An 18-year-old female suffered a headache for 2 weeks. Computed tomography and magnetic resonance image revealed a fusiform aneurysm on the lower BA trunk. Digital subtraction angiography (DSA) revealed a 7.1×11.0 mm-sized fusiform aneurysm located just below the anterior inferior cerebellar artery (AICA) (). Right side posterior inferior cerebellar artery was not seen. Both posterior cerebral arteries (PCA) and superior cerebellar arteries (SCA) filled normally. Both posterior communicating arteries (PComA) were patent and the diameter of both arteries was more than 1 mm in diameter (). For the proximal portion of the aneurysm forming a severe acute angle, flow diversion with multiple stent is difficult to enforce. Endovascular coiling of aneurysm with occlusion of dysplastic parent vessel is planned if the patient tolerate complete preoperative occlusion test.
The location of aneurysm is just distal to vertebrobasilar junction and proximal to the origin of AICA, and it is somewhat related perforating artery injury to perform a BTO at basilar artery. We planned the BTO on both vertebral arteries. BTO was performed under local anesthesia and accessible for neurological examination. At the time of the BTO, the neurological status of the patient was continuously monitored by a trained neurosurgeon that assessed speech, motor strength, sensory extinction, and level of alertness ().
Bilateral femoral artery (FA) punctures were performed and guide catheter placed in both vertebral arteries (VA). Additional right FA puncture was performed for diagnostic cerebral angiogram. The patient was anticoagulated by the intravenous injection of 5000 IU heparin. Over-the-wire microballoon (Hyperfrom, Hyperglide; ev3, Irvine, CA, USA) was used for BTO and these balloons were located at VA just around the C2 vertebra foramen (). The balloon was then inflated. Complete balloon occlusion of VA was verified by a proximal contrast injection. The BA and both AICAs were visible through the retrograde blood flow from the PComA, which were visualized by an angiographic injection in the both internal carotid artery (ICA).
Bilateral BTO of the each VA were performed over 20 min under a normotensive state. Baseline blood pressure was 109/72 mm Hg (mean arterial blood pressure, 84.33 mm Hg). The patient tolerated 20 minutes under the normotensive state and hypotension was induced pharmacologically by the intravenous infusion of nicardipine (starting dose of 5 mg/hr with 2.5 mg/hr increase every 5 min if the blood pressure did not reach the target range). After the mean arterial pressure was reduced by 20% of baseline (92/56 mm Hg; mean arterial blood pressure, 68.0 mm Hg), hypotension was maintained for 20 minutes.
Before BTO, the patient underwent a complete TCD investigation. The patient-customized TCD headset was mounted. BTO was performed with concomitant monitoring of the blood velocity and direction of the blood flow in the middle cerebral artery (MCA) and P1 segment of the PCA.
Baseline Tc-99m hexamethylpropylenamineoxime (Tc-99m HMPAO) qualitative SPECT was performed one day prior to the BTO. Another SPECT after BTO also checked. Tc-99m HMPAO was injected intravenously approximately 10 min after the balloon was inflated.
Baseline neurophysiologic monitoring steps included electroencephalography, somatosensory evoked potential, and brain stem auditory evoked potential. During the BTO, NPMs was closely observed.
DSA during BTO, the right ICA angiogram showed retrograde filling of the distal part of the BA, PCAs, SCAs and AICASs through the PComA (). The patient tolerated 20 minutes under the normotensive state and induced hypotensive state with no developing neurological deficits.
Baseline mean blood flow velocity of MCA was 74 cm/s (). The velocity during BTO dropped immediately to 48 cm/s upon balloon inflation, but returned to the baseline value (72 cm/s) within several seconds without additional treatment and was maintained during the BTO. After induced hypotension, it dropped to 65 cm/s; the patient tolerated in induced hypotensive condition. After deflation of the balloon, the velocity of the MCA recovered to the baseline value. The reduction of flow velocity was 2.7% under the normotensive state and 12.2% under induced hypotensive state.
Baseline SPECT images did not show any focal perfusion reduction (). SPECT images obtained after BTO also did not showed hypoperfusion of the cerebral hemisphere and cerebellum compare to the baseline SPECT.
During the BTO, there is no NPMs change in this patient.
Coil embolization was performed with multiple coils in the usual way. Care was taken to preserve the both AICAs, which arose close to the distal aspect of the aneurysm. After coil embolization, a left side ICA angiogram () showed regrograde filling of the BA (arrow) and both AICAs (arrowhead) through the PComA. A right side VA angiogram showed complete obliteration of the aneurysm and no filling of the BA though the VA.
The patient was discharged without any neurological symptoms. After 6 months after treatment, the patient was doing well without any focal neurological deficits.


A 16-year-old girl with β-thalassemia major presented with painless bilateral cervical lymphadenopathies for 1 month. She had been on regular transfusion since 9 months of age and was HCV positive due to regular transfusions. Her parents were heterozygotes for β-thalassemia minor. Initial evaluations including complete blood count, biochemistry tests, Epstein barr virus infection (EBV) and toxoplasmosis studies were not conclusive. white blood cells and platelets were normal. serum ferritin levels of the patient were 3,200 ng/mL and 3,000 ng/mL at two occasions. She was receiving subcutaneous desferrioxamine at least 5 nights/week as iron chelation since 2.5 years of age. After about 3 weeks of persistent cervical lymphadenopathy, computerized tomography (CT) scan of the neck and chest was performed revealing bilateral cervical and upper anterior mediastinal lymph nodes with para-tracheal involvement. Abdominopelvic imaging was normal. An excisional lymph node biopsy was performed which was compatible with Hodgkin's lymphoma, nodular sclerosis subtype. Clinical staging revealed stage 2A (spleen was considered enlarged due to thalassemia since it was not a new finding). She was scheduled to receive systemic chemotherapy with 6 alternating cycles of MOPP/ABVD followed by radiotherapy and was off-therapy thereafter. Her mother also developed colon adenocarcinoma 6 years later and her brother (who was hematologically normal) died of brain tumor 8 years later. She also received antiviral treatments including α-interferon and ribavirin for HCV and changed to be negative. Fortunately, the patient is in remission for Hodgkin's lymphoma and still on regular blood transfusion after 10 years of follow-up.


A 13-year-old girl with β-thalassemia intermedia was referred to our clinic due to hyperleukocytosis (WBC: 154,000/mm3). She was receiving hydroxyurea since 8 years of age and had been kept transfusion independent.
At presentation she had Hb 8.5-9 gr/dL, MCV 78 fL and MCH 20 pg. Hemoglobin electrophoresis revealed HbA 45%, HbF 51% and HbA2 4%. Spleen was palpated about 4cm below costal margin. Splenomegaly was evident as it was before. Due to the persistence of hyperleukocytosis (WBC: 50,000-150,000/mm3) and aggravation of anemia, a bone marrow aspiration was performed which showed hypercellular marrow with increased myeloid to erythroid ratio. Significant degrees of reticulin stain-measured fibrosis were noted accompanying marrow eosinophilia. The histopathological findings were in favor of chronic myelogenous leukemia (CML). Real time polymerase chain reaction examination for the detection of BCR-ABL translocation on bone marrow aspirate revealed positive reaction for 342 bp amplified product compatible with t(9;22)(q34;q11.2) translocation (Philadelphia chromosome). Treatment with Imatinib mesylate (Gleevec) was started at a dosage of 400 mg/day; however, she developed accelerated blastic crisis and proceeded towards acute myeloid leukemia (AML) and did not respond to chemotherapy regimens and thus died about 3 months after diagnosis.


A nine-year-old female patient reported unilateral flushing associated with ipsilateral
sweating when submitted to prolonged physical exercise. It started at the age of six,
being unaltered since then. The opposite side of the face remains pale and anhidrotic.
These skin changes could be reproduced during her examination, 30 minutes after
exercising, with flushing and sweating ().
In contrast to the patient's beliefs, the affected side is not the one with normal
vascular and sweat response, but the pale and anhidrotic side. Her medical history had
no other diseases or accidents. No birth problems (such as forceps usage) were
identified. A neurological and ophthalmological evaluation made by a neuropediatrician
and an ophthalmologist was normal (with no need for additional tests).


A 35-year-old woman presented with an abdominal wall mass that was palpable one week prior to her visit. The mass was not painful and did not show a change in size. Her past medical history was remarkable because she had undergone laparoscopic bilateral ovarian cystectomy due to endometriosis 3 years prior to her visit. Physical examination revealed a surgical scar on her left lower abdomen and an index finger tip-sized mass was palpable in the abdominal wall under the scar. It was smooth and movable, and was not tender. The laboratory findings were unremarkable.
Ultrasonography demonstrated an approximately 1.6×0.9 cm-sized hypoechoic mass in the subcutaneous adipose tissue (). An excisional biopsy was performed. The mass was well demarcated from the adjacent soft tissue by a thin fibrous capsule. The cut surface showed a yellow, glistening lobular parenchyma with areas of blood-filled cystic spaces. Histologically, the parenchyma was composed of luteinized granulosa and theca cell layers, suggesting corpus luteum of the ovary (). There was no evidence of endometriosis.
A review of the operation record of the laparoscopic surgery that was performed 3 years prior showed that the patient had 5.4×4.5×4.0 cm- and 3.2×3.0×1.5 cm-sized endometriotic cysts in bilateral ovaries with an associated dense pelvic adhesion. After a cystectomy, the specimens were fragmented and extracted using forceps through the trochar on her left lower abdomen.
The patient was discharged after the abdominal wall mass excision and was healthy with no recurrence of the lesion at a follow-up at 32 months.


Here is a case of a 5-year-old girl who reported to us 2 days after encountering trauma due to fall from an elevated platform. Clinical examination revealed a discontinuity of the lower border of the mandible on the left side. Her mouth opening was restricted to 10 mm and the mandible was seen to deviate to the left side on opening. Intra oral examination revealed a derangement of occlusion. There was no mobility of the fractured segments. A hematoma was present on the buccal aspect in the parasymphyseal region on the right side []. Further, the orthopantomograph confirmed our clinical observations []. A diagnosis of fracture of the body of the mandible on the left side associated with a fracture of the parasymphysis on the right side of the mandible was made.
The treatment approach chosen was a unique one, involving a modification of the conventional transosseous wiring technique. The procedure was carried out under general anesthesia. The body fracture was exposed using a buccal vestibular incision. Two holes were drilled on either side of the fracture line into the buccal cortical plate; one at the region of external oblique ridge to avoid damage to the permanent tooth germ of the first molar and the other close to the lower border of the mandible. Two intermaxillary fixation (IMF) screws with eyelets, 1.5 mm diameter and 10 mm in length were inserted into the holes such that they were perpendicular to the cortical plate following which a 26 gauge stainless steel wire was passed into the eyelets and fastened. Having assuring reduction and fixation of the fractured ends, wound closure was achieved. Fracture reduction and stabilization was concluded satisfactory as shown by the post-operative occlusion. Recovery from general anesthesia was uneventful. An orthopantomograph was made post-operatively to confirm the approximation of the fractured ends []. The patient was discharged on the third post-operative day with instructions for a soft diet and maintenance of good oral hygiene.
Subsequently, satisfactory healing was observed. Four weeks after placement of the screws, following the confirmation of the stability of the fractured ends both clinically and radiographically a decision was made to remove the screws []. The patient was followed periodically, and presently shows satisfactory occlusion [].


