Case 314: Cerebral Proliferative Angiopathy.

HISTORY: An 18-year-old man presented to the neurosurgery outpatient department with recurrent episodes of epistaxis for the past 8 years and altered behavior for the past month. Epistaxis was scanty in amount, intermittent, spontaneous, and not associated with any trauma or nasal obstruction or breathing difficulties. Bleeding used to stop spontaneously after some time. There was no history of associated headache, seizures, vomiting, fever, or loss of consciousness. On physical examination, the patient was afebrile, with normal vital signs and normal Glasgow Coma Scale score (15 of 15) at the time of presentation. Multiple dilated engorged veins were visible on the forehead; however, there was no evidence of abnormal skin pigmentation. Neurologic examination yielded findings that were within normal limits. Laboratory examinations revealed a hemoglobin level of 11 g/dL (normal range, 13.2-16.6 g/dL), with the rest of the parameters within normal limits. This patient underwent unenhanced CT of the brain and paranasal sinuses followed by contrast-enhanced MRI of the brain for further evaluation.

Case 314.

HISTORY: An 18-year-old man presented to the neurosurgery outpatient department with recurrent episodes of epistaxis for the past 8 years and altered behavior for the past month. Epistaxis was scanty in amount, intermittent, spontaneous, and not associated with any trauma or nasal obstruction or breathing difficulties. Bleeding used to stop spontaneously after some time. There was no history of associated headache, seizures, vomiting, fever, or loss of consciousness. On physical examination, the patient was afebrile, with normal vital signs and normal Glasgow Coma Scale score (15 of 15) at the time of presentation. Multiple dilated engorged veins were visible on the forehead; however, there was no evidence of abnormal skin pigmentation. Neurologic examination yielded findings that were within normal limits. Laboratory examinations revealed a hemoglobin level of 11 g/dL (normal range, 13.2-16.6 g/dL), with the rest of the parameters within normal limits. This patient underwent unenhanced CT of the brain and paranasal sinuses followed by contrast-enhanced MRI of the brain for further evaluation (Figs 1-3).

51-year old man with tingling, burning and progressive limb weakness.

Peripheral neuropathy is a common reason for referral to neurology. Chronic acquired demyelinating neuropathies are an important and varied group with overlapping presentations, and may have an immune-mediated cause. Their correct diagnosis is important as they respond to different treatments; timely intervention can prevent irreversible axonal degeneration. We present a case that highlights the approach to an adult presenting with a chronic demyelinating neuropathy.

Endovascular Occlusion of Cervical Internal Carotid Artery Pseudoaneurysm in a Child Treated by N-Butyl Cyanoacrylate: A Rare Case Report.

We report a rare case of spontaneous extracranial cervical internal carotid artery (ICA) pseudoaneurysm in a female child aged 3 years who presented with a swelling in the neck which had bled following an attempted incision as it had been thought to be an abscess. A CT angiogram and an MR angiogram were not very conclusive to diagnose the exact site of origin and the morphology of the aneurysm. Digital subtraction angiography revealed a dissecting pseudoaneurysm of the right extracranial cervical ICA. The right ICA was ending as a pseudosac, and the right cerebral circulation was filling up through the right posterior cerebral artery. To minimize the radiation exposure, a microcatheter was placed inside the diagnostic catheter. The aneurysm sac was occluded using N-butyl cyanoacrylate since there was no distal flow to the brain from the artery beyond the aneurysm. It was a safe, effective and cheaper alternative to open surgery or to other endovascular management options available. Not all neck swellings are abscesses, and they should be examined and evaluated to exclude a vascular cause.

Spinal dural arteriovenous fistula in a child: a rare presentation.

Spinal dural arteriovenous fistula (AVF) is an abnormal shunting between the segmental artery and radicular vein adjacent to the dural root sleeve in the spine. This is the most common vascular malformation of the spinal cord and is a rare but treatable cause of para or quadriplegia. It most commonly occurs in elderly men and often affects the thoracolumbar region. These patients clinically present with progressive myelopathies, and other autonomic symptoms (e.g., bladder and bowel dysfunction) subsequently in the later course of the disease. Computed tomography angiography and magnetic resonance imaging remain the modality for initial evaluation. Herein, we present a rare case of spinal dural AVF in a child along with a review of imaging modalities. To the best of our knowledge, there are few case reports of this condition in a paediatric age group.

