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Case Reports in Pediatrics
Volume 2017, Article ID 2171974, 4 pages
Case Report

Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome

Department of Pediatrics, Division of Pulmonary Medicine, McGovern Medical School at University of Texas Health Science Center, Houston, TX, USA

Correspondence should be addressed to W. De Jesus-Rojas; ude.cmt.htu@sajorsusejed.oderfliw

Received 18 April 2017; Accepted 17 July 2017; Published 13 August 2017

Academic Editor: John W. Berkenbosch

Copyright © 2017 W. De Jesus-Rojas et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score. We present a 6-year-old girl with Caroli Syndrome and End-Stage Renal Disease who presented with persistent hypoxemia. The goal of this report is to increase awareness of HPS in children.