Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure

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,...

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Main Authors: Eldad Elnekave, Eugenia Belenky, Lindsley Van der Veer
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Radiology
Online Access:http://dx.doi.org/10.1155/2015/852853
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author Eldad Elnekave
Eugenia Belenky
Lindsley Van der Veer
author_facet Eldad Elnekave
Eugenia Belenky
Lindsley Van der Veer
author_sort Eldad Elnekave
collection DOAJ
description 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.
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series Case Reports in Radiology
spelling doaj-art-9020bce002054ff6b8b991ec4d876bec2025-02-03T05:45:34ZengWileyCase Reports in Radiology2090-68622090-68702015-01-01201510.1155/2015/852853852853Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular ClosureEldad Elnekave0Eugenia Belenky1Lindsley Van der Veer2Department of Radiology and Oncology, Rabin Medical Center, 49100 Petah Tikva, IsraelDepartment of Radiology, Rabin Medical Center, 49100 Petah Tikva, IsraelDepartment of Gastroenterology, Easton Hospital, 250 S 21st Street, Easton, PA 18042, USAA 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.http://dx.doi.org/10.1155/2015/852853
spellingShingle Eldad Elnekave
Eugenia Belenky
Lindsley Van der Veer
Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure
Case Reports in Radiology
title Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure
title_full Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure
title_fullStr Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure
title_full_unstemmed Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure
title_short Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure
title_sort noncirrhotic extrahepatic portosystemic shunt causing adult onset encephalopathy treated with endovascular closure
url http://dx.doi.org/10.1155/2015/852853
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AT lindsleyvanderveer noncirrhoticextrahepaticportosystemicshuntcausingadultonsetencephalopathytreatedwithendovascularclosure