A 12-year-old girl was referred to a private dental office due to pain and swelling of the right vestibule and palate of maxilla (). She received right kidney transplantation five years ago and has been using immunosuppressive drugs. The patient was afflicted by ALL six months ago and underwent chemotherapy including rituximab, cyclophosphamide, vincristine and prednisolone. The chemotherapy lasted six months. The result of complete blood work-up did not show any sign of blood cell alteration.
A biopsy was performed () on the mucosal tissue of the buccal vestibule. The tissue structure was beige to white, completely soft like fish meat, with differential diagnosis including leukemic infiltration and secondary tumor.
The pathological testing procedure of immunohistochemistry and C-Kit (CD 117) confirmed the diagnosis of ALL and the patient underwent chemotherapy again with stronger drugs. The patient died due to severe side effects of chemotherapy after 3 months.


A 48-year-old male was brought to the casualty after he was found hanging from a ceiling fan, with one leg on a stool and the other hanging free. The duration of hanging was not known, but the time to hospital presentation was about 60 min. He was a known case of depression. On arrival, he was drowsy, restless, with saliva drooling out of his mouth and glasgow coma scale (GCS) score of 6. A partial ligature mark was noted around his neck. His respiratory rate (RR) was 44/min, pulse rate (PR) 122/min, blood pressure (BP) 200/100 mm Hg, arterial oxygen saturation (SPO2)on room air 85%, pupils dilated bilaterally and reacting sluggishly to light. There was no sub-conjunctival haemorrhage. Bilateral crepitations were observed on chest auscultation. After securing intravenous access, he was sedated with midazolam 0.04 mg/kg and Propofol 1 mg/ kg, his neck was stabilized using cervical collar and the airway was secured by endotracheal intubation (using McCoy blade). Assisted controlled ventilation was provided with tidal volume 8 ml/kg, RR 12/min, positive end-expiratory pressure (PEEP) 5 cm water (H2O) and inspired fraction of oxygen (FiO2) 1. He was nursed in a head-up position (30°–45°) maintaining sedation and muscle paralysis with infusions of Midazolam and Vecuronium, respectively. Blood samples were sent for routine biochemical and haematological investigations, including arterial blood gas (ABG) analysis, urinary catheterization were performed and central venous line was secured. Because central venous pressure was elevated, fluid administration was restricted and Frusemide was administered. Short-duration intravenous infusion of nitroglycerine was administered to control the elevated blood pressure, and was discontinued soon thereafter. Other elements of management included maintaining normocarbia partial pressure of carbon dioxide in arterial blood (PaCO2) (30–35 mmHg), prophylactic antibacterials, ensuring euglycemia, intravenous Ranitidine and Methylprednisolone (1 g infused over 10 min). Chest and cervical spine X-rays were performed in bed, while computed tomography (CT) scan of the head and neck was performed after the patient was stabilized haemodynamically. All biochemical, haematological and radiological investigations were reported as normal. With improvement in chest condition and reduction in FiO2 support after 28 hrs of presentation, Vecuronium infusion was discontinued. Thereafter, the patient was gradually weaned off assisted ventilation over the next 8 hrs and was extubated when he regained full consciousness and good spontaneous respiratory efforts. The patient was kept under observation for another 24 hrs in the intensive care unit and was finally discharged from the hospital without any neurological sequela after a total of 4 days of care.
The clinical presentation of cases 2 and 3 has been summarized in . Whereas their management was broadly on lines similar to that followed in case 1, variations specific to their clinical condition and management have been described in the following section.


A 77-year-old female accidentally noticed a progressively enlarging lump in her right abdominal wall one year earlier and she didn't receive any treatment. Loose stools appeared for the past half year without any obvious weight loss. A month previously, the patient suffered from shortness of breath after physical activity and eventually she visited our hospital, where the physical examination revealed a giant irregular tumor in the right chest, abdominal and pelvic walls. The upper edge of the tumor reached the lateral margin of the right breast and the lower edge was at the level of the pubic symphysis. The mass was hard without tenderness and poorly circumscribed with limited mobility. The local skin temperature was normal. The patient was a housewife: she denied any history of exposure to asbestos dust and poisonous chemicals and she had no symptoms, including fever, sweating at night, cough and chest or abdominal pain. The routine blood tests and serum carcinoembryonic antigen (CEA), CA12-5 and CA19-9 were normal. The plain chest X-rays showed a large amount of pleural effusion in the right thorax accompanied with mediastinum shift. Ultrasound-guided drainage was performed after admission. Cytological examination of the pleural fluid revealed malignant tumor cells. Chest, abdominal and pelvic computed tomography (CT) showed a giant irregular soft-tissue mass in the right thoracoabdominal wall and bilateral pelvic walls that had extensively invaded the muscular layers of the wall and extended to the inside of the abdominal cavity with peritoneum thickening (). The mass had poorly-defined margins. There was no clear demarcation between the mass and the surrounding body wall tissue. The abdominal and pelvic lesions extended to the subcutaneous tissue with spiculated margins. The tumor contained no calcium tissue and the tumor showed inhomogeneous slightly decreased attenuation with a CT value between 32 and 48 Hounsfield units on the unenhanced CT, and heterogeneous mild to moderate enhancement with a CT value that was increased 21-26 Hounsfield units on the contrast-enhanced CT. The pleural fluid causing the effusion was completely drained. There was no significant evidence of pleural thickening (). No abnormal lesion was seen in both lungs. We noted ascites and part of the small bowel was dilated in the abdominal and pelvic cavity, but no soft-tissue tumor was seen inside (). The mass on abdominal magnetic resonance imaging (MRI) was iso-intense on the precontrast T1-weighted images (), heterogeneous iso- to hyper-intense on the T2-weighted images () and it was heterogeneously enhanced with multiple patchy non-enhancing necrotic areas after contrast medium administration (). The abdominal wall part of the lesion was bigger and more inhomogeneous than the other parts. The tumor itself contained no fat, yet there were multiple patchy areas of fat tissue in the superficial part of abdominal lesion due to subcutaneous tissue infiltration, and surrounding subcutaneous tissue swelling was also noted on the fat-suppressed T2-weighted images.
Biopsy was performed and specimens were taken from three different sites of the abdominal wall mass. Hematoxylin & Eosin staining showed papillary and tubular malignant tumor cells and proliferating mesothelial cells that had extensively invaded the muscular tissue (). The tumor cells were positive for high molecular weight cytokeratin (34βE12) (), HBME-1, calretinin (), Wilm's tumor-1 (WT-1) and vimentin, and the tumor cells were negative for thyroid transcription factor-1 (TTF-1) and villin. These results confirmed the diagnosis of MM.
As the tumor was very large and extensively invasive, surgical resection was not possible and radiotherapy was also not done. The patient was treated symptomatically and sadly, she died six months later due to gradual deterioration of her condition.


A 64-year-old, 164 cm, 70 kg woman had a five-year history of hypertension and diabetes mellitus. She experienced insomnia, nausea, visual hallucination and left-side tingling sensation 4 months ago and memory impairment and gait disturbance became evident clinically 2 months ago. At that time, she underwent brain magnetic resonance imaging, which did not demonstrate abnormality. The patient presented with generalized myoclonus, progressive gait disturbance, and deviation of the left eye 1 month ago. Clinically, her condition resembled that of terminal vegetative state. Brain MRI showed T2 hyperintensity of the right dominant cortex, caudate nucleus, putamen, and thalamus on diffusion-weighted sequences. Electroencephalogram showed nearly continuous high voltage 1-1.5 Hz periodic sharp waves in the frontal area bilaterally. Under the suspicion of CJD the patient was admitted for brain biopsy.
The patient was scheduled last on the operating list. The operating room was cleared of unnecessary equipment and the wall of operating room was covered with vinyl chloride monomers. All staff members involved with the operation wore liquid-repellent gown, face-masks with plastic transparent shield visor, and double gloves ().
The mental status of the patient ranged from stupor to semicoma and vital signs were stable. Standard monitoring were applied before induction of anesthesia. Anesthesia was induced with a bolus injection of propofol 1.5 mg/kg in combination with remifentanil infusion at 0.5 µg/kg/min. Endotracheal intubation was facilitated by vecuronium 6 mg. Anesthesia was maintained with oxygen 5 L/min, propofol 50-150 µg/kg/min, and remifentanil 0.05-0.1 µg/kg/min total intravenous anesthesia. Almost every equipment applied was of single-use nature, including face-masks, oropharyngeal airway, laryngoscopic blade, endotracheal tube, Ambu bag, O2 flowmeter, and stethoscope. The surgeon used hand-drill instead of air-drill, and the entire surgical procedure took 40 minutes. Extubation was performed following in the operating room after gaining of spontaneous ventilation. The patient recovered in the operating room and was subsequently transferred to the private ward. All disposable equipment was destroyed and incinerated postoperatively.
Brain biopsy revealed neuronal loss, vaculoation, and astrocytosis in the brain tissue. Western blot demonstrated a misfolded, protease-resistant, pathogenic form of prion protein in the brain tissue. A diagnosis of sporadic CJD was made based on the above findings.


A 61-year-old man presented with approximately a five-year history of multiple scattered non-tender palpable firm indolent subcutaneous nodules on both palms and soles. He recognized the initial skin lesion on his right palmar area after he felt discomfort and pain upon grabbing an object. The lesions have increased in size and number over the last four years on both palms, and, recently, the same multiple lesions appeared on the soles. He complained from time to time of morning stiffness and numbness on his hands and feet. There was no recollection of associated trauma. His family history and past medical history were unremarkable.
Physical examination revealed that the number of nodules was approximately ten, which were located mainly in the center of both palms and soles, and these ranged in size from approximately 0.5 cm to 1.5 cm in diameter (). As the nodules of the soles were deeply located, they were not visible, but palpable. No limitation of hand or finger motion was observed. No joint swelling or other specific skin lesions were found.
Laboratory tests, including blood cell count and blood chemistry, were all within normal ranges. However, a rheumatologic evaluation yielded a positive antinuclear antibody (homogeneous pattern) and positive serum rheumatoid factor with a titer of 5+ (1:16) (positive range 1+~5+). Hand X-ray showed no remarkable findings, except for mild osteoarthritic changes in distal interphalangeal joints. For histological diagnosis, a 4 mm punch biopsy was performed from one of the lesions on the left palm. Histopathological findings showed relatively well-demarcated cellular proliferation, including slit-like vascular channels and haphazardly-arranged, spindle-shaped fibroblasts located among the dense collagenous matrix ().
Based on these clinical and histological findings, he was diagnosed as multiple FTSs. The patient did not want to be referred to a hand and foot surgeon for surgical excision. He has been followed up for almost 1 year and his pain and numbness have been treated with oral anti-inflammatory agents (aceclofenac). The number of FTSs has since remained stationary.


