A 40-year-old adult male reported to the outpatient department with the chief complaint of a firm mass in the left cheek region. The duration of the swelling was approximately 6 months and the growth was continuous, slow, and painless. The patient was a farmer by profession and had no history of illness. On further probing, the patient gave the history of decay and infection in the left maxillary second molar a year ago. He underwent an unwarranted and irregular course of antibiotics and other drugs for almost 2–3 months prescribed by traditional practitioners in his village followed by extraction. The swelling appeared on the left cheek after approximately 2 months of extraction.
On examination, the swelling was firm, nontender, sessile, mobile and oval in shape. It measured 4 × 3 cm and was not fixed to the surrounding soft tissue. There was no paraesthesia over the distribution of infraorbital nerve and the overlying skin was unaffected. Intraorally, the left maxillary teeth were vital on pulp testing with no periapical radiolucency in dental radiographs.
An occipito-mental view (OMV) of skull showed generalized opacification of the left maxillary sinus. The computerized tomography (CT) scan showed evidence of well-defined, homogeneously enhancing soft tissue mass lesion seen in the left maxillary sinus [Figures and ]. No evidence of any necrosis or calcification was seen. The lesion was causing lytic destruction of anterior wall of the sinus and also the erosion of left floor of orbit. However, no intraorbital/intracranial extension was noted. Nasal septum was deviated mildly toward the right with relative inferior turbinate hypertrophy. Routine hematological evaluation showed mild leucocytosis with eosinophilia and an increased erythrocyte sedimentation rate (ESR) []. The chest x-ray was normal. A differential diagnosis of a chronic sinusitis, benign cyst or tumor, and maxillary antrum mycosis was made, and an incisional biopsy was performed under local anesthesia. The pathological tissue was creamish in color, firm in consistency, and hyalinized in texture [].
The histopathological picture showed diffuse areas of fibrosis and granulation tissue with patchy infiltrate of acute and chronic inflammatory cells. There were few septate organisms, giant cells, macrophages, epitheloid cells, and foci of hemorrhage. The report was suggestive of a fungal granuloma. Microbiological studies included KOH (potassium hydroxide) wet mount, LCB (Lactophenol Cotton Blue) preparation, and culture on Sabouraud's dextrose agar (SDA). Growth on SDA showed two types of colonies. One thin and white at first and then became gray tan in color and second golden colored, fast growing and powdery. Microscopic finding in the first culture showed chains of single-celled conidia produced in basipetal succession from annellide (basocatenate). Annellide were solitary with globose, truncate, smooth, and brown colored conidia []. Microscopic finding of second colony showed conidiophores bearing dense, verticillately arranged branches bearing phialides. Phialides were ellipsoidal tapering abruptly into long cylindrical neck. Conidia were spherical, smooth walled, yellowish, and in long divergent chains. Chlamydospores were present in short chains, brown, sub spherical and thick walled []. Based on all these findings, the isolates were identified as Paecilomyces and Scopulariopsis species.
The patient was put on oral itraconazole 200 mg twice daily for a week which resulted in a significant reduction of the extraoral swelling. A thorough sinus debridement and lavage was planned under general anesthesia. The sinus was approached by the standard Caldwell-Luc method because of the evident bony erosion of the anterior wall which had to be removed simultaneously []. A plastic drain was fixed oroantrally for irrigation after sinus clearance. In the postoperative phase, the patient was again put on oral itraconazole 200 mg twice daily and the sinus was irrigated with 1% acetic acid once daily for 7 days. The patient is been followed regularly for the last 1 year and a check OMV x-ray of skull confirmed repneumatization of the left maxillary sinus with complete resolution of clinical sign and symptoms.


A 21-year-old female presented with the chief complaint of an unesthetic facial and dental appearance [Figures –]. Her parents pointed that she was greatly dissatisfied by her appearance. She had a severe class III malocclusion with 2-mm anterior crossbite and 2-mm reverse overbite. The family had no history of skeletal class III malocclusion. When viewed from the front, the patient's face was oval. Lateral view and oblique view showed pronounced mandibular prognathism and midface deficiency with concave profile. Intraorally, the molar relationship was class III with a complete anterior crossbite [Figures –].
Cephalometric analysis [] showed maxillary deficiency and mandibular protrusion. The ANB angle was –4°, suggesting a skeletal class III malocclusion.
The skeletal problem was due to a combination of maxillary deficiency and mandibular prognathism [Figures –].
Bilateral sagittal split osteotomy with presurgical and postsurgical orthodontics was planned to achieve esthetically acceptable and functionally optimum occlusion with straight facial profile and minimum traumatic surgical exposure to the patient. Presurgical orthodontics in both the arches was done to relieve maxillary and mandibular crowding. Maxillary and mandibular arches were aligned upto 0.019 × 0.022 stainless steel wire with 0.022 slot edgewise appliances. The mandibular incisors were decompensated by proclining them in normal inclination and the archforms were coordinated [Figures –]. Mandibular third molars were extracted one month prior to the orthognathic surgery.
Before orthognathic surgery, the template was prepared using tracing paper. Skeletal profile of maxillae and mandible was traced. Profile tracing was then transferred using a carbon paper to a thin cardboard. This outline was then cut to produce cardboard template. From these templates, trial sections were made until desirable location and amount for osteotomy was found. The cut section of mandible was then fitted back to tracing in desired occlusal relation and the probable postsurgical changes were checked. In this case, 8 mm of mandibular setback brought class I molar relation with an esthetically pleasing profile. Hence, 8 mm setback of mandible was planned for osteotomy.
Retromolar area was exposed using modified third molar incision. Bilateral sagittal split osteotomy with short lingual split was carried out using surgical saws.[] Medial pterygoid muscle was detached after performing the split and 8 mm setback was achieved.
Fixation was done using four hole miniplates and screw on both sides. Intermaxillary elastics were placed on braces for 14 days in immediate postoperative phase. The patient was followed closely after the procedure and was guided to perform opening and lateral movements. Orthodontic treatment was resumed 6 weeks after surgery. One year later, fixed appliances were removed and a retention appliance was delivered [Figures –].
Patient's cooperation was excellent throughout the treatment. Cephalometric finding show the normal jaw relationship [, ].


A 45-year-old female was admitted with the complaint of generalized edema. She got about 6 kg of weight gain during recent one week. She had dizziness, dyspnea, anorexia, nausea and epigastric discomfort. She presented physical examination findings including neck vein engorgement, abdominal distension, hepatomegaly and pitting edema. She had history of syncope about one month ago, but brain magnetic resonance imaging (MRI) showed no abnormal finding. The laboratory findings showed leukocyte count of 11,500/mm3, hemoglobin level of 9.1 g/dL and platelet count of 284,000/mm3. Biochemical tests showed that the serum level of alanine aminotransferase was 30 IU/L, aspartate aminotransferase was 55 IU/L, and alkaline phosphatase elevated up to 398 IU/L. Serum albumin was 3.7 g/dL, total bilirubin was 1.2 mg/dL, and creatinine was 2.3 mg/dL. Alpha fetoprotein was less than 1 ng/mL. The results for hepatitis B surface antigen and anti-HBs were negative. Urine analysis showed albumin positive, and otherwise negative.
Renal disease was suspected and abdominal ultrasonography was done. Liver showed diffuse coarse echo and hypoechoic pattern with hepatomegaly (). Both kidneys showed also diffuse increased cortical echo suggesting renal parenchymal disease ().
Dynamic contrast enhanced CT was taken because of abnormal liver echo and size. On arterial phase, there was no enhancing lesion in liver (). On portal phase, size of liver increased, and especially left lobe showed bulging contoured enlargement and ill-defined hypoattenuation (). There were multiple ill-defined hypoattenuatted areas in right lobe. Spleen showed normal enhancement, but size increased. Both kidneys also showed multiple irregular decreased attenuation lesions (). Because liver showed decreased echo on ultrasonography, it was assumed that it was not fatty infiltration. These CT findings suggested diffuse infiltrative disease, so liver biopsy was done. Percutaneous fine-needle-aspiration biopsy was performed under ultrasound guidance, and a histological examination of the biopsy specimen showed amorphous material stained by Congo red stain. The deposit demonstrated characteristic positive birefringence with polarized light. Biopsy results led to a final diagnosis of extensive amyloidosis.
Additional examinations were done to evaluate the extent of amyloidosis involvement. 2D Doppler echocardiography showed enlarged both atrium and thickened left ventricle wall with normal vetnrcular contractility, which suggested infiltrative disease such as amyloidosis. Bone marrow aspiration and colonic mucosa biopsy showed also amyloidosis. Urine Immunoelectrophoresis showed abnormal zone of restriction in kappa light chain, suggesting Bence-Jones protein, free kappa type.
She was diagnosed as systemic amyloidosis, and chemotherapy was done. However, hepatic failure with hepatic encephalopathy was aggravated and she got discharged in a moribund state.


A 62-year old man who had a history of excessive alcohol consumption presented with massive hematemesis and melena. He had no medical history of other systemic disease. On arrival at emergency room, his systolic blood pressure was about 45 mmHg. Melena was seen on digital rectal examination and fresh blood was drained through L-tube during gastric irrigation. Complete blood counts showed hemoglobin of 3.6 g/dL and platelet count of 68,000/mm3. Other laboratory tests were followings; total bilirubin 0.9 mg/dL, albumin 1.5 g/dL, prothrombin time 18.8 seconds. Child-Pugh's classification was B. Hepatitis B surface antigen, anti-hepatitis C virus antibody and anti-human immunodeficiency virus antibody were all seronegative.
An emergent upper endoscopy was immediately performed following fluid resuscitation with blood transfusion. It revealed minimal esophageal varices without a stigmata of hemorrhage () and blood-stained gastric mucosae with no evidence of the cardiac or fundal varices (). Duodenal ulcer scar with marked luminal narrowing was noted on the bulb, however, the endoscope can pass through the narrow lumen. Dumbbell-shaped ruptured varices with an adhering whitish fibrin clot were seen on the postbulbar portion of the duodenum (). The duodenal varices were collapsed during air inflation and engorged with suction of the air. Abdominal computed tomography (CT) demonstrated liver cirrhosis with splenomegaly and moderate amount of ascites. Venous collaterals around the duodenum with extravasated contrast in the second and third portion were seen and the collaterals originated from main portal vein and drained throughout the inferior vena cava via the right renal vein ().
For hemostasis, we intravariceally injected 1.0 mL of cyanoacrylate into the duodenal varices. After EIS, the bulging varices were not collapsed during luminal aeration. The patient was getting hemodynamically stable and hemoglobin level was recovered up to 8.9 g/dL with transfusion of the packed red blood cells.
An endoscopy after 5 days showed a tubular-shaped venous bulging, by previous injection of cyanoacrylate, with a hard consistency and hyperemic covering mucosa. Endoscopic biopsy was done at the ulcer scar on the bulb, showing a chronic inflammation with granulation tissue microscopically. After 2 weeks, an endoscopy revealed post-EIS ulceration with yellow plaque and venous bulging.
Six months later, a follow-up endoscopy revealed much collapsed duodenal varices with small yellow plaque on the surface (). Abdominal CT disclosed the collapsed and much decreased portosystemic venous collaterals in the retroperitoneum of the paraduodenal space (). No bleeding has been detected by this time as of 9 months after the initial bleeding episode.


