TY - JOUR