A 53-year-old female presented with a several year history of a right painless breast mass in the right upper outer quadrant that had grown in size significantly during the last two years until referral for treatment. The patient had no increased risk factors and family history was negative for both breast and ovarian cancers. The patient had been started on progesterone 10 mg daily one year prior to presentation for oligomenorrhea. A core biopsy taken nine years prior to presentation was reported as fibroadenoma. The patient was then followed biennially by the Ontario Breast Screening Program until the significant growth increase when a second core biopsy was recommended and was also consistent with fibroadenoma. Given the sudden increase in size, with the lesion now measuring 5.5×1.8 cm by mammogram (, ), the radiological/pathological correlation rounds recommended a surgical assessment and excisional biopsy for definitive diagnosis. An excisional biopsy was undertaken.
Grossly, the well-circumscribed pale-tan mass measured 6.5×3.6×2.4 cm, which in areas had a whorled appearance. Histologically, focal areas of fibroadenomatous changes were noted () while areas of leaf like architecture with variable cellularity of stromal component were present as well. Cytologic atypia was present throughout the stromal component ranging from minimal to moderate atypia and a variable number of mitoses were present (up to 7 to 8 per 10 high power fields), consistent with a diagnosis of phyllodes tumor with borderline malignant potential (). At the lateral portion of the excised mass there was a tubular carcinoma area with a greatest dimension of 2.4 cm (). The carcinoma cells were immunohistochemically positive for epithelial membrane antigen, estrogen receptor, progesterone receptor and E-cadherin, but negative for S100 protein. Absence of basal myoepithelial lining in tubules was confirmed by negativity for smooth muscle myosin heavy chain and P63 (). Lobular carcinoma in situ was also noted in this area and the diagnosis was confirmed by negativity of immunohistochemical stain for E-cadherin (). Microcalcification was observed within the tubular carcinoma. The surgical margins were not involved by the tumor, but the tubular carcinoma was very close (0.1 mm) to the inked surface at the lateral and posterior edges.
Following the pathology review the patient had complete staging workup, including bone scan, liver ultrasound, and chest X-ray. These were all negative for metastases. A dedicated breast magnetic resonance image (MRI) was completed prior to reexcision that demonstrated a few, scattered enhancing masses of unknown significance. Subsequently the patient underwent a re-excision for margin safety and sentinel lymph node biopsy. Pathology review of the re-excision found scerlosing adenosis and atypical ductal hyperplasia with no evidence of residual invasive or in situ carcinoma or phyllodes tumor components. The previously close margins of tubular carcinoma at the lateral and posterior aspect are now 4.0 cm and 2.8 cm from those margins, respectively. All three sentinel nodes were negative for malignancy.
This patient's unique presentation was reviewed at breast tumor rounds at the Juravinski Cancer Centre with the decision for the patient to next be evaluated by the radiation oncology team for adjuvant therapy.


A 11 year old right handed high school girl presented with sudden development of uneasiness, perception of smell of kerosene, followed by loss of awareness of surroundings for a period of 1-2 minutes for last 20 months. Initially the episode of seizure were around 3-4 attacks per week but later it increased to 10-12 attacks a day. She had no significant past events related to present disease. There was no significant event in her birth process and family history was negative for such type of disease. She had no complaints suggestive of intracranial space occupying lesion or raised intracranial pressure. Complete physical and neurological examination including higher mental functions revealed no abnormality. Scalp EEG recording showed epileptic spikes originating from left centro-temporal region. MRI of brain showed features of left amygdalo-hippocampal mass lesion (1×1.5×1cm) suggestive of low grade glioma. She was on two anti epileptic drugs (AED) [Carbamazipine and sodium valproate] for the last 16 months but seizure frequency did not reduce rather it increased further.
She underwent amygdalohippocampectomy with lesionectomy plus standard anterior temporal lobectomy. Histopathology revealed grade-1 ganglioglioma. Postoperatively she recovered uneventfully. She was put on carbamazipine 100mg tablet thrice daily that was tapered and stopped 4 months after operation. She has been seizure free for last seven months. Post operative MRI of brain at the end of 4 months after operation showed no residual or recurrent tumor and there was no visual field defect or nominal aphasia.


A 13 years old girl presented with sudden staring followed by unconsciousness and tonic-clonic convulsions involving left upper and lower limbs since birth. All these events persisted for only 40-50sec.She had history of mild birth asphyxia. Her family history for such kind of illness was negative. Her parents visited several physicians both in the country and abroad. She took several AEDs in combination for several years without significant improvement in seizure control. There were 25-30 seizure attacks per day even with multiple AEDs (Carbamazepine + Phenobarbitone + Valproic acid).Scalp EEG revealed abnormal epileptic spikes originating from right centro-temporal region. MRI of brain showed features suggestive of right mesial temporal sclerosis.
She underwent right amygdalohippocampectomy plus standard anterior temporal lobectomy. Postoperatively she recovered uneventfully. She was put on carbamazipine 100mg tablet thrice daily that was tapered 4 months after operation. She is seizure free for last six months while on carbamazipine 100mg 12 hourly.


A 12 years old boy presented with sudden transient global headache, nausea followed by unconsciousness for 1-2 minutes. He regained consciousness but was unaware of time, place and person for another 3-5 minutes. He was suffering from this event repeatedly for last five years. Initially there were 12-15 attacks per year that increased to 3-5 attacks per day even while he was on adequate AEDs treatment (Carbamazipine+Sodium Valproate). His family history was negative for such kind of illnesses and his birth history was normal. Scalp EEG revealed abnormal epileptic spikes originating from right posterior centro-temporal region along with right anterior temporal slowing. MRI of brain showed features of right mesial temporal sclerosis (Right temporal horn apparent dilatation, decreased hippocampal volume and punctuate hyperintensity in hippocampus) and right temporal lobe hypoplasia along with large anterior temporal arachnoid cyst (4x4x5cm) without mass effect.
He underwent right amygdalohippocampectomy plus standard anterior temporal lobectomy with excision of arachnoid cyst. Postoperatively, he recovered uneventfully. He was put on carbamazepine 100mg tablet thrice daily after operation. He is seizure free for last three month with carbamazepine. He has no clinical or perimetric visual field defect.


Our patient, a 25-year-old male, presented with complaints of cough with minimal expectoration for the past four months followed by fever for the past four weeks, which was low-grade, continuous, and associated with a weight loss of around 10 kg and loss of appetite. He also had right hypochondrial pain, which was non-colicky, non-radiating, not associated with any bowel symptoms and not associated with jaundice. He was on treatment from another hospital where he was started presumptively on anti-tubercular therapy (isoniazid, rifampicin, pyrazinamide, ethambutol, and pyridoxine) in appropriate doses, in view of right pleural effusion. He was taking the medication regularly for four weeks before he presented to our hospital. There was no history of tuberculosis in the past or any case of active pulmonary tuberculosis in the family.
Examination revealed a thin young man, afebrile, with normal pulse, blood pressure, and respiratory rate. He had pallor, with decreased air entry in the right infrascapular and infra-axillary area, with tender hepatomegaly about 4-5 cm below the right costal margin. The rest of the general physical and systemic examination was essentially normal.
His initial investigations revealed a hemoglobin of 10.6 gm/ dL, leucocytosis (TLC = 24,000/cumm) with neutrophilia, with normal platelet count. His ESR was 60 mm in the first hour by the Wintrobe's method. Serum LDH was 798 U/L. The remaining biochemical parameters were essentially normal. The enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV) was non-reactive. His chest radiograph revealed right pleural effusion. Analysis of pleural fluid showed 500 cells with a differential count of 80% lymphocytes and 20% polymorphs. The pleural fluid sugar level was 50 mg/dl and protein level was 3.2 gm/dl. Serum protein was 6.0 gm/dl. The Adenosine Deaminase (ADA) level in the pleural fluid was 48 U/L (Units/Liter) (Normal: <30 U/L, 30-60 was equivocal and >60 was considered highly suggestive of tuberculosis). Pleural fluid was negative for malignant cells and did not show any organisms either. The sputum was negative for acid fast bacilli. Ultrasonography of the abdomen showed an enlarged liver with multiple hypoechoic areas of variable sizes in both the lobes of the liver with minimal ascites. The rest of the abdominal ultrasound was essentially normal.
An initial impression of multiple liver abcesses with right-sided exudative pleural effusion (pleural fluid protein/ serum protein > 0.5) was made. Tubercular etiology was considered in view of long history and pleural fluid analysis and hence the antitubercular therapy was continued and he was started on intravenous antibiotics to cover possible pyogenic etiology of multiple liver abcesses.
An ultrasound-guided tap from the hypoechoic liver lesions was done and the aspirated fluid was sent for cytology, acid fast bacilli staining, Gram staining, and culture for bacterial pathogens.
Even as the above reports were expected, he began developing breathlessness along with abdominal distention, which on clinical examination was actually an enlarging liver. Repeat blood investigations revealed anemia (Hb = 10.2 g/dL) with leucocytosis (TLC = 22,800/cumm) with neutrophilia. The serum LDH increased to 1098 U/L. The rest of hematological and biochemical parameters were essentially normal. A repeat chest radiograph was done, which showed mediastinal widening with homogenous opacity in the left upper zone, along with the pre-existing right pleural effusion. A repeat pleural fluid analysis was negative for malignant cells. Bone marrow aspiration and biopsy were done. Contrast enhanced commuted tomography (CECT) of the chest and abdomen and a guided biopsy from the left lung mass were planned.
The cytology of the liver lesion aspirate yielded cells suspicious of being round cell tumor. The liver lesion aspirate showed no acid fast bacilli and on culture grew no pathological organisms. CECT of the chest and abdomen performed subsequently showed nodular lesions in the mediastinum and left upper lung, along with hepatomegaly with multiple hypodense lesions scattered within it []. A CECT-guided biopsy of the left lung mass was done, which revealed a cellular tumor comprising of nests and groups of polygonal-to-spindle-shaped pleomorphic cells, separated by fibrous septa, having a high nuclear to cytoplasmic ratio, high mitosis, and some areas of necrosis []. On immunohistochemistry, the tumour cells were negative for LCA (Leucocyte Common Antigen), strongly expressed Cytokeratin, Vimentin, Desmin, Neuron-specific enolase, and Epithelial Membrane Antigen (EMA), suggestive of DESMOPLASTIC SMALL-ROUND-CELL TUMOR []. Bone marrow aspirate and biopsy received subsequently were essentially normal.
On being told about the illness and its prognosis, the patient and his family members decided against getting any chemotherapy or surgery and also declined to get polymerase chain reaction (PCR) done for detection of t(11;22) (p13;q12) translocation. He left against medical advice and expired after a few weeks in his native village.