A 72-year-old man having recurrent lymph node metastases from gastric cancer was admitted to Saiseikai Sendai Hospital to receive chemotherapy for gastric cancer. He had a history of distal gastrectomy plus right hepatic lobectomy for advanced gastric cancer with liver metastases (). As paraaortic lymph node metastases had re-grown despite 17 courses of combination chemotherapy of paclitaxel (Bristol-Myers Squibb, NY, US) and S-1 (Taiho Pharmaceutical CO., LTD, Tokyo, Japan), the chemotherapy regime was changed to drip infusion of docetaxel (Sanofi-aventis, Paris, France). He showed no definite adverse effects at the first administration of docetaxel in short term hospitalization. This was the second treatment of docetaxel. On admission, he looked well-being and blood chemistry data showed no inflammatory disease. Before chemotherapy, abdominal CT showed neither metastasis nor abscesses in the liver. A total of 95 mg of docetaxel was infused intravenously. Before docetaxel administration, 8 mg of methyl predonine was routinely pretreated. One day after chemotherapy, the patient presented with a high-grade fever up to 39℃ and low blood pressure (80/55 mmHg). At that time, laboratory findings revealed a white blood cell count of 13,550/L, a red blood cell count of 2,420,000/L, a blood platelet count of 109,000/L, aspartate aminotransferase of 1,112 IU/L, alanine aminotransferase of 774 IU/L, and CRP of 4.6 mg/dl. Since he was diagnosed with septicemia unknown etiology, continuous dopamine infusion and broad-band antibiotics were started. The next day, blood chemistry examination revealed serious liver and renal dysfunction. Although sepsis was strongly suspected from leucocytosis and the high value of C-reactive protein, the origin of sepsis could not be identified. Despite intensive care including hemodialysis and respiratory support, the patient died 5 days against strong medical care after docetaxel administration. Blood bacterial culture revealed strongly positive E. coli. An autopsy was performed to check the cause of rapid multiple organ failure. There was no evidence of myelo-suppression by sequential chemotherapy from the result of bone marrow histology. Residual lymph node metastases from gastric cancer were histologically confirmed. Multiple occult liver abscesses which could not identified macroscopically were diagnosed by the histological examine (). They consisted of gram-negative rods and fungi (). Therefore, we concluded that fatal E. coli septicemia had been induced from multiple occult liver abscesses as a result of immuno compromised host induced by anticancer drug treatment.


A 49-year-old female, who was a heavy smoker, but with negligible previous medical history, was admitted to a hospital due to stridor, dyspnea, cough, and hoarseness. Physical examination revealed no pathological signs, blood tests were normal, and the patient was treated with corticosteroids without a definite diagnosis. Ten days later, she developed epigastric pain that radiated to the left scapula and the left supraclavicular fossa. She was admitted to a hospital, where an echocardiogram and a chest CT scan revealed the presence of pericardial effusion (). During her hospitalization, she developed cardiac tamponade, for which she urgently underwent pericardiocentesis. Blind biopsies were taken from the pericardium and the upper lobe of the left lung, which showed malignant cells forming papillae and invading lymphatic and blood vessels (). However, it was not possible to identify the primary origin of the neoplasm, despite the thorough investigation with abdominal CT scan and technetium-99m-MDP bone scan. In addition, blood tests were normal, apart from the values of CEA (20.6 ng/mL (normal value: <4.7 ng/mL)) and CA-125 (341.4 IU/mL (normal value: <34 IU/mL)). The patient received one cycle of chemotherapy with carboplatin 5AUC, docetaxel 60 mg/m2, and bevacizumab 5 mg/kg for a neoplasm of unknown primary origin, which improved her symptoms.
Afterwards, she was admitted to our department for further investigation and treatment. The physical examination revealed a palpable nodule of the right lobe of the thyroid gland, and the ultrasound demonstrated diffuse heterogeneity of the same lobe. Nevertheless, the ultrasound failed to reveal any specific suspicious area, and the serum concentration of thyroglobulin was normal (17.7 ng/mL (normal value: <78 ng/mL)). The cytological examination after a blind fine-needle aspiration biopsy of the right lobe of the thyroid gland revealed the presence of a papillary thyroid carcinoma. Subsequently, a cervical MRI was performed, demonstrating extensive lymphadenopathy. The patient underwent total thyroidectomy and bilateral lymph node dissection. The histological examination verified the presence of a stage IVC (T3N1bM1) papillary carcinoma with high-grade (grade III) malignancy and sporadic anaplastic changes, lymphovascular and perineural invasion, and metastatic infiltration of the cervical lymph nodes (). A reexamination of the pericardial fluid from the pericardiocentesis revealed the expression of thyroglobulin by the malignant cells. An I131 scan was also performed, but without demonstrating abnormal I131 uptake. The patient's treatment was continued with the use of the same chemotherapy regimen for a total of six cycles without any major adverse events and with excellent tolerance.
After the completion of chemotherapy, a FDG-PET scan was performed with negative results. Afterwards, the follow-up was based on physical examination, blood tests, and imaging examinations. Unfortunately, the disease relapsed six months later with a very aggressive clinical course due to lung metastases and cardiac tamponade. It was not possible to administer any anticancer treatment at that time, and she passed away due to respiratory and cardiac failure.


A 45-year-old female presented with a painless mass in the right parotid gland. The patient had neither any systemic diseases nor any trauma on the region. The overlying skin was normal in color and appearance. A course of antibiotic therapy had been prescribed to rule out infection causes without any beneficial effect. An ultrasound imaging showed a relatively solid hypoecho nodule in the right parotid gland at the superficial lobe []. Fine needle aspiration (FNA) was performed. Under general anesthesia, superficial parotidectomy was done, superficial lobe with tumor was freely separated from adjacent tissue. The gross specimen contained a soft, brown-yellowish lobulated mass with definitive borders measuring 7.5 × 5 × 2.5 []. Microscopically, the specimen showed mature adipose tissue with some salivary gland structure. The ratio of mature adipose tissue to salivary gland tissue was 2:5 []. A one-year follow-up period showed no evidence of recurrence [].


An 18-year-old female was admitted to the Ghaem hospital, Mashhad, Iran, with a one-year history of painless swelling in the left parotid region. The mass did not grow significantly in the past year. Clinical examination showed a soft movable mass approximately 6 cm × 5 cm in size. Ultrasound imaging revealed a hypoecho nodule []. The gross specimen was 5 × 4 × 3 in size. The lesion was removed by superficial parotidectomy under general anesthesia. Histopathologic examination revealed a well-defined lobular structure, containing mature fatty tissue, within salivary gland element. The ratio of mature adipose tissue to salivary gland tissue was 1:3 []. Eight-month follow-up after surgery showed no sign of recurrence.


A 65-year-old African man presented to our surgical emergency with a week history of bleeding per urethra following nocturnal penile erections. He admitted to have introduced a broken transistor radio antenna into his urethra a week before to serve as an improvised ‘itchstick’ to ease a bothersome itchy urethral condition. The object subsequently migrated proximally out of reach. He made several unsuccessful attempts at self-retrieval prior to presentation and remarkably reported no difficulty with voiding. He had no history of psychiatric disorder, alcoholic or drug intoxication and had no prior history of similar or other self-injurious behaviour. He, however, had a bothersome perineal and itchy urethral condition which was worse at nights.
Physical examination revealed a hard rod-like mass in the proximal part of the penis extending to the perineum; the mass had only minimal mobility. A pelvic X-ray done showed a rod-like radio-opaque foreign body in the region of the penile and bulbar urethra []. A diagnosis of foreign body in the male urethra was made. After obtaining informed consent, removal of the foreign body was planned. Under caudal regional block with 2% Xylocaine gel instilled into the urethra, an attempt at ‘milking out’ the foreign body was made but failed. Endoscopic removal was considered but deemed inappropriate as the distal end of the object was jagged (irregular) and appeared impacted in sub-urethral tissue. External urethrotomy of the anterior urethra was performed with the incision made into the proximal penile urethra at the distal tip of the foreign body which was then easily extracted []. The extracted foreign body, an aluminium tube transistor radio antenna measured 13 cm in length [], urethral swab for microscopy and culture was taken and urethrotomy closed over a 16 Fr Foley's catheter. The patient was discharged after 48 hours and the catheter removed after a week at the clinic. The urethral swab grew Klebsiella pneumoniae and he was treated with sensitive antibiotics, tabs Augmentin for 2 weeks. At 1 month follow-up, he complained of a decreased but persistent urethral itch. Four-bottle urine microscopy revealed numerous pus cells, few epithelial cells and spermatozoa and only scanty growth of K. pneumoniae. No worm or ova was found. Antibiotics (Augmentin) were continued for a further 2 weeks and empirical treatment for pin worm infestation was given (Albendazole) resulting in resolution of symptoms. A psychiatric evaluation revealed him psychologically normal. At 9 month follow-up, he had no recurrent itch and normal voiding with good uroflow.