Jejunal strongyloidiasis mimicking crohn's colitis: A diagnostic dilemma!

ABSTRACT: We report an unusual case of jejunal strongyloidiasis presenting as chronic malabsorption and intractable small bowel diarrhea in an immunocompetent adolescent boy who posed a diagnostic challenge for pathologists, radiologists, and gastroenterologists. Histopathology revealed chronic active colitis and was consistent with the clinicoradiological diagnosis of Crohn's colitis but nonresponse to immunomodulators warranted full-thickness jejunal biopsy through laparotomy which showed numerous larvae and eggs of Strongyloides. There is a need to increase the awareness of Strongyloides colitis given its high rate of misdiagnosis and mortality as the correct diagnosis can avoid a fatal outcome of this curable disease.

Natural History of Asymptomatic Walled-off Necrosis in Patients With Acute Pancreatitis.

Background and objectives Studies on the natural history of asymptomatic walled-off necrosis (WON) in acute pancreatitis (AP) are scarce. We conducted a prospective observational study to look for the incidence of infection in WON. Material and methods In this study, we included 30 consecutive AP patients with asymptomatic WON. Their baseline clinical, laboratory, and radiological parameters were recorded and followed up for three months. Mann Whitney U test and unpaired t-tests were used for quantitative data and chi-square and Fisher's exact tests were used for qualitative data analysis. A p-value <0.05 was considered significant. Receiver operating characteristic curve (ROC) analysis was done to identify the appropriate cutoffs for the significant variables. Results Of the 30 patients enrolled, 25 (83.3%) were males. Alcohol was the most common etiology. Eight patients (26.6%) developed an infection on follow-up. All were managed by drainage either percutaneously (n=4, 50%) or endoscopically (n=3, 37.5%). One patient required both. No patient required surgery and there was no mortality. Median baseline C-reactive protein (CRP) was higher in infection group 76 (IQR=34.8) mg/L vs asymptomatic group, 9.5 mg/dl (IQR=13.6), p<0.001. IL-6 and tumor necrosis factor (TNF)-alpha was also higher in the infection group. The size of the largest collection (157.50±33.59 mm vs 81.95±26.22 mm, P<0.001) and CT severity index (CTSI) (9.50±0.93 vs 7.82±1.37, p<0.01) were also higher in infection group as compared to the asymptomatic group. ROC curve analysis of baseline CRP (cutoff 49.5mg/dl), size of WON (cutoff 127mm) and CTSI (cutoff of 9) showed AUROC (area under ROC) of 1, 0.97, and 0.81 respectively for the future development of infection in WON. Conclusion Around one-fourth of asymptomatic WON patients developed an infection during three-months follow-up. Most patients with infected WON can be managed conservatively.

Undifferentiated carcinoma with osteoclast-like giant cells of pancreas on cytology: A case report with review of literature.

Undifferentiated carcinoma with osteoclast-like giant cells (UC-OGC) is a rare malignant neoplasm accounting for <1% of pancreatic masses. Very few case reports and small series have described the cytomorphological features of this entity. We report a case of UC-OGC arising in the pancreas presenting with liver metastasis in a 56-year-old man diagnosed by guided fine-needle aspiration cytology (FNAC). A characteristic biphasic pattern comprising of malignant mononuclear cells with scattered bland giant cells were the hallmark features for cytological diagnosis. Our case along with review of cytology literature emphasize the utility of FNAC and the cell block in the diagnosis and management of this rare entity.

Paraparesis in adult aortic coarctation: Reversal by stent supported angioplasty.