A 38-year-old man, nonsmoker presented with the history of multiple episodes of hemoptysis of 18 months duration. With each episode of hemoptysis he coughed out 50–100 ml of blood. He had no symptoms of fever, breathlessness, chest pain/discomfort, hoarseness of voice, difficulty in swallowing or weight loss. He had been previously treated with antitubercular treatment on one occasion in the past 18 months with an empirical diagnosis of pulmonary Koch's. Since he did not have any clinical or symptomatic benefit with the antitubercular treatment, his anti-Koch's drugs were stopped after a period of 4 months. He had history of recurrent oral ulceration treated as aphthous ulcers multiple times. He also had history of recurrent genital ulceration.
On examination, he was afebrile, had no lymphadenopathy and had no signs of pulmonary disease. He was found to have multiple oral ulcers on the lower lip and a healed ulcer on the tongue. He had a scrotal ulcer measuring 2 × 2 cm [].
On investigation, he was found to be severely anemic (hematocrit – 18) with total WBC count of 8500/cumm. His platelet counts were normal, so were his bleeding and clotting parameters. Peripheral blood smear was suggestive of microcytic hypochromic anemia. Sputum examination by gram stain and Ziehl–Neelsen stains were negative. His serum ANA, ANCA, RA factor, VDRL, and TPHA were reported negative. His chest X-ray PA view showed right hilar mass []. Ultrasound abdomen was normal. Biopsy of the oral ulcer was done which showed spongiosis and features of acute inflammation. A fiber-optic bronchoscopy was done to evaluate for the source of hemoptysis. Bronchoscopy revealed extrinsic compression of right intermediate bronchus however no focus of bleed was found. Contrast CT scan of the chest was done which showed marked thickening of the right pulmonary artery along with aneurysmal dilatation with thrombus in situ extending along the descending branch of the left main pulmonary artery proximally []. Pathergy skin test was negative. Ophthalmic examination excluded eye involvement. Since our patient had oral and genital ulceration with features of systemic vasculitis, a diagnosis of Behcet's disease was made. He was started on pulse dexamethasone and cyclophosphamide. He has been clinically stable with no further episodes of hemoptysis after 6 months of treatment. He was subsequently lost for follow up.


We examined a 33-year-old male with progressive painless decrease in vision and nystagmus since childhood and no history of night blindness. His best corrected visual acuity was 20/80 with 0.00 diopter sphere (D Sph) / -4.00 diopter cylinder (D Cyl) X 10° in the right eye and 20/60 with 0.00 D Sph / -3.50 D Cyl X 170° in the left eye respectively. Color vision was normal on examination with Ishihara plates. A positive history of similar abnormality was obtained in his sibling (elder brother had similar abnormality). There was no history of consanguinity. Slit-lamp examination was normal and iris did not show any transillumination defect. Intraocular pressure using applanation tonometry was 15 mm of Hg in both eyes. Fundus examination showed macular vascularization, macular hypoplasia and hypoplasic optic nerve head with diffuse retinal pigment epithelial atrophy and prominent choroidal vessels in both eyes. Patient was clinically diagnosed to have foveal hypoplasia. A genetic analysis revealed an X-linked recessive inheritance pattern.
SD-OCT (Copernicus, Italy) was done in both eyes using asterisk scan protocol (7 mm length scan, 6 B-scans with 2743 A-scans per B-scan). SD-OCT was recorded with the eyes in the null zone, to overcome nystagmus. The scan location was assured by positioning the asterisk scan acquisition pattern over the foveal area and this location was assured as the scan passes through the fovea in the fundus image obtained in SD-OCT. The scan revealed absence of foveal dip, preservation of the inner retinal layers with thickened fovea, 270 microns in the right eye and 245 microns in the left eye. The qualitative and quantitative characteristics of the retina were similar in macular and peripheral areas (2° and 7° respectively). The small elevation of the inner segment/ outer segment (IS/OS) junction was absent in the fovea which is otherwise present in normal eyes.
Microperimetry (MP1, Nidek Technologies, Padova, Italy) using 33 stimuli in the central 20° of the macula and 4-2 threshold strategy revealed reduction of retinal sensitivity in the central retina. Mean retinal sensitivity was 7.2 dB in the right eye and 14.2 dB in the left eye. Relative scotoma was present in both eyes at the foveal area. Fixation was relatively unstable in both eyes, with abnormal fixation location (central fixation in the right eye and eccentric fixation in the left eye). Average eye movements during the microperimetry were 1.27°/sec in the right eye and 1.49°/sec in the left eye. Figs. and describe the color fundus photography, microperimetry and SD-OCT findings in our case. Fundus Autofluorescence (FAF) imaging with confocal scanning laser ophthalmoscope (Heidelberg Retina Angiograph, HRA II, Heidelberg Engineering, Heidelberg, Germany) did not show the typical foveal darkening in both eyes [].


A 30-years-old lady (P2L2) with last childbirth 7 years back presented with amenorrhea and anorexia of 1-year duration. She denied any history of abdomen pain, weight loss, chronic cough or fever. Her menstrual history was normal prior to onset of symptoms. On examination, she was found to have a mobile, non-tender lump in the suprapubic region. Gynecological evaluation revealed a healthy cervix with retroverted uterus. A mass of 22-24 weeks size pregnancy was felt separately from the uterus in the anterior fornix. Groove's sign was positive.
Routine investigations were within normal limits. Serum CA-125, alpha-fetoprotein, carcino-embryonic antigen and β-human chorionic gonadotrophin levels were normal. Ultrasonography of the pelvic region revealed a complex left adnexal mass measuring 12.7 × 9 × 11.6 cm with central solid and cystic areas with calcification. Computerized tomography scan of the abdomen was suggestive of a neoplastic lesion of the left ovary, with a differential diagnosis of teratoma and ovarian carcinoma. The patient was taken up for staging laparotomy. Intra-operatively, resected ovarian tumor was sent for pathological examination. On macrosopy, the tumor measured 18 × 11 × 8 cm in size, encapsulated with a smooth external surface. The cut surface was solid with small cystic spaces and yellowish in appearance. Scrape smears were prepared and the specimen was also processed for frozen section.
On microscopy, the smears were hypercellular with cells arranged in a follicular and trabecular pattern. The cells were also seen dispersed in loose cohesive sheets with focal tight micro-follicular pattern occasionally traversed by delicate vasculature. Individual tumor cells were small to moderate sized, round to oval with occasional large cells having high nuclear: cytoplasmic ratio. The cytoplasm was delicate, with occasional fine vacuoles. The nuclei were central, round to oval with fine, evenly distributed chromatin and with occasional prominent nucleoli. Focally nuclear grooves were also noted. Wisps of metachromatic stroma in between tumor cells and cellular follicular arrangements reminiscent of Call-Exner bodies with central metachromatic stroma were present [Figure –].
A diagnosis of GCT was offered, which corroborated with frozen section examination [].
The patient underwent right-sided oophorectomy with bilateral lymph node dissection. The contralateral ovary was preserved.
Subsequent formalin-fixed paraffin-embedded (FFPE) sections confirmed the above findings and diagnosis of GCT [].


A 30-year-old systemically healthy male patient presented with a chief complaint of growth in the mouth involving lower-left back teeth region of the jaw. The patient had noticed a small painless growth about few years back. There was a very gradual increase in size, which led to discomfort while eating as the extent of growth had reached the occlusal plane [] since 3 months. Patient also complained of interference of growth while chewing and food lodgement between molars. Patient complained purulent discharge and constant dull pain in the same region. There was no history of intake of any hormonal supplements, but the patient was given antibiotics by some dentist for the same.
The extraoral examination did not reveal any facial asymmetry. Lymph nodes were not palpable. Intraoral examination revealed a solitary diffused growth, pale bluish red in color, measuring around 2×1.5 cm seen in the interdental region in relation to the left first molar and second molar region that did not extend lingually []. The superior surface of lesion showed indentation of the upper teeth as a result of surface ulceration. The growth was pedunculated. There was no mobility or pathological migration of any of the molars was not present. Pus discharge while probing []. Oral hygiene of the patient was fair.
The intraoral periapical radiograph of teeth 46 and 47 region revealed widening of the periodontal ligament space, marked interdental bone loss with change in the trabecular pattern of bone. Roots of the involved teeth did not show any signs of resorption []. The occlusal radiograph did not show the expansion of bony plates.
Blood examination revealed normal values. The treatment comprised of oral prophylaxis and surgical excision of the growth by gingivectomy procedure under local anesthesia.
Although many treatment techniques have been described for PG, when it is large or occurs in a surgically difficult surgically area, choosing an appropriate treatment modality can be difficult. Excisional biopsy is indicated for the treatment of PG. Except when the procedure would produce marked deformity: in such a case, incisional biopsy is mandatory. Conservative surgical excision and removal of causative irritants (plaque, calculus, foreign materials, and source of trauma) are the usual treatments[] for gingival lesions.
Here, local anesthesia 1 : 80,000 given to the patient and abscess was drained with the help of the periodontal probe. In this case, PG was associated with periodontal abscess and bone loss, so interval bevel gingivectomy was performed. The flap is designed in the manner that removed lesion form interdental papilla. Periosteum reflected and exposed the underlying bone [].
After complete degranulation and scaling, root planning angular defect was filled with G-BONE® bone alloplast (hydoxyapatite + tricalcium sulphate) [] and flap was sutured. Periodontal pack was given for facilitate healing. The biopsy specimen was sent for the microscopic examination.
Ten days later, periodontal pack and suture were removed and satisfactory healing of the gingiva was seen.
Patient revisited after 3 months. Complete healing of the operated area was observed at 3 month follow-up visit []. Radiograph also shows sign of bone fill [].
Histopathological findings in the photomicrograph shows hematoxilin–eosin-stained section showing the overlying epithelium had hyperplastic parakeratinized stratified squamous epithelium exhibiting areas of pseudoepitheliomatous hyperplasia []. Highly vascular connective tissue exhibiting numerous small and large endothelium-lined channels engorged with red blood cells. Thickened wall of blood vessels, proliferating endothelial cells and few lymphatic vessels were evident. Mixed inflammatory cell infiltrate consisting predominantly of neutrophils and lymphocytes were also seen []. There was no evidence of atypia or malignancy. The clinical and histopathological features were suggestive of PG.