A 5-year-old girl born out of a nonconsanguineous parentage, deaf and dumb from birth with other milestones being normal, was brought to our hospital for evaluation of three episodes of bacterial meningitis.
During the first episode, the child presented to a pediatric general hospital (GH) with high-grade continuous fever and vomiting for 3 days followed by altered sensorium for 2 days. On examination, the child had mild left orbital cellulitis, was drowsy, and meningeal signs were present without features of raised intracranial pressure or focal deficits. Routine investigations were done which showed mild anemia; computerized tomography (CT) of brain showed diffuse cerebral edema with left ethmodial sinusitis and orbital cellulitis on the left side. Lumbar puncture (LP) revealed turbid cerebrospinal fluid (CSF) with 350 cells (90% polymorphs, 10% lymphocytes), protein of 780 mg/dl and glucose less than 10 mg/dl. Gram stain was negative, but CSF culture showed growth of Streptococcus pneumoniae sensitive to penicillin, gentamycin, cefotaxime, ceftriaxone, vancomycin, and ciprofloxacin. She was treated with sensitive antibiotics for 2 weeks and she recovered. She was referred to our institute by the pediatrician who treated her during the first episode, but the patient’s father got her discharged within 12 hours because of social problems.
She presented once again to the same pediatric hospital after 4 months with high-grade, continuous fever and headache of 3 days duration. On examination, she was conscious and had meningeal signs with no focal deficits. Routine investigations were normal except for mild anemia. CSF showed 250 cells (100% polymorphs), protein of 65 mg/dl, and glucose less than 20 mg/dl. Gram stain was negative; culture did not grow any organism. She received injectible crystalline penicillin and ceftriaxone for 14 days and became fully asymptomatic.
Approximately 1 month later, she once again developed high-grade, continuous fever, vomiting, and altered sensorium of 2 days duration. This time, the child was referred to our hospital for evaluation and treatment, without being admitted at GH. On examination, the child was drowsy and meningeal signs were present without any features of raised intracranial tension or focal deficits. Lumbar puncture revealed turbid CSF with cell count of 2000 (90% polymorphs), protein of 168 mg/dl, and glucose of 23 mg/dl. Pneumococcal antigen was positive in the CSF. Blood and CSF cultures were negative. She was treated with ceftriaxone for 2 weeks in meningitic doses and improved totally. There was no history of any ear discharge, rhinorrhea, head injury, and recurrent respiratory or skin infection. On examination, there was no splenomegaly, sinus, or tuft of hair over spine. Her routine investigation showed mild anemia, leukocytosis, normal biochemistry, and nonreactive HIV test. Brainstem-evoked auditory responses were absent bilaterally with audiometry suggestive of bilateral severe sensory neural hearing loss.
She was evaluated with high-resolution computerized tomography (HRCT) scan of the temporal bone and magnetic resonance imaging (MRI) of the brain. On the left side, the cochlea was showing a featureless single cavity. There was complete absence of modiolus and interscalar septa. The vestibule was grossly dilated. The cystic cochlea and vestibule together gave a “figure-of-eight” appearance. The lateral semicircular canal was dysplastic and dilated. Superior and posterior semicircular canals were normal. Internal auditory canal (IAC) was dilated and contained normal facial and vestibulocochlear nerves. The vestibular aqueduct was normal. The left tympanum and the mastoid air cells were filled with soft tissue density, indicating either CSF leakage or otitis media with mastoiditis [Figures and ].
The cribriform bony plate between the cochlea and IAC was deficient, producing an open fundus communicating with the cochlea. This could be a probable site of communication between the subarachnoid space and inner ear. CT cisternography, which reveals the abnormal transotic CSF leak, was not performed in our case. However, the oval and round windows were wider. These could be the probable sites of communication between the inner ear and middle ear. The middle ear ossicles (malleus, incus, and stapes) and tympanic membrane were normal. The right inner ear also revealed similar abnormality. However, the tympanum and mastoid were normal.
With these features, the patient was diagnosed as a case of congenital deaf-mutism with RBM, cystic vestibulocochlear anomaly, and probable infection arising from left middle ear cavity. She was evaluated by the ENT surgeon, and after 1 month of the last episode, she underwent left middle ear exploration and a communication at the stapes foot plate with inner ear was identified and closed. The patient has had no recurrences from the last 1½ years.


A 45-year-old man, alcohol consumer, non-smoker, without co-morbidities, presented with abnormal behavior for 3 days and global headaches with vomiting for 6 days developing few hours after a cross-country run of 13 km at 7,000 ft. There was no seizure, loss of consciousness, fever or head injury. He was afebrile, conscious, abulic and uncooperative with normal vitals. Rest of the examination was normal.
A non contrast computed tomography scan of head was normal. Magnetic resonance imaging (MRI) brain showed hyperintense signal in bilateral thalami, left basal ganglia and periventricular white matter on T1 and T2-weighted images (T1WI, T2WI) and fluid attenuated inversion recovery image (FLAIR), and hyperintense internal cerebral veins suggestive of subacute thrombosis [Figure and ]. DWI revealed heterogeneous signal intensity without hypointensity on ADC map corresponding to vasogenic edema [Figure and ]. Gradient echo image showed susceptibility artefacts compatible with hemorrhagic component of venous infarct []. Magnetic resonance venography (MRV) did not reveal the deep venous system, which was visualized on T1WI indicating thrombosis, while superior sagittal and lateral sinuses were patent [].
His condition steadily improved on low molecular weight heparin (LMWH) bridged with acitrom. Mini mental status examination improved from 20/30 to 29/30 by 3 weeks. At 2 months, he was asymptomatic with serum homocysteine of 31.51 μmol/l (5.46-16.2 μmol/l), and at this time, after discontinuation of acitrom for 2 weeks, the protein S was 49.00% (77-143.00%), and protein C 118.40% (70-140.00%); factor V Leiden mutation, methylene tetrahydrofolate reductase (MTHFR) gene mutation (C677T) and prothrombin gene G20210A mutation were not detected. Anti-phospholipid IgG antibody was 4.53 GPL U/ml (0.5-10.0) and IgM antibody was 2.77 MPL U/ml (0.5-10.0). Anti-neutrophil cytoplasmic antibody and rheumatoid factor were negative.
He was treated with methylcobalamin, pyridoxine and folic acid. Oral anticoagulation was continued for 1 year only. After 16 months, he remained asymptomatic with normal MRI brain [] and partially recanalized deep cerebral venous system on MRV, and serum homocysteine fell to 16.50 μmol/l (5.46-16.2 μmol/l).


A 17-year-old boy presented to our hospital with high spiking fever, chills rigors associated with vomiting and headache for the past 3 days. He also had a history of intermittent purulent right ear discharge since 6 years of age. There were no focal neurological signs or papilledema to suggest raised intracranial tension. A CT scan of the head done by the referring doctor was normal. Lumbar puncture showed increased proteins (210 mg/dL) with normal sugars. Cerebrospinal spinal fluid analysis (CSF) showed 3000 cells, 84% of them being neutrophils. Gram staining was negative for bacteria and CSF cultures were sterile. He was treated as pyogenic meningitis and was started on a combination of Ceftriaxone, Amikacin, and Tinidazole. His fever promptly responded to the antibiotics, but he repeatedly complained of a persistent headache. He was also diagnosed with bilateral atticoantral CSOM with a right-sided cholesteatoma. In view of the persistent headache, a contrast MRI study of the brain was obtained and it revealed a right-sided enhancing subtentorial subdural collection suggestive of a subdural empyema [].
Neurosurgical consult was sought and the patient was transferred under our care for further management. As the boy was afebrile with no evidence of raised intracranial tension, he was initially managed conservatively with antibiotics. Over the next few days his headache and vomiting subsided, but he kept complaining of repeated episodes of severe neck pain every night at around 3 am.
A repeat contrast-enhanced computed tomography scan (CECT) done 10 days later using a 64 slice scanner revealed that the collection had greatly increased in size and now measured 4.5 × 2.5 cm and was associated with secondary hydrocephalus []. The boy was taken up for a right retromastoid suboccipital craniectomy and about 15 ml of frank subdural pus was aspirated, and the subdural space gently washed out with antibiotic solution. Pus was sent for culture but later failed to show any bacterial growth.
Post-operative period, he did well with no recurrence of fever or neck pain. Intravenous antibiotics were continued for the next 10 days. At the time of discharge he was afebrile and neurologically intact with no focal deficits. He was advised to attend the ENT outpatient department period for further management of his middle ear infection.


A 40-year-old white male was referred to our clinic due to a 15-day history of bilaterally blurred vision and metamorphopsia. He had a history of myasthenia gravis for which he had been treated with oral prednisolone and azathioprine during the last 2 years. His best-corrected visual acuity (BCVA) was 8/10 in the right eye (OD) and 9/10 in the left eye (OS).
Fundoscopy revealed a few small, yellow-whitish circumscribed macular elevations and one larger, irregularly shaped parafoveal elevation in OD (fig. ). Similar small macular lesions were also noted in OS. Fluorescein angiography showed multiple areas of early hyperfluorescence and late leakage in OD (fig. ). Similar but less pronounced angi-ography findings were seen in OS (fig. ). Optical coherence tomography (OCT) revealed RPE detachment, elevation of the neurosensory retina and highly reflective subretinal fluid mostly in OD (fig. ).
The diagnosis of multifocal CSCR was made. As the patient's myasthenia could not be optimally controlled at the time, the treating neurologist advised against prednisolone discontinuation. Unfortunately, we noticed further deterioration of the BCVA and subfoveal expansion of the subretinal fluid in OD, so it was decided to administer an intravitreal injection of ranibizumab in this eye. One month later, his BCVA had deteriorated to 3/10 in OD and 7/10 in OS. Fundoscopy revealed that the area below the fovea in OD was enlarged with the deposition of material presumed to be fibrin in the subretinal space. OCT demon-strated further elevation of the neurosensory retina and a more irregular subretinal accumulation of highly reflective material (fig. ). Indocyanine green angiography excluded neovascularization and polyp-like vascular abnormalities. Low-fluence photodynamic therapy (PDT) was performed. We administered verteporfin i.v. at a dose of 6 mg/m2 over 10 min, waited for 5 min and then directed the diode laser at the larger lesion of leakage in OD, using a wavelength of 689 nm and laser energy of 25 mJ/cm2. After the first application, the laser spot was directed at the second and the third lesion of leakage in OD (fig. ). As the patient's myasthenic symptoms improved, it became possible for the attending neurologist to reduce the dose of prednisolone, increase the dose of azathioprine and add neostigmine. One month later, and while undergoing prednisolone tapering, his BCVA in OD had improved to 6/10 with no subretinal fluid, while the area with the fibrin deposition had nearly disappeared (fig. ). The BCVA in OS improved to 10/10 over the next couple of months. To date, 1 year after treatment, the patient's BCVA remains stable at 6/10 in OD and 10/10 in OS with no signs of active disease. The patient's myasthenia is currently well controlled with azathioprine and neostigmine.


In April 2004, a 58-year-old male presented with a PSA of 17.9 ng/ml, T3 and Gleason 7 (3 + 4) prostate adenocarcinoma. He underwent pelvic lymph node ablation, showing an invasion of the right ilio-obturatory nodes. A bone scan as well as a thoraco-abdominal CT were normal. The patient was treated with prostatic (70 G) and pelvic (50 G) radiation therapy, followed by gosereline acetate (10.8 mg every 3 months for 6 months). His post-treatment PSA nadir was 1 ng/ml in July 2004.
In October 2007, progression occurred with an increase in PSA, CT showing enlarged lumbo-aortic nodes as well as lumbar bone metastases. The patient began ADT with gosereline acetate (10.8 mg every 3 months) and bicalutamide 50 mg/day from June 2007 to December 2007, followed by spine radiation therapy. In January 2008, as PSA progressed, docetaxel (75 mg/m2 every 3 weeks with prednisone 10 mg daily/6 cycles) was started. A partial response was obtained, and ADT was resumed after chemotherapy. In January 2009, while PS was 1, a new progression occurred with bone pain, radiographic progression (bone, liver, hydronephrosis) as well as PSA increase. A mitroxantrone/prednisone treatment was proposed (10 mg/m2, 3 cycles), but treatment was poorly tolerated and the disease continued to progress.
From 2009 through 2011, the patient received a total of 5 separate regimens for his progressing metastatic disease: zoledronic acid, stilboestrol, oral vinorelbine (80 mg weekly, 6 cycles), docetaxel (60 mg/m2, 3 cycles), and carboplatin/VP16 (3 cycles: carboplatin AUC 4 and VP16 100 mg/m2 for C1, carboplatin AUC 4 and VP16 80 mg/m2 for C2 and carboplatin AUC 3.75 and VP16 75 mg/m2 for C3). However, the disease progressed and PS deteriorated to 3.
In March 2011, a named-patient treatment with abiraterone was started at a dose of 1,000 mg/day together with prednisone 10 mg/day. Before abiraterone, PSA was 504 ng/ml and PS was 3; main metastatic lesions were located in the bones, lumbo-aortic nodes, liver, urinary tract (hydronephrosis) and lungs. Moreover, the patient had ascite and lower limb edema.
Quickly after abiraterone initiation, the patient had a sharp clinical improvement (PS 1). Biological response (PSA decrease at 281 ng/ml) was obtained after 1 month of treatment. No side effects were attributed to abiraterone treatment. After 3 months of treatment, radiological partial response was achieved (fig. ) and PSA was 54 ng/ml with PS 1.
In August 2011, PS deteriorated, lower limb edema increased, and the patient died.


