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Abernethy malformation is a rare congenital anomaly in which there is direct communication between the portal and systemic venous circulation. The clinical presentation ranges from asymptomatic with incidental detection on imaging to secondary complications of disease or related to associate anomalies. This is a retrospective analysis of data from nine patients with Abernethy malformation at a single center. This is a referral center for Pediatric Cardiology and for Hepatobiliary and Pancreatic Surgery. The patients presented to the Pulmonary Hypertension Clinic/the Hepatobiliary Surgery Clinic. Out of nine patients, four were male. Type II Abernethy malformation was present in five patients whereas three patients had type I malformation. One of the patients had communication between inferior mesenteric vein and internal iliac vein. Five out of nine patients were erroneously diagnosed as idiopathic primary pulmonary hypertension and were treated with vasodilators. One patient required living donor liver transplant. One patient was managed with surgical shunt closure whereas two patients required transcatheter shunt closure. The rest of the patients were managed conservatively. Abernethy malformation is more common than previously thought and the diagnosis is often missed. There are various management options for Abernethy malformation, which includes surgical or transcatheter shunt closure and liver transplant. Management of Abernethy malformation depends upon type, presentation, and size of shunt.

Background: Malrotation with midgut volvulus is a common surgical emergency in children. A diagnostic tool for malrotation with characteristics ideal in emergency settings such as non-invasiveness and rapidity remains a keenly debated issue among surgeons and radiologists alike., Methods: Fifty-two pediatric patients of suspected malrotation with midgut volvulus were studied between 1998 and 2016. All patients underwent plain abdominal X-ray and Color Doppler; contrast upper gastrointestinal (GI) studywas done in some patients. All cases were operated and diagnosis was confirmed. A subset of 60 pediatric patients with nonspecific GI complaints were also examined to see relative position of superior mesenteric vein (SMV)/superior mesenteric artery (SMA) in control population., Result: A total of 52 suspected cases of malrotation were admitted from May 1998 to November 2015, 43 had inversion of SMA/SMV and nine had SMV anterior of SMA in Color Doppler. All 43 cases of inversion of SMA/SMV were cases of malrotation after surgical confirmation; while five out of nine cases of SMV anterior to SMA had malrotation., Conclusion: In appropriate clinical settings, Color Doppler documenting the reversal or aberrant SMV/SMA axis is not only predictive but also diagnostic of malrotation of gut.

Porto-mesenteric venous thrombosis following a trans-arterial occlusion of a superior mesenteric arteriovenous fistula is a rare occurrence. We present a case of endovascular management of one such case treated pharmacomechanically with catheter-directed mesenteric thrombolysis and transjugular intrahepatic portosystemic shunt creation without long-term successful outcome.