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Clin Gastroenterol Hepatol. 2006 Nov;4(11):1411-4. Epub 2006 Sep 18.

Biliary atresia with choledochal cyst: implications for classification.

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  • 1Division of Gastroenterology, Hepatology & Nutrition, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.

Abstract

BACKGROUND & AIMS:

To illustrate the limitations of the embryonic and perinatal classification system of biliary atresia (BA), we present a child with an antenatal diagnosis of choledochal cyst (CC) associated with BA and review the published literature.

METHODS:

Medline and Pubmed were searched for "BA and CC," "cystic biliary atresia," "BA and cysts," and "biliary cystic malformations."

RESULTS:

A 7-week-old with an antenatal diagnosis of CC was found to have BA associated with CC. The literature search identified 88 cases of BA with CC. Sixty-seven cases had type 1 BA (atresia of the common bile duct), 2 had type 2 BA (atresia of the common hepatic duct), and 19 had type 3 BA (atresia of the porta hepatis). Of the 27 cases of antenatal diagnosis of BA with CC only 1 had associated congenital anomalies. Outcome analysis showed poor outcomes were significantly more common (P = .009) and occurred earlier (P = .0249) in patients with type 3 BA. Children with type 3 BA were 5.4 times more likely to develop poor outcomes compared with type 1 (hazard ratio, 5.4; 95% confidence interval, 1.03-27.8).

CONCLUSIONS:

BA associated with CC forms a distinct subtype of BA, characterized by a preponderance of type 1 BA, a relatively good clinical outcome after surgery, and an absence of associated congenital anomalies. Antenatal diagnosis of many affected infants supports their inclusion within the embryonic BA group and suggests that a broader interpretation of the embryonic phenotype and further classification of BA based on genetic susceptibility may be required.

PMID:
16979949
DOI:
10.1016/j.cgh.2006.07.005
[PubMed - indexed for MEDLINE]
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