In 2000, a 49-year-old man presented with prostate adenocarcinoma with PSA at 6 ng/ml and Gleason score of 7 (3 + 4). He underwent a radical prostatectomy with pelvic lymph node ablation. After radical prostatectomy, the patient was staged pT2N0, but surgical margins were positive and intraprostatic lymphovascular invasion was diagnosed with no invasion of capsule or seminal vesicles. Gleason score was confirmed.
In September 2007, PSA monitoring showed an increase at 57 ng/ml. A CT showed enlarged iliac lymph nodes without metastases. Bicalutamide was started and PSA decreased to 1.2 ng/ml. In August 2008, upon increase in PSA, the patient began ADT (leuprolide 45 mg every 6 months). PSA dropped, but 1 year later, in August 2009, PSA was at 122 ng/ml and a CT showed retroperitoneal node enlargement. The patient began chemotherapy with docetaxel (75 mg/m2 every 3 weeks with prednisone 10 mg daily − 10 cycles). Partial node response was obtained and PSA dropped to 15 ng/ml after 10 cycles of docetaxel. In September 2010, progression was evident with bone and lung metastases and PSA at 666 ng/ml. Zoledronic acid was given and a second line of chemotherapy was proposed with cisplatin + VP16 (CDDP 75 mg/m2 and VP16 100 mg/m2 for C1, CDDP 67.5 mg/m2 and VP16 90 mg/m2 from C2/6 cycles). Stabilization was obtained until May 2011 when PSA rose to 1,069 ng/ml together with bone pain. PS was good at 0. A named-patient treatment with abiraterone was started at a dose of 1,000 mg/day together with prednisone 10 mg/day. After 1 month of abiraterone, PSA dropped to 143 ng/ml. Abiraterone was well tolerated. In December 2011, the patient remained in very good shape (PS 0, and PSA 14.8 ng/ml). A CT showed response of lung lesions (fig. ). Then, the disease remained stable until May 2012 with a bone scan indicating progressive bone metastases, while CT lung and node lesions remained stable. In August 2012, PSA was at 2,025 ng/ml and abiraterone was stopped. Chemotherapy was resumed with cabazitaxel (25 mg/m2 − 3 cycles). Tolerance was good and PSA dropped to 1,208 ng/ml after C2.


A 10-month-old boy with a body weight of 9.6 kg presented with progressive lethargy. His past medical history was unremarkable. He had suffered from gastritis 2 weeks before and started vomiting again 2 days before admission. The patient had no fever, excessive sweating or diarrhea, and his urine output was normal. Physical examination revealed a sleepy but easily arousable infant with a heart rate of 160 per minute, a respiration rate of 32 per minute and a body temperature of 37.7°C. On all four extremities, a blood pressure of 135/95 mm Hg was measured, a value above the 99th percentile (P) by age and height (e.g. P 95th is 103/56 mm Hg). There were no signs of meningitis or intracranial hypertension, no abnormalities in sensibility and strength, and reflexes were normal. Cardiopulmonary examination revealed no heart murmurs, and breath sounds were normal. The abdomen was not painful to palpation and there were no masses. No edema was found, and the skin was unremarkable. Based on the combination of hypertensive crisis and the neurological findings, the diagnosis of malignant hypertension was made and the patient was admitted to our pediatric intensive care unit for evaluation and intravenous antihypertensive therapy [, , ]. Nicardipine at 1 mcg/kg/min lowered his blood pressure to an acceptable level of 105/55 mm Hg (P 95th) within a few hours.
Laboratory results showed a normal complete blood cell count, serum electrolytes (Na 137 mmol/l, K 4.1 mmol/l, Cl 104 mmol/l), creatinine 13 µmol/l, urea 3.6 mmol/l and glucose 5.6 mmol/l. Glomerular filtration rate was 118 ml/min, and blood lactate as well as ammonia levels were normal. An arterial blood gas analysis revealed a mild meta-bolic acidosis with respiratory compensation (pH 7.40, bicarbonate 17.7 mmol/l, pCO2 29 mm Hg).
A computed tomography of the brain revealed no intracranial bleeding, no tumor and no signs of elevated intracranial pressure. An abdominal ultrasound showed normal kidneys without hydronephrosis, and Doppler studies were unremarkable. On the ultrasound, an abdominal mass with the characteristic ‘target sign’ of an ileocecal intussusception was visualized in the right lower quadrant (fig. ). Radiographically guided hydrostatic reduction with water-soluble contrast failed. Laparotomy was performed, which confirmed the diagnosis of intussusception. After repositioning, intestinal perfusion was adequate and bowel resection was not necessary. The anesthetics used during the surgery had no major influence on the blood pressure. Following surgery, the mental status returned to normal and antihypertensive medication could be discontinued. His blood pressure decreased to a normal level (P 50th 90/40 mm Hg).


A 17-year-old-male patient reported to the Department of Dental Sciences with a gross asymmetry over the right anterior region of the mandible. The patient gave a history of loosening of teeth since 1 year. Intra-oral examination revealed a bony hard swelling in the right mandibular region extending from central incisor to first molar []. Right mandibular lateral incisor, canine, first premolar, and second premolar were mobile and gave no response on electrical pulp testing. On palpation the swelling was non-tender, irregular in shape, and measuring 2 cm × 3 cm. Orthopantomogram showed a large well-circumscribed radiolucency extending from right mandibular central incisor to the mesial root of first molar []. Due to the expansive growth there was deviation of the roots of right mandibular canine and first premolar. Root resorption was also evident in relation to the first premolar, second premolar, and mesial root of first molar on the right side. Based on these features, a differential diagnosis of odontogenic cysts and tumors (like odontogenic keratocyst, lateral dentigerous cyst, AOT, calcifying epithelial odontogenic tumor) was given. Other differential diagnosis included central giant cell lesions, lateral periodontal cyst, lateral radicular cyst, and the much rarer central benign mesenchymal neoplasms. The lesion was operated under local anesthesia and the specimen was sent for histopathologic examination. The mobile teeth, that is, mandibular lateral incisor, canine, premolars, and the first molar were removed along with the lesion []. Grossly, the lesion was white to tan, nodular tissue with cystic spaces containing yellowish brown semisolid material []. Microscopic examination revealed extremely vascular encapsulated lesion showing multivariate patterns of cellular arrangements ranging from sheets of polygonal cells arranged in ductal pattern, rossetes to solid sheets of cells []. In the center of these ducts, eosinophilic amyloid-like material was also seen. The solid lobular masses showed numerous spindle to columnar hyperchromatic cells with interspersed deposits of eosinophilic hyaline-like material. Mitotic figures were also seen in the lobules [Figures and ]. The above features were consistent with the diagnosis of AOT.


A 31-year-old female patient presented with blurred vision in both eyes which was more severe in her left eye. The problem had started 3 years prior to presentation. The condition had been slowly progressive 6 months prior to her first visit. The patient did not use glasses or any optical aids.
Family and past history were unremarkable. Best corrected visual acuity (BCVA) was 2/10 and counting fingers at 2 meters with -18.0-5.0 × 70 and -19.0 in her right and left eyes respectively.
Slit lamp biomicroscopy showed inferior corneal thinning bilaterally about 2 mm above the limbus with a stromal scar involving the central cornea, extending to the inferior area of thinning in her left eye. The remainder of the ocular examination was unremarkable bilaterally. Corneal topography, showed a crab- claw pattern in the right eye and a very irregular pattern in the left one ().
PK was performed in the left eye and a 7.50 mm sized recipient bed received a 7.75 mm donor with small inferior graft decentration.
On the first postoperative day, the patient was found to have a positive Seidel test and mildly shallow anterior chamber (AC); the microleakage was from a suture tract located at the 6 o’clock position of the host-graft junction. There was no apparent wound gap but a small host-graft mismatch was present. The patient was followed while receiving steroid and antibiotic drops; a few days later the leakage ceased and the AC was fully formed.
The rest of her postoperative course was uneventful up to 8 months when her last sutures including the one at 6 o’clock were removed. Several days later, the patient developed acute hydrops at the inferior host-graft area mostly involving the recipient cornea (). Seidel test was positive but the AC was formed. Antibiotic drops were started and the eye was patched; 2 days later the leakage stopped and edema in the inferior recipient cornea decreased. One week later there was no leakage and the hydrops disappeared with final uncorrected BCVA of 2/10 ().


A 54-year-old male was seen in our nephrology clinic for progressive increase in his serum creatinine from 1.38 to 3.66 mg/dl over 9 months. There was no history of fever, diarrhea, steatorrhea, urinary tract symptoms, renal stones, or use of nephrotoxic agents. His past medical history included hypertension for 15 years, type 2 diabetes for 11 years, obstructive sleep apnea, depression, and treated cellulitis. He had undergone a duodenal switch procedure for morbid obesity (body mass index >50 kg/m2) 20 months earlier. Subsequently, he had lost more than 100 kg of body weight, and there was significant improvement in the control of blood pressure, sleep apnea, and blood sugar. His serum creatinine was 1.38 mg/dl and urinalysis was normal at the time of surgery.
His clinical examination was unremarkable. Laboratory investigations showed hemoglobin of 9.9 g/dl, white cell count of 6.9 × 103/μl, blood urea nitrogen of 51.82 mg/dl, and serum creatinine of 3.66 mg/dl. Serum electrolytes, calcium, phosphate, and bicarbonate were within normal limits. Urinalysis showed 5-10 white cells/hpf and 1-2 white cell casts without any proteinuria or hematuria. Ultrasound of kidneys showed normal sized kidneys with a few small bilateral calculi without any evidence of obstruction. Doppler of renal arteries was normal.
In view of progressive unexplained increase in creatinine, a kidney biopsy was done. The kidney biopsy [Figures and ] demonstrated 13 glomeruli of which six were globally sclerosed. The remaining glomeruli were histologically normal. The tubular interstitial compartment showed foci of tubular damage with deposition of translucent crystals of different shapes which are predominantly intraluminal with few intracellular and focal interstitial distributions. Under polarized light, the deposits appeared strongly birefringent forming fan-like, sheaf-like, or irregular shapes consistent with calcium oxalate crystals. There was an associated mild interstitial inflammation with moderate interstitial fibrosis and tubular atrophy. Blood vessels exhibited moderate arteriosclerosis and arteriolar hyalinosis. The immunofluorescence was negative and electron microscopy did not show any electron dense deposits but did reveal tubular damage with intratubular crystal deposition. His 24-h urinary oxalate level was found to be significantly elevated at 98.7 mg/day (normal range upto 40 mg/day). Urinary calcium and citrate were undetectable.
A diagnosis of oxalate nephropathy secondary to enteric hyperoxaluria post-duodenal switch operation was made and he was started on low-oxalate, low-fat diet, with high-fluid intake (>3 liters per day). In addition, he was prescribed calcium citrate 1000 mg tid with meals as an oxalate binder. He was also started on cholestyramine 4 g twice daily and repeat urine oxalate improved markedly to 63 mg/day and his serum creatinine stabilized.


A 57-year-old woman was admitted to the emergency department for diffuse maculopapular rashes on the whole body and anaphylactic shock. She had been complaining of sudden- onset of abdominal pain, dyspnea, pruritus, and urticaria. On physical examination, it revealed tenderness, guarding, and rigidity all over the abdomen. Her blood pressure was 90/60 mmHg, heart rate 118/min, and temperature 37.0°C. Blood test analysis was normal and subsequent leucocytosis with eosinophil dominance. Hydration, hydrocortisone, and antihistamines were used to treat the possible anaphylactic reaction. The patient received oxygen as supportive treatment. The patient recovered after emergency medications and then abdominal computed tomography (CT) revealed a splenic cystic lesion measuring about 9’ 6 cm2 in diameters and containing floating membranes. The anterior wall of the cyst showed discontinuity at a point []. Diffuse pelvic and minimal perisplenic fluid were detected. Our fundamental concerns were to preserve the spleen. Then, the patient underwent a laparoscopic partial cystectomy and omentoplasty, since the spread of the disease into the peritoneal cavity of lesions was strong contraindications for a radical surgical approach. At the time of laparotomy, the cyst's outer membrane was seen ruptured []. The postoperative course was uneventful, and the patient was discharged 10 days after the surgery. We applied albendazole, 10 mg/kg/day for 6 months, and the patient was followed up with CT that displayed no recurrence of the disease during a follow-up of 6 months []. She is still symptom-free in the 12-month follow-up.


A 26 year old unmarried male was referred to us with history of left sided chest pain for two days. He had consulted at local hospital immediately after trauma. A skiagram chest taken there [] showed non homogenous opacity with central lucency in the region of left hilum. On examination, the patient was afebrile. Except for a few abrasions on anterior chest wall, general examination and respiratory system examination was normal.
The patient gave history of trauma - a tractor wheel hitting him on the anterior chest wall, while he was lying down; following which he developed the pain. There was no history of breathlessness, fever, cough, hemoptysis, cough or expectoration. There was no history suggestive of bronchial asthma or exposure to pets at home or work place. A repeat chest X ray on 31/08/2011 showed cavitations in the left hilar region. The margins of the lesion were now thinned out and smooth [].
His sputum for AFB was negative. Gram stain showed few gram positive cocci but was sterile on culture. A mantoux test was done and read 8 mm at end of 72 hours. Other lab investigation revealed haemoglobin 14.4, leukocyte count 7900, and differential count of 78 % polymorphs and 19% lymphocytes. His total eosinophilic count was 88/cc. His serum was non reactive for HIV antibodies. A CT scan of thorax done on 03-09-2011 showed a single smooth walled cavity abutting left chest wall with surrounding areas of ground glass haziness, probably suggestive of lung contusion [].
Fibre optic bronchoscopy was performed to achieve a microbiological diagnosis and to inspect the trachea-bronchial tree in view of previous reports being inconclusive. Bronchoscopy revealed a bleeding spot in left main bronchus, but no active bleeding was seen. Bronchial brushings taken from left upper lobe were negative for malignant cells and AFB, and the lavage fluid also was sterile. In view of the patient's history of ancedent recent trauma, negative past and present history for any infectious process, and negative microbiological reports, a diagnosis of Post traumatic pulmonary pseudo cyst (TPP) was made. The patient was reassured and advised analgesics and bed rest for 3 days. Serial follow up chest skiagram showed rapid resolution of the cavities [] without any antibiotics, thus confirming our diagnosis of TPP.


A 19-year-old girl presented with complaints of weight gain, hypertrichosis, menstrual irregularities of 2 years and hypertension of 4 months duration. She also gave history of easy bruisability, broad voilaceous striae over abdomen and thighs, recurrent vaginal candidiasis. Her height was 154 cm, weight 77 kg, BMI of 32 kg/m2, and waist hip ratio 0.95 on examination. She was normotensive on antihypertensive medications. There was thinning of scalp hairs, mooning of face, facial plethora, hypertrichosis over face, increased supraclavicular and dorsocervical fat pad thickness, voilaceous abdominal striae, thinning of skin over dorsum of hands, and proximal muscle weakness. There was no neurological deficit including visual fields. OGTT detected IFG (fasting plasma glucose 102 mg/dl) and IGT (postglucose load 156 mg/ dl). Her bone mineral density estimation by DXA revealed osteopenia. Her hormonal evaluation revealed normal basal ACTH levels (38.7 pg/ml) in the presence of basal cortisol levels of 21.3 μg/dl (8 am) and evening cortisol of 23 μg/ dl (4 pm). Serum cortisol levels remained unsuppressed following overnight (18.6 μg/dl) and low dose (15.7 μg/dl) dexamethasone suppression test. However, it decreased by 73% on high dose dexamethasone suppression test (5.8 μg/ dl). MRI brain did not reveal any pituitary abnormality. Since neurosurgeons were reluctant to operate without localization and also to find source of ACTH, bilateral inferior petrosal sinus sampling (BIPSS) was planned with vasopressin stimulation due to nonavailability of CRH and desmopressin. The central to peripheral ACTH ratio on the right side was 28/1 and 32/1 in basal and postvasopressin respectively (normal <2 and <3 respectively). The interpetrosal sinus gradient was also significant (right IPS/left IPS = 13; normal <1.5) suggesting pituitary microadenoma on the right side of adenohypophysis. The patient was diagnosed as having Cushing's disease caused by an ACTH-secreting pituitary adenoma, which was extirpated (adenomectomy) by transphenoidal surgery. The sixth postoperative day basal Cortisol level was 0.33 μg/dl, suggestive of possible remission of disease. She was discharged on replacement doses of hydrocortisone with advice on stress dosing and she is planned for gradual tapering of hydrocortisone doses.
Histopathological examination on light microscopy showed more than 95% of cells arranged in a large sheet of polyhedral cells with eccentric nuclei and moderate amount of bright eosinophilic, granular cytoplasm consistent with oncocytes, which confirmed the diagnosis of oncocytoma []. These cells were arranged in diffuse, solid, and sinusoidal pattern []. These cells were strongly positive for ACTH []; prolactin; GH; S100; neuron specific enolase; chromatogranin A; and synaptophysin on immunohistochemical staining and displayed a bright brown positivity universally. Phosphotungstic acid hematoxylin (PTAH) staining revealed dark blue oncocytic cytoplasm [].


A 65-year-old female was presented with dyspnea on exertion (New York Heart Association, NYHA class III) for over a period of 1 year. For 5 years, she had complained of dry mouth, intermittent dry eye, and gritty feeling under the eyelids. However, she did not have Raynaud's phenomenon or parotid swelling. She denied taking anorexiants or other drugs. Further, she did not have any sort of family history.
Blood pressure, pulse rate, and respiration rate were 114/78 mm Hg, 92 per minute, and 24 per minute, respectively. Cardiovascular examination revealed a grade 2/6 systolic murmur in the left low parasternal area, a more accentuated P2 than A2, and both jugular veins that are both slightly distended.
Laboratory exam disclosed polyclonal hypergammaglobulinemia identified by slightly increased total protein (8.2 g/dL, normal 6.5-8.1 g/dL) and gamma globulin (22.4%, normal 11.1-18.8%). Autoantibody tests showed positive results for anti-nuclear antibodies (1 : 640, speckled, anticytoplasmic antibody: positive), rheumatoid factors (57.10 IU/mL, normal <14 IU/mL), and anti-Ro (SSA) antibodies (3+), while anti-neutrophil cytoplasmic antibodies (ANCA), antibodies to La (SSB), double stranded DNA, Sm, ribonucleoprotein (RNP), topoisomerase-I (Scl-70), histidyl-tRNA synthetase (Jo-1) were negative. Antibodies to cardiolipin and lupus anticoagulant were also negative. Serum complements protein levels were all slightly decreased (C3 87.0 mg/dL, normal 90-180 mg/dL; C4 7.2 mg/dL, normal 10-40 mg/dL; CH50 21.6 U/mL, normal 34-71 U/mL). Anti-human immunodeficiency virus antibodies, hepatitis B virus antigen and antibodies, and anti-hepatitis C antibodies were all negative. Erythrocyte sedimentation rate was increased (42 mm/hour, normal 0-20 mm/hour). Hemoglobin, white blood cell and platelet counts, and urine test were within normal limits. N-terminal pro B-type natriuretic peptide level was 1469.1 pg/mL (normal 0-125 pg/mL). Creatinine kinase-MB and troponin-I were also within normal limits. Arterial blood gas analysis in room air showed pH 7.44, pO2 78 mm Hg, and pCO2 34 mm Hg.
Pulmonary function test revealed normal ventilatory pattern with normal CO diffusion capacity. Chest X-ray demonstrated grossly normal lung fields with a mildly enlarged right ventricle. Electrocar-diogram showed tall, peak P waves in lead II, III, and aVF, suggesting right atrial enlargement. Transthoracic echocardiography showed a mildly enlarged and thick right ventricle, hypokinetic right ventricular free wall, grade II tricuspid regurgitation, and peak tricuspid regurgitation jet velocity of 4.64 m/s with an estimated right ventricular systolic pressure (RVSP) of 91.1 mm Hg (). Transthoracic and transesophageal echocardiography excluded any other congenital, valvular, and myocardial diseases. Right heart catheterization also revealed severe PH. Pulmonary artery pressure was 74/27 mm Hg (mean 46 mm Hg) with a pulmonary capillary wedge pressure of 7 mm Hg (). Cardiac index was calculated as 2.27 L/min/m2 and pulmonary vascular resistance was 11 Wood units. Elevated pulmonary artery pressure was not significantly decreased with adenosine infusion. Chest computed tomography and lung perfusion scan did not show abnormal findings compatible with pulmonary embolism. Schirmer's test was positive. Salivary gland scintigraphy disclosed severely decreased tracer uptakes in both parotid and submandibular glands.
Considering her clinical, laboratory, cardiologic, imaging findings and the Revised International Classification Criteria for Sjögren's Syndrome by the American-European Consensus Group, she was clearly diagnosed with severe PH associated with primary Sjögren's syndrome. She was treated with 62.5 mg endothelin receptor antagonist (Bosentan®) twice a day for the initial month and 125 mg twice a day for the following months, 200 mg hydroxychloroquine twice a day, 50 mg azathioprine twice a day, and topical corticosteroid and sodium hyaluronidate eye drops. A follow-up transthoracic echocardiography after 6 months of treatment showed somewhat improved right ventricle contractility and decreased estimated RVSP (65.5 mm Hg) compared to the initial exam (). Equally, her dyspnea symptom was much improved and uneventfully, she is being followed up at an outpatient clinic.