Aortic coarctation presenting with neurological complications as compressive myelopathy is rare. We report a case of a 43-year-old, hypertensive, female who presented with gradually progressive paraparesis over 4 years. She was diagnosed to be having coarctation of the aorta with intra-spinal collaterals causing compressive myelopathy. She underwent successful percutaneous endovascular implantation of a balloon-expandable aortic stent to relieve her aortic coarctation. This led to regression of her intra-spinal collaterals relieving her cord compression. This nonsurgical modality treatment proved to be safe and effective in relieving her hypertension and neurological complication of paraparesis. Learning objectives: •To recognize that paraparesis can be a rare manifestation of coarctation of the aorta.•To highlight the importance of treating the primary pathology of coarctation of the aorta in such critically ill therapeutically challenging patients.

A study of correlation of angioarchitecture of intracranial blood vessels with difficulty during endovascular coiling of aneurysms.

OBJECTIVE: The duration and complications of coiling are dependent on several factors. These include experience with the armamentarium and the expertise of the operator. Also, the angioarchitecture of the blood vessel can play an important role in the duration of the procedure and outcome. METHODS: 41 patients underwent endovascular coiling. The angioarchitecture of the vessels in terms of the course of the blood vessels from the arch of the aorta to the aneurysm, and the angles between the arch and its branches, between the parent and feeding arteries, and between the feeding artery and the aneurysm were measured. During coiling, duration, attempts, complications, and outcome were recorded. A correlation was made between angioarchitecture and difficulty during coiling. RESULTS: Based on number of attempts of coiling, two groups were defined. In group I, 26 patients underwent a single attempt with a mean duration of 61 min and in group II, 15 patients had more than one attempt with a mean duration of 98 min. The mean angle between the arch and its branches, between the internal carotid artery and the anterior cerebral artery, and between the anterior communicating artery and the aneurysm was more acute in group II compared with group I. Spearman's correlation suggested that as the angle at different vessel levels decreased, duration, attempt, and complications increased, and vice versa. CONCLUSIONS: Angioarchitecture analysis revealed that the sharper the angle (acute angle) between various vessels, the greater the difficulty in negotiating a microcatheter through the vessels and the more complications, duration, number of attempts, and poor outcome. We therefore feel that angioarchitecture analysis should be done carefully in all patients in whom the decision to undergo coiling is taken.

Lipomatous ependymoma: report of a rare differentiation pattern with a comprehensive review of literature.

We report the case of a 13-year-old girl presenting with left-sided hemiparesis, altered sensorium and episodic headache with bouts of projectile vomiting. Imaging revealed a large heterodense intraventricular mass lesion displaying focal calcification and hyperintensity on T1- and T2- weighted fluid attenuated inversion recovery (FLAIR) magnetic resonance images suggesting the presence of intratumoral fat. Histologically, the tumour showed sheets of glial cells, focal perithelial rosettes and individual cells showing fat vacuoles. The morphological impression was of an ependymoma with lipomatous differentiation. Glial fibrillary acid protein (GFAP) immunohistochemistry revealed positivity in the cytoplasmic processes of the tumour cells as well as in the cytoplasmic rim of the cells having an adipocytic appearance. S100 and vimentin were also immunoreactive. Ultrastructural studies confirmed the ependymal differentiation of the tumour and the presence of an osmiophilic fat component confirming the diagnosis. After 1 year of follow-up, the patient presented with similar complaints and MRI evidence of recurrence of the tumour. A comprehensive literature review revealed that half of the reported cases of this pattern recurred suggesting a possibly tenacious clinical course.

High patency of proximal splenorenal shunt: A myth or reality ? - A prospective cohort study.

BACKGROUND: Proximal splenorenal shunt (PSRS) is a well-accepted surgical procedure for non-cirrhotic portal hypertension (NCPH). Though a patent shunt is important for good long term outcome, there are very few studies on patency of these shunts. We analysed shunt patency using dynamic computed tomographic (CT) portography and compared it with other modalities. METHODS: From 2004 to 2014, 50 patients with PSRS were evaluated prospectively for shunt patency using dynamic CT portography, clinical parameters and ultrasound Doppler. RESULTS: The causes of NCPH were extrahepatic portal vein obstruction (EHPVO) in 38 patients and non-cirrhotic portal fibrosis (NCPF) in 12 patients. The shunt patency rate using clinical parameters, ultrasound Doppler and dynamic CT portography were 70%, 40% and 60% respectively. Clinical parameters overestimated while ultrasound Doppler underestimated the shunt patency rate. Dynamic portography had 100% correlation with conventional angiography in the five patients when this was done. The site of shunt could be demonstrated convincingly by dynamic CT portography. The shunt patency rate decreased over time. It was 64%, 60% and 43% in <1 year, 1-5 years and >5 years respectively. Our NCPF patients had a greater shunt patency rate compared to EHPVO patients (9/12 vs. 21/38) though the difference was not significant. Only size of the splenic vein had a significant impact on the shunt patency rate on statistical analysis. CONCLUSIONS: Dynamic CT portography is useful for evaluation of shunt patency. Proximal splenorenal shunts have a high blockage rate which has hitherto not been reported.