A 63-year-old male presented with swelling in the right inguinal region for the past 10 years with a sudden increase in size since two months. On examination, a single, firm three cm swelling was felt in the right inguinal region. No abnormality was detected in the left inguinal region. No other lesion such as an ulcer or swelling was noted in the right leg or thigh.
There was no history of fever, rigors and loss of weight or appetite in the recent past. Patient gave no history of exposure to tuberculosis.
On general examination, patient was well built and nourished. There was no pallor or generalized lymphadenopathy. No abnormality was detected on systemic examination. Base line blood investigations were found to be within normal limits. An ultrasonogram of the right inguinal mass showed circumscribed hypoechoic mass suggestive of a lymph node shadow. An ultrasound guided aspiration was done and the smears were submitted for cytological examination. Ultrasonogram of the abdomen was unremarkable.
Cytosmears examined were moderately cellular with dual cell population, composed predominantly of oval to polygonal cells with abundant cytoplasm and round bland nuclei resembling histiocytes []. These cells were arranged in loose clusters and as isolated forms. The background showed few discrete round cells with round nuclei and scanty cytoplasm suggestive of mature lymphocytes []. No precursor lymphoid cells were seen on cytosmears. In correlation with clinical and image findings, an impression of reactive lymph node was suggested on cytology. A clinical follow-up and excision was advised if clinically suspicious or lesion further increased in size.
An excision biopsy was performed which showed skin with a neoplasm located in lower dermis and subcutis. Tumor cells were arranged in nests with focal diffuse pattern. Individual cells appeared round to oval with abundant granular eosinophilic cytoplasm and centrally placed bland nuclei []. Focal collections of lymphocytes were also seen []. Tumor cells were seen surrounding the adnexal structures and nerve fibres. Immunohistochemistry showed S100 positivity in nucleus and cytoplasm ([] inset). Immunostains for CD68 and inhibin showed cytoplasmic positivity. A final diagnosis of granular cell tumor was given based on histology and immunoprofiling.


A 26-year-old man, presented with a one-week history of polymorphous eruptions over face, trunk, and extremities, associated with painful sores in the mouth as well as fever. He had no history of any systemic illness. The lesions started around the mouth with greasy scaling, which gradually progressed to involve scalp, neck, trunk, and extremities, including genitals.
On general physical examination, jaundice, and inguinal lymphadenopathy were present. His vital statistics were stable. Systemic examination was within normal limits, except hepatosplenomegaly.
On cutaneous examination, multiple skin colored to yellowish papules and pustules were present over the scalp, retro-auricular area, face, neck, trunk, extremities, and genitalia, which were associated with facial edema [Figures and ]. Diffuse greasy scaling was present over the beard area and retro-auricular region. Oral mucosa showed multiple vesicles, pustules, and hemorrhagic crusting of lips [].
Laboratory data showed: Hb-16.1 gm/dl; Total leukocyte count (TLC)-33,600/cu.mm; Neutrophil 54%, Lymphocyte 25%, Eosinophil 21%; total bilirubin-5.9 mg/dl; direct bilirubin-3.5 mg/dl; serum glutamic oxaloacetic transaminase (SGOT)-179 U/L; serum glutamic pyruvic transaminase (SGPT)-269 U/L; alkaline phosphatase (ALP)-167 U/L; renal function test- within normal limits. USG of the abdomen showed fatty changes in the liver with hepatosplenomegaly.
Biopsy from the skin, liver, and bone marrow showed dense infiltrate of histiocytes with longitudinal nuclear groove and numerous eosinophils and lymphocytes [Figures and ]. Occasional mitoses were also seen in the histiocytic infiltrate. Immunohistochemistry was positive for S-100 [].
With the history, clinical findings, and investigation, the diagnosis of multisystem LCH was made. The patient was started on chemotherapy under Medical Oncology Department. He was given Vinblastine and Etoposide weekly for 6 weeks with daily Prednisolone, followed by maintenance therapy of 12 cycles with same drugs at intervals of 3 weeks and daily 6-Mercaptopurine. The patient responded well and is on continuous follow-up till date.


A 41-year-old male patient with persistent hiccups that started suddenly and lasted for 10 days was referred to our department. His past medical history was unremarkable. The patient had no history of repeated hiccupping episodes. Chest X-ray, abdominal ultrasound, gastroscopy, and electrolyte analysis showed no abnormalities. General neurological and otorhinolaryngological examinations also showed no abnormal findings, except for hiccups with a frequency of 15-20 per minute, which continued during the patient's sleep. The hiccups did not respond to conservative treatment methods, such as drinking cold water, breath holding, and pharynx stimulation. Cisapride 30 mg, omeprazole 40 mg, and baclofen 30 mg were administered daily for 9 days, but these medications had no effect. Gabapentine 300 mg daily was added, but the patient suffered from dizziness and refused the medication. The patient's hiccup contractions were so severe that he complained of upper abdominal discomfort. Therefore, left phrenic nerve block with 4 ml of 1% lidocaine was performed using the neurostimulation method []. The hiccups stopped after the phrenic nerve block, but they recurred 40 minutes later. We repeated the phrenic nerve block, but the hiccups reappeared 45 minutes later. Finally, we elected to use positive pressure ventilation therapy to treat the hiccups. After 8 hours of NPO status, propofol 2.0 mg/kg was administered intravenously. After loss of the eyelash reflex was confirmed, succinylcholine 70 mg was administered, and ventilation was maintained via a facial mask with an air/oxygen mixture containing 50% oxygen and no inhalational anesthetic. A tidal volume of 650 ml was delivered at a rate of 8 breaths per minute, and peak inspiratory pressure (PIP) was 25 cm H2O. Two minutes after positive pressure ventilation was begun, the patient began spontaneous ventilation. Ten minutes after injection of propofol, the patient regained full consciousness, and the hiccups did not reappear. The patient discontinued all oral medications, and at the 10 week follow-up, no further episodes of hiccups had occurred.


A 30-year-old woman presents with progressive weakness of both legs for last 10 days. She has difficulty in walking, climbing stairs, and standing up from a sitting position. She has developed decreased sensation up to the level of umbilicus and urgency of micturition. All muscles of both lower limbs have decreased tone and power (grades 3 to 4/5) with flexors weaker than extensors and absence of involuntary movements. There is a graded (distal more than proximal) sensory loss involving all modalities of sensation with a sensory level of D10. All deep tendon reflexes are elicitable and plantars are extensor. Her abdominal reflexes are all absent and there is no spinal tenderness. An MRI of the dorsal spine rules out a diagnosis of dorsal transverse myelopathy. Subsequent MRI of the brain reveals seven white matter T2 hyperintensities of variable size and location. There is no enhancement with gadolinium and antimyelin antibodies are positive. A diagnosis is made of “demyelinating disease of the central nervous system” and intravenous methylprednisolone is prescribed. The patient is informed that her symptoms and test results will be monitored over time. She would like to know what her chances are of developing multiple sclerosis (MS) in the next few years.
As evident from the clinical scenario, the primary question or concern of the patient pertains to the domain of prognosis. That is, among patients (problem) with the described symptoms (intervention/exposure) and those who do not have these symptoms (control/no exposure), the question pertains to the incidence of MS (outcome) over time. It is obvious that in this case, an RCT would be impossible to conduct. Since the presence of symptoms cannot be manipulated by an investigator, an experimental design can be ruled out. In this case, investigators cannot randomize patients according to certain symptoms which brings observational study designs into consideration. Another possible observational design is the cross-sectional study, which examines both exposure and outcome at the same time in the same group of people. This is clearly not a relevant study design for this scenario since the aim is to measure the incidence of the outcome between two groups over time. The next possibility is a case-control design. In the case-control study design, participants could be separated into those with MS and those without and then go retrospectively to assess the differences in terms of presence or absence of risk factors between the two groups. While this study design is feasible, there are two limitations. First, because the primary interest is associated with several risk factors (i.e., composite outcome), this study could be subject to recall bias.[] Second, incidence cannot be calculated in a case-control study as patients cannot be followed retrospectively. This leaves the cohort study as the best study design to answer the clinical research question on prognosis. In this scenario, subjects would be separated into those that have the described symptoms and those that do not. They would then be followed over time and the incidence of MS in the exposed group would be compared to the incidence of MS in the unexposed group. From this data, the relative risk would be calculated.


A 37-year-old pregnant woman comes to the clinic for a routine checkup. This is her second pregnancy. She had her first child when she was 35 years old and had an amniocentesis at 18 weeks to test for Down syndrome. The amniocentesis was negative. She is currently 8 weeks pregnant and would like to know as soon as possible of any abnormalities. An ultrasound can be done in the first trimester for diagnosing Down syndrome, but it is unknown if the results are as reliable as the conventional test of amniocentesis.
This scenario represents the domain of diagnostic accuracy. The question can be formulated as follows: Among pregnant women over the age of 35 (patient/problem), what is the accuracy of ultrasound (intervention/experimental test) compared to amniocentesis (control/conventional test) in detecting Down syndrome in fetus (outcome)? Again, the RCT can be eliminated because, in order to determine diagnostic accuracy in an unbiased manner, both tests need to be administered to the same subset of the population. In the case-control study, subjects with the outcome of interest are selected and then matched with healthy controls. Since we do not know whether or not the outcome of Down syndrome is present, a case-control study cannot be used. The cohort study group's subjects are selected based on exposure status followed over time and the incidence of the outcome is calculated. In this clinical scenario, no exposure is described and we are not interested in calculating the incidence of Down syndrome. Accordingly, the characteristics of the cross-sectional study make it the best choice to answer this clinical research question. A cross-sectional study would allow two observations from the same group to be determined. In this case, a sample of pregnant women over the age of 35 would be given both an ultrasound and amniocentesis. The results of the tests would be recorded and the number of true positives and negatives versus the false positives and negatives would be calculated along with positive and negative predictive values.


