Portal hypertensive biliopathy: a rare cause of childhood cholestasis View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2008-11

AUTHORS

Wael El-Matary, Eve A. Roberts, Peter Kim, Michael Temple, Ernest Cutz, Simon C. Ling

ABSTRACT

Portal hypertensive biliopathy (PHB) is defined as abnormal biliary changes that take place most likely secondary to extrahepatic portal vein obstruction (EHPVO) with portal hypertension. This condition may be asymptomatic or could lead to a cholestatic state, which is not well-described in children. We report a child who developed a cholestatic nature with portal hypertension some time after having neonatal surgery for duodenal atresia. We discuss the differential diagnosis and management of this rare condition. Symptomatic PHB has been only rarely reported in children. It should be suspected in patients with portal hypertension and having features of biliary obstruction. Hepaticojejunostomy may have a therapeutic role in selected patients in whom endoscopic or percutaneous manipulation of the biliary tree is unsuccessful and who have not responded to a surgical portal-systemic shunt procedure. More... »

PAGES

1339-1342

References to SciGraph publications

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00431-008-0675-4

DOI

http://dx.doi.org/10.1007/s00431-008-0675-4

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1031950222

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/18270735


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