Abstract

RUIZ N, Patricia; AGUIRRE R, Karen; MESA M, Catalina  and  LARA L, Lilian. Case Report of Syndromic Biliary Atresia in a Pediatric Patient. Rev Col Gastroenterol [online]. 2015, vol.30, n.4, pp.447-455. ISSN 0120-9957.

Biliary atresia is an obstructive neonatal cholangiopathy of unknown etiology that produces damage to the parenchyma of the liver and to the intrahepatic and extrahepatic bile ducts. It is the most common cause of neonatal cholestasis and liver transplantation in the pediatric population. In most cases it manifests as an isolated malformation. This article presents the case of a 75 day old patient with biliary atresia associated with abdominal heterotaxy. Biliary atresia syndrome together with splenic malformation has been previously described, as have biliary atresia associated with anatomical malformations of the spleen, pancreas, and heart. It occurs with genitourinary malformations less frequently. The prognosis of patients with biliary atresia has significantly improved with early recognition of signs and symptoms and timely performance of hepatoportoenterostomy (Kasai portoenterostomy).

Keywords : Biliary atresia; visceral heterotaxy.

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