A 28-year-old female had experienced intermittent abdominal pain for 3 months and visited the Asan Medical Center, Seoul, South Korea, for further evaluation and treatment. An abdominal-pelvic computed tomography scan taken in January 2010 at the Asan Medical Center revealed a huge mass involving most of the lower abdomen and pelvic cavity with multiple septations and areas of enhancement (). The provisional diagnosis on the basis of the image findings was either borderline or malignant ovarian neoplasm. Neither ascites nor metastatic lymphadenopathy was found.
The patient had a history of radical nephrectomy for Wilms' tumor of the right kidney 25 years previously, at the age of 3 years, in April 1985 at an outside hospital. After surgery, she had been treated with intravenous injection of dactinomycin 25 mcg for 5 days and vincristine 1 mg on the 7th day after the operation. Since then, she had been healthy with no evidence of recurrence or metastasis until she noticed abdominal pain in September 2009.
Under the clinical diagnosis of an ovarian tumor, the pelvic mass was resected in February 2010. During surgery, the mass was noted to occupy the entire lower abdominal cavity without involving both adnexa. Intraoperative consultation was made and the frozen section diagnosis was malignant gastrointestinal stromal tumor or other malignant mesenchymal tumor such as leiomyosarcoma with pending evaluation of permanent sections.
The original slides of the Wilms' tumor, resected 25 years ago, could not be reviewed because the slides were not available in the file of the outside hospital. However, the outside pathology report stated that the size of the tumor was 12 cm and it was located at the right kidney. The tumor was abutting the head of the pancreas, and in the operation record it was written that the tumor was not completely excised. Microscopically, the tumor was shown to be a classic Wilms' tumor with predominant epithelial tubular elements and minor blastemal and mesenchymal components. No anaplasia or lymphovascular invasion was recorded, and the mitotic count was stated as scant. The final diagnosis of the nephrectomy specimen was Wilms' tumor, stage III.
Grossly, the resected tumor in February 2010 was a 30-cm, previously ruptured, solid and cystic mass. On the cut surface, the tumor was heterogeneous, pinkish tan to yellow, and had both solid and friable areas. Multifocal hemorrhagic, cystic, and necrotic changes were also noted (). Microscopically, the tumor was composed of three different cell types, epithelial, mesenchymal, and blastemal components (). Areas of tumor necrosis were seen (). The mesenchymal tumor cells were short, spindle-shaped to round cells with scanty cytoplasm in a myxoid background (). The epithelial cell component formed tumor cell nests with glandular architecture (). The majority of the tumor compartment was an undifferentiated blastemal cell component. Mitoses were frequently observed up to 30/10 HPFs, particularly in the blastemal component. Hyperchromatism was also observed, but significant pleomorphism was not obvious (). Immunohistochemical staining was done and the results were as follows: focal positive for cytokeratin in the epithelial component but negative in the blastemal and mesenchymal components (). The Ki-67 labeling index was positive in about 50% of the tumor cells (), and p53 staining was positive in about one third of the tumor cells in all three elements. The immunohistochemical staining for WT-1, S-100 protein, SMA, desmin, myogenin, myoglobin, CD117, and CD34 was negative in all three components.
On the basis of the histologic findings in conjunction with the immunohistochemical findings, the tumor was diagnosed as a recurrent classic Wilms' tumor without anaplastic changes.


A 27-year-old male agricultural laborer from Tamil Nadu, India, presented with alleged consumption of 50 ml of an unknown poison as an act of deliberate self-harm following a quarrel with his spouse. Following consumption he had several episodes of vomiting. Subsequently, he developed twitching movements of his limbs, urinary incontinence, and altered sensorium. He was brought to the Emergency Department 4 hours after consumption. On examination he was found to be stuporous (GCS 11/15). He had a systolic blood pressure of 80 mm of Hg, a heart rate of 120/min, and a respiratory rate of 32/min. He was documented to have hypoxia (pulse oximetry saturation of 70%). The pupillary size was 3 mm bilaterally and reacting to light. He was noted to have occasional fasciculations. Systemic examination was otherwise unremarkable. He was intubated and mechanically ventilated in view of altered sensorium and hypoxia. A gastric lavage was administered followed by a single dose of activated charcoal instilled through the nasogastric tube. The provisional diagnosis made in the emergency department was of an organophosphate poisoning with acute cholinergic syndrome. The chest X-ray was normal and ECG revealed sinus tachycardia. Basic blood investigations including serum electrolytes, creatinine, liver function tests, and complete blood counts were normal. The serum pseudo-cholinesterase level was normal (3839 U/l). The patient was admitted into the semi-intensive care unit and managed conservatively with fluids and cardiac monitoring. The plan was to use atropine if the patient developed bradycardia or miosis and to repeat serum pseudo-cholinesterase levels after 24 hours.
Twenty-four hours after presentation, the relatives were able to provide the name and container of the poison which was Cartap hydrochloride 4 gm/50% SP. At 24 hours the patient had shown improvement in sensorium and ventilatory supports were rapidly reduced. He was subsequently extubated. Due to the fact that there is insufficient data regarding effective timing of the antidote, and the significant clinical recovery of our patient, the antidote was not administered. The patient was discharged after counseling 3 days after admission with no complications.


A 72-year-old woman presented to the consulting room with the complaint of proptosis in the left eye. The patient referred that proptosis was initially observed several years ago [].
Visual acuity examination was performed on both eyes (Snellen optotype at 6 m distance) and yielded 20/25 with correction of +0.50 D sphere in the right eye and 20/25 with correction of +1.75 D sphere in the left eye. Color perception found to be normal.
Anterior segment examination for both eyes did not show any severe pathology. Fundoscopic examination did not reveal edema or atrophy of the optic disc.
Standard visual field examination was performed in the patient twice and showed peripheral scotoma in the left eye [].
Magnetic resonance imaging (MRI) study of the orbital cavities was ordered. In the MRI protocol T2, T2 with fat saturation, T1, T1 with fat saturation sequences, pre- and post-gadolinium injection on three different planes (axial, sagittal, and coronal) were performed. A fusiform mass of 3.4 cm was demonstrated in the left orbit. The mass appeared as hypointense to brain and optic nerve tissue on T1-weighted images and slightly hyperintense on T2-weighted images. There was no intracranial extension of the lesion or any sign of neighboring structure invasion [].
The benign noninvading growth pattern and the relatively steady clinical appearance corroborated the possibility of meningioma, and as a consequence diagnosis of optic nerve sheath meningioma was suggested.
After completing the examinations, the therapeutic strategy suggested to the patient by ophthalmologists and neurosurgeons included a systematic follow-up every 6 months in view of the fact that visual performance remained stable for a certain number of years.


A 47-year-old man with a history of smoking and being overweight presented with an acute anterior ST-segment elevation myocardial infarction. He was more than three hours away from the nearest catheterization laboratory and thus received systemic thrombolysis associated with a loading dose of clopidogrel and aspirin. The treatment was successful during the transfer period. The deferred coronary angiography showed a significant stenosis with thrombus of the proximal left anterior descending coronary artery with Thrombolysis in Myocardial Infarction 3 flow and a tight stenosis of the proximal right coronary artery. Moreover, the coronary angiography highlighted substantial coronary-pulmonary fistulas involving all three major coronary arteries (). The patient received curative unfractionated heparin for 8 days with the standard pharmacological treatment. Echocardiography showed septal akinesis with a left ventricular ejection fraction of 50%. A 99mTc-methoxyisobutylisonitrile single photon emission computerized tomography scintigraphy was performed at day 14 and showed anterior myocardial infarction with inferior myocardial ischemia. The patient was referred for surgery, but he refused intervention. He was discharged under medical treatment.


A 29-year-old unmarried female presented with complaints of bilateral breast swelling for past 6-month duration. There was no associated history of pain, nipple discharge or swelling in axilla. There was no positive family history. During October 2006 she had a similar swelling in the right breast, FNAC of which showed phylloides tumor. She underwent wide local excision in December 2006, biopsy of which showed angiosarcoma of the breast with free margins. She received no further treatment before she reached our hospital. The swelling in the right breast had recurred with another new swelling in left breast. FNAC from both breast swellings showed spindle cell neoplasm. Her metastatic work-up was negative. She underwent bilateral mastectomy with axillary dissection on March 2008. Histopathology report of surgical specimen showed low-grade angiosarcoma of the left breast [] and high-grade angiosarcoma of the right breast []. Immunohistochemistry of right breast was done which showed cells positive for factor VIII. On microscopic examination, histology of low grade showed irregularly dilated vascular channels lined by flat endothelial cells and histology of high grade showed proliferated polygonal or spindle-shaped, pleomorphic endothelial cells with anastomosing vascular channels. None of the bilateral axillary node was positive for malignancy. In view of the histopathology, she was advised further adjuvant therapy with local irradiation over right breast. During evaluation before radiotherapy, CT scan thorax revealed right hilar and right mediastinal lymph. CT-guided FNAC was done from mediastinal node, which showed metastatic deposits. Radiotherapy was given to right breast and mediastinum. Post-radiotherapy CT scan thorax showed much regression in size of mediastinal nodes. The patient was given adjuvant chemotherapy with anthracycline/ifosfamide-based regime in dose of ifosfamide 2 g/m2 IV (days1-5) and doxorubicin 25 mg/m2 IV (days 1-3) every 3 weeks for six cycles. Now this patient is under close follow-up.


A 17-year-old female patient presented with increasing abdominal discomfort in the left upper quadrant of the abdomen and abdominal distention due to ascites. A paracentesis was performed and fluid was submitted for chemical and cytological evaluation. Laboratory evaluation of the peritoneal fluid showed glucose of 85 mg/dl, total protein of 4.1 g/dl, and fluid albumin of 2.2 g/dl. Twenty-one months prior to the current presentation, the patient was admitted to the hospital with increasing perirectal pain and an enlarging mass, which on a computed tomography (CT) scan was seen to be located between the posterior margin of the anus and the gluteal crease. The CT scan also revealed bilateral inguinal, iliac, and subcarinal lymphadenopathy, along with four masses in her right lung. An excisional lymph node biopsy was performed and a diagnosis of alveolar rhabdomyosarcoma was rendered, based on the cytomorphology and immunophenotype. G-banded karyotyping and fluorescence in situ hybridization (FISH) studies revealed a FOXO1 locus rearrangement of t(2;13)(q36,q14) in the background of a near-tetraploid karyotype, with multiple other abnormalities.
Wright-stained cytospin preparations, Papanicolaou-stained Surepath® preparations, and hematoxlin and eosin-stained cellblock preparations were made from the peritoneal fluid. All preparations showed abundant mesothelial cells, lymphocytes, and occasional neutrophils. Rare, tight, three-dimensional clusters of 10 to 20 cells were present in the Papanicolaou-stained preparation. All preparations showed small-to-very large single cells, with single or multiple hyperchromatic nuclei, and high nucleocytoplasmic ratios. No mitoses or necrosis was seen. The neoplastic cells were typically seen intimately associated with mesothelial cells and inflammatory cells, forming mixed cell clusters. This dyshesive, polymorphous population of cells was most easily appreciated in the Wright-stained cytospin preparations [], where the cells were rounded-to-ovoid, and ranged from small (mean, 18 μm) to large (mean, 22 μm) in size. The nuclei of the larger cells were ovoid and measured 18 × 13 μm on an average. Some of the cells showed prominent nucleoli, which were frequently irregular in shape, and ranged in size from 3.5 to 6 μm. In the same Wright-stained preparation, the mesothelial cells were larger (mean, 25 × 22 μm), but their nuclei (mean, 12 μm in diameter) and nucleoli (mean, 3 μm) were smaller than those of the neoplastic cells. The nucleocytoplasmic ratio of mesothelial cells was also lower compared to that of the tumor cells. The cytoplasm of the tumor cells ranged from scant rims of bluish, dense, non-vacuolated cytoplasm to a more ample, eccentric cytoplasm, with perinuclear metachromatic pink-staining areas. The tumor cells were slightly smaller in the Papanicolaou-stained preparation, where the nuclear details were better appreciated. The nuclei were coarsely hyperchromatic with thick chromatinic rims and had single to multiple irregularly shaped nucleoli. The cytoplasm appeared dense, glassy, and cyanophilic, occasionally with pink hues. Multilobated and multinucleated cells were easily identified, sometimes with ‘wreath-like’ eccentrically located nuclei. Large, pleomorphic cells, measuring up to 30 μm, were also present
[]. Some nuclei had irregular, ‘nose-like’ protrusions and some had complex, convoluted ‘embryo-like’ shapes. Occasional pairs of tumor cells separated by apparent ‘windows’ were present and mimicked the mesothelial cells.
Cell-block sections showed pleomorphic tumor cells, with high nucleocytoplasmic ratio, coarse hyperchromatic nuclei, and prominent nucleoli [Figures and ]. Occasional cells with plasmacytoid appearance and eosinophilic cytoplasm were present. No strap cells or classic rhabdomyoblasts were seen.
The cytological features of the peritoneal fluid were initially interpreted as atypical, favoring the reactive mesothelial cells; however, on review of the case by the cytopathologist, they were considered malignant, and immunoperoxidase stains were performed to confirm the diagnosis. Immunoperoxidase stains performed on the cellblock preparation showed that the neoplastic cells were positive for muscle-specific actin and myogenin [], but were negative for cytokeratin AE1/AE3 []. Both the reactive mesothelial cells and neoplastic cells were strongly positive for desmin. A diagnosis of alveolar rhabdomyosarcoma involving the peritoneal fluid was made based on the cytomorphology and immunoperoxidase stain results.


