Case Report
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World J Hepatol. Mar 27, 2013; 5(3): 137-144
Published online Mar 27, 2013. doi: 10.4254/wjh.v5.i3.137
Portal hypertensive biliopathy: A single center experience and literature review
Vanessa Suárez, Andrés Puerta, Luisa Fernanda Santos, Juan Manuel Pérez, Adriana Varón, Rafael Claudino Botero
Vanessa Suárez, Luisa Fernanda Santos, Adriana Varón, Rafael Claudino Botero, Gastroenterology and Hepatology Service, Department of Internal Medicine, Fundación Cardioinfantil, Bogotá 110131, Colombia
Andrés Puerta, Juan Manuel Pérez, Department of Diagnostic Imaging, Fundación Cardioinfantil, Bogotá 110131, Colombia
Author contributions: Suárez V contributed to acquisition of data, initial drafting of the paper, final approval of the paper; Puerta A contributed to acquisition of imaging, initial drafting of the imaging literature, final approval of the article; Santos LF contributed to acquisition of patients’ data, initial revision of the data, initial editing of the draft, final correction and approval of the paper; Pérez JM contributed to initial design of the draft, selection and description of imaging, revising the imaging literature, final approval of the article; Varón A contributed to acquisition of data, initial drafting the paper, final approval of paper; Botero RC contributed to conception of the article and design, critical revision of the content, final correction and approval of the article.
Correspondence to: Rafael Claudino Botero, MD, Gastroenterology and Hepatology Service, Department of Internal Medicine, Fundación Cardioinfantil, Carrera 13B 161-85, Consultorio 17, Bogotá 110131, Colombia. claudino48@mac.com
Telephone: +57-1-2167813 Fax: +57-1-6791147
Received: July 4, 2012
Revised: September 25, 2012
Accepted: November 14, 2012
Published online: March 27, 2013
Abstract

Portal hypertensive biliopathy (PHB) is characterized by anatomical and functional abnormalities of the intrahepatic, extrahepatic and pancreatic ducts, in patients with portal hypertension associated to extrahepatic portal vein obstruction and less frequently to cirrhosis. These morphological changes, consisting in dilatation and stenosis of the biliary tree, are due to extensive venous collaterals occurring in an attempt to decompress the portal venous blockage. It is usually asymptomatic until it progresses to more advanced stages with cholestasis, jaundice, biliary sludge, gallstones, cholangitis and finally biliary cirrhosis. Imaging modalities of the biliary tree such as Doppler ultrasound, computed tomography, magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography are essential to establish the diagnosis and the need of therapeutical interventions. Once the diagnosis is established, treatment with ursodesoxycholic acid seems to be beneficial. Decompression of the biliary tree to dilate, remove stones or implant biliary prosthesis by endoscopic or surgical procedures (hepato-yeyunostomy) usually resolves the cholestatic picture and prevents septic complications. The ideal treatment is the decompression of the portal system, with transjugular intrahepatic porto-systemic shunt or a surgical porto-systemic shunt. Unfortunately, few patients will be candidates for these procedures due to the extension of the thrombotic process. The purpose of this paper is to report the first 3 cases of PHB seen in a Colombian center and to review the literature.

Keywords: Bile duct diseases; Biliary obstruction; Banti’s syndrome; Cholestasis in children; Portal vein obstruction; Interventional endoscopy