Long-term prognosis in biliary atresia after hepatic portoenterostomy: analysis of 35 patients who survived beyond 5 years of age.
The long-term prognosis in 35 patients with biliary atresia, who had undergone successful hepatic portoenterostomy and survived beyond 5 years of age, was studied. Eighteen (51%) patients developed ascending cholangitis after surgery. Jaundice recurred or increased in half of the patients, mainly as a result of ascending cholangitis. Another serious problem was the development of portal hypertension. Esophageal varices developed in 16 (46%) patients, with rupture and massive hemorrhage in seven. Leukopenia resulting from hypersplenism was common. Eighteen patients had WBC counts less than 5000/microliter. Thrombocytopenia was less common than leukopenia. Height was slightly below normal, although weight was within the normal range. Three patients died of hepatic failure; all had previously had ascending cholangitis. These observations indicate that ascending cholangitis is critical in the long-term and short-term prognoses in patients who have undergone successful repair of biliary atresia.
D000293:Adolescent; D001652:Bile Ducts / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D002761:Cholangitis / Q000209:etiology / Q000401:mortality; D005260:Female; D005500:Follow-Up Studies; D006471:Gastrointestinal Hemorrhage / Q000209:etiology / Q000401:mortality; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000401:mortality; D007583:Jejunum / Q000601:surgery*; D008099:Liver / Q000601:surgery*; D008107:Liver Diseases / Q000209:etiology / Q000401:mortality; D008137:Longitudinal Studies; D008297:Male; D011183:Postoperative Complications; D011379:Prognosis; D012008:Recurrence