title: Long-term follow-up of patients with biliary atresia successfully treated with hepatic portoenterostomy. The importance of sequential treatment.
abstract: 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.
authors.affiliation: Hospital Infantíl "La Paz", Dto de Cirugia Pediátrica, Paseo de la Castellana 261, E-28046 Madrid, Spain.
authors.forename: M
authors.initials: M
authors.lastname: López-Santamaria
authors.forename: M
authors.initials: M
authors.lastname: Gamez
authors.forename: J
authors.initials: J
authors.lastname: Murcia
authors.forename: J
authors.initials: J
authors.lastname: Diez-Pardo
authors.forename: M
authors.initials: M
authors.lastname: Diaz
authors.forename: N
authors.initials: N
authors.lastname: Leal
authors.forename: R
authors.initials: R
authors.lastname: Lobato
authors.forename: L
authors.initials: L
authors.lastname: Martinez
authors.forename: L
authors.initials: L
authors.lastname: Hierro
authors.forename: C
authors.initials: C
authors.lastname: Camarena
authors.forename: A
authors.initials: A
authors.lastname: De la Vega
authors.forename: E
authors.initials: E
authors.lastname: Frauca
authors.forename: P
authors.initials: P
authors.lastname: Jara
authors.forename: T
authors.initials: T
authors.lastname: Berrocal
authors.forename: C
authors.initials: C
authors.lastname: Prieto
authors.forename: P
authors.initials: P
authors.lastname: Cortés
authors.forename: J
authors.initials: J
authors.lastname: Tovar
mesh_terms: D000293:Adolescent; D000328:Adult; D001656:Biliary Atresia / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D002648:Child; D004932:Esophageal and Gastric Varices / Q000209:etiology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007565:Jaundice / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery; D008099:Liver / Q000098:blood supply / Q000000981:diagnostic imaging; D016031:Liver Transplantation / Q000401:mortality; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000401:mortality; D012008:Recurrence; D012039:Regional Blood Flow; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014463:Ultrasonography