A 16-year-old girl was referred to our clinic with a diagnosis of neuroretinitis or juxtapapillary chorioretinitis. The patient had a 3-week history of decreased vision and metamorphopsia in her left eye. Her medical and family history were unremarkable. There was no history of trauma or high myopia. Before referral, systemic work-up for tuberculosis, sarcoidosis, syphilis, Lyme disease, brucellosis, and toxoplasmosis was negative. At our initial visit, best-corrected visual acuity was 20/20 in the right eye and counting fingers in the left eye. There was a mild relative afferent pupillary defect in the left eye. Biomicroscopic examination was within normal limits. There was no cellular reaction in the anterior chamber or vitreous cavity. The intraocular pressure was 16 mmHg in both eyes. Fundus examination of the left eye revealed a juxtapapillary, elevated, white chorioretinal lesion consistent with CNV that was associated with exudation and retinal edema []. The juxtapapillary choroidal nevus underlying CNV was not visible with color fundus photography [], but we were able to detect it with the brightest illumination of the indirect ophthalmoscope. Fundus fluorescein angiography (FFA) demonstrated extrafoveal, juxtapapillary CNV, and late diffuse hyperfluorescence superior to this lesion. Optic disc was normal in all phases of the angiography [Fig. –]. Optic coherence tomography (OCT) confirmed CNV with overlying intraretinal cysts and minimal subretinal fluid. A diagnosis of juxtapapillary CNV associated with a choroidal nevus was made. After a written informed consent was obtained, three consecutive monthly intravitreal bevacizumab (1.25 mg/0.05 ml) injections were performed. After treatment, best-corrected visual acuity improved to 20/50. CNV appeared inactive and smaller in size with complete resolution of exudation exposing an underlying choroidal nevus with 6 × 6 × 2.6 mm in dimensions []. OCT demonstrated gradual resolution of subretinal fluid and intraretinal cysts []. FFA revealed burnt-out fibrotic CNV with late staining and juxtapapillary hypofluorescence due to underlying choroidal nevus []. The lesion was still inactive and final visual acuity was stable throughout 2 years of follow-up.


A 78-year-old male with chronic ischaemic heart disease was referred for recurrent episodes of VT treated by implantable cardioverter defibrillator (ICD) discharges. He had previously undergone an endocardial VT ablation. His recent CAG showed a chronic total right coronary artery occlusion with good collateral flow and no progression over time. Under treatment with bisoprolol, amiodarone, phenytoin, and lidocaine the clinical VT with a cycle length of 430 ms was reproducibly induced during the invasive EP study. Endocardial and epicardial mapping indentified a substrate in the inferior scar of myocardial infarction.
Thirty-three RF pulses were applied with a mean energy of 34 W from both epicardial and endocardial access in order to obtain scar homogenisation. At the conclusion of the procedure the VT was no longer inducible. There was no significant pericardial bleeding during and after the procedure. The patient was discharged home on oral bisoprolol, amiodarone, and phenytoin. At 3-month follow-up the patient was free of sustained ventricular arrhythmias.


A 50-year-old male presented with frequent PVCs causing marked exercise intolerance. His Holter recording revealed a high number of monomorphic PVCs (27,000/24 h) with bigeminy. Left ventricular ejection fraction was normal. Neither syncope nor sustained ventricular arrhythmias were documented, and the family history was unremarkable. During the previous invasive EP study an ablation had not been performed due to the absence of an adequate pace-map. Using the MNS to guide the catheter, a perfect 12 out of 12 pace-map was gained from the anterior epicardial aspect of the LVOT (Fig. ).
Delivery of three RF applications with an average energy of 27 W terminated the arrhythmia and rendered it non-inducible (also with isoproterenol infusion). The successful ablation site was 7 mm away from the left main coronary stem (Fig. ). No acute complications were observed, but in the early post-procedure period the patient developed a self-limiting sterile pericarditis with an insignificant amount of pericardial fluid. At follow-up after 3 months, PVCs were absent and the patient reported an improved physical condition. His prior antiarrhythmic medication was discontinued.


A 29-year-old male with frequent palpitations underwent three unsuccessful RF ablation procedures of a left-sided accessory pathway. Mapping ablation catheters were introduced epicardially and retrogradely in the left ventricle. After 39 unsuccessful ablation attempts with a mean energy of 27 W, thorough epicardial mapping revealed the earliest atrial retrograde activation site in the left lateral aspect of the mitral annulus.
A single RF application at the corresponding endocardial site by the retrograde catheter abolished the conduction over the accessory pathway. The post-procedural period was uneventful and he was discharged without antiarrhythmic medication. In his 3-month follow-up he was completely free of arrhythmias.


A 33-year-old female was presented to our out-patient clinic with a history of left hemifacial spasm for 5 years. Her past medical history was unremarkable. The patient underwent microvascular decompression in 2010 at our medical center ().
Intra-operatively, bilateral facial electromyography and brain stem auditory evoked potential was monitored. After the dura incision, gentle retraction of the cerebellum was applied using Greenburg retractor and we have exposed the lower cranial nerves. Dissection of the arachnoid was done from the rostral border of the lower cranial nerves. The arachnoid band was very tough and difficult to dissect, but no obvious injury occurred to the lower cranial nerve during the dissection. The left anterior inferior cerebellar artery was the offending vessel compressing the facial nerve from the caudal side and decompression was successfully completed without any adverse events and with no change in the intraoperative monitoring of brainstem auditory evoked potential. Patient recovered well from anesthesia with reduced facial spasm, compared to the pre-operative state. Postoperatively, neurologic exam revealed no abnormalities and also patient's swallowing and vocalization function was intact. Immediate postoperative brain CT scan performed on postoperative day showed no sign of hemorrhage.
On postoperative 5th day, the patient started to exhibit hoarsness with swallowing difficulty. Barium swallowing test was done to evaluate dysphagia, and minimal nasal regurgitation was demonstrated due to unilateral soft palate palsy. On endoscopic exam, performed by an otolaryngologist, the vocal cord was intact in both sides with unilateral left soft palate palsy, which suggested tensor and/or levator veli palatini muscle weakness (). Under the impression of possible lower cranial nerve palsy caused by postoperative edema of the nerve, steroid (prednisolone) was prescribed and we decided to observe the symptoms. Symptoms persisted with slight improvement with steroid therapy but repeatedly aggravated with steroid tapering. She received speech rehabilitation therapy for persistent difficulty of phonation and swallowing.
On postoperative 19th day, patient complained of difficulty breathing during sleep. Polysomnography was done to evaluate sleep apnea. Mild obstructive sleep apnea syndrome was observed secondary due to soft palate palsy. We encouraged her to sleep on her side; then her symptom improved.
Despite persistent speech rehabilitation, symptoms still persisted. We performed cranial nerve MRI to evaluate for the possibility of any structural abnormalities, such as delayed hematoma or possible infection. However, MRI showed no abnormal structural lesions or abnormal enhancement, which may suggest infection (). The patient continued to receive speech rehabilitation with subjective improvement of swallowing and speech.
On postoperative 25th day, the patient developed grade II facial palsy by House-Brackmann grade on the left side. Patient's facial palsy improved with administration of acyclovir for 5 days, but hoarseness with swallowing difficulty showed little improvement.
The patient received prolonged speech and swallowing rehabilitation therapy, and follow up examination was done before discharge showed improvement of nasalization, but persistent hypernasality. Patient was discharged with recommendation for prolonged rehabilitation.
Patient visited our out-patient for a follow up in postoperative 2 years. Endoscopic examination revealed normalized soft palate palsy and no abnormality in the vocal cord mobility (). However, patient's subjective difficulty of speech still persisted and we decided to observe her symptoms for any changes in the future.


A 34-year-old woman presented in our ED with chief complaints of severe acute epigastric pain referred to back, vomiting, and sudden onset breathlessness followed by Type I respiratory failure (arterial blood gas report). Somehow, intubation was delayed and she developed hypoxic cardiac arrest. Immediate critical care help was asked. After receiving initial 5 cycles of CPR she was intubated and mechanically ventilated with 100% oxygen and tidal volume and respiratory rate being 450 ml and 10, respectively. Adequate depth chest compression with full recoil and at a rate of hundred was performed at typically described on sternal position. Her carotid pulse was feeble and non-sustainable. ECG in monitor showed ventricular fibrillation (VF). 200 J Direct current (DC) shock was given in the conventional apex-sternal position (left antero-lateral) after 5 cycles of CPR. But even after DC shock, rhythm was not reverted into sinus. ABG showed severe acidosis with mild hyperkalemia. Intravenous adrenaline (ADR) 1 mg and sodium bicarbonate (8.4%) correction were given. Repeat DC shock after 1 minute of CPR and ADR failed to revert into sinus rhythm and no return of spontaneous circulation (ROSC). Then one intensivist continued CPR and other quick screened precordium with echocardiography (MicroMaxx Sonosite, Gurgaon, India) to see cardiac contractility if any. Intensivist found no heart chamber view in parasternal long axis, short axis, apical four chamber, and subcostal view as in all conventional site and surprisingly heart chamber was found visible in right side of the chest (CPR was stopped for few seconds only). We started CPR with slight modification. We place the heel of right hand on the right sternal margin (lower half of the sternum) and left heel of the other hand on top of the first (right handed) and tried to depress it 5 cm with each compression and allowing adequate chest wall relaxation. After 5 cycle effective CPR, we delivered DC shock again. During defibrillation, sternal paddle was on the right sterna margin and the apex paddle was under right breast tissue (right antero-lateral). This time rhythm reverted to sinus after defibrillation with 200 J (biphasic). We continued CPR for another few minutes and ultimately ROSC was achieved. ADR 0.1-0.5 mcg/kg/min was continued as infusion in titration. ROSC time was 8 minutes. She was shifted in ICU. Invasive monitoring lines were inserted under ultrasonography (USG) vision. There were no other features suggestive of Kartagenar syndrome in this patient or any congenital anomaly except this situs inversus and also had no other congenital structural heart disease. She was managed in ICU with mechanical ventilation, antibiotics, renal replacement therapy, and enteral-parenteral nutrition. Detailed radiological and cardiological evaluation and consultation were taken. Her brain MRI study was normal. Echocardiography (Echo) shown improved ejection fraction. Her pancreatitis required 40 days to resolve without any surgical intervention. She was then extubated uneventfully with intact neurological status and discharged from hospital next 5 days.


