Document Detail


Congenital absence of portal vein presenting as hepatopulmonary syndrome.
MedLine Citation:
PMID:  15670231     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A 9-year-old girl presented with cyanosis and decreased exercise tolerance. Chest X-ray, lung function and echocardiogram were normal. Contrasted echocardiogram showed intrapulmonary right to left shunt and computerized tomography of the thorax showed dilated pulmonary vasculature. There was no arteriovenous malformation. Computerized tomography of the abdomen revealed absence of the intrahepatic portion of the portal vein. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained into the right atrium. We concluded that the patient had hepatopulmonary syndrome secondary to absence of the portal vein. This is the first report of hepatopulmonary syndrome in a female paediatric patient with a congenital absence of the portal vein. As all portal blood entered directly into systemic circulation, the condition was equivalent to congenital portosystemic shunt. Cases of congenital portosystemic shunt complicated by hepatopulmonary syndrome were also reviewed.
Authors:
K M Cheung; C Y Lee; C T Wong; A K H Chan
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Journal of paediatrics and child health     Volume:  41     ISSN:  1034-4810     ISO Abbreviation:  J Paediatr Child Health     Publication Date:    2005 Jan-Feb
Date Detail:
Created Date:  2005-01-26     Completed Date:  2005-05-04     Revised Date:  2007-09-24    
Medline Journal Info:
Nlm Unique ID:  9005421     Medline TA:  J Paediatr Child Health     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  72-5     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Caritas Medical Centre, Hong Kong, China. jennykmcheung@hotmail.com
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MeSH Terms
Descriptor/Qualifier:
Child
Female
Hepatopulmonary Syndrome / classification,  etiology*
Humans
Portal Vein / abnormalities*,  radiography
Tomography, X-Ray Computed

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