A 46-year-old black female presented with clinical lesions consistent with DLE. She denied systemic symptoms of arthralgia, arthropathy, myalgia, fatigue, Raynaud's phenomenon and gastrointestinal complaints. Lesions were present on the head, trunk, and upper extremities, and presented as reddish-purple, atrophic plaques. Laboratory data showed a normal complete blood count (CBC) with differential analysis, and a normal erythrocyte sedimentation rate. Antiphospholipid antibody testing was negative ; serum electrolytes, blood urea nitrogen, creatinine, and liver function tests, as well as urinalysis and chest radiographs were within normal limits. The antinuclear antibody (ANA) titer was normal. Specific ANA screening yielded negative results for anti-Smith, anti-double stranded DNA (dsDNA), and anti-histone antibodies. Tests for anti ribonuclease-sensitive antigen (RNAse), extractable nuclear antigen (ENA), small nuclear antigen) (sn), ribonucleoproteins (RNP), and U1, and U2 complexes were negative, as was testing for anti-SS-A (anti-Ro) and anti-SS-B (anti-La). Levels of Complement C3 and C4 were normal. Biopsies of the lesions were performed, with subsequent Hematoxylin and Eosin (H&E) staining, multicolor direct immunofluorescence (DIF) and immunohistochemistry (IHC) studies.
H & E: Routine H&E staining was performed, and examination of the tissue sections revealed mild epidermal atrophy with focal follicular plugging. A mild interface infiltrate of lymphocytes and histiocytes was noted. Within the dermis, a superficial and deep, perivascular and periadnexal infiltrate of lymphocytes, histiocytes, and plasma cells was observed. Neutrophils and eosinophils were rare. Increased dermal mucin was not found; disorganization of the dermis was observed on H & E (Figs. and ).
DIF: Skin cryosections were prepared, and incubated with multiple fluorescein isothiocyanate (FITC)-conjugated secondary antibodies. The secondary antibodies were of rabbit origin and included: a) anti-human IgG (γ chain), b) anti-human IgA (α chains), c) anti-human IgM (μ-chain), d) anti-human fibrinogen, and e) anti-human albumin (all used at 1:20 to 1:60 dilutions and obtained from Dako (Carpinteria, California, USA). We also utilized secondary antibodies of goat origin, including: a) anti-human IgE antiserum (Vector Laboratories, Bridgeport, New Jersey, USA) and b) anti-human C1q (Southern Biotech, Birmingham, Alabama, USA). Finally, monoclonal anti-human IgG4 FITC conjugated antibody was used to test for possible intercellular staining between keratinocytes (Sigma-Aldrich Saint Louis, Missouri, USA). The slides were then counterstained with 4’,6-diamidino-2-phenylindole (Dapi) (Pierce, Rockford, Illinois, USA) or TO-PRO®-3/DNA combined with (ANA Hoesch H1399. We also counterstained with Hoechst 33342 (Invitrogen Corporation, Carlsbad, California USA). Slides were then washed, coverslipped, and dried overnight at 4°C. In Figures and , we demonstrate some of the most prominent H&E and DIF patterns observed, as well as the improvement of the quality and power of DIF staining by the use of simultaneous multicolor fluorescence and counterstaining of cell nuclei. The DIF results were classified as follows: (0, negative; +, weak positive; ++ and +++, positive; and ++++, maximum positivity). We found 1) deposits of IgG, in a granular pattern at the basement membrane zone (BMZ) of the dermal/epidermal junction, as well as at the BMZ of the sebaceous glands (+++); 2) deposits of IgA surrounding the superficial dermal blood vessels (++); 3) deposits of IgM in a granular pattern at the BMZ of the skin (+++); 4) deposits of complement C3 in a granular pattern at the BMZ of the skin and of the sebaceous glands (+++) and 5) deposits of fibrinogen, in a granular pattern at the BMZ of the skin (++).
IHC: To study correlation with our vimentin DIF data, we performed IHC utilizing a Dako dual endogenous peroxidase blockage system, with the addition of an Envision dual link. We utilized Dako technical instructions for staining. We applied 3, 3 diaminobenzidine as an IHC chromogen, and counterstained with hematoxylin. highlights our results, including the compartmentalization of vimentin around several skin appendices where the DIF autoreactivty was detected. The degree of compartmentalization of vimentin by IHC parallels the deposition of immunoglobulins, complement and other immunosurfactants (such as fibrinogen) by DIF.


A 27-year-old unmarried woman, not a known diabetic, presented to our outpatient department with osmotic symptoms for 1 month and several episodes of vomiting and fever with mild dyspnea for 3 days.
On examination, she was found to have a body mass index (BMI) of 14.22 kg/m2, pulse of 115/min, and blood pressure (BP) of 110/70 mmHg (supine) and 80/50 (erect). Dehydration was marked and she had acidotic breath. Pallor was present, edema was absent, and jugular venous pressure (JVP) was normal. Gastrointestinal system examination was normal except for mild splenomegaly. Examination of other systems did not reveal any abnormality.
Initial laboratory data showed fasting blood sugar 494 mg/dl, postprandial blood sugar 568 mg/dl, glycated hemoglobin (HbA1c) 18.7%, urinary ketones strongly positive, serum creatinine 0.6 mg/dl; arterial blood gas showed acidosis (pH 7.29, pCO2 33, pO2 90, HCO3 15 mEq/l). Total leukocyte count was 21,000/mm3 with a left shift, Hb was 9 g/dl, and erythrocyte sedimentation rate (ESR) was 65 mm at the end of first hour. Chest X-ray showed a consolidation in the left lung. Anti-glutamic acid decarboxylase (anti-GAD) antibody was subsequently found to be positive.
She was diagnosed to be a case of Type 1 diabetes mellitus, left lung consolidation, and sepsis with diabetic ketoacidosis. She was started on the standard protocol for diabetic ketoacidosis along with antibiotics. After the initial treatment, her condition stabilized gradually. However, from the 5th day of admission, she started complaining of abdominal pain and distension of abdomen along with swelling of the feet. Physical examination now showed development of hepatomegaly, increase in splenomegaly, ascites, and pedal edema. Investigation revealed a deranged liver function: total protein 7.1 g/dl, serum albumin 2.7 g/dl, total bilirubin 1.1 mg/dl (indirect 0.8), aspartate transaminase (AST) 147 U/l, alanine transaminase (ALT) 99 U/l, serum alkaline phosphatase 1431 U/l, and gamma-glutamyl transpeptidase (GGT) 1075 U/l. Workup included negative hepatitis B and C viral serologies. Echocardiography, 24-hour urinary albumin, and thyroid stimulating hormone (TSH) were normal.
Contrast-enhanced computed tomography (CECT) scan of the abdomen showed nodular liver surface with attenuated hepatic veins and proximal inferior vena cava (intrahepatic and suprahepatic regions) with intrahepatic collaterals, dilated portal vein, splenic vein, dilated mesenteric venous channels, splenomegaly, and moderate ascites, indicating hepatic parenchymal changes with portal hypertension and compromised hepatic venous outflow suggestive of Budd–Chiari syndrome.
Gastroenterology consultation was taken, and as advised, the following investigations were done. Antinuclear antibody (ANA) and dsDNA were negative. Platelet count, prothrombin time (PT) and activated partial thromboplastin time (APTT) were normal. Antithrombin III, α-fetoprotein, protein C and protein S, and cardiolipin levels were normal. Analysis of ascitic fluid showed total protein 1.7 g/dl, glucose 95 mg/dl, lactate dehydrogenase (LDH) 145 U/l, WBC 46/mm3, and serum-ascites albumin gradient of 1.8 g. Myeloproliferative disease and hyperhomocysteinemia were ruled out. Upper gastrointestinal (GI) endoscopy was normal.
She was started on anticoagulation, sodium restriction, diuretic therapy, and paracentesis. She was advised transjugular intrahepatic portosystemic shunt, which she refused. There was resolution of the edema and ascites with medical management. She was discharged with insulin, warfarin, and diuretics, and advised to attend endocrinology and gastroenterology OPD regularly. At 6 months follow-up, there was no edema or ascites, but no alteration in the size of thrombus.


