Refractory hypoxemia caused by hepatopulmonary syndrome: a case report

Hepatopulmonary syndrome is a clinical syndrome that can affect patients of all ages with liver disease and is more common in children with biliary atresia. Contrast echocardiography is the test of choice to diagnose the presence of intrapulmonary vascular dilatation. The established treatment for h...

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Bibliographic Details
Published in:Journal of medical case reports 2014-12, Vol.8 (1), p.418-418, Article 418
Main Authors: Govindan, Morgen L, Kuo, Kevin W, Mahani, Maryam Ghadimi, Shanley, Thomas P
Format: Article
Language:English
Subjects:
Biliary Atresia - complications
Case Report
Case studies
CT imaging
Hepatopulmonary Syndrome - complications
Humans
Hypoxia - etiology
Infant
Liver
Male
Transplantation of organs, tissues, etc
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Summary:Hepatopulmonary syndrome is a clinical syndrome that can affect patients of all ages with liver disease and is more common in children with biliary atresia. Contrast echocardiography is the test of choice to diagnose the presence of intrapulmonary vascular dilatation. The established treatment for hepatopulmonary syndrome is liver transplantation. We present the case of an 8-month-old Caucasian baby boy with a history of biliary atresia, polysplenia, and interrupted inferior vena cava who presented with hypoxemia and cyanosis that progressed rapidly. A chest computed tomography angiogram revealed significant dilatation of the pulmonary vasculature, prompting further evaluation and diagnosis of hepatopulmonary syndrome with contrast echocardiography. He was maintained on a milrinone infusion while awaiting liver transplantation. His hypoxemia improved slowly following liver transplantation, requiring tracheostomy and prolonged ventilator dependence. Hepatopulmonary syndrome should be included in the differential for progressive hypoxemia in children with liver disease, particularly those with biliary atresia. Imaging with chest computed tomography angiogram and contrast echocardiography should be considered in cases of unexplained refractory hypoxemia.
ISSN:1752-1947
1752-1947
DOI:10.1186/1752-1947-8-418