Long-term follow-up of patients with biliary atresia successfully treated with hepatic portoenterostomy. The importance of sequential treatment

• Published in: Pediatric surgery international 1998-07, Vol.13 (5-6), p.327-330
• Main Authors: LOPEZ-SANTAMARIA, M, GAMEZ, M, DE LA VEGA, A, FRAUCA, E, JARA, P, BERROCAL, T, PRIETO, C, CORTES, P, TOVAR, J, MURCIA, J, DIEZ-PARDO, J, DIAZ, M, LEAL, N, LOBATO, R, MARTINEZ, L, HIERRO, L, CAMARENA, C
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Biliary Atresia - complications; Biliary Atresia - diagnostic imaging; Biliary Atresia - surgery; Biological and medical sciences; Child; Esophageal and Gastric Varices - etiology; Esophageal and Gastric Varices - surgery; Female; Follow-Up Studies; Humans; Jaundice - diagnostic imaging; Jaundice - etiology; Jaundice - surgery; Liver - blood supply; Liver - diagnostic imaging; Liver Transplantation - mortality; Liver, biliary tract, pancreas, portal circulation, spleen; Male; Medical sciences; Portoenterostomy, Hepatic - mortality; Recurrence; Regional Blood Flow; Retrospective Studies; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the digestive system; Survival Rate; Treatment Outcome; Ultrasonography
• ISSN: 0179-0358; 1437-9813
• DOI: 10.1007/s003830050331

The outcome of 18 biliary atresia (BA) patients (5 male, 13 female; age range 10.7-22.5 years; mean 15.4+/-0.7 years) treated with hepatic portoenterostomy (HPE) and jaundice-free for more than 10 years without liver transplantation (LT) is analyzed retrospectively. Eight of these patients subsequently required LT (age at LT 12. 8+/-0.5 years, range 10.5-15.2 years); 3 children (aged 11.6, 13.2 and 14.1 years, respectively) had episodes of gastrointestinal variceal bleeding associated with other signs of severe disease and are now candidates for LT; and among the 7 asymptomatic patients (age range 11.2-22.5 years; mean 15.9+/-2.1 years), 5 had sonographic and biochemical signs of moderate portal hypertension (PH). In order to analyze whether the age at transplantation influences the survival of children transplanted for BA, we also reviewed the outcome of 71 BA patients transplanted at our hospital between 1986 and 1996. All the children older than 10 years at the time of LT were alive; only patients younger than 10 years died following LT (n = 15). We conclude that the natural outcome of extrahepatic BA is toward PH, fibrosis, and cirrhosis, even in those cases successfully treated with HPE. In our experience, the results of sequential treatment with HPE and LT were excellent.