A 49-year-old female patient visited the department of rehabilitation 1 month after suffering a cerebellar hemorrhage () affecting more than two thirds of her anterior ventral of pons on both sides, midbrain, and medullar right of cerebellum. The upper and lower extremity strengths were MRC (medical research council) right 2 and left 0-1, and there was ataxia on her right side. The patient was able to communicate by blinking her eyes, had limb and facial paralysis, dysarthria, dysphagia, sweating disorder, and had a Levin tube and the urinary catheter installed. She had a body temperature of 36.5℃, pulse of 82 beats/min, and average blood pressure of 140/80 mmHg, which was not well controlled. Before visiting the hospital, the patient hadn't had bowel movement for more than 7 days and she complained of nausea, vomiting, and abdominal pain. She was experiencing abdominal distension, but abdominal tenderness and rebound tenderness were not observed, and had low bowel sounds. In a plain radiograph, the colon and small intestine were filled with gas and feces, and the ascending colon's diameter was increased to 95 mm (). On computed tomography, there were no tumors or vascular abnormalities that would cause mechanical obstruction in the intestinal tract, and there wasn't any endoscopic finding in the stomach. The blood tests showed hemoglobin (11.8 g/dl), leukocytes (9,500/mm3), ESR (54), and CRP (0.88). In biochemical tests including an electrolyte-specific test; there were no specific findings. A Clostridium difficile toxin test was negative. Although fasting, fluid therapy, bowel stimulant, intestinal massage, and glycerin enema using an installed rectal tube were conducted, none of these procedures were effective. Starting on day 9 after admission, glycerin enema was conducted using a 170 cm extended-length rectal tube. The extended-length rectal tube was inserted with 4 French 45 cm rubber intestinal tubes overlapped 5 cm with one another and wound with Tegaderm™ (Healthcare, 3M). At 1-2 day intervals, enemas were repeatedly administered for 2 weeks. However, after 2 weeks, enemas were done less often and yet the oral dietary food intake was increased, hence, the symptoms worsened. Erythromycin (500 mg) was orally administered 4 times daily and the patient showed rapid improvement of symptoms, but due to the increased oral dietary food intake, nausea, vomiting, and gas distension were observed. To ease the condition, metoclopramide (10 mg) was intravenously injected 2 times daily and erythromycin was changed to bethanechol and administered. Bethanechol was administered at a starting dose of 5 mg, and then increased to 3 times daily or a total of more than 30 mg, along with a 1-2 times weekly enemas using the extended-length rectal tube, and regular stool movement was possible. After enemas, the extended-length rectal tube was forced out along with feces, and regular rectal tubes were then used. Common side effects of bethanechol such as sweating, flushing, and hypotension were not observed, and the dosage was reduced to 35 mg and maintained after observing drooling of saliva at a dose of 40 mg. After 1 month of treatments starting from the 75th day after the first admission, the patient's symptoms did not worsen even without the use of rectal tubes. At 3 months after the first admission, stool movement was possible by using bisacodyl (20 mg) once every 2-3 days. After 9 months, when the patient was re-admitted for rehabilitation treatment, her right-side muscle strength was grade 3. And there was an improvement of dysphagia sufficient for her to swallow porridge. She was able to urinate by herself and there was frequent urination. When her amount of exercise was reduced, although bowel obstruction symptoms such as gas bloating were observed during abdominal radiography, they were easily improved after a daylong use of rectal tubes and treatment with magnesium hydroxide, mosapride, and bisacodyl suppositories.


A 56-year-old female presented with best corrected visual acuity (BCVA) of 20/120 and nuclear sclerosis (nuclear opacity 3, nuclear color 2 using Lens Opacification Classification System III)[] in the left eye (LE). She was posted for PE with foldable PC IOL implantation (LE). Preoperative endothelial cell count was 2653/mm2.
The surgery was performed using proparacaine drops (Paracain Ophthalmic Solution 0.5%, Sunways Pvt. Ltd., Mumbai, India) through a 2.8-mm temporal clear corneal incision. All surgical steps including PE were uneventful. A foldable IOL of +22.5 D of the Sensar AR40e variety [Abbott Medical Optics Inc (AMO), 1700 E. St. Andrew Place, Santa Ana, CA 92705 USA] was loaded into the Emerald C Cartridge (Lot CH00841 of AMO Inc., USA) by the first assistant outside the field of the operating microscope to save the surgical time. The cartridge was inserted in the injector and the loaded injector was handed to the surgeon. The tip of the cartridge was just inserted snugly by slight rotatory motion into the anterior chamber through the 2.8-mm incision. However, while removing the injector system, it was noticed that there was a gross downward beaking of the bevelled anterior end of the cartridge [] which had earlier passed unnoticed. Immediately, the incision site was inspected under the operating microscope and a Descemet's tear was detected with a rolled out flap of about 2 mm in length. The viscoelastic was meticulously washed out and the main incision wound and the two side port entries were carefully hydrated and an air bubble was injected into the anterior chamber.
On the first postoperative day, the patient's LE had an uncorrected VA of 20/60 with a small rolled out Descemet's flap and adjacent Descemet's striae at the site of the main clear corneal incision (temporally) [] with mild corneal edema. The edema persisted at 7 days post-op with mild resolution. However, the rolled out flap was not evident any more. A corneal opacity remained at the deeper corneal layers with mild surrounding edema, even at the time of last check-up at 4 weeks post-op, with a BCVA of 20/30 []. The corneal endothelial cell count 4 weeks after surgery was 2332/mm2.


A 69-year-old male visited the hospital for diarrhea and abdominal discomfort lasting about 2 weeks. He did not have a medical history of hypertension or diabetes mellitus, but had a past medical history of tuberculosis diagnosis and complete recovery 1 year before the visit. He did not have any history of operation in the abdomen nor a symptom during defecation. He did not smoke or drink alcohol either. Diarrhea and abdominal discomfort started 2 weeks before the visit, without other specific symptoms. Vital signs at admission were measured as blood pressure 120/70 mm Hg, pulse rate 68/min, respiration rate 20/min, and body temperature 36.5℃. Not pale conjunctiva and anicteric sclera was found at physical examination. There was no splenomegaly or palpable mass from abdominal palpation, either. There was a mild tenderness at the left lower quadrant with indeterminable rebound tenderness. CBC result showed WBC 3,220/mm3, hemoglobin 13.0 g/dL, hematocrit 38.0%, platelet 263,000/mm3, PT 12.7 seconds (INR 1.09), and aPTT 29.8 seconds. The blood chemistry was analyzed as total bilirubin 0.75 mg/dL, total protein 7.5 g/dL, albumin 4.1 g/dL, AST 25 IU/L, ALT 19 IU/L, ALP 66 IU/L, γ-GTP 42 IU/L, triglyceride 72 mg/dL, cholesterol 149 mg/dL, BUN 28 mg/dL, and creatinine 0.9 mg/dL. Electrolyte was measured as Na 135 mmol/dL, K 3.9 mmol/dL, and Cl 101 mmol/dL. Urinalysis was normal but the stool occult blood test was weakly positive. Colonoscopy was performed for the suspected infectious colitis, where a huge mass was found protruding to the middle of lumen of the sigmoid colon toward the anus. This mass was migrated toward the proximal by the air infusion and was cleared through the colonoscopy. Ileocecal lesion was found to have an edematous and erythematous mucosal change, and a cystic submucosal mass was found at the appendiceal orifice from histological examination after the colonoscopy (). An abdomen-pelvis computed tomography was performed, then, for it was suspected of an intussusception reduced by colonoscopy, and a wall enhancing cystic lesion was found at the appendiceal base (). When a follow-up colonoscopy was performed on day 4, ileocecal mucosal lesion appeared to have improved (). A laparoscopic appendectomy was performed on day 9, and a histologic examination shows appendiceal cyst is lined by columnar mucinogenic cells and inflamed wall (). The patient was discharged after the operation without particular complications.


A 3-year-old boy with an unremarkable previous medical history was referred to our department in January 2004 with rapidly progressive weakness of the bilateral upper and lower limbs throughout the prior five days. There was a history of head rotation and leaning consistently to the right that had developed over a three-week period. A clinical examination revealed neck stiffness and head rotation similar to that of spasmodic torticollis: the face turning to the left side and the head pulling to the right shoulder. Muscle strength was decreased and muscle tone increased in the bilateral upper and lower limbs. All the deep tendon reflexes became brisk, and ankle clonus was observed. Hemogram and routine blood chemistry were normal. Plain radiographs of the cervicothoracic spine did not reveal any abnormality, and chest radiograph was also normal. MRI study revealed an oval, unilocular, well-defined extradural lesion posterior to the thecal sac opposite the C6-T2 vertebrae. The lesion was hyperintense on both the T1W and T2W images. The spinal canal at the same level was slightly enlarged with a smooth edge. The lesion was compressing the underlying spinal cord, which showed hyperintensities on T2W images consistent with myelomalacic changes ().
Post-contrast study did not reveal any enhancement of the lesion. Vertebral bodies, pedicles, and posterior elements appeared normal. No pre- or paravertebral soft tissue masses were seen. Based on these imaging findings, we made a diagnosis of a spontaneous extradural hematoma with evidence of underlying compressive myelomalacia (at the C4-T3 level). Surgery was performed with the patient in the sitting position. C6 to T2 laminectomy was performed, and a cystic lesion was seen extradurally on the left side. The cyst contained black blood within a wall which was more than 1mm thick. The hematoma was evacuated and the cyst wall was removed. Cord decompression was performed. The postoperative course was uneventful, and the child made a gradual neurological recovery following surgery. The diagnosis of hydatid disease was confirmed upon histopathology ().
Postoperative sonography of the abdomen revealed no evidence of hydatid disease, thus albendazole was given orally for one month for prophylactic purposes. During the follow-up period of 50 months, repeated clinical, radiological, and serological examinations (every 6 months) did not show any evidence of local recurrence or systemic hydatidosis. MRI scanning showed that the spinal cord had recovered completely by 50 months after surgery (Figs. , ).
Post-contrast study did not reveal any enhancement of the lesion. Vertebral bodies, pedicles, and posterior elements appeared normal. No pre- or paravertebral soft tissue masses were seen. Based on these imaging findings, we made a diagnosis of a spontaneous extradural hematoma with evidence of underlying compressive myelomalacia (at the C4-T3 level). Surgery was performed with the patient in the sitting position. C6 to T2 laminectomy was performed, and a cystic lesion was seen extradurally on the left side. The cyst contained black blood, within a wall which was more than 1mm thick. The hematoma was evacuated, and the cyst wall was removed. Cord decompression was performed. The postoperative course was uneventful, and the child made a gradual neurological recovery following surgery. The diagnosis of hydatid disease was confirmed upon histopathology ().
Postoperative sonography of the abdomen revealed no evidence of hydatid disease, thus, albendazole was given orally for one month for prophylactic purposes. During the follow-up period of 50 months, repeated clinical, radiological, and serological examinations (every 6 months) did not show any evidence of local recurrence or systemic hydatidosis. MRI scanning showed that the spinal cord had recovered completely by 50 months after surgery (Figs. , ).


