Long-term outcome in type I biliary atresia.
OBJECTIVE
This retrospective study reviews the long-term outcome of type I biliary atresia (BA).


METHODS
Three hundred twenty-three patients with BA, including 50 with type I, underwent corrective surgery. The surgical results, role of cholangiograms during the corrective surgery, late complications, and current statuses were evaluated.


RESULTS
The overall survival rate of the nontransplant type I patients was better than that of the type II/III patients (52% vs 33%, P = .0009). Cholangiograms of 32 patients were classified into 3 types: cloudy (48%), treelike (13%), and mixed (39%). Of 26 patients who underwent corrective surgery in 1972 or later, 7 (50%), 7 (78%), and 3 (100%) patients of the cloudy type, mixed type, and treelike type, respectively, have survived without liver transplantation (LTx). Of 18 type I patients who survived more than 20 years without LTx, 7 developed severe late complications. Two of them eventually required LTx after 20 years old.


CONCLUSIONS
Use of cholangiograms during corrective surgery might have a long-term prognostic value. The overall survival rate of type I BA was better than that of type II/III. The incidence of late complications was, however, considerably high in the type I survivors. All patients required careful long-term follow-up.
Nio
Masaki
M
Department of Surgery, Miyagi Children's Hospital, Sendai 989-3126, Japan. mnio@miyagi-children.or.jp
Sano
Nobuyuki
N
Ishii
Tomohiro
T
Sasaki
Hideyuki
H
Hayashi
Yutaka
Y
Ohi
Ryoji
R
D000714:Anastomosis, Surgical; D001656:Biliary Atresia / Q000145:classification / Q000000981:diagnostic imaging* / Q000601:surgery*; D002758:Cholangiography; D006801:Humans; D011171:Portoenterostomy, Hepatic; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome