EXTRAHEPATIC biliary atresia is an infrequent entity that presents a variety of clinical features. Although the pathogenesis remains the subject of some debate, it is thought to begin in utero and may continue after birth, resulting in the segmental or total obliteration of the intrahepatic and/ or extrahepatic biliary system.1 Clinical experience would suggest that surgical intervention in some manner interrupts this progressive process, particularly in the "noncorrectable" forms. The patient described herein is illustrative and presents anatomic variations not previously described, to the best of our knowledge. Longterm survival is attributed to cholecystohepatic channels.
Report of a Case
A 5-year-old girl was seen in 1956 because of slow development and at least three episodes of jaundice, attributed to "infectious hepatitis," first described at 3 years of age. At operation in 1956 for a suspected congenital deformity, the gallbladder was noted to be large, thick walled, distended, and moderately
Schorlemmer GR, Wild RE, Mandell V, Newsome JF. Cholecystohepatic Connections in a Case of Extrahepatic Biliary Atresia: A 27-Year Follow-up. JAMA. 1984;252(10):1319–1320. doi:10.1001/jama.1984.03350100049030
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