Juvenile Nasopharyngeal Angiofibroma: Case report with review on role of imaging in diagnosis.

Juvenile nasopharyngeal angiofibroma is a locally aggressive benign vascular neoplasm, composed of vasogenic and myofibroblastic elements, accounts for 0.05-0.5% of all the head and neck neoplasms. There are very few case reports of nasopharyngeal angiofibroma involving the oral cavity; we report a case involving both the maxilla and mandible in a 17-year-old patient who reported with a large firm swelling on right side of face with recurrent epistaxis and headache. Magnetic resonance angiography revealed a large lobulated enhancing soft tissue mass, which was hypointense on T1-weighted image and heterogeneously hyperintense on T2-weighted image causing expansion of pterygopalatine fossa and sphenopalatine foramen with extension into the sphenoid sinus, ethmoid air cells, right nasal cavity, right infratemporal fossa and right maxillary sinus with remodeling of right zygomatic arch and part of body and ramus of mandible. It was supplied by the right external carotid artery. Patient was referred to the department of neurosurgery for further management. The diagnosis at an early stage is important because it is associated with high risk of morbidity, but advances in imaging, and surgical methods of treatment have changed the sites associated with high risk of morbidity.

Absent right coronary artery: A case of single coronary artery or congenital ostial atresia?

Atresia of the right coronary artery ostium is a rare anatomic variant of the coronary circulation. It is often difficult to differentiate from single coronary artery. Its presence unassociated with any other anomaly has never been described in an adult individual. We report this unusual anomaly and discuss its anatomical and pathophysiological significance and possible ways to differentiate from single coronary artery.

The current approach to the diagnosis of vascular anomalies of the head and neck: A pictorial essay.

Throughout the years, various classifications have evolved for the diagnosis of vascular anomalies. However, it remains difficult to classify a number of such lesions. Because all hemangiomas were previously considered to involute, if a lesion with imaging and clinical characteristics of hemangioma does not involute, then there is no subclass in which to classify such a lesion, as reported in one of our cases. The recent classification proposed by the International Society for the Study of Vascular Anomalies (ISSVA, 2014) has solved this problem by including non-involuting and partially involuting hemangioma in the classification. We present here five cases of vascular anomalies and discuss their diagnosis in accordance with the ISSVA (2014) classification. A non-involuting lesion should not always be diagnosed as a vascular malformation. A non-involuting lesion can be either a hemangioma or a vascular malformation depending upon its clinicopathologic and imaging characteristics.

Malignant extra-renal rhabdoid tumor with unusual presentation: A report of two cases.

Malignant extra-renal rhabdoid tumor (MERT) is a rare highly aggressive tumor that occurs in young children with the very poor clinical outcome. The tumor is characterized by a diffuse proliferation of "rhabdoid cells," which are round or polygonal with eccentric nuclei, prominent nucleoli, and glassy eosinophilic cytoplasm containing hyaline-like inclusion bodies. However, rhabdoid cells are also seen in certain other soft tissue sarcomas such as proximal-type epithelioid sarcoma, rarely synovial sarcoma, and extra-skeletal myxoid chondrosarcoma. Because of its poor prognosis and histomorphological similarities with other soft tissue tumors, an accurate diagnosis is required using a wide immunohistochemical panel. Very few cases of MERT have been reported in the literature and to our knowledge none in the supra-glottis area. Due to the rarity and poor outcome of this tumor, we are reporting two cases of MERT.