title: Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure.
abstract: A 54-year-old woman presented with a six-month history of episodic confusion and progressive ataxia. A comprehensive metabolic panel was notable for elevated values of alkaline phosphatase (161 U/L), total bilirubin (1.5 mg/dL), and serum ammonia of 300 umol/L (normal range 9-47). Hepatitis panel, relevant serological tests, tumor markers (CA-19-9, CEA), and urea cycle enzyme studies were unrevealing. Lactulose and rifaximin therapy failed to normalize serum ammonia levels. Imaging revealed a structural vascular abnormality communicating between an enlarged inferior mesenteric vein and the left renal vein, measuring 16 mm in greatest diameter. The diagnosis of congenital extrahepatic portosystemic shunt was made and endovascular shunt closure was performed using a 22 mm Amplatzer II vascular plug. Within a day, serum ammonia levels normalized. Lactulose and rifaximin were discontinued, and confusion and ataxia resolved.
authors.affiliation: Department of Radiology and Oncology, Rabin Medical Center, 49100 Petah Tikva, Israel.
authors.forename: Eldad
authors.initials: E
authors.lastname: Elnekave
authors.affiliation: Department of Radiology, Rabin Medical Center, 49100 Petah Tikva, Israel.
authors.forename: Eugenia
authors.initials: E
authors.lastname: Belenky
authors.affiliation: Department of Gastroenterology, Easton Hospital, 250 S 21st Street, Easton, PA 18042, USA.
authors.forename: Lindsley
authors.initials: L
authors.lastname: Van der Veer