A 54-year-old female school teacher reported with a complaint of intermittent swelling inside the mouth on the left side. Repeated exacerbations of the swelling occurred 4-5 times in a span of 8 months. Itching of the cheek always preceded the swelling, which got resolved within 1-2 days. There was no change in sensation over the affected area. She had consulted many clinicians prior to reporting to our hospital and had taken medications. However, the patient's symptoms had persisted. The detail of past consultations or medications regarding presenting complaint was obscure. She gave a medical history of hypertension and dyslipidemia for which she was on medication since 5 years. General physical examination did not show any relevant findings. No cervical or generalized lymphadenopathy was found.
Extra-orally, diffuse swelling of left cheek was noted with no color changes. Intra-orally, examination of the left buccal mucosa revealed a hard movable mass of 1 cm × 0.5 cm anterior to the anterior border of ramus almost at the level of 27 []. Slight tenderness was present on palpation of the mass. Although the parotid papilla was prominent, it was not tender on palpation. Blood investigations were unremarkable. The erythrocyte sedimentation rates were mildly raised, Mantoux test was normal. Blood smear for staining malarial and filarial parasites were negative.
An occlusal radiograph placed vertically in the upper gingival sulcus in contact with the buccal mucosa to check for any soft-tissue calcifications did not reveal any findings.
Differential diagnoses of a parotid sialolith, minor salivary gland tumor, cysticercosis, calcified facial node, and lipoma were made.
Ultrasound scan of the cheek revealed a well-defined round hypo-echoic lesion in the muscular layer of the left cheek measuring 0.8 cm × 0.5 cm, with small specks of calcification. There were no obvious vascularity within the lesion and no perilesional edema []. The parotids appeared normal bilaterally. The probability of inflammatory pathology was considered.
Excisional biopsy of the nodule was carried out. Histological examination revealed a dense fibrous capsule showing infiltrate of mixed inflammatory cells consisting of lymphocytes, macrophages, eosinophils, and neutrophils. Within the capsule, a single female filarial worm showing “double uterus” appearance containing numerous larval forms was seen []. Further, magnification revealed a thick layed cuticle with multiple longitudinal ridges and centrally placed intestinal tubule within the well-developed musculature [Figures and ]. A definitive diagnosis of Dirofilariasis repens was arrived at.
Patient was treated with diethylcarbamazine (DEC) 100 mg tid for 3 weeks, albendazole 400 mg hs and cetrizine 5 mg Once daily for 5 days.
Following medication, patient was reviewed regularly for 8 months and found to be completely asymptomatic.


A 5-year-old boy presented to the Department of Pedodontics and Preventive Dentistry, with an abnormal upper front tooth. Past history and the family history were not relevant and there was no history of trauma. Examination revealed irregular morphology of teeth in the upper anterior region and there was fusion of left maxillary central incisor, lateral incisor and a supernumerary tooth and the fused teeth were free of caries []. The remaining primary teeth in both arches were normal. Occlusal radiograph revealed fusion of the central and lateral incisors to the supernumerary tooth with no clear distinction of root canal in the supernumerary tooth and agenesis of succedaneous upper left lateral incisor []. Parents were counseled regarding the anomaly and were made conscious about the concomitant problems expected with these anomalies. At 1-year follow-up, the succedaneous tooth germ of central incisor was found to be developing normally and he was advised regular follow-up.


A 46-year-old male patient presented with history of decreased appetite and diarrhea of 3 months duration. He gave a history of acute pancreatitis one year before at which time multi-detector computerized tomography (MDCT) imaging showed evidence of acute pancreatitis with no necrosis and a bulky tail of pancreas. A MDCT of the abdomen done now showed a well-circumscribed lesion in the tail of pancreas with infiltration into the splenic hilum []. The maximum diameter of the tumor was 3.5 cm. His Serum Chromogranin A level was 970ng/L. His rest of the lab parameters were within normal limits. He received pneumococcal and meningococcal vaccine two weeks before the planned surgery. A single incision laparoscopic distal pancreatectomy with splenectomy was performed.
Patient was placed in the supine position with a 45 degree left up position. A 3 cm vertical incision was chosen at the umbilicus and subcutaneous tissue was dissected off from the sheath. CO2 pneumoperitoneum of 15 mm Hg was achieved using a veress needle. One 12 mm and two 5mm ports were inserted as previously described by us []. A 10 mm, 45 degree scope was used for camera vision. Hook cautery, Harmonic shears (Ethicon – Endo-surgery, Johnson and Johnson) and Enseal device (Ethicon – Endo-surgery, Johnson and Johnson) were used for avascular dissection. At first the splenic flexure of colon was mobilized completely. The lesser sac was entered and widely opened by dividing the gastro-colic omentum. A gastric traction suture was placed to retract the posterior wall of stomach to the right, thereby exposing the lesser sac widely by a suture technique previously described by us to facilitate SIL splenectomy.[] The short gastric vessels were divided with Enseal device. The inferior border of pancreas was defined. Next, the superior border of pancreas was defined in the proximal and mid-body region of the pancreas. The dissection was next carried out from inferior border onto the posterior surface of pancreas in an avascular plane. The body of pancreas in the midbody region including the splenic vein was looped with a ribbon tape and a surgeons knot loosely tied the same anteriorly. A 2-0 prolene suture was passed into the abdomen from the subcostal region in the left anterior axillary line, through the ribbon tape loop and then exited from an area close to its previous entry point. The ends of the prolene loop were held with a hemostat. Traction on the hemostat facilitated anterior displacement of the body of pancreas facilitating further dissection. The distal body and tail were mobilized from the retroperitoneum in a plane anterior to the kidney in view of intense desmoplasia encountered adjacent to the tumor. The lienorenal ligament was divided using Harmonic shears. At this stage the distal body of pancreas and spleen were completely freed of all attachments. The pancreatic transection plane was chosen 2 cm proximal to the tumor. A 5 mm laparoscope was used during this step with an Endo GIA linear cutter 60 mm, white cartridge (Autosuture), passed through the 12 mm port for stapled transection at the chosen site []. The splenic artery was separately clipped with hemlock clips and divided. The pancreatic stump was overseen with running 2-0 prolene sutures. Fibrin sealant (Tisseal, Baxter) was applied at the pancreatic transection site. A 20F drain tube was placed adjacent to the transected end and the same was exited subcostally at the posterior axillary line. Hemostasis was checked. Keeping anterior leaf of the bag open using two interrupted prolene sutures hitching it to the anterior abdominal wall facilitated bagging of specimen. The specimen was placed in an endobag and was retrieved through the umbilical incision by joining the three port sites []. Sheath was closed with no 1 PDS suture and skin was approximated with staples []. On opening pancreas along long axis, solid cystic tumor was noted in the tail region of pancreas with distance of 2 cm from transected pancreas margin [Figures and ]. The nasogastric tube was removed on postoperative day 1. Patient was started on oral liquids on day 2 and gradually increased to a solid diet. The surgical drain was removed on day 4 patient was discharged on day five after surgery. Wound healed and skin clips were removed on 9th postoperative day. Two weeks following surgery, patient presented with fever and leukocytosis and was readmitted. MDCT showed evidence of 20 cc collection in relation to the pancreatic stump, which was drained by percutaneous catheter. He was treated with culture sensitive antibiotics and discharged after removal of percutaneous catheter after seven days. Histopathology of the resected specimen showed a very well differentiated neuroendocrine carcinoma with negative resected margin of pancreas and no lymphnode metastasis. At a postoperative follow-up of 30 months he remains asymptomatic, has chromogranin A levels within normal limits, with hardly discernable scar [] and has no recurrence on MDCT imaging.


A 31-year-old male presented with 1 week history of anorexia, headache, and gross hematuria. He did not have history of fever, urinary tract infection, stone disease, rash, arthralgia, night sweats, and weight loss, nor was there any significant past history. He was a non-smoker and non-alcoholic, and family history was not contributory. On examination, his blood pressure was 180/100 mmHg, heart rate was 100/min and he had mild pallor. Systemic examination was normal and fundus examination did not reveal any changes of hypertensive retinopathy.
Investigations revealed normal hemoglobin (11.5 g/l), total leukocyte count (9500/dl) and platelet count (2.4 × 106/dl), but increased serum creatinine (4.8 mg/dl). Urine examination revealed proteinuria (4+) and dysmorphic RBCs (>80% of RBCs), and his 24-h urine protein excretion was 3.8 g. For evaluation of gross hematuria, he underwent cystoscopic examination, ultrasonography and non-contrast computerized tomography of the genitourinary tract, which were normal. Anti-nuclear antibodies, anti-neutrophil cytoplasmic antibodies, anti-glomerular basement membrane antibodies and serum cryoglobulins were negative, so were hepatitis B surface antigen, anti-hepatitis C antibody and human immunodeficiency virus. His renal function further deteriorated, requiring initiation of hemodialysis, and subsequently, a kidney biopsy was performed. The kidney biopsy specimen contained nine glomeruli, and consisted of both cortex and medulla. Light microscopy examination of the biopsy revealed that all the glomeruli showed mesangial expansion, hypercellularity and mesangial nodules. Cellular crescents were present in two glomeruli along with focal glomerular basement membrane thickening. The tubulointerstitial compartment showed fibrosis and atrophy involving <30% of the biopsy area, while the blood vessels were normal []. The mesangial nodules were periodic acid-schiff (PAS) positive but Congo red negative. Immunofluorescence examination revealed 2+ to 3+ diffuse linear staining for kappa light chains in mesangium and tubular basement membrane []. Electron microscopy examination showed powdery electron-dense material in the lamina rara interna of the glomerular basement membrane as well as on the outer aspect of the tubular basement membrane [ and ]. With these findings, a diagnosis of LCDD was made and he was investigated further.
Serum electrophoresis was performed, which revealed a monoclonal band in the gamma region that, on immunofixation, was confined to the kappa and IgG regions. Serum kappa free light chain level was elevated and bone marrow examination revealed 8% plasma cells. Thus, the LCDD was thought to be non-myeloma related. He was started on bortezomib, thalidomide and dexamethasone regimen. After four cycles, his hematuria settled; however, renal functions did not improve. Repeat serum immunofixation was negative. He was initiated on maintenance hemodialysis on which he is doing well since the last 3.5 years and is planned for deceased donor renal transplantation.