A 10-year-old, 22 kg, girl presented with drooping of both upper eyelids since birth along with bilateral narrow horizontal palpebral fissure and skin tags of both lower eyelids over the medial canthus. On ophthalmic evaluation, the child was diagnosed to have blepharophimosis syndrome and was posted for sequential bilateral sling surgery.
There was history of mild breathing discomfort on climbing two flights of stairs for last 1 year. She denied any history of palpitation, chest pain, syncope, or swelling of feet. She had normal developmental milestones. On general examination, the girl maintained a chin up and head tilt backward posture along with raised eyebrows to clear the visual axis. The nasal bridge was flat. Her pulse rate was 90/ min and blood pressure was 100/65 mmHg. There was no pallor, edema, or cyanosis. On systemic evaluation, an ejection systolic murmur, most prominent over the pulmonary area, was auscultated over the precordium. Chest X ray did not show any abnormal finding and electrocardiogram (ECG) revealed right axis deviation and incomplete right bundle branch block (RBBB). Echocardiography revealed 5 mm ostium secundum ASD with a left to right shunt. Biventricular function was normal. Hematological and biochemical investigations were normal. Airway examination revealed small mouth opening and high arched palate.
The child was premedicated with oral midazolam 0.5 mg/ kg 45 minute before surgery. In the operating room, routine monitoring included pulse oximetry, ECG, noninvasive blood pressure, and end-tidal carbon dioxide and gas monitoring. Intravenous access was obtained and radial artery cannula was inserted for invasive blood pressure monitoring. Anesthesia was induced with oxygen, sevoflurane and fentanyl 40 μg. Atracurium 10 mg was injected for muscle relaxation after ability to mask-ventilate the patient was confirmed. Insertion of proseal laryngeal mask airway (LMA) size 2.5 was unsuccessful despite two attempts (classical technique and direct-laryngoscopy guided technique consecutively). Direct laryngoscopy revealed a Cormach-Lehane grade IV. However, mask ventilation was adequate and subsequently Cobra perilaryngeal airway (CobraPLA) #2 was successfully inserted. Anesthesia was maintained with oxygen, air and sevoflurane with intermittent dose of atracurium and fentanyl. Pressure-controlled ventilation was used and normocarbia maintained. The surgery lasted for 75 minutes. The patient was hemodynamically stable throughout the intraoperative period. Ringer's lactate 500 ml was infused. At the end of surgery, neuromuscular blockade was reversed with neostigmine and glycopyrrolate and the CobraPLA was removed. There was minimal blood stain on the cuff of the CobraPLA. She was shifted to postanesthesia care unit and monitored for 2 hours. Postoperative analgesia was provided with paracetamol 500mg intravenously q6hr. Postoperative course was uneventful.
She was rescheduled for surgery of the other eye after 7 days. Airway was secured by CobraPLA #2 in the first attempt. Rest of the anesthetic management was similar and uneventful. Arterial cannula was not used at that time as the clinical course of the patient was stable and we expected minimal hemodynamic variation based on our previous experience.
She was discharged home 4 days after the second surgery and advised follow-up in the cardiology department for management of the ASD.


A 36-year-old woman was admitted to SICU after resection of a hepatic mass. She was intubated, ventilated, and sedated with propofol and remifentanil infusion provided analgesia. On the second postoperative day, the abdominal packs were removed and hemostasis was achieved. She remained hemodynamically stable, was weaned from the ventilator, and extubated on day 4.
Propofol and remifentanil infusions were stopped 1 hour and 15 minutes before the extubation, respectively. After tracheal extubation, the patient was fully awake, breathing spontaneously, respiratory rate was 20-24 breaths per minute, oxygen saturation was 100%, and she was hemodynamically stable. Thirty minutes after extubation, the lumen of the central venous catheter, used for the remifentanil infusion up to 15 minutes prior to extubation, was used to infuse a plasma protein fraction bag at a rate 125 ml/hour.
Within 2-3 minutes, the heart rate of the patient decreased to 48 beats per minute, oxygen saturation dropped to 60%, she stopped breathing, and systolic blood pressure decreased to 86 mm of Hg []. She was immediately ventilated with bag-valve-mask. Atropine was given, and within 3 minutes her heart rate increased to 60 beats per minute and her blood pressure improved to 108 mm of Hg. The patient started to breathe and was fully awake after 8 minutes. Concurrently, her heart rate and blood pressure improved (72-74 beats per minute and 112-120 mm of Hg, respectively) oxygen saturation reached 100% with a respiratory rate of 22-24 breaths per minute (Oxygen supplementation 5 liters per minute was given by nasal canula). The patient remained awake, hemodynamically stable, and breathed spontaneously. She was transferred to the surgical ward on day 6 and later discharged home.


A 49-year-old man presented with a 4-year history of low back pain and referred pain in his right leg. While standing, he felt sensory sensations from the lateral side of his right upper leg to the knee and the medial side of his lower leg and ankle. Coughing and sneezing worsened these symptoms. He complained about some weakness of his leg, but he had no pain or other symptoms of his knee. Apart from a trochanteric bursitis 5 years earlier, his medical record reported no relevant details. Physical examination showed a restricted flexion of his lumbar spine, with pain at the level of the right sacroiliac joint during lateroflexion and extension of the lumbar spine. Local tenderness was noticed at the level of the right sacroiliac joint, gluteal and trochanteric region. Neurologic examination did not reveal any abnormalities: no weakness was noticed in his right leg, and the straight leg and femoral stretch tests were negative.
Plain radiographs of the SI-joint, pelvis and hip did not reveal any abnormality. Also an MRI of the lumbar vertebrae revealed no abnormality, especially no herniated disc. Laboratory data showed no abnormal blood levels. A technetium scintigraphic scan showed no increased isotope uptake in the lower spine, but revealed a hot spot at the level of the proximal tibiofibular joint (Fig. ).
At a second physical examination, local tenderness at the level of the proximal tibiofibular joint was present. There was a normal mechanical axis of the right leg and the knee function was optimal. Radiographs of the right knee showed proliferative bone formation between the proximal tibia and fibula. There was no dislocation or subluxation of the proximal tibiofibular joint, and the proximal fibula had a normal shaft without any bowing. A CT scan showed eccentric bone growth of both the proximal tibia and fibula, causing a synostosis at the edges of the bone formations (Fig. ).
An excision of the synostosis was performed with an additional peroneal nerve release. At surgery, no cartilage cap was found, indicating eccentric bone growth rather than an osteochondroma. The synostosis was removed until the fibula could move entirely free from the tibia. The peroneal nerve was not compressed by the synostosis, and it had a normal appearance.
Postoperatively, the pain at the level of the tibiofibular joint decreased, and surprisingly, the low back pain symptoms and referred pain disappeared entirely. After 2 months, he started giving soccer training again, which was impossible preoperatively. Postoperative radiographs and CT scan revealed a little remnant of the tibial bone growth, but the synostosis appeared to have been removed completely. After 7 months, he complained of pain in the fibular head again, with referred pain to the ankle. He did not complain about low back pain or other related symptoms. At the last follow-up examination at 10 months postoperatively, he was free of symptoms.


A 10 years 6 months-old girl, born on 06/02/1998, presented in the Department of Dentistry, Bhatia General Hospital, under parental guidance with a complaint of protruding upper front teeth. Her medical history was non-contributory. According to the history charting questionnaire, her mother revealed her daughter's lip biting habit while studying or reading. The habit had developed since childhood and, even after repeated counselling, efforts taken to quit the habit by her previous general practitioner proved in vain.[]
Her extraoral examination [Figures ] revealed a shortened lower facial height with a convex profile. A clinically flat mandibular plane was also noted, signifying a horizontal growth pattern, favorable for Activator therapy. The face was symmetrical and the nasolabial angle was approximately 90°.On temporomandibular joint (TMJ) examination, all movements were found to be normal. Her Visual Treatment Objective (VTO) clinically showed a marked improvement in profile guaranteeing a successful result.
On intraoral examination [Figures –], the molar/canine relation was end-on tending toward Angle's Class II relation with a partially impacted lower left canine. Clinically, the overjet measured 6 mm.
Cephalometric analysis showed the sella-nasion-point A angle (SNA) to be 82.4° and sella-nasion-point B angle (SNB) to be 76.1°. The resulting Point A-nasion-point B angle (ANB) of +6.3° suggested a posterior placement of the mandible. Upper incisor to the sella-nasion (SN) plane was 120°and lower incisor to mandibular plane (IMPA) was 110°, reconfirming upper and lower incisor proclination. The interincisal angle was 110°, confirming a convex profile. Frankfort's mandibular plane angle (FMA) of 17° suggested a horizontal growth pattern, which was very favorable for the use of a myofunctional appliance.
The Cervical Vertebrae Maturity Index showed a substantial growth potential of about 80%.
The orthopantomogram showed continuous posterior and lower borders, normally positioned condyle, a pre-requisite for myofunctional treatment, adequate alveolar bone height and good root parallelism with a partially impacted lower left canine.
The Ashley Howe's Model Analysis turned out to be 44%, revealing a borderline case for an extraction decision.
Thus, the patient was diagnosed as a case of developing skeletal Class II malocclusion, exhibiting an end-on molar and canine relation.
The treatment approach was a two-phase treatment with periodic oral prophylaxis. Andreasen's modified Activator with a lower lip pad was the appliance of choice for treating the Class II skeletal base malocclusion.[] This was finished with pre-adjusted fixed appliances using McLaughlin, Bennett and Trivisi's 0.022 slot prescription.[]
On post-myofunctional therapy [Figures and ], the facial profile straightened and the lower one-third facial height increased significantly.
Intraorally [Figures –], the molar/canine relation turned into Angle's Class I relation bilaterally.
Cephalometrically, SNA stayed constant whereas SNB increased to 79.6°,which resulted in an ANB of +2.8°, normal for our Indian population. Both upper incisor to SN plane and IMPA reduced dramatically, resulting in an interincisal angle of 128°, corroborating a decrease in the upper/lower incisor proclination. FMA increased to 22°, also adding to the near-normal vertical component of growth.[]
shows the Andreasen's Activator placed intraorally – the appliance which resulted in the above mentioned desired changes.


A 4-year-old boy was referred with sporadic bilateral ocular retinoblastoma. In the right eye, we observed International Classification of Retinoblastoma (ICRB) group E, Reese-Ellsworth Classification (R-E) group V retinoblastoma, occupying the entire globe. In the left eye, ICRB group B, R-E group IV, a retinoblastoma larger than 8 DD size mass was situated anterior to the equator without subretinal or vitreous seeding. After the immediate primary enucleation of the right eye, chemotherapy with cisplatin, etoposide, doxorubicin, cyclophosphamide and vincristine was initiated and the biopsy results showed choroid and optic nerve invasion over the lamina cribrosa but resection margin was clear. Before the third cycle of chemotherapy, we observed an increase in size of the retinoblastoma in the left eye and topotecan was added to the original chemotherapy regimen. After one cycle of the new regimen, the tumor continued to grow. Brachytherapy with I-125 was performed but there was no change in tumor size for six months then we decided to perform PBRT ().
PBRT with total dose of 46 Gy delivered in 23 fractions was performed (). One month after the end of PBRT, the tumor started to shrink, and two months later had decreased further, allowing us to perform thermotherapy with diode laser. After additional thermotherapy, six months after the end of PBRT, the tumor had regressed completely ().