A 47-year-old woman presented with a 3-day history of central colicky abdominal pain, abdominal distension and vomiting. She was treated with oral spasmolytic drugs without improvement. Medical history revealed no history of digestive bleeding. The patient underwent no surgical procedure in the past. Examination of her abdomen revealed tenderness and distension of the abdomen and no palpable mass. A 1cm uncomplicated umbilical hernia was present. Digital rectal examination was normal. Laboratory tests indicated an elevated white blood count (23500/mm3), normal serum sodium and potassium levels. A plain X-ray abdominal film showed distended small bowel loops and multiple gas fluid levels (). Computed tomography of the abdomen revealed multiple distended small-bowel loops distributed radially. The thickened small bowel wall and the congestive appearance of mesenteric fat were suggestive of a strangulated obstruction ().
We decided that an emergent laparotomy was required after a brief and intensive resuscitation. Upon exploration, we found a moderate clear ascites and an epiploic adhesion band overriding the jejunum with ischemic changes in the proximal jejunum (). After surgical removal of the adhesion band we noted that it was connected to a 5cm long diverticulum arising from the mesentery side 20 cm distal to the ligament of Treitz (). A diverticulectomy without small bowel resection was performed because the swollen, purple intestines were well perfused after 20 min of observation and there was no suspect endoluminal tissue. An umbilical herniorraphy were also performed. Final pathology exam confirmed the jejunal diverticulum with no sign of malignancy. The patient had an uneventful post-operative course and was discharged 5 days after the procedure.


A 10-year-old boy presented with a 4-day history of fever, sore throat, and neck pain. The boy had taken oral medication (acetaminophen, 500 mg QID) before presentation without effect. A physical examination revealed inflamed tonsils with a yellowish purulent coating () and bilateral neck masses of approximately 5×3 cm2. The neck masses were firm and movable, tender upon palpation, and associated with neck stiffness. There were no other abnormal physical examination findings. Non-specific complaints included cough, rhinorrhea, and abdominal cramping and pain with nausea and vomiting. Blood testing revealed the following: hemoglobin, 14.6 g/dL; white blood cell (WBC) count, 13,720/mm3, with neutrophil predominance (84.3%); platelet count, 197,000/mm3; and C-reactive protein (CRP) concentration, 9.4 mg/dL. Soft-tissue neck films showed no evidence of retropharyngeal abscess or masses, whereas a neck ultrasound revealed bilateral hypoechoic masses suggestive of cervical lymphadenitis (). Acute exudative tonsillitis with cervical lymphadenitis was suspected, and treatment with intravenous benzyl penicillin (10×104 U/kg IV Q6H) was initiated.
Despite 72 hours of intravenous antibiotic treatment, a persistent high-spiking fever was noted. Three days after admission, the patient developed a rough-textured and sandpaper-like erythematous maculopapular skin rash with pruritus on the lower body (). Vesicular and bullous formations were apparent in the axillary region. The skin rash resolved and reappeared intermittently during hospitalization. Four days after admission, the patient developed strawberry tongue and bilateral conjunctivitis (). Blood tests performed four and seven days after admission revealed WBC counts of 16,580/mm3 (day 4) and 22,460/mm3 (day 7) and CRP concentrations of 25.7 mg/dL (day 4) and 25.4 mg/dL (day 7). The patient's condition had not improved, and the neck masses remained painful.
Four days after admission, the patient's temperature spiking had not resolved, and he exhibited enlarged cervical nodes and acute exudative tonsillitis. Because the patient had failed to respond to initial therapy with benzyl penicillin within 72 hours, a bacterial-resistant infection was suspected, and the treatment regimen was changed to intravenous amoxicillin/clavulanic acid. The change in medication resulted in a significant improvement of the patient's condition. Seven days after admission, the interval between febrile episodes was prolonged, with lower peak temperatures. Reductions in neck mass size and tenderness were also apparent.
Nine days after admission, the patient's fever had subsided, and all of the other symptoms had resolved. The patient was discharged. Testing before discharge revealed the following: WBC count, 14,410/mm3; platelet count, 508,000/mm3; CRP concentration, 10.7 mg/dL; erythrocyte sedimentation rate, 102 mm/h and 112 mm/2 h; and antistreptolysin O and Epstein-Barr virus (EBV) antibody titers within normal limits. The throat swab cultures taken at the time of admission were positive for Streptococcus viridans, whereas blood cultures were negative for all organisms.
Fourteen days after the onset of fever, the patient presented to our outpatient department with sheet-like desquamation of the hands, feet, and perianal region (). Laboratory analyses revealed normocytic, normochromic anemia (hemoglobin 11 g/dL, hematocrit 34.8%) and thrombocytosis with a platelet count of 718,000/mm3. On the same day, echocardiography revealed bilateral coronary artery dilatation with a right coronary artery aneurysm (). The diameters of the dilated vessels were as follows: left main coronary artery (LMCA) 5.2 mm; left anterior descending artery 5.5 mm; left circumflex artery 4.4 mm; and right coronary artery (RCA) 6.5 mm. The patient was diagnosed with KD based on the echocardiographic findings and was readmitted. Immediate treatment with intravenous gammaglobulin was initiated (1 g/kg for 2 days), and the patient was subsequently discharged with a prescription for ongoing treatment with aspirin (5 mg/kg/day) and dipyridamole (6 mg/kg/day). Testing before discharge revealed the following: WBC count, 8,540/mm3; neutrophils, 55.5%; platelet count, 270,000/mm3; and erythrocyte sedimentation rate, 8 mm/h and 10 mm/2 h. These findings indicated that the inflammation associated with the disease was controlled.
Follow-up echocardiography examinations during a 6-month period after discharge revealed diffuse coronary artery dilatation and aneurysms as follows: LMCA 4.8 mm and RCA 6.7 mm (1 month after discharge); LMCA 4.1 mm and RCA 6.2 mm (two months after discharge); and LMCA 3.9 mm and RCA 4.6 mm (six months after discharge).


A four-year-old girl (body weight 16 kg) presented with fever for three days. On examination, she showed fine crepitations over both lower lung fields (anterior and posterior). A plain X-ray of the chest on PA view demonstrated non-homogenous opacities in both lower lung zones. Blood examination report showed a normal hemoglobin and RBC count. Total WBC counts were 12,400/μl (N - 79%, L - 18%, E - 2%, M - 1%, B - 0). The diagnosis of lower respiratory tract infection was made and she was prescribed cefditoren pivoxil 10mg/kg/day (75 mg twice daily) for 10 days and an ambroxol-salbutamol combination elixir. She was also advised paracetamol 15mg/kg/dose (250 mg thrice daily) for fever.
Her fever subsided within the first two days of therapy after which paracetamol was discontinued. On the fourth day of therapy with cefditoren, she developed wide spread maculopapular rash []. She also complained of pain over the right knee joint and refused to walk. There was no history of fall or injury to the knee. Local pain and tenderness were noted on flexion and extension of the right knee joint. There was erythema but no appreciable swelling. X-ray showed no bony or joint space abnormality []. Other joints like hips, elbows, and wrists were not involved.
All medicines that the child was receiving including cefditoren pivoxil were discontinued promptly. Paracetamol 15 mg/kg three times a day was prescribed again to provide relief from pain. The rash, local pain, and tenderness resolved within three days. However, there was still some residual discomfort with joint mobility and difficulty in walking. She eventually recovered full function of the knee joint and started walking without any discomfort on the seventh day following discontinuation of cefditoren pivoxil.
The child was suspected to have suffered an episode of rash and arthralgia with cefditoren pivoxil treatment. None of the concomitant medications, e.g., ambroxol, salbutamol, and paracetamol is known to cause arthropathic symptoms or joint damage. An exanthematous febrile illness of viral origin, albeit rarely, is known to usually affect older teens and adults. Known as the fifth disease as the rash appears on the fifth day of the fever, it is also characterized by swelling and pain of joints.[] The clinical presentation in this patient excludes the diagnosis of fifth disease. In this case there was reasonable temporal relationship between the adverse event and drug exposure. Also, pain disappeared and functional joint disability was resolved on dechallenge. Rechallenge did not occur. Further, no alternative cause, including any disease, was likely to have caused the adverse event. As per the WHO and Uppsala Monitoring Center standardized case causality assessment criteria,[] this event can be considered as a “probable” reaction to due to cefditoren pivoxil. The Naranjo's ADR probability score,[] 7, also confirmed this causality as “probable”.


A 42-year-old lady presented to the surgical OPD with complaints of a swelling with multiple discharging sinuses over her right thigh for the past two years. The swelling was insidious in onset and was gradually increasing in size. There was no history of any trauma or insect bite at the site of the lesion. The patient had no past history suggestive of tuberculosis or diabetes mellitus. She was postmenopausal for the last four years and had no past history of intra-uterine contraceptive device insertion. She had been treated by multiple doctors in various centres, over the last 6 months and had taken extended courses of oral anti-microbial therapy.
On examination there was a 15 cm × 20 cm, irregularly shaped, indurated swelling in the medial aspect of the right thigh extending from the perineum to mid-thigh (Figure ). There were multiple sinuses draining yellow coloured pus along with sulphur granules. There were no other lesions elsewhere on the body.
The pus from the discharging sinuses was sent for microscopy and a biopsy from the lesion was sent for histo-pathological examination. Gram staining revealed Gram positive bacilli, but no organisms could be cultured. Histopathological examination confirmed the diagnosis of actinomycosis (Figure ).
A MRI scan was done and it revealed multiple sinuses in the medial aspect of the right thigh extending to the underlying muscles (Figure ). There were no lesions in the underlying bone.
The patient was started on co-trimaxozole and cindamycin for a period of 1 month, after which the patient was taken up for surgical excision of the lesion followed by split skin grafting to cover the created defect (Figure ). During excision, the whole lesion along with the involved muscle was removed. Multiple sinus tracks were encountered during surgery.
The patient had a wound infection in the postoperative period, which responded to parentral antibiotics. The patient was discharged on oral co-trimaxazole and clindamycin for 15 days following which only co-trimaxozole was continued for 2 months.
The patient has been in regular follow-up and there has been no recurrence till after 1 year of surgery (Figure ).


