Electrocardiogram (ECG) displayed inverted P waves in I, aVL and aVR, which were suggestive of situs inversus. Chest X-ray revealed evidence of bronchopneumonia and the cardiac shadow was on the right side. Ultrasonography abdomen also indicated situs inversus. Echocardiography was suggestive of situs inversus with dextrocardia with normal cardiac anatomy. Hepatobiliary iminodiacetic acid (HIDA) scan was indicative of extra hepatic biliary atresia (EHBA) with the liver in left upper abdomen. Child was diagnosed as situs inversus with dextrocardia, bronchopneumonia, and extrahepatic biliary atresia.
Bronchopneumonia was managed with intravenous antibiotics, paracetamol, and steam inhalation. The child was placed on therapy with oral phenoparbitone 10 mg twice a day and intravenous Vitamin K 5 mg once a day for 10 days. Investigations revealed hemoglobin 9.5 g/dL, total leucocyte count (TLC) 17400/mm3, platelet count 4.5 lac/mm3, serum sodium 131 meq/L, serum potassium 4.4 meq/L, random blood sugar 70 mg/dL, serum bilirubin 19.2 mg/dL (normal 0.2-1.2) (direct-12, indirect 7.2), SGOT 148IU/L (normal 15-50) , SGPT 93 IU/L (normal 15-50), alkaline phosphatase 1232 IU/L (normal 50-300), blood urea 18 mg/dL (15-45), serum creatinine 0.6 mg/dL (0.6-1.2), prothrombin time (PT) 11.3 sec (test), 12 sec (control), and aPTT test 38.7 sec Control 29 sec (normal 20-40 sec). The child was listed for corrective biliary surgery.
The operating room was kept warm prior to shifting of the child. In the operating room, routine monitors (electrocardiogram, pulse oximeter, non invasive blood pressure) were attached. Left-sided ECG leads were attached on the right side and vice versa. Anesthesia was induced with intravenous fentanyl (9 μg) and thiopentone (25 mg), and lungs were ventilated with isoflurane in oxygen and nitrous oxide (50:50) (MAC 1.2) using Jackson Rees modification of Ayre's T piece. After achieving neuromuscular blockade with atracurium (2.5 mg), trachea was intubated with endotracheal tube size 3.5 mm ID. Capnography and temperature monitoring was also initiated. Urethra was catheterized and urinary output was monitored. Anesthesia was maintained with desflurane in oxygen and nitrous oxide (50:50) (MAC 1) using paediatric close circuit along with boluses of atracurium (0.5 mg) and fentanyl (1 μg) as required. The child was kept warm using warm fluids, warming mattress and by adequately covering the child with cotton.
The surgeon found the liver on left side with right lobe on the left side, malrotated gut (caecum, appendix on the left side), two atretic gall bladders, annular pancreas with preduodenal portal vein, cirrhotic enlarged liver, and a multi-lobulated spleen. Hepatic roux-en-y portoenterostomy with Ladd's procedure was done. The surgical incision site was infiltrated with 6 mL of 0.125% bupivacaine. The hydration was maintained using normal saline. The surgery lasted 5 hours, and blood loss of 100 mL was replaced with packed red blood cells.
At the end of surgery, the airway resistance increased. The airway pressures increased from 18 cm H2O to 28 cm H2O. Endotracheal tube suctioning was done. On auscultation, breath sound was reduced on the right lung fields. Repeat suction was done. We suspected a mucus plug to be obstructing the endotracheal tube and changed the tube after repeat laryngoscopy. A mucus plug was seen at the tip of the tube. The ventilation became better. Residual neuromuscular blockade was reversed and trachea was extubated. The child had good respiratory efforts with bilateral equal air entry. The child was shifted to the pediatric intensive care unit for further management. The child was nursed in the incubator and ECG and pulse oximeter monitoring were continued. The vitals and urinary output were within normal limits. The humidified oxygen was supplemented using an oxygen hood. The postoperative hemoglobin was 9.9 g/dL. The serum electrolytes were normal. The analgesia was provided with rectal paracetamol suppository. The child had an uneventful recovery and was discharged on the 18th postoperative day.