A 1-year-old girl without any family history of retinoblastoma visited for evaluation of left leukocoria. There were multiple tumors around the optic disc and macula with subretinal seeding classified as ICRB group D, R-E group II in right eye and large peripapillary endophytic mass with total retinal detachment classified as ICRB group B, R-E group V in left eye. Chemotherapy with cisplatin, etoposide, doxorubicin, cyclophosphamide and vincristine was initiated, which led to the regression of multiple tumors, the reattachment of the retina and complete regression finally. Despite the application of thermotherapy to the subretinal seeding area, the lesion grew and new lesions of subretinal seeding inferior to the macula developed. We had to consider EBRT in that case and decided to perform PBRT, considering her age.
PBRT, with total dose of 46 Gy delivered in 23 fractions, was performed (). One month after completion of PBRT, the subretinal seeding had regressed completely (). The patient was tumor-free for seven months. However, vitreous hemorrhage was observed at routine examination under anesthesia after eight months of PBRT. We closely observed the patient and found newly developed small mass developed at the area of previously regressed subretinal seeding. The mass have grown rapidly to near the optic nerve during three months, so we carefully decided to enucleate the eye. Pathology result of the enucleated eye showed dysplastic retina with dystrophic calcification, consistent with near complete necrosis of tumor without invasion of choroid, anterior chamber, sclera and lamina cribrosa.


A 5-year-old boy was presented with left leukocoria. There was sporadic unilateral ICRB group E, R-E group V, massive retinoblastoma with diffuse subretinal seeding and total retinal detachment around huge mass in the left eye (). Chemotherapy with carboplatin, etoposide and vincristine was started and one cycle of chemotherapy led to reattachment of retina and significant shrinkage of tumor volume with reduced subretinal seeding. But before starting third cycle of chemotherapy there was recurrence of diffuse subretinal seeding especially around optic disc (). Instead of enucleation, which was refused by the patient's parents, we performed PBRT.
PBRT, with total dose of 50.4 Gy delivered in 28 fractions, was performed (). At the end of PBRT, all subretinal seeding had disappeared around the optic disc and peripheral retina with further reduced volume of the original tumor ().
However, three weeks later, during the examination before the planned thermotherapy to treat the residual tumor, an increased volume of the retinoblastoma and multifocal recurrence at the area around the tumor and around almost the entire ora serrata were discovered (). We decided to perform enucleation carefully after consultation with radiation oncologist and pediatric oncologist. Pathology report described there were tumor involvement of about 40% of retina containing 5% necrosis without invasion of choroid, sclera and lamina cribrosa.


A 42-year-old female reported to the Adhiparasakthi Dental College and Hospital with chief complaint of pain in the left lower back tooth for 2 weeks. The pain was dull and continuous in nature and reduced on taking analgesics. Her medical and family history was noncontributory. On intraoral examination the patient had a poor oral hygiene, generalized attrition, and grade II mobility in 36. The patient was referred to Department of Oral Surgery for extraction of periodontally affected 36. The tooth extraction was done by intra-alveolar technique. The extracted tooth showed a hard irregular mass attached to the mesial root of 36. The cavity was curetted and the wound closed primarily. The specimen was submitted for histological evaluation. Gross examination showed a noncarious left mandibular first molar with the apex of the mesial root embedded in an irregular spherical mass of tan hard tissue measuring 15 mm []. A radiograph of the specimen showed a radio-opaque mass attached to the mesial root of the teeth []. The specimen was fixed in 10% neutral formalin, subjected to decalcification in formic acid, bisected in a mesio-distal direction and then processed for light microscopic examination. Histopathology revealed decalcified section of tooth showing the physiological architecture of dentin along with a calcified tumor mass attached to the root of the teeth []. The calcified tumor mass was composed of sheets of cementum like tissue with lack of interstitial tissue []. Basophilic reversal line was seen []. After radiographic and microscopic evaluation a diagnosis of cementoblastoma was made. The treatment of choice for cementoblastoma is surgical removal. As the tooth has already been removed for dental reason no further treatment was done in this case. However, patient was asked to come for a follow-up but the patient never turned up.


A 14-year-old Korean boy presented with a 2-year history of asymptomatic multiple horizontal linear lesions on his lumbar area. His familial and personal histories were unremarkable, and he did not take any significant medications such as steroids. He denied any history of trauma on his lumbar area, obesity, rapid growth, and systemic illness. He recalled that the hypertrophic lesions arose from the atrophic bands, and the atrophic bands on the upper back occurred recently. Physical examination revealed several palpable hypertrophic yellowish bands on the lower lumbar region. The lesions at the middle level of the back showed a transitional change, each of which revealed central hypertrophic bands that joined to peripheral atrophic bands. Above those lesions, slightly atrophic shiny erythematous bands with pinkish hue were seen (). Routine laboratory tests were within normal limits.
A skin biopsy was taken from a palpable hypertrophic band. On histopathologic examination, hypertrophic collagen bundles were separated by basophilic wavy fibers in the dermis without significant epidermal changes (). Verhoeff-van Gieson staining revealed irregular clumped or fragmented elastic fibers between collagen bundles in the reticular dermis. Elongated elastic fibers with splitting of ends that looked like a paintbrush were observed (). Those results confirmed the diagnosis of LFE. In order to elucidate the role of TGF-β in the pathogenesis of LFE, we performed immunohistochemical study for TGF-β, but the result was negative ().


A 54-year old man with asymptomtic, tiny, multiple, flesh-colored papules on both cheeks () was referred to us from the cardiothoracic surgery department, where he had been treated for recurrent pneumothorax of the left lung. He did not remember the onset of the lesions, but stated that the number was increasing. He had no special medical or family history. The papules were thought to be sebaceous hyperplasia or verruca plana and a punch biopsy was performed. The specimen showed an epithelial cord and fibrous stroma in the dermis and an anastomosing band from follicular epithelium (). A diagnosis of fibrofolliculoma was made, which is the major feature of BHDS.
There was no family history of the skin lesions, lung disease, and of benign or malignant tumors. Furthermore, multiple variable-sized lung cysts were found on his chest following computed tomographic scan and a right renal cyst 2 cm in diameter was found on abdominal sonography (). Molecular analysis of FLCN was performed after informed consent from the patient. Sequencing revealed a single nucleotide frameshift duplication occurring within the polycytosine tract located in exon 11, c.1285dupC (p.H429Pfs*27), resulting in a change of the 429th amino acid from histidine to proline in the FLCN gene product. Based on this, it is possible to accurately diagnose BHDS.
His pneumothorax was treated with thoracostomy and pleurodesis, and active treatment was not required for the skin lesions, lung cysts and renal cyst. He is on regular follow-up because renal and colorectal cancer can occur in this syndrome.


A 78-year-old female presented to the department of dermatology for evaluation of a scalp lesion. Since birth, she had a solitary 3×5 cm yellow, verrucous plaque on the scalp (). She had no family history of similar lesions and had no previous relevant medical history, including history of local trauma or previous surgeries to that site. She only complained of mild tenderness in the area of the lesion. The lesion was completely excised for diagnosis and treatment.
On histopathologic examination, the epidermis showed hyperkeratosis, irregular acanthosis, and papillomatosis. Incompletely differentiated hair structures, which are typical features of nevus sebaceous, also were observed in the dermis (). On the basis of clinical and histopathologic findings, the lesion was diagnosed as a NS.
In addition, there was cystic invagination extending downward from the epidermis within the lesion. Papillary projections, lined by two rows of cells, extended into the lumina in the lower portion of the invagination (). From these findings, the cystic invagination was diagnosed as syringocystadenoma papilliferum. Moreover, basaloid epithelial proliferations, trichoblastoma-like lesions that showed PAS negative arising from the NS were observed (). There was a parakeratotic column overlying the trichoblastoma like-lesion, resembling cornoid lamella of porokeratosis (). In addition, there were fat cells in the upper dermis, which were unusual findings in NS (). The lesion was removed completely, and there were no signs of recurrence.


A 44-year-old Egyptian man presented with chest pain of 1 h duration. His electrocardiogram showed ST depression and T inversion in anterolateral leads, and his Troponin T was positive at 0.7 ng/ml. He was a nonsmoker with a history of hyperlipidemia and hypertension. The patient was not on any medications. On hospital admission, he had a normal complete blood count (CBC) including platelet count (411 × 109/l [n = (150--450) × 109/l]). His renal function was normal. He was sent for PCI after he had received a loading dose of clopidogrel (300 mg), aspirin (325 mg), and bolus dose of enoxaparin 80 mg subcutaneously. Coronary angiography revealed 90% tight stenosis of mid-left anterior descending coronary artery with fresh large thrombus and other coronary arteries were normal. Since the clot burden was large, it was decided to administer tirofiban and enoxaparin for 48 h and then perform PCI. Tirofiban was intravenously administrated at a dose of 0.4 μg/kg/min for 30 min followed by 0.1 μg/kg/min continuous infusion. CBC at 6 h was normal. After 24 h of infusion, CBC revealed very severe thrombocytopenia (platelet count, 7 × 109/l). Immunoplatelet count CD61 was 6.4 × 109/l [n = (150-400) × 109/l]). A thorough examination of the blood film under a microscope confirmed the finding of marked thrombocytopenia without evidence of microangiopathic hemolytic anemia. Accordingly, tirofiban, clopidogrel, and aspirin were discontinued. After 6 h of stopping tirofiban, the platelet count had increased to 18 × 109/l, and at 12 h it was 38 × 109/l. There was no fall in hemoglobin. There were no symptoms or signs of bleeding. He complained of right loin pain and a computed tomographic scan was negative for retroperitoneal hematoma or any other source of internal hemorrhage. The platelet count increased gradually. shows the time course of platelet recovery. At 72 h, platelet count was back to normal and the patient was started again on aspirin and clopidogrel. He underwent successful PCI with drug-eluting stent implantation to left anterior descending artery. There was no significant clot in the artery. The patient's further hospital course was uneventful, and he was discharged next day of intervention with normal hematological test results (platelet count, 362 × 109/l). His repeat platelet counts at 1 and 3 months were 302 × 109/l and 335 × 109/l, respectively, while taking aspirin and clopidogrel.


