Cystic Biliary Atresia: A Wolf in Sheep’s Clothing

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Abstract:

Neonatal obstructive jaundice is frequently explained by biliary atresia (BA) or the presence of a choledochal cyst (CC). Cystic biliary atresia (CBA) has been a proposed as a subtype of BA with projected improved outcomes. We aimed to characterize these lesions further. We conducted an Institutional Review Board-approved review of all patients treated for obstructive jaundice at our tertiary children’s hospital over 10 years. Over the decade we evaluated 91 children with obstructive jaundice: 13 CBA, 52 BA, and 26 CC. Patients with isolated CBA and BA were diagnosed significantly earlier than those with CC (15.9, 54, and 281 days, respectively; P = 0.0001). There was a significant delay between diagnosis and surgical intervention for patients with CBA compared with BA: 17 days versus 5.7 days (P = 0.004). There was no difference in rate of transplant between CBA and BA (31 vs 50%; P = 0.35). The time from surgery until transplant was 13.9 and 18.6 months for CBA and BA, respectively (P = 0.62). Although radiographically similar to CC, CBA behaves similarly to isolated BA. Delay in recognition and surgical treatment may affect outcomes and lead to an increased incidence of liver failure. The presence of a cystic biliary malformation in the setting of neonatal jaundice should be regarded as CBA until proven otherwise.

Document Type: Research Article

Affiliations: Emory University School of Medicine, Atlanta, Georgia, USA

Publication date: September 1, 2013

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  • The Southeastern Surgical Congress owns and publishes The American Surgeon monthly. It is the official journal of the Congress and the Southern California Chapter of the American College of Surgeons, which all members receive each month. The journal brings up to date clinical advances in surgical knowledge in a popular reference format. In addition to publishing papers presented at the annual meetings of the associated organizations, the journal publishes selected unsolicited manuscripts. If you have a manuscript you'd like to see published in The American Surgeon select "Information for Authors" from the Related Information options below. A Copyright Release Form must accompany all manuscripts submitted.
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