A 34-year-old white woman was referred to the Department of Periodontics at Tehran Medical University in 2006. Her chief complaints were mobility and spacing between her maxillary teeth that had developed during the past year, gingival bleeding, and swelling. The patient was especially concerned about all four maxillary incisors (Fig. ).
She had a history of periodontal disease and had undergone periodontal surgery three times, at the ages of 14, 22, and 27 years, in the posterior regions of both arches. All previous treatments had been performed by a general dentist. The medical history of the patient’s family revealed that her mother had lost her teeth in her youth because of loosening; her brother had also suffered from severe periodontal disease.
All the teeth were present except for the mandibular right third molar. The patient had poor oral hygiene, with a Plaque Index of 97%, and bleeding on probing (BOP) was observed in all regions. Radiographic evaluations revealed moderate to severe generalized bone loss (Fig.). The mobility was grade I at teeth #5, #14, #15, #26, and #30; grade II at teeth #1, #2, #3, #4, #7, #10, #12, #13, #23, #24, and #25; and grade III at teeth #8 and #9. In addition, tooth #3 had grade I distal furcation involvement, and tooth #30 had grade I buccal furcation involvement. According to the clinical and radiographic findings, the patient was diagnosed with GAP. The objectives of treatment were to control the advancement of the disease, reduce tooth mobility, re-establish aesthetics and function, and, in general, obtain predictable and sustainable results.
Treatment began with instruction in oral hygiene. Next, scaling and root planing were performed. Amoxicillin (250 mg) and metronidazole (250 mg) three times a day and 0.2% chlorhexidine mouth rinse (two times a day) were prescribed for 7 days afterward.
Since poor oral hygiene had caused recurrence of the disease, the patient entered a maintenance phase before initiating any further treatment. After 6 months, the Plaque Index had decreased to 16%, but BOP was still about 40%. Mobility of the teeth had decreased, except in the anterior maxilla. In addition, although a decrease in pocket depth (average of 2 mm) was observed, pockets deeper than 5 mm and BOP were still present in all regions.
During phase II of treatment, to eliminate the deep pockets, periodontal flaps and correction of bone contours (osteoplasty) were performed in all but the anterior maxilla. No significant decrease in the mobility of the maxillary incisors was observed. Grade III mobility of the central incisors made mastication very difficult. The crown/root ratio of the central incisors was about 2/1, which explained the severe mobility (Fig. ). Because of these problems and the unpleasant appearance of the central incisors, we decided to extract them. The patient insisted on replacement of her lost teeth without any damage to adjacent teeth.
In agreement with the patient’s wishes, and taking into consideration the crown/root ratio of the canines and premolars and the extent of the future edentulous area (Fig. ), the decision was made to place implants. However, pronounced, severe bone loss had occurred in the anterior maxilla as a result of the GAP, such that the extractions might lead to collapse of the soft tissue, aggravation of vertical defects of the ridge, and vertical bone resorption. For these reasons, forced eruption of the hopeless teeth was used to decrease the amount of bone resorption.
Tomography and standard periapical radiography were used to evaluate the extruded bone and assess alterations in the crestal bone. Before orthodontic treatment, the distance between the floor of the nose and the marginal bone of each hopeless tooth was evaluated and registered. These distances ranged between 12.8 and 15.7 mm for the maxillary incisors (Fig. ). Edgewise brackets were bonded to the surfaces of all maxillary teeth between and including the first molars (Fig. ).
Extrusion was performed over the course of 4 months. The patient was checked every 2 weeks, and all interferences between the palatal surfaces of the anterior maxillary teeth and the incisal edges of the anterior mandibular teeth were eliminated. When the original and final casts (after 16 weeks) were compared, the teeth were determined to have been displaced 4 mm from their baseline locations.
In extrusion of the hopeless teeth, the bone level of the adjacent teeth dictates the amount of extrusion. Comparison of the marginal bone levels of the lateral incisors with those of the canines revealed that the amount of extrusion was sufficient after 4 months. To compensate for the bone resorption that would inevitably follow implant placement, the orthodontic extrusion was allowed to progress somewhat beyond the marginal bone levels of the canines (slight overtreatment).
Comparison of radiographs obtained before and after extrusion revealed that the relationship between the cementoenamel junction and the marginal bone remained constant. In addition, the depth of the vertical defect at the mesial of the left central incisor had decreased.
After 4 months of stabilization, the maxillary incisors were extracted. Before extraction of the teeth, standardized tomography was performed using a template. The amount of bone that had been regenerated in the incisor area ranged between 2 and 3.9 mm (Fig. ). The incisors were extracted gently. The sockets of the central incisors were filled with bone grafting material (Bio-Oss) up to the level of the crestal bone (Fig. ).
Extraction of the teeth without preservation of the soft tissue will lead to soft tissue collapse and loss of the interdental papillae, and the resultant dark spaces in the interproximal regions will compromise the aesthetic results of treatment. Therefore, to preserve the soft tissue architecture and ensure a better aesthetic result, the extracted teeth were used as pontics during healing of the extraction sites. The extracted teeth were cut 2 mm below the gingival zenith, pulpal tissues were removed, and the cut surfaces were shaped into ovate pontics using composite resin. After the crowns were placed into their respective sockets, they were attached to each other using flowable composite. The positions of the teeth were registered using a silicon index. The reshaped extracted teeth were attached to a previously fabricated removable acrylic resin partial denture in their original positions (Fig. ), and the denture was delivered to the patient about 3 hours after extraction of the teeth. The patient was instructed to wear the prosthesis continuously except for cleaning. This continuity of use was essential to maintain soft tissue form (Fig. ).
To keep the Bio-Oss granules in place, the patient was advised not to remove her denture earlier than 1 week after surgery to perform oral hygiene. Amoxicillin 250 mg and Metronidazole 250 mg (every 8 hours for 7 days) were prescribed. No abnormal clinical signs or symptoms of inflammation were seen during the first week.
One month after extraction of the teeth, epithelialization of the socket surfaces was complete (Fig. ). During the healing period, minor soft tissue recession of about 1 mm was observed at the gingival margin and interdental papillae.
Because raising flaps in the edentulous region for the placement of implants might increase the risk of tissue recession, a model-based, flapless implant placement technique was used. For this reason and to evaluate the bone volume after extraction of the teeth and determine the ideal paths of implants, a template was made using transparent acrylic resin (Fig. ). Four holes were made at the buccal and lingual surfaces of the template at the lateral incisor sites. After local anesthesia had been administered, bone mapping was achieved via the holes made on the template. The template was inserted back onto the cast, and the stone was reshaped based on the recorded measurements. The outcome of the reshaping thus accurately represented the patient’s residual bone (Fig. ). Model surgery was done, and implant analogues were inserted into the cast to determine the primary paths of the implants. Access holes to the implant analogues were prepared in the template and were filled with gutta-percha. After a cone beam computed tomogram was obtained with the template in place, the final paths of the implants were corrected on the template (Fig. ).
On the day of surgery, two implants, each 3.5 mm wide and 15 mm long, were placed in the lateral incisor sites using the template with the flapless method. Immediately after surgery, an implant-level impression was taken. Within 2 hours, a provisional prosthesis had been prepared and was delivered to the patient without any functional occlusion. The patient was instructed to maintain oral hygiene and to avoid biting with the anterior teeth. After 1 week, the sutures were removed. The definitive prosthesis was delivered to the patient after 4 months (Fig. ).


A 25-year-old man presented with a 3-week history of a gradually enlarging painful swelling of the right forehead, headache, fever, and rhinorrhea. He had a history of allergic rhinitis and asthma, but no head trauma. The physical examination revealed a fever and a 4×2 cm fluctuant mass above the right eyebrow and frontal sinus with surrounding cellulitis (). On nasal endoscopic examination, bilateral nasal polyps and a purulent discharge were noted (). Clinical examination of the cranial nerves, visual fields, and vision were normal. Complete blood counts showed neutrophil predominant leukocytosis (a leukocyte count of 16×103 per ml with 79.3% neutrophils). Erythrocyte sedimentation rate was elevated, measuring 105 mm/hour. An ECG and a chest X-ray were unremarkable.
A paranasal sinus X-ray revealed opacifications of the right frontal sinus, the ethmoid sinuses, and the left maxillary sinus. A contrast-enhanced computed tomographic scan of the paranasal sinuses showed right frontal sinusitis with an anterior bone defect, bilateral ethmoid sinusitis, and left maxillary sinusitis. However, no abnormalities in the brain parenchyma were noted (). There was evidence of bony destruction in the anterior wall of the frontal sinus and a subperiosteal abscess. The patient was treated with parenteral antibiotics (ceftriaxone 2G IV daily), intranasal decongestant and oral steroid. However, the frontal headache and frontal bulging mass did not improve with medical treatment and the patient became febrile again after 2 days of antibiotic therapy. A T2-weighted magnetic resonance imaging scan of the paranasal sinuses showed a high signal lesion of the right frontal bone marrow under the right subperiosteal abscess without intracranial involvement ().
Surgical treatment was indicated because of the gradually enlarging mass of the right frontal area. Under general anesthesia, the right nasal polyps were resected with ethmoid forceps. After the right ethmoidectomy, the right frontal sinus natural ostium was opened, purulent material oozed out, and the abscess was drained. Samples were taken for culture and gram staining and were found to be positive for Staphylococcus aureus. The main surgical finding was a polypoid mass which had occluded the frontal recess. The frontal sinus natural ostium was opened and drained. The interval between surgery and disappearance of main clinical symptoms was 2 days and the deformity resolved during this time. One week later, a technetium-99m methylene disphosphonate (99mTc-MDP) bone scan and gallium scan were obtained. These scans were consistent with resolved acute osteomyelitis () and the patient was discharged 7 days after surgical management. The antibiotics were continued orally for 8 weeks. Antihistamine was needed to control the allergic rhinitis. Six months postoperatively the deformity had not returned () and the patient was free of symptoms at the one-year follow-up examination.


A 65-year-old man, 163 cm in height and 60 kg in weight, was admitted to the emergency room with a cough, blood tinged sputum and dyspnea. He underwent percutaneous mitral balloon valvuloplasty 15 years earlier due to MS. He had a history of a cerebral infarction and hypertension. Electrocardiography revealed atrial fibrillation (A-Fib). Minimal coronary artery occlusive disease was identified during coronary angiography. Transthoracic echocardiography (TTE) revealed severe MS (mitral valve area of 0.79 cm2; mean diastolic pressure gradient of 12.5 mmHg; left atrial volume index [LAVI] of 115 ml/m2), mild tricuspid regurgitation, and a right ventricle systolic pressure of 47 mmHg and ejection fraction (EF) of 72%. Despite mild swirling in the left atrium (LA), no mass or thrombi were observed. The patient was medicated with digoxin, torasemide, simvastatin, and 3 mg of warfarin. For MVR, warfarin was discontinued and heparinization was initiated four days before surgery until midnight of the day before surgery. The international normalized ratio (INR) and activated partial thromboplastin time (aPTT) one day before surgery were 1.00 and 26.3 seconds, respectively.
Before CPB, in TEE, severe spontaneous echo contrast (SEC) in the LA was noted with no evidence of thrombus at any angle (). CPB was instituted and after injecting 18000 units of heparin, the activated clotting time (ACT) was increased to 489 seconds. During CPB, the ACT was maintained at more than 500 seconds with an additional 3000 units of heparin. The body temperature was decreased to 33℃. The surgeon confirmed the absence of any removable organized thrombus in the LAA and performed MVR with a Carpentier-Edward 27 valve.
After unclamping the aortic cross clamp (ACC), a well functioning bioprosthetic mitral valve (MV) without paravalvular leakage was confirmed through TEE. The pump flow rate was lowered to wean the patient from CPB and during de-airing of the heart, TEE revealed a newly formed non-mobile mass in the LAA (). At that time, the ACT was 569 seconds. After discussion with the surgeon, a re-bypass was planned, and the mass was removed via a left atriotomy after ACC. The clot was removed as a single piece, which measured approximately 33 × 33 mm, which had the appearance of a fresh, newly formed thrombus by a visual inspection (). Weaning from the CPB was performed uneventfully. The total CPB and ACC time was 81 minutes and 53 minutes, respectively.
The patient awoke six hours after arriving at the intensive care unit with no neurological deficits and was extubated at postoperative day (POD) 1. Anticoagulation was started with warfarin at POD 2. He was transferred to the general ward at POD 3 and discharged at POD 9. His postoperative TTE revealed a well functioning bioprosthetic MV, a slightly decreased LA size with a normal LV function, and no remaining MS or mitral regurgitation.