AM, a 45-year-old was admitted for alcohol de-addiction. He had a history of alcohol dependence for 12 years and dysthymia for 7 years. Off and on, he was on escitalopram 10-20 mg for past one year. At admission (day 1), all his physical parameters were within normal limits except for bilateral fine tremors of hands (post-detoxification diagnosed by neurologist as essential tremors) and a mild hepatomegaly (with fatty change on ultrasound). Alcohol detoxification was done using lorazepam 8 mg/day, tapered off between days 4 and 22. Escitalopram (20 mg/day) was continued and for tremors long-acting propranolol (40 mg/day) was added on day 1, increased to 80 mg on day 24, and 120 mg on day 29. For persisting sleep initiation disturbance, he was prescribed zolpidem (10 mg) on day 13, increased to 15 mg and 20 mg on days 17 and 18, respectively. Persistence of sleep problems led to addition of mirtazapine 7.5 mg at bed time from day 21. His sleep normalized, and on day 24, prescription instructions were revised to initiate taper-off of zolpidem from 20 mg/day at the rate of 5 mg every fourth day. On day 36, about 20 hours after the last dose of zolpidem, he was observed to be pacing in the ward and misrecognizing the treating staff. He became agitated, refused medication, and soiled his clothes. He was disorientated to time and place, and refused to change soiled clothes. His delirium rating scale score was 29.[] All medications were stopped and his vitals were monitored. He was afebrile with normal respiratory rate. Blood pressure varied from 130/90 to 160/110 mmHg, and pulse from 84 to 100 beats per minute. Neurological and fundus examination were normal. Arterial blood gas analysis, ECG, full blood count, blood sugar, serum electrolytes, liver function test, blood urea, serum creatinine, and urine analysis were within normal limits. Over next 24 hours, his condition persisted despite haloperidol being increased from 0.5 mg to 2 mg daily.
A review of medication over past 72 hours revealed an inconsistency between the prescription order and the actual administration of zolpidem. On day 24, zolpidem prescription was written in two brand names and either one was to be administered depending on availability. The nurse, however, administered both the brands concurrently resulting in doses of 25 mg and 20 mg instead of 10 mg and 5 mg on days 28 to 32, respectively. On day 35, when zolpidem was to be omitted (from 5 mg/day), the nurse realized her mistake and reduced the ‘additional’ zolpidem from 15 mg to 7.5 mg, making the actual dose reduction from 20 mg to 7.5 mg []. After this discovery, lorazepam 1 mg twice daily (b.i.d.) was initiated. Over next two days, delirium subsided completely. After proper relapse prevention counseling, AM was started on disulfiram and later discharged.


A 56-yr-old man with liver cirrhosis caused by the hepatitis B virus visited our hospital on December 30, 2008 for evaluation of weakness in his lower legs of 1-week duration. He first experienced discomfort and myalgia in his lower legs, which worsened over time and made him unable to walk. He had undergone a percutaneous coronary intervention 10 yr previously at the local hospital and had taken simvastatin (20 mg/day) regularly since then. He was diagnosed with Child-Pugh C cirrhosis due to hepatitis B virus a year ago and followed-up at the same hospital with stable liver function in a compensated state. Ten days before visiting our hospital, he was switched from simvastatin to fluvastatin (20 mg/day) by his doctor, as the patient was concerned about his liver disease. In addition to fluvastatin (20 mg/day), his medications included aspirin (100 mg/day), betaxolol (10 mg/day), candesartan (16 mg/day), lactulose (30 mL/day), and entecavir (0.5 mg/day) without diuretics or fibrates. His medical history was significant for no alcohol use and 10 pack-years of smoking, but he had quit smoking 10 yr previously.
The patient's blood pressure was 122/76 mmHg, his temperature was 36.8℃, his pulse was 63 beats/min, weight was 98 kg, and his body mass index was 29.8 kg/m2. He had mild tenderness of the lower extremities but all other assessments were unremarkable. Laboratory findings included sodium 125 mM/L, potassium 5.1 mM/L, chloride 97 mM/L, phosphorus 3.1 mg/dL, CO2 24.4 mM/L, serum creatinine 1.0 mg/dL, blood urea nitrogen 21 mg/dL, glucose 176 mg/dL, calcium 8.3 mg/dL, phosphorus 3.1 mg/dL, aspartate transaminase 1,303 IU/L (normal < 40), alanine transaminase 354 IU/L (normal < 40), alkaline phosphatase 145 IU/L (normal 40 to 120), total bilirubin 4.6 mg/dL, direct bilirubin 1.4 mg/dL, protein 8.0 g/dL, albumin 2.7 g/dL, and uric acid 3.5 mg/dL. His hematocrit was 38.5%, platelet count was 80,000/µL, and white blood cell count was 11,100/µL, with 18.5% lymphocytes, 69.7% polymorphonuclear leukocytes, 10.% monocytes, 1.4% eosinophils, and 0.2% basophils. His prothrombin time (PT) was 1.78 INR (40.7%) and activated partial thromboplastin time (aPTT) was 36.5 seconds. His total serum creatine kinase (CK) was 36,804 IU/L (normal 50 to 250 IU/L) and his CK-MB was 157.0 ng/mL (normal < 5 ng/mL). Thyroid function test was within normal limits. Urinalysis revealed no red or white blood cells, positive dipsticks for protein, occult blood, bilirubin and urobilinogen, but negative for glucose. A 99mTc-diphosphonate scintigram bone scan showed increased uptake over all extremities and muscles of the thorax and abdomen ().
The patient was diagnosed with fluvastatin-induced rhabdomyolysis. Treatment with fluvastatin, betaxolol and candesartan was discontinued, and he received massive hydration and urine alkalization. He was treated with intravenous sodium bicarbonate (100 mEq in 1,000 mL of half-normal saline at a rate of up to 500 mL/h or 50 mEq/h) titrated to a urine pH > 6.5. He was administered a 20-80 mg dose of intravenous furosemide to maintain diuresis. The extent of rhabdomyolysis progressed despite supportive treatment. His CK concentration was increased to 166,160 IU/L, and renal failure occurred. He underwent continuous renal replacement therapy on day 7, but his renal function did not recover and hepatic function worsened, with PT prolonged to 3.8 INR (18%) and total bilirubin increased to 24.5 mg/dL (). He died due to rhabdomyolysis complicated by hepatic failure on day 15.


A 26-year-old man was admitted to our clinic with history of severe back and left leg pain. Neurological examination revealed mild plantar flexion deficit, absent Achilles reflex and positive Lasègue sign on the left. Magnetic resonance imaging (MRI) showed an extruded discal herniation at the level of L5-S1 on the left, and an intradural caudal lipoma (). We performed microsurgical subtotal lipoma excision after L5-S1 discectomy in one session. The lipoma operation was uneventful, and surgical microscope was used throughout all excision steps. The lipoma was removed partially because of adhesion to the conus medullaris. During general anesthesia recovery, the patient complained of severe burning pain in his lower extremities that increased with painless stimuli. Pain was more prominent on the right and below the knees bilaterally. Also, the patient could not move both lower extremities due to induced pain. Analgesia did not relieve his symptoms. Eventually, high dose (250 mg/day) meperidine and gabapentine (400 mg/day t.d.s.) were given after Pain Clinic consultation. His symptoms resolved totally two weeks after the operation without neurological sequelae.


A 20-year-old man was diagnosed with HHT (Osler-Weber-Rendu disease). He was known to have cyanosis since infancy, as well as multiple cutaneous telangiectasias. His oxygen saturation was 84% by pulse oximetry. His father also had the same cutaneous telangiectasias without cyanosis. The patient had been catheterized in the past and was found to have a large arteriovenous malformation in the left upper lobe, as well as a smaller one in the right upper lobe. He had required 2 partial exchange transfusions because of intense polycythemia with a hematocrit of 73% and hyperviscosity symptoms (headaches and blurred vision). At the age of 24, cardiac catheterization was performed and an attempt was made to place 2 Gianturco-Grifka vascular occlusion devices simultaneously in the main feeding artery. Because of the large size of the proximal feeding artery, it was impossible to stabilize the devices so as to achieve complete occlusion. One device dislodged to a small branch of the fistula without affecting significantly the blood flow to the remaining branches and the other was retrieved back in the sheath. Despite heparin administration, the patient developed deep vein thrombosis of the right leg and required long-term anticoagulation. He remained cyanotic and returned for repeat catheterization at 26 years of age. After demonstration of the fistula with selective injection in the feeding artery (), a 10 Fr long Mullins sheath was placed through the left femoral vein and two Cardio-Seal atrial septal defect occlusion devices (23 and 27 mm respectively), were deployed in the proximal feeding artery of the arteriovenous fistula which came off of the left upper lobe branch ().
These devices achieved near complete occlusion of the fistula (), except for some small branches, which arose very proximally, after the take-off of the feeding artery, near the left-upper lobe branch. The oxygen saturation increased immediately from 85 to 94%, and stabilized later at 90-92%. The patient remained stable and has been followed clinically for 2 years after device placement. Follow-up catheterization has not been performed yet, although it is planned because of the well-known progressive nature of the arteriovenous malformations in Rendu-Osler-Weber disease and the possibility of recanalization after initially successful embolization.


A 15-year-old female presented with cyanosis and clubbing. She also had exophthalmos, scoliosis and mild mental retardation. The chest x-ray showed widening of the mediastinum and marked scoliosis. A 2-D echocardiogram showed significant dilatation of the ascending aorta above the sinuses of Valsalva. Although she did not have excessively long extremities and arachnodactyly, a variant of Marfan's syndrome versus homocystinuria was considered. Because of the severe cyanosis and the aortic aneurysm, she underwent cardiac catheterization. The pulmonary veins and left atrium were entered through a patent foramen ovale. The systemic arterial saturation, as well as that of the left lower pulmonary vein was decreased (80%) and was not changed after administration of oxygen. Pulmonary arterial pressure was mildly elevated at 35/10, with a mean 20 mmHg. The Qp:Qs ratio was markedly decreased at 0,66. Aortography revealed a very large aneurysm of the ascending aorta sparing the sinuses of Valsalva, with a transverse diameter of 7,7 cm and mild aortic insufficiency. Pulmonary angiography showed a large saccular aneurysm arising from the left lower pulmonary artery and connecting directly to the left lower pulmonary vein. There was no significant atrial level shunt. The right pulmonary artery was normal. Because of the large size of the arteriovenous fistula and the coexisting aortic aneurysm, transcatheter embolization was not attempted. The patient was taken to the operating room where a left lower lobectomy was performed. The ascending aorta was replaced by a Hemashield graft above the sinuses of Valsalva. Postoperative recovery was uneventful and her oxygen saturation normalized to 99%. She has been followed for 6 years and remains in relatively good condition although she requires orthopaedic therapy for her scoliosis. The ascending aortic graft has calcified, but there is no progression of the mild aortic regurgitation.


