This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Zukin, D. D. Articles by Lake, A. M. Search for Related Content PubMed PubMed Citation Articles by Zukin, D. D. Articles by Lake, A. M. Social Bookmarking                         What's this?

Extrahepatic Biliary Atresia Associated with Cyanotic Congenital Heart Disease

Three Case Reports and a Review

Departments of Pediatrics and Medicine (Cardiac and Gastrointestinal Units), Massachusetts General Hospital, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts

Richard R. Liberthson

Departments of Pediatrics and Medicine (Cardiac and Gastrointestinal Units), Massachusetts General Hospital, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts

Alan M. Lake

Departments of Pediatrics and Medicine (Cardiac and Gastrointestinal Units), Massachusetts General Hospital, Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts

Three patients with extrahepatic biliary atresia associated with cyanotic con genital heart disease are presented and their clinical courses reviewed. In these patients, the coincidence of cyanosis and jaundice caused an atypical green- appearing cutaneous discoloration which masked the early clinical detection of biliary atresia in one infant and cyanotic heart disease in the other two. In all infants, either partial or complete correction of the congenital cardiac lesion was possible. Two infants died from hepatic disease despite attempted surgical revision and one has satisfactory bile drainage one month following portoenterostomy. Awareness of the rare concurrence of cyanotic congenital heart disease and extrahepatic biliary atresia is helpful to the clinician when evaluating infants with atypical cutaneous discoloration. Early oversight of either underlying disease process can be avoided, and early evaluation and management of both entities can be possible.

Clinical Pediatrics, Vol. 20, No. 1, 64-66 (1981)
DOI: 10.1177/000992288102000110


CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?