A 55-yr-old male presented with complaints of dizziness and dyspnea. The initial laboratory findings revealed a leukocyte count of 3.4×109/L, a hemoglobin level of 8.1 g/dL, and a platelet count of 37×109/L. On a peripheral blood smear, 63% immature cells were found with 2% segmented neutrophils and 35% lymphocytes. The bone marrow aspirate was hypercellular and replaced by 90% blasts. The remaining cells were lymphocytes. Megakaryocytes were decreased in number. Accordingly, our case did not have sufficient non-blast cell elements in the bone marrow to adequately assess for multilineage dysplasia. The biopsy could not be evaluated due to an inadequate specimen. Flow cytometry showed that the blasts were positive for cytoplasmic myeloperoxidase, CD13, CD33, CD34, HLA-DR, and CD7 and negative for CD3, CD5, CD19, CD20, CD22, CD10, CD56, and CD14. Cytochemistry demonstrated that nonspecific esterase was positive. Finally, the patient was diagnosed with AML with myelodysplasia-related changes based on the 2008 WHO classification, because he had a MDS-related cytogenetic abnormality, the -5/del(5q). He was treated with induction chemotherapy involving idarubicin and arabinofuranosyl cytidine (Ara-C). Complete hematologic remission with a normal karyotype was achieved. After the second consolidation chemotherapy (daunorubicin+Ara-C), the patient received an autologous peripheral stem cell transplantation and remained in remission status for 3 months. However, 9 months after the diagnosis, relapse occurred (). Even after 3 reinduction therapies, remission could not be achieved and clonal evolution developed. The proportion of blasts increased up to more than 80% by 12 months after the diagnosis. The patient subsequently refused to receive continuous treatment and was lost to follow up.
For the cytogenetic analysis, unstimulated short-term cultures were set up using bone marrow aspirates, and at least 20 metaphases were analyzed. G-banded karyotype results were described according to the International System for Human Cytogenetic Nomenclature (ISCN) 2009 [].
We applied a genome-wide SNP 6.0 array (Genome-Wide Human SNP array 6.0; Affymetrix Inc., Santa Clara, CA, USA) using genomic DNA according to the manufacturer's instructions, and the data were analyzed using Genotyping Console 3.1 software (Affymetrix Inc.). Aberrations that were identified by SNP-A were described according to ISCN 2009 []. In order to detect the somatic origin of the copy number alterations that were distinguished from constitutional polymorphic copy number variants (CNVs), we adopted our stringent and conservative algorithm. We selected only the lesions that were more than 100 kb and with more than 10 SNP/CNV probe markers of the array involved within those regions. The lesions that were then identified by SNP-A were compared with the Database of Genomic Variants () and with an internal control series from healthy normal controls with Korean ethnicities (N=200) in order to exclude known CNVs. For the copy neutral loss of heterozygosity (CN-LOH), we excluded homozygous stretches of the DNA region that were less than 25 megabases (Mb) in the interstitial chromosomal regions, except for those encompassing the telomeric regions of the chromosome, according to the algorithm adopted in the previous studies [].
Interphase FISH studies were done on the bone marrow aspirates using commercially available probes (BCR/ABL1, RUNX1/RUNX1T1, PML/RARA, CBFB/MYH11, MLL, EGR1/D5S23, D5S721, and CEP7/D7S486; Abbott Laboratories, Abbott Park, IL, USA), according to the manufacturer's instructions.
The cytogenetic analysis showed that 90% of the metaphase cells were bearing -5, del(11)(q22), +r in 20 metaphase cells analyzed (). Genomic aberrations identified by SNP-A were arr 5p15.33p15.2 (423,554-11,003,428)×1, 5q33.2q35.3 (153,225,007-180,652,396)×1, 11q14.1q23.2 (84,514,776-113,765,153)×1, and 21q22.13q22.2 (37,018,986-39,492,610)×3. These results indicated a 5p-terminal deletion (11 Mb), a 5q-terminal deletion (27 Mb), an 11q-interstitial deletion (29 Mb), and a 21q gain (3 Mb) (). Therefore, the G-banded karyotype was finally revised as 46,XY,r(5)(p15.2q33.2),del(11)(q14.1q23.2),dup(21) (q22.13q22.2)[18]/46,XY[2].
As for the chromosome 5 abnormality, FISH revealed nuc ish (D5S721×1, D5S23×1, EGR1×2)[129/200]. This result corresponded to the signal patterns of the 5p15.2 deletion in 65% of the nuclei examined, whereas 2 EGR1 (early growth response 1) signals were indicative of no deletion of 5q31 on both chromosomes 5 (). This finding was compatible with the result that the deleted region of 5q that was identified by SNP-A was more telomeric to 5q31, which was spanned by the commercially available EGR1/D5S23, D5S721 FISH probe.
Interphase FISH using the commercial MLL (mixed lineage leukemia) probe demonstrated normal hybridization patterns of the 11q23 regions, indicating that the deleted region of 11q by SNP-A was located more proximal to the MLL gene. FISH using BCR/ABL1, RUNX1/RUNX1T1, PML/RARA, CBFB/MYH11, and CEP7/D7S486 probes demonstrated normal hybridization signal patterns.
During the follow up, additional chromosomal abnormalities, including add(4)(q32) were found, suggesting clonal evolution ().


A 72-year-old woman presented with headache and was admitted to our hospital. Neurologic and physical examinations demonstrated no abnormality. Computed tomography scans showed an abnormal mass lesion in the right frontotemporal convexity region. The lesion was surrounded by marked cerebral edema. Mass effect was noted on the midbrain. Magnetic resonance (MR) imaging showed a mass with a similar signal intensity on the T1-weighted image and high signal intensity on the T2-weighted image [], respectively. Contrast-enhanced axial T1-weighted images showed a well-enhanced, dural-based mass compressing the right temporal and frontal lobes, and extending into the sylvian cistern and filling the cortical sulci. It is specifically these subarachnoid components of the tumor in the sulci that showed relatively little mass effect. Coronal and sagittal enhanced T1-weighted images also showed unusual enhancement patterns []. Cerebral angiography showed a mild tumor blush supplied by the right middle meningeal artery and middle cerebral artery.
The patient underwent a right frontotemporal craniotomy, and the tumor was partially resected. The removed specimens consisted of the tumor tissues only, and did not include the surrounding brain tissues. Microscopically, dyscohesiveness of tumor cells was observed. The tumor was globally composed of rhabdoid cells showing large eosinophilic cytoplasm and eccentrically placed, medium-sized round nuclei with prominent nucleoli []. There were many tumor cells with an inclusion-like round structure in their cytoplasm. The patterns seen in conventional meningiomas, such as whorl or psammoma body formations, were not observed. Neither necrosis nor microvascular proliferation was present in the tumor, although scattered mitotic cells (3 mitoses per 10 high-power fields) were seen. Immunohistochemically, the cytoplasm was strongly positive for vimentin []. Epithelial membrans antigen was focally expressed. GFAP, S-100, and AE1/3 were negative. The MIB-1 staining index was approximately 8%. Postoperative course was uneventful and MR images demonstrated resection of the temporal mass lesion []. The patient was discharged home for outpatient radiotherapy treatment. MR imaging performed 24 months later did not revealed regrowth of the residual tumors.


A 42-year-old male visited the emergency department complaining of chest pain. He had previously been admitted 3 months ago due to similar symptoms, and was diagnosed with acute pericarditis according to the features of typical chest pain and findings of electrocardiography (ECG) and echocardiography (). Ibuprofen and colchicine were administered and the symptoms improved within 24 hours. At the time, he was discharged after 5 days, and he had stopped taking the medications by himself after 2 weeks as he did not feel any continuing symptoms.
At admission, he complained of severe chest pain which had begun 4 hours prior to admission. The pain increased with deep breathing and position change. He also complained of palpitation which had been present prior to the development of chest pain. The patient had no relevant abnormal medical history and no current medication, and reported to have lost 2 kg during the past 2 months. He was a 14-pack-year smoker, but denied any alcohol use.
Upon general examination, he appeared acutely ill. His vital signs in the emergency department were as follows: body temperature 36.9℃, pulse rate of 121 beats per minute, and blood pressure 140/89 mm Hg. The heartbeat was regular and no cardiac murmur including precordial friction rub could be identified. In addition, the lung sound was clear. There was no thyroid enlargement or neck vein engorgement.
Laboratory results including complete blood count, biochemistry profile, and cardiac enzymes were within normal limit. The chest radiograph finding was normal. ECG showed sinus tachycardia and diffuse concave ST-segment elevation in all leads except aVR and V1. PR segment was normal (). Transthoracic echocardiography showed normal systolic and diastolic function of the left ventricle (LV) and a scanty amount of pericardial effusion at the LV posterior side (). Echocardiography that had been performed at the previous admission had shown constrictive physiology, but no such finding was visible this time. Cardiac magnetic resonance imaging showed a slightly thickened pericardium with small amounts of pericardial effusion (). Autoimmune antibodies (ANA and Anti-ds-DNA) showed normal results. According to the characteristics of the chest pain, which was sharp and pleuritic in nature, and the diffuse concave ST elevation without relevance to any certain coronary artery which was out of proportion to the pain intensity, the patient was diagnosed as acute pericarditis rather than coronary spasm-related angina.
The patient was given ibuprofen and colchicine. Due to a history of palpitation and weight loss, tests for thyroid function were done on hospital day 3. Serum thyroid hormone examination showed evidence of hyperthyroidism with a T4 level of 14.2 µg/mL, T3 level of 182 ng/dL, and TSH level of 0.06 µIU/mL (normal value T4 4.7-12.5 µg/mL, T3 76-190 ng/dL, TSH 0.3-6 µIU/mL). Thyroid autoantibody evaluation and a thyroid scan were performed. TSH receptor antibody was 5.4 IU/L (normal 0-1 IU/L), thyroglobulin antibody 89 U/mL (normal 0-60 U/mL), and microsomal antibody 136 U/mL (normal 0-60 U/mL) all showed elevated levels. Thyroid scan showed 17% (normal 5 to 15%) uptake and the patient was diagnosed with Graves' disease, after which methimazole 10 mg bid was prescribed. Chest pain subsided within 24 hours, and the patient showed marked clinical improvement within 7 days with improvement of palpitation and general weakness. Elevated ST-segment was improved. The patient was discharged with medications for anti-hyperthyroidism and colchicine. No clinical evidence of recurrence was found during the following 3 months. The ST-segment eventually normalized on the follow up electrocardiogram ().