A 4-year-old female was diagnosed soon after birth with complex cyanotic congenital heart disease. Chest x-ray showed situs inversus of the abdominal viscera, dextrocardia and decreased pulmonary vascular markings. Two-dimensional echocardiography and cardiac catheterization revealed the following anatomy: situs inversus of the abdominal viscera; hepatic venous drainage directly to the left-sided atrium; left-sided inferior vena cava, continuing through a left-sided azygos vein to a left superior vena cava that connected to the left-sided atrium; right superior vena cava connecting to the coronary sinus, which drained to the left-sided atrium; drainage of the pulmonary veins to the right-sided atrium; common atrioventricular valve; unbalanced AV canal with hypoplastic left ventricle; supero-inferior ventricles; double outlet right ventricle with anterior and rightward aorta and posterior and leftward pulmonary artery; severe subpulmonary and valvar pulmonary stenosis.
She had moderate cyanosis, but was relatively well and thriving until 4 years of age. Subsequently, she developed progressively severe cyanosis and polycythemia, with a hematocrit of 60% and oxygen saturation of 65-70%. She had moderate clubbing with a harsh IV/VI systolic ejection murmur at the right upper sternal border. Cardiac catheterization at 4 years of age showed the above-described anatomy, with low pulmonary artery pressures (mean of 10 mmHg). It was decided to proceed with bilateral anastomoses of the superior venae cavae to the pulmonary arteries which also connected the inferior vena caval flow to the pulmonary circulation through the left azygos vein (Kawashima operation), thus leaving only the hepatic veins draining to the systemic circulation. The main pulmonary artery was disconnected from the right ventricle. The postoperative oxygen saturation was 94%. Over the next 2 years, there was a progressive drop in the arterial oxygen saturation, to 79%. A repeat cardiac catheterization was performed which showed well functioning bilateral cavopulmonary anastomoses, normal central pulmonary arteries, with a diffuse opacification of the lung parenchyma and rapid appearance of contrast in the pulmonary venous circulation, with opacification of large pulmonary veins at the same time with central pulmonary arteries, suggesting diffuse pulmonary arteriovenous communications (). The pulmonary artery pressure was normal. She was reoperated, and the hepatic veins were anastomosed through a 22 mm Gore-tex tube graft to the right pulmonary artery. Immediately postoperatively the saturation increased to 85-90%, and over the next six months, it increased further to 97%, suggesting resolution of the pulmonary arteriovenous communications.


A 56-year-old male patient went under follow-up for moderately differentiated adenocarcinoma of upper lobe of left lung (T1N0M0) since January 2009. He had undergone left upper lobectomy with lymph nodal clearance in January 2009. He had received three cycles of adjuvant chemotherapy with gemcitabine and cisplatin from April to June 2009. Further, chemotherapy was precluded by neutropenia initially and then the patient defaulted.
The patient again reported in October 2010 for complaints of pain in the abdomen and the lower limbs. On examination, dilated superficial abdominal veins with features of IVC obstruction were seen. The patient was started on symptomatic and supportive care. The patient was referred for F18-FDG PET/CT.
Whole body PET/CT imaging was done after intravenous administration of 370MBq (10 mCi) of F18-FDG. The images were acquired after 45 minutes of uptake period.
PET/CT did not reveal any FDG avid lesion at the site of the primary. A large FDG avid left suprarenal mass measuring-10.6AP × 9.9TR × 13.0CC cm was noted [] with extension of tracer uptake along dilated left adrenal vein into the inferior vena cava []. The tumor thrombus was extending from the level of 3rd lumbar vertebra in the inferior vena cava up to the level of right atrium []. Left kidney was displaced inferiorly by the suprarenal mass. FDG avid lesions were also noted in segment I, II, IV and VII of the liver [].


A 55-year-old male patient weighing 65 kg was scheduled for an elective mitral valve replacement and tricuspid annular plasty for treatment of severe mitral and tricuspid valve regurgitation. His significant past medical history included atrial fibrillation and diabetes mellitus. His current medications included warfarin, glimepiride, torasemide, verapamil, and isosorbide dinitrate.
Warfarin was discontinued 7 days before elective surgery. Routine preoperative laboratory tests revealed a normal clotting screen with an activated partial thromboplastin time (aPTT) of 37.8 s, a prothrombin time (PT) of 1.08 INR and a normal platelet count of 168,000/mm3.
On the day of surgery, an epidural catheter was placed at the T7-8 level after the induction of general anesthesia for postoperative analgesia. After the patient was positioned in the right lateral decubitus position by an assistant, the epidural space was identified using a paramedian approach and a loss of resistance to air by a 16 G Tuhoy needle at T7-8 level. An epidural catheter was threaded 3 cm into the epidural space and secured over the patient's back. These procedures were atraumatic, with no bloody tap or blood in the catheter. The patient was anesthetized for surgery with total intravenous anesthesia using midazolam and fentanyl. Intravenous unfractionated heparin 19,500 U (300 U/kg) started on for cardiopulmonary pump 130 minutes after epidural catheterization. The activated clotting time (ACT) was monitored and maintained adequate level to keep the anticoagulated state during the operation. Upon completion of the valve replacement and intravenous protamine (293 mg) was administered to reverse the effect of heparin. A continuous epidural infusion of preservative free sufentanil 500 µg and 0.18% ropivacaine 250 ml was used for postoperative analgesia with baseline infusion rate 1 ml/hr, bolus 2 ml and lockout time 15 minutes.
On the examination 1 hour after surgery while conscious in the intensive care unit, the patient showed clear mental status and neurologically intact movement. His immediate postoperative PT (INR) and aPTT were 1.52 and 42.2 s, respectively, and his platelet count was 77,000/mm3. After 16 hours from the end of cardiac surgery, the patient complained of flaccid paraplegia with sensory deficit and back pain. There was oozing at the insertion site of the epidural catheter. At this time, his aPTT was 53.3 s, and his PT was 1.30 INR. An initial examination revealed posterior midthoracic tenderness to palpation, flaccid paraplegia, loss of deep tendon reflexes in the lower extremities and anus, hyperesthesia in bilateral T5-7 dermatomes, and anesthesia of both lower extremities. Immediately, he was taken an emergency magnetic resonance imaging (MRI) of the spine, which revealed an epidural hematoma at the T5-6 level (). The patient underwent an emergency T5-T7 laminectomy and the spinal epidural hematoma was completely removed within 6 hours after the symptoms first developed. Seven days after laminectomy, the patient had almost fully regained his motor and sensory functions, but he still experienced dysuria and right foot drop. Six months after laminectomy, his motor and sensory functions had fully recovered, while dysuria was slight.


A 45-year-old male with severe acute pancreatitis (SAP) was referred to the ICU with acute physiology and chronic health evaluation (APACHE II) score 20 and intra-abdominal hypertension. About 5 years back, he had had a TBI (Marshal grade III)[] with significant intracranial bleed. It was a focal lesion and he had a complete clinical recovery with conservative management. After surgical necrosectomy performed in the sixth week of SAP, there was fresh bleeding from the drains requiring an urgent re-exploration. His haemoglobin dropped from 10 gm% to 6 gm% within few hours, and his prothrombin time was 6 s prolonged, with a platelet count of 60,000/cmm. General anaesthetics included isoflurane, besides fentanyl, thiopental, midazolam and vecuronium bromide. The intraoperative period was uneventful from the point of adverse events like hypoxia, hyperventilation, severe hypotension (mean blood pressure <60) or hypertension. However, he required significant volume resuscitation (about 5 L in 2 h) comprising of 1.5 L of crystalloid solution, 0.5 L of colloid solution, 4 units of packed red blood cells (PRBC), 4 units of fresh frozen plasma (FFP), 10 units of random donor platelet and 10 units of cryoprecipitates, to maintain haemostasis and haemodynamics. Soon after shifting him back to the ICU, he developed sinus bradycardia with hypertension (150/90 mmHg). Pupillary examination revealed left-sided pupillary dilatation with minimal reaction to light. The right pupil was normal. Difference in pupillary size was more than 3 mm. Fundoscopy did not reveal papilloedema. His acid base and electrolytes were normal. A negative pilocarpine test ruled out Adie syndrome and he did not exhibit features of Horner's syndrome. Absence of any appreciable localizing sign was suggestive of a nonvascular major intracranial event as the cause of acute anisocoria. In the background of existing coagulopathy, intracranial haemorrhage was kept as a probable diagnosis. But, an urgent computed tomography scan revealed an old, healed haemorrhage in the left fronto-temporal region as sequelae to the past TBI and no new organic lesion or bleed []. Soon thereafter, interventions were started to decrease ICP and 1 g/kg intravenous bolus mannitol and 20 mg frusemide were administered. Repeat doses were given every 4-hourly for 24 h and he was nursed in the head-up position. Within 12 h of initiating measures for ICP reduction, hypertension and bradycardia resolved and his left pupil started to decrease in size and reaction to light was restored. After normalization of pupillary size and response to light, mannitol and frusemide were stopped. On day 3 of the event, he was fully conscious and his trachea was extubated.