In 2009, a 11-year-old Indian Muslim girl was referred to the Oral Medicine Clinic, CSM Medical University, Lucknow, India by Public Health Dentist during “School oral health care program”, with a complaint of reduced mouth opening, discomfort and a burning sensation particularly when eating spicy foods.
Patient showed history of areca nut chewing since seven year; the reduced mouth opening was noticed from past one year, with discomfort and a burning sensation particularly when eating hot and spicy foods.
Intraoral examination revealed that her entire oral mucosa was pale, especially the buccal mucosa, which does not show areas of erosion and the hard palate, which was completely blanched or opaque. Her tongue, uvula and soft palate were normal.
Dental examination reveals carious lesion in posterior tooth and poor oral hygiene.
Physical examination reveals a mouth opening of 14 mm and absence of fibrotic bands.


In 2011, another 10-year-old Indian Muslim boy who had not been attending school at all was referred to the Oral Medicine Clinic, CSM Medical University, Lucknow, India by Public Health Dentist during the “Community oral health screening program”, with a complaint of reduced mouth opening, discomfort and a burning sensation particularly when eating spicy foods.
Patient showed history of areca nut chewing since six year; the reduced mouth opening was noticed from past eight month, with discomfort and a burning sensation particularly when eating hot and spicy foods and loss of gustatory sensation.
Clinical examination of soft tissue of oral cavity reveals blanched or opaque appearance of mucosa, reduced mobility of the soft palate and tongue, loss of tongue papillae and dryness of the mouth.
Dental examination reveals dental fluorosis in the anterior teeth, carious lesion in posterior tooth and a poor oral hygiene.
Physical examination reveals mouth opening is 13 mm, with presence of palpable fibrous band especially in the retro molar area but does not involve the uvula and tonsil.


A 15 year old girl was referred to urogynecology department (April’08) with severe urinary incontinence since childhood.
Cloacal anomaly was detected soon after birth (March’93) which was managed successfully by the paediatric surgeon with colostomy, anal transposition and colostomy closure. In view of non visualisation of vaginal opening, vaginoplasty at a later date was suggested by the paediatric surgeon.
A complete workup was done in the urogynecology department. Local examination under anaesthesia showed a very wide and patulous urethra with urinary leakage. A narrow vaginal opening was lying lateral to the urethral orifice (instead being inferior) with a bridge of tissue separating the two []. The vaginal opening could be dilated to expose a normal cervix and adequate vaginal length that did not require vaginoplasty. Radiological imaging (Ultrasonography, Computed Tomography imaging, Micturating Cystourethrogram, intravenous urogram) confirmed normal upper urinary tract. Bladder appeared normal in capacity as well as contour. Features of stress urinary incontinence with markedly decreased valsalva leak point pressure were depicted in the urodynamic assessment. There were no features of bladder outlet obstruction or detrusor overactivity. Cystourethroscopy confirmed patulous short urethra with significant sphincter deficiency.
Developmental urethral anomaly as part of urogenital sinus maldevelopment was the cause of her incontinence so reconstruction of urethra and augmentation of urethral sphincter with autologous fascia lata as pubovaginal sling was done on 11.1.10. During the procedure the dense fibrous scar tissue between urethra and vagina was dissected. After mobilizing the remaining urethra vaginal wall flap was used to lengthen the urethra and reconstruct the external urethral meatus. A harvested strip of fascia lata was laid under the proximal urethra as pubovaginal sling.
Persistence of significant urinary incontinence was observed in the post operative period. Re-evaluation was done after six months and confirmed persistent short (approximately one cm) urethra with sphincter deficiency. A second reconstruction was planned to form a neourethra. Continent diversion was also an available option but was deferred considering her age and child bearing prospective. Restoring continence with a near normal functional anatomy remained the prime objective.
On 21.01.11 examination revealed a nonexistent urethra and bladder neck and the only opening discharging urine was identified as bladder. It was decided to reconstruct the urethra from tubularised anterior bladder wall (modified Tanagho flap). Parallel incisions were made in the anterior wall of the bladder to form a 5 cm × 2 cm rectangular bladder flap preserving its proximal attachment. Scarring around the bladder opening due to previous surgery demanded abdominal mobilization of the bladder. Further extraperitoneal mobilization of the bladder made the anterior wall flap tension free which was then tubularised to create the neourethra. The distal end was brought down at the expected site of meatus to form the external urethral meatus.
Laparotomy also revealed a lateral fusion defect that included a relatively well developed communicating left horn, rudimentary right horn and a right endometriotic cyst of 4 cm × 5 cm which was drained.
Urinary drainage was ensured by suprapubic and urethral catheter post operatively. Continuous periurethral leak (leak around the catheter) was observed in second post operative week which was treated with antimuscarinics. Menstrual suppression was given for six months in view of endometriotic cyst.
Initially frequency, urgency and urge incontinence was significant which improved with antimuscarinics and bladder training. Four months following the surgery, patient has achieved a continent period of 2 hours which is socially acceptable to her and is likely to improve further with bladder training. A normal urinary flow rate and no residual urinary volume excluded bladder outlet obstruction. However, regular and longer follow up with symptomatic and functional assessment is required.


A 77-year-old man presented with gait disturbance that had persisted for 1 month. He had a history of dementia and depression for which he had received medication. Brain magnetic resonance imaging (MRI) revealed atrophy and venous congestion. Computed tomography (CT) angiography revealed DAVF of the superior sagittal sinus (SSS) (). Transfemoral cerebral angiography (TFCA) showed that the SSS DAVF was supplied via multiple branches of the external carotid artery (ECA), the right anterior cerebral arteries, and the meningeal branches of the internal carotid artery (ICA) and vertebral artery (). Anterograde drainage was not observed through the SSS, while drainage through the vein of Labbe and sphenoparietal sinus was main. TFCA revealed venous hypertension and galenic venous drainage. Furthermore, TFCA showed CVR (). The DAVF was large and complex. According to Borden classification, the DAVF was type 2.
Embolization of DAVF was performed under general anesthesia using a biplanar fluoroscopic unit. Right femoral artery and left femoral vein were punctured with an angiography needle (18 gauge). A 6Fr Envoy guiding catheter (Codman, Miami Lakes, Florida, USA) was then introduced to the right ECA. Another Envoy catheter was introduced to the right internal jugular vein through the venous root. Heparin was administered in order to achieve a targeted activated coagulation time of 250-300s throughout the procedure. Using a transvenous approach, a microcatheter (Echelon 10) was placed at the fistulous sac of the SSS (). Eleven coils (GDC 360 Standard 24 mm × 40 cm (×7), 22 mm × 40 cm (×3), 20 mm × 33 cm (×1)) were inserted through the microcatheter (Echelon 10). Another microcatheter (Prowler Select 18) was subsequently used to insert non-detachable coils; this was placed using Synchro 14 microwire. Other coils (Interlock Coil 14 mm × 30 cm (×2), 14 mm × 20 cm (×1), 12 mm × 30 cm (×1), 12mm × 20 cm (×1), Nester Coil 10 mm × 14 cm (×2), 6 mm × 14 cm (×3), 4 mm × 14 cm (×3)) were inserted through this microcatheter. Following intravenous coil embolization using various coils, DAVF was occluded partially. Angiography revealed that retrograde drainage through the fistula remained (). A Scepter C balloon catheter (4×15 mm) was placed at the most distal segment of the middle meningeal artery (MMA). The tip of the balloon microcatheter was positioned at the DAVF. After that, balloon inflation and deflation was successfully carried out. () In order to occlude any residual fistula, Onyx18 was then injected through the dual lumen balloon catheter positioned in the MMA. Over a period of 22 minutes, a total of 4.5 cc of Onyx18 was injected, after which the DAVF was almost completely occluded (). Following embolization, angiography revealed no arteriovenous shunting or CVR (). The balloon was deflated by syringe suction without difficulty. The catheter was then immediately removed without any noticeable adherence to the Onyx cast. Procedure related complications were not observed. The patient symptoms were unchanged during hospitalization.


A 49-year-old Greek Cypriot male was referred to us with a history of four episodes of meningitis over the past 15 years (1998, 1999, 2002 and 2009). The first three episodes had been treated elsewhere, each time presenting with headache, fever, photophobia but no rash. Though no bacterial diagnosis was ever made and no organism isolated, his episodes were associated with high cerebrospinal fluid (CSF) protein and CSF neutrophil infiltrate. He responded well to intravenous antibiotic treatment on each occasion with no neurological deficit. Computerized tomography (CT) and magnetic resonance imaging (MRI) head imaging had not detected the cause of his recurrent meningitis.
He had had two Staphylococcus aureus infections of his umbilicus in 2002 and 2009 but otherwise his medical history was unremarkable. There was no history of head trauma nor of recurrent ear or sinus infections. He did report some non-specific upper respiratory tract symptoms but none suggestive of CSF rhinorrhoea.
Detailed investigation of his immune system revealed low mannose-binding lectin (MBL) levels (0.06 mg/L, normal range 1.0–4.0 mg/L). The rest of his humoral and cellular immune tests were normal. A coronal CT sinus scan done to investigate his non-specific upper respiratory tract symptoms showed a 4 × 3 mm defect in the cribriform plate adjacent to the vertical attachment of the middle turbinate, a relatively common site for ‘congenital’ defects (). A small rim enhancing CSF intensity sac was shown on MRI just beneath the cribriform plate defect, consistent with a meningocoele. This was confirmed at surgery. The defect was surgically repaired with temporalis fascia and inferior turbinate mucosa by an endonasal endoscopic route.


A 21-year-old Caucasian male was reviewed in our clinic with a history of Streptococcus pneumoniae meningitis and bacteraemia complicated by pneumonia requiring intensive care unit admission. He recovered well following treatment with intravenous ceftriaxone. Assessment of his immune system revealed an isolated borderline low IgG level (6.3 g/L, normal range 7–16 g/L), borderline low C4 level (15 mg/dL, normal range 16–54 mg/dL) and low pneumococcal antibody titres (16 mg/L, protective range >50). At the time of his first episode, his IgG level was also noted to be low (5.1 g/L, normal range 7–16 g/L). Viral testing was negative. He was vaccinated with 23-valent pneumococcal polysacharide vaccination and made an appropriate antibody response.
He continued to report tiredness and general malaise and just over a year later, at age 23, developed a second episode of pneumococcal meningitis. This again responded well to intravenous antibiotics. Serotype-specific pneumococcal antibodies were measured after the second episode and low titres to 7 of the 13 measured pneumococcal serotypes were found. There was no history of head trauma with either episode nor of CSF rhinorrhoea.
The past medical history included autoimmune hepatitis and Crohn’s disease, requiring low-dose maintenance oral steroids. Other medication intermittently used included mesalazine, mercatopurine and methotrexate. He had had recurrent tonsillitis as a child and had a tonsillectomy at the age of 20.
Initial routine CT head was reported as normal. However, detailed sinus CT showed an anomalous, prominent right agger nasi ethmoid air cell extending up to the skull base with sclerotic thickened bony walls and opacification within (). This was diagnosed as a mucocoele of this anomalous air cell. It was surgically decompressed.
Long-term pneumococcal antibiotic prophylaxis and pneumococcal vaccination were given as preventative measures. Currently, his IgG and complement levels are within normal range.