A 64-year-old woman presented to her general practitioner (GP) with a three-year history of a right frontal skull swelling. Over the past year this swelling had increased in size. The patient was referred to the neurosurgery clinic where examination revealed a sessile non-tender mass (approximately 4.3 x 1.6 cm). The overlying skin was intact with normal hair growth. There was no headache and there were no focal neurological signs or symptoms.
The initial skull X-ray was suggestive of an osteoma. Bone scintigraphy was normal. A CT scan of the head showed a sclerotic thickened area of the right frontal bone with no significant mass effect on the underlying cerebrum (). No brain or meningeal enhancement was noted on the scans. The findings were suggestive of an osteoma, with fibrous dysplasia as another possibility. Review of the scans by a second radiologist confirmed an area of sclerosis and non-destructive expansion of the right frontal bone. However, this was thought to represent an intraosseous meningioma.
The patient was treated surgically and underwent a small skin incision overlying the lesion. The latter was chiselled off and sent for pathological examination in order to obtain a tissue diagnosis. The contour of the skull was restored.
Histologically the sample contained fragments of mature bone, whereby some of the bone marrow spaces were replaced by a spindle cell tumour forming lobules and whorls (). It was composed of cells with oval nuclei, some nuclear pseudoinclusions, eosiniophilic cytoplasm and indistinct cytoplasmic borders. Mitotic figures were not present. The features were consistent with an intraosseous meningioma of meningothelial subtype (WHO Grade I).
The patient was subsequently readmitted and underwent a total excision of the tumour and cranioplasty. The involved right frontal bone was excised and replaced with a titanium mesh.


An 88-year-old woman who had been suffering from ITP for several years was admitted for resection of a small tumor of the tongue. Platelet count remained at a level of around 20×109/L. Initial laboratory data showed reduced platelet counts (24×109/L). The patient was referred to our department for management of platelet count, which needed to be increased to about 50×109/L before tumor resection. Although a positive result was obtained for anti-H. pylori IgG antibodies, the patient could not be treated by eradication therapy because of an allergy to penicillins. As the patient was suffering from osteoporosis and refused platelet transfusion, she was treated using CAM without prednisolone (PSL). The clinical course is shown in . CAM was given at 400 mg/day. Platelet count had increased to 42×109/L by 5 days after CAM treatment and then resection was safely performed. Little bleeding was seen during and after resection. After resection, the platelet count had decreased to 28×109/L despite CAM treatment. The patient was treated with EM at a dose of 600 mg/day as an alternative to CAM. The platelet count had increased to 35×109/L by 7 days after starting EM treatment. After the patient was discharged, the platelet count remained at about 34-39×109/L. We tried stopping EM prescriptions for 1 month, but the platelet count decreased to 26×109/L. Re-treatment with CAM did not improve the platelet count, so she was re-treated using EM at a dose of 800 mg/day instead of CAM. After 1 week, platelet count increased from 21×109/L to 31×109/L. The dose of EM was therefore reduced to 600 mg/day. By the end of the observation period, platelet count had increased to 44×109/L.


A 61-year-old man was referred with purpura, which had appeared several months before. Initial laboratory data showed a reduced platelet count (14×109/L), so he was admitted for further investigation. Anti-nuclear antibody was positive (titer 1:640, speckled). Testing for anti-dsDNA antibody, anti-SSA antibody, anti-RNP antibody, anti-Sm antibody, and anti-Topo I antibody all yielded negative results, as did testing for anti-H. pylori IgG antibody. A bone marrow specimen revealed hyperplasia of normal megakaryocytes. Neither articular swelling nor skin rash suggestive of collagen disease was present. Based on clinical and laboratory findings, ITP was diagnosed. The clinical course is shown in . PSL was given at 60 mg/day for 4 days, but the platelet count still decreased to 6×109/L. Methylprednisolone pulse therapy was therefore performed and PSL reduced to 30 mg/day because of hyperglycemia. By 1 week after pulse therapy, platelet count had only increased to 20×109/L. The patient was treated with CAM at a dose of 400 mg/day after obtaining informed consent. About 4 weeks after treatment for ITP, platelet count did not increase and remained at 17×109/L. The patient was therefore administered EM at 600 mg/day as an alternative to CAM. After 2 weeks, platelet count increased to 77×109/L and oral PSL could be gradually tapered. By the end of the observation period, platelet count increased to 208×109/L.


A 75-year-old woman suffering from ITP was admitted with a gradual decrease in platelet count. Prior to admission, platelet count had remained at a level of around 40×109/L. Laboratory data on admission showed reduced platelet counts (18×109/L). Anti-H. pylori IgG antibody and urea breath tests were negative. The clinical course is shown in . CAM was given at 800 mg/day after informed consent was obtained. Platelet count had rapidly increased to 131×109/L by 7 days after CAM treatment. However, after 1 month, platelet count had decreased to 40×109/L despite CAM treatment. Because liver dysfunction occurred, we stopped prescribing CAM. After the patient was discharged, the platelet count remained at about 35-50×109/L without medication. The patient was treated with EM at a dose of 600 mg/day for a common cold. After 1 week, platelet count had increased from 43×109/L to 97×109/L. Because the patient recovered from the cold, EM prescription was stopped. After 1 month, the platelet count had decreased to 41×109/L. By the end of the observation period, platelet count had remained at a level of around 40×109/L without medication.


A 4-year-old male child having severe hemophilia A (recurrent epistaxis, bleeding from gums, echymosis and easy bruisability in the past) on regular treatment and follow-up in the hematology department of the institute was referred to the ENT OPD with the chief complaints of foul-smelling nasal discharge and persistent excessive sneezing for 2 months. He had suffered from one recent episode of upper respiratory tract infection. He was getting supplemental iron tablets and had received IV recombinant factor VIII once in the past.
An infected growth, bacterial or fungal sinusitis or foreign body (FB) in the nose was suspected. An X-ray of the paranasal sinuses revealed an irregular soft tissue density in the left (L) maxillary sinus. A computed tomography (CT) scan of the paranasal sinuses confirmed the presence of FB. Laboratory investigations performed were serum creatinine 0.6 mg%, B+ blood group, Hb 11.8 gm%, TLC 10,400 (P66 L29 E1), normocytic and normochromic general blood picture, reticulocyte count (0.8%), mean corpuscular hemoglobin (MCH) 78.7%,mean corpuscular hemo concentration (MCHC) 32%, platelet (4.1 lakhs) and prothrombin time (PT) 12.6/11.8 s. Raised aPTT (1 min 28 s/29.7 s) was the only significant finding. Factor VIII was 0% and no inhibitor against factor VIII was found.
The patient was jointly reviewed by an ENT surgeon, an anesthesiologist, a hematologist and a pediatrician and a management strategy was charted out. In view of the tender age and severe bleeding disorder hemophilia, it was decided to remove the FB under general anesthesia with a secure airway. To prevent excessive perioperative bleeding, 30 U/kg of human recombinant factor VIII was administered IV (one vial having 220–400 monoclonal purified activity units in Hemofil–M, Baxter product code, 1501805) 1 h before operation. In the operation theater, one more IV line was established for drugs and fluids while the other was reserved for factor VIII infusion. Injection tranexamic acid 150 mg was given as infusion before the induction of anesthesia. Colloids, packed red cells and fresh frozen plasma (FFP) were kept at hand. Anesthesia was induced with thiopental (3 mg/kg) and endotracheal intubation (4.5 mm cuffed endotracheal tube) was performed after achieving paralysis with 25 mg of succinylcholine. A throat pack was gently inserted, avoiding any trauma, to prevent aspiration. Vecuronium bromide 1.5 mg was used after recurrence of respiratory efforts.
Using a flexible endoscope, the FB was successfully removed in 10 min without any bleeding. Extra care was taken to avoid even minimal trauma. Residual muscle paralysis was reversed with neostigmine and glycopyrrolate. Before extubation, the oral and nasal cavities were carefully examined for any active bleeding by both the surgeon and the anesthesiologist. After extubation, the patient was shifted to the intensive care unit and nursed in the recovery position. Six hours after surgery, the patient received another vial of factor VIII and one vial once a day thereafter for 4 days. The postoperative period was uneventful. After 7 days, the patient was discharged from the hospital.


A 33-year-old lady with secondary infertility of 7-year duration, with bilateral polycystic ovaries and history of complete septal resection came for further treatment to our clinic. She had conceived with first cycle of IUI but aborted at 8 weeks. She further took four cycle of IUI but failed to conceive. In the third cycle of IUI she had mild ovarian hyperstimulation syndrome (OHSS) but managed conservatively. She was on eltroxin 50 mcg daily since 5 years for hypothyroidism and her body mass index was 35. She was planned for controlled ovarian stimulation for IVF and was given long protocol. Combined estrogen, progesterone pill was given from day 2 to day 22 of previous cycle and inj. Leuprolide acetate (Lupride®, Sun Pharmaceutical, Halol, Gujarat, India) 0.5 ml subcutaneously was started on day 21 of her previous menstrual cycle. This was followed by 0.2 ml lupride from day 2 of menstrual cycle stimulation cycle after confirming down regulation. Stimulation with recombinant FSH (recagon – Organon) was started on day 2 of menstrual cycle, starting recagon dose was 175 IU s/c for duration of 10 days. Coasting was done for next 2 days by avoiding recagon with continuation of lupride. Peak estrogen level on day of HCG was–4995 pg/ml and progesterone – 2.7 nmol/l.HCG 5000 IU intramuscularly was given for oocyte maturation. Oocyte retrieval was done on day 14 of cycle. Total of 30 oocytes were retrieved. Right ovary was 7×7 cm and left ovary was 6×7 cm. She had moderate OHSS managed conservatively with i/v albumin, fluids and high-protein diet. In view of OHSS extended culture of embryos done and three blastocysts were cryopreserved. Five days after oocyte retrieval, she came with sudden onset of severe right-sided abdominal pain with increasing severity in next 2 hours. On examination, she had tachycardia+ 108/min, blood pressure – 100/70, respiratory rate – 20/mnt. She had moderate abdominal distension, severe right-sided guarding and tenderness and abdominal girth 102 cm. Abdominal ultrasound done suggested enlarged bilateral ovary-right 14×10 cm and left 8×9 cm with multiple cysts, fluid in paracolic gutter and Morrison's pouch. She was diagnosed with severe OHSS. Doppler study was not conclusive about torsion. Pain decreased with injection Tramadol. With further episode of severe abdominal pain after 2 hours of admission, decision was made for emergency laparoscopy.
The findings were - uterus normal in size, left ovary 9×8 cm, right ovary was enlarged and gangrenous and twisted twice over the pedicle 15×15 cm, with 500 ml of hemoperitoneum. Detorsion was not possible due to large size and friability of right ovary. Right salpingo-ooporectomy was done. Specimen was retrieved through endobag. Thorough peritoneal lavage was given and Port closure done []. Postoperative period was uneventful. Histopathology report was - ovarian tissue with hemorrhage and necrosis (hemorrhagic infarct).
Blastocyst transfer was done 3 months later, but she failed to conceive.


A 31-year-old female, with primary infertility since 3 years, bilateral polycystic ovary, previous 4 failed cycles of ovulation induction and timed intercourse came to our clinic for further treatment. Hysterosalpingogram was normal with bilateral tubal spill. Semen analysis of husband was normal. Antral follicle count was 20. Ovulation induction with clomiphene-100 mg×5 days and human menopausal gonadotrophin (total dose 300 IU i/m) was given. On day 13 there were two dominant follicles in left ovary. Injection human chorionic gonadotrophin (HCG) 5000 IU was given i/m and IUI was done twice at 24 and 48 hrs of injection. Ovulation confirmed on 2nd day IUI. Luteal phase support was given with vaginal progesterone for 14 days. Twelve days after IUI she came with severe left-sided abdominal pain with outside scan suggesting hemorrhagic cyst in left ovary. Pain was severe and not relieved with analgesic tramadol. On doing Doppler ultrasound enlarged left ovary compared with right was seen. Right ovary was 4.17×2.78cms and. Left ovary was 11.39×7 cm with two corpus luteal cyst. Color Doppler suggested enlarged cystic left ovary with heterogeneous reduced echo texture and scanty blood flow suggestive of torsion (partial torsion) and minimal ascitis [Figures and ].
Emergency laparoscopy was done []. Left ovary and tube was twisted thrice over the pedicle, enlarged and ischemic. Detorsion was done and the change of color of ischemic bluish tube and ovary was remarkable. Peritoneal lavage was given and port closure done. Owing to enlarged and friable ovary, fixation of pedicles of ovary could not be done. Postoperatively, 2 weeks after, repeat Doppler and USG suggested normal sized left ovary with normal blood flow and follicles. Unfortunately patient failed to conceive in this cycle. She was advised to avoid ovulation induction for next 3 months.