A 20-year-old primigravida at 38 weeks of gestation presented at the emergency department with a hypertensive crisis. An urgent lower segment caesarean section (LSCS) was planned by the obstetrics team and anaesthetic consultation was sought for it. Her antenatal history revealed spontaneous conception with no complications in the first trimester. During her antenatal visit in the 18th week of gestation, hypertension was detected without any other feature of pre-eclampsia. She was advised Methyldopa 500 mg thrice daily and Nifedipine 10 mg twice daily, but she did not take them regularly. She had occasional headache and palpitation, but did not seek any medical advice. In the 37th week, she started having frequent episodes of headache and dizziness. She resumed her anti-hypertensive and was admitted for LSCS at a private hospital where her blood pressure (BP) was found to be 220/130 mm Hg. Her ultrasound abdomen revealed an adrenal mass lesion (5.6 × 3.9 cm cystic lesion) suggestive of a phaeochromocytoma and she was referred to our tertiary care facility. On admission, she had palpitation and headache, her blood pressure was 200/120 mm Hg, heart rate 110/min with no evidence of non-reassuring foetal heart rate pattern. Her investigations done at the previous hospital revealed Hb 10 gm/dl, platelets 3.52 lakhs/mm3, and normal renal and liver function tests. Electrocardiogram suggested sinus tachycardia and left ventricular hypertrophy with no ST-T changes. Urine dipstick examination showed proteinuria (3+) and glycosuria, and fundoscopy suggested grade 4 hypertensive retinopathy with early papilloedema. Subsequent foetal cardiotocograph revealed nonreassuring heart rate pattern. The decision for an emergency LSCS was made by the obstetrician.
Informed high risk consent was obtained from the patient in view of recently diagnosed phaeochromocytoma, an unoptimised hypertensive crisis, and need for postoperative stay in the intensive care unit. Anti-aspiration prophylaxis was given with ranitidine 150 mg intravenous (i.v) before shifting to the operation theatre. Emergency arrangements including invasive monitoring and vasodilator infusions were made in the operating room. The paediatrician was informed regarding planned modified rapid sequence induction with fentanyl and possible need for neonatal resuscitation. Oxygen supplementation was continued. Pre-induction, right radial artery and internal jugular vein were cannulated for haemodynamic monitoring after local anaesthetic infiltration and aseptic precautions. Her baseline central venous pressure was16 mm Hg and BP was 186/120 mm Hg, with a heart rate of 108/min. Magnesium sulphate 2 g i.v. was administered. Pre-oxygenation was started. Fentanyl 100 mg was given i.v., followed by two more boluses of 50 mg each at 3-min intervals. Her BP decreased to 160/100 mm Hg. Subsequently, lignocaine 80 mg i.v. was administered. Anaesthesia was induced with thiopentone titrated to loss of eyelash reflex and succinylcholine 100 mg was given to facilitate tracheal intubation. Cricoid pressure was maintained throughout induction till bilateral air entry was confirmed. BP remained 150/92 mm Hg at intubation. Anaesthesia was maintained with O2 and N2O (50% and 50%) with isoflurane, to attain a minimum alveolar concentration (MAC) of 0.7, and atracurium in bolus of 7.5 mg when the patient had respiratory efforts. After the delivery of the baby, isoflurane was decreased and N2O increased to 60%. A 2.25 kg weight baby was delivered after 6 min which cried immediately at birth with an Apgar score of 9. Oxytocin, 10 units, was administered as a slow infusion. Nitroglycerine (NTG) infusion had to be started immediately after delivery at 1 mg/kg/min as the BP increased to 200/126 mm Hg and was titrated to a mean arterial pressure of 95-100 mm Hg approximately. Residual neuromuscular block was reversed with neostigmine and glycopyrrolate. Diltiazem 10 mg was administered before extubation to blunt the response. The patient was awake and comfortable after extubation and NTG infusion continued while the patient was shifted to the intensive care unit (ICU) for postoperative monitoring and haemodynamic management. Urinary catecholamine levels were sent for confirming the diagnosis. She was started on prazosin 2.5 mg thrice daily and NTG infusion stopped. She was shifted to ward and prazosin dose titrated as per the endocrinologist's opinion to 3 mg four times a day and atenolol 50 mg once daily was added after 2 weeks. Meanwhile, the magnetic resonance imaging (MRI) of her abdomen revealed a multiloculated solid cystic lesion (T2 hyperintense) in right adrenal gland and she was referred to the surgical unit for resection of phaeochromocytoma after a week of beta blockade as her blood pressures were optimised by that time.


The patient was a 14-year-old boy who was a known case of CGD, with osteomyelitis due to Nocardia asteroides of the proximal femur with right hip pain, inability to walk, and overlying cellulitis. The mycological and bacteriological diagnosis was determined by microscopy, culture, and biochemical tests of clinical samples.
The isolate had been identified as N. asteroides by standard methods,[] the strain was negative on cellobiose, positive on glycerol, and negative on hypoxanthine and sorbitol. It was identified as a strain of N. asteroides [].
Our patient survived after treatment with trimethoprim-sulfamethoxazole (TMP-SMX) and ceftriaxone.


A 12-year-old girl with CGD presented with 1 week of painful red fluctuant swelling over the upper right neck and fever. She had noted a gradually increasing mobile mass over the right neck. CGD had been diagnosed at age 2 complicated by multiple pneumonias and liver abscesses. She had an erythematous, warm, tender, tense, fluctuant mass in the right submandibular area without other lymph node enlargement. She was admitted with a high grade fever (40°C orally). The patient had received one or two different antibiotics prior to the isolation of A. israelii like the other patiens.
The biopsy of the submandibular mass indicated chronic granuloma. Because of the possibility of actinomycosis, a culture and smear were requested. Gram stain of the purulent discharge of the lymph node revealed irregular gram-positive filamentous microorganisms. After 4 weeks, microorganisms grown on anaerobic culture media and biochemical testing confirmed A. israelii (positive glucose, mannitol and sucrose fermentation but negative hemolysis, catalase, starch hydrolysis, DNase indole and urease) []. Aspiration of her lymph node yielded A. israelii in pure culture. Leukocyte count was 8200/mm3 and sedimentation rate 86 mm/h. She was treated with intravenous levofloxacin with gradual improvement. Levofloxacin and ceftriaxone were given for a total of 4 weeks with complete resolution.


An 83-year-old man presented to the prosthodontic specialty clinic to enhance the existing maxillary complete denture and/or to make a new denture with or without implant utilization. The patient was referred to prosthodontic clinic by an undergraduate student because of the patient's continual complaint about poor retention and stability of the maxillary complete denture. The patient was healthy and independent with hypertension that was medically controlled. The existing prosthesis, which was fabricated by the undergraduate student one year earlier, included a maxillary complete denture and a mandibular transitional RPD Kennedy Class I modification 1 which were made after extraction of his mandibular incisors keeping only the two mandibular canines and the first left premolar. The patient was not happy with the existing prosthesis and was not totally satisfied with a previous denture made by his dentist four years earlier. His major complaint with the first and second prostheses was mainly that the maxillary complete denture loosens while he is speaking. Multiple chair side hard and soft relines were performed over the previous year to enhance the retention and stability of the existing maxillary complete denture. Because of patient financial limitations, he wanted to avoid implant therapy if a new denture could be fabricated with adequate retention and resistance.
Upon intraoral examination of the maxilla, the maxillary right and left buccal frena appeared as thick/multiple fibrous bands giving them a "fan-shape" and along with the labial frenum, they were considered low and located close to the crest of the residual ridge (). The vestibules were considered shallow because of the frena anatomy; otherwise, the rest of the soft tissues appeared within normal limits. The edentulous ridge appeared broad, rounded, and covered by firm soft tissue. The patient was informed about the observed frena and had been told that they could be a causative factor for his complaint and for maxillary denture looseness. Therefore, he was advised that the frena should be removed surgically by a procedure called "frenectomy" before a new denture was made. Additionally, he was told that if low satisfaction with the new complete denture continued, implant therapy would be considered. Before the patient was referred to the oral surgeon, the existing soft liners on the maxillary complete denture were removed and a new chair side hard reline (Flexacryl Hard, Lang Dental Mfg. Co, Inc., Wheeling, IL, USA) was performed. shows the significant amount of relief (labial and buccal notches) needed to accommodate the frena after the chair side reline procedure. After the hard reline, clinical evaluation of occlusion and vertical dimension for the existing removable prosthesis found to be adequate. However, during subsequent visits patient was still complaining about the maxillary denture looseness at different time of day while he was speaking.
Then the patient was referred to the oral surgeon to perform the frenectomy. At the time of appointment for frenectomy, the treating prosthodontist was available to utilize the existing denture as a stent to support and stabilize the tissue attachments in the new position. Because of the existence of broad frena and shallow vestibules, the oral surgeon decided to perform the frenectomy with the Z-plasty technique because of its usefulness in such situation where simultaneous frenum elimination and vestibule lengthening can be achieved. When simultaneous frena excision and vestibule deepening with the Z-plasty technique were completed, and after final suturing, a soft denture reline material (Coe-Comfort, GC America, Inc., Alsip, IL, USA) was placed on the existing denture and carefully inserted into the patient's mouth. A gentle border molding was performed before the denture was removed from the mouth and then the excess reline material was trimmed around the borders. According to a previously suggested protocol,, the patient was instructed to keep the denture in place for the next 24 hours including sleep time and to minimize the in-out placement of the denture during the initial stages of healing (normally the first 3 - 5 days). The sutures were removed after 7 days. During post-surgical week one and two, the initial soft denture reline material was replaced with new material twice. On the third post-surgical week, a different denture reline material was used (Coe-Soft, GC America, Inc.) and left in place for an additional three weeks., The definitive maxillary complete denture construction was initiated six weeks following the surgical frenectomy procedure.,,
The full mouth rehabilitation of the patient was achieved following the proposed treatment plan and the rendered treatment was performed according to classical recommendations in text books and literature, for delivering such prosthodontic therapy. The final complete maxillary denture had longer border extensions ( and ). Post-treatment follow-up appointments indicated that the patient was fully satisfied with the final treatment outcome. Currently, it has been over a year since the treatment was completed and the patient is functioning well with the denture.


A premature, 34-week-old, 930 g, male neonate was born by cesarean section to a hypothyroid mother. The first one month was complicated by hypoglycemia, seizures and low hemoglobin. The patient was on breast milk and also supplements. He had physiological jaundice on day 3 of life which required phototherapy. After phototherapy, his bilirubin was 4.6 mg% out of which direct component was 1.2 mg%. At the age of one and half months, the patient passed clay-colored stool and was icteric. It was negative for stercobilinogen. His obstructive jaundice work up showed raised bilirubin of 9.5 mg% with direct bilirubin of 6.5 mg% and raised alkaline phosphatase 1020 IU. The ultrasonography had normal biliary tract with no dilatation of biliary radicals. Common bile duct diameter was 4 mm. At this point, surgical opinion was sought by the pediatrician. Hepatobiliary imino-diacetic acid (HIDA) scan was not conclusive as there was uptake by liver as well as early excretion, but there was no progress of dye in the intestine during 24 hours. So, exploratory laparotomy was done keeping in mind about the biliary hypoplasia or inspissated bile syndrome.
The patient's weight was 1.2 kg when he was taken for surgery. Gallbladder was distended and there was bile on aspiration. The extrahepatic biliary apparatus was normal. Intraoperative cholangiogram suggested dye was going in the pancreatic duct, but there was no dye in the intestine with terminal dilatation of CBD []. After kocherization, longitudinal duodenotomy was done in the second part of duodenum. On duodenotomy, there was choledochocele of size of less than 1 cm in diameter []. A vertical incision was taken on the cyst. There was gush of bile []. Because of very small choledochocele, we were not able to excise the mucosal lining of the cyst nor were we able to suture incised edges and take biopsy of the wall. Also, we could not identify opening of pancreatic duct. On table cholangiogram confirmed the presence of dye in the intestine []. Later, duodenotomy was closed. The postoperative period was uneventful. His bilirubin after 2 weeks of surgery was normal and stool was normal looking. A follow-up HIDA scan after 6 months had good uptake and free flow of dye in the intestine. At 12 months follow-up, the child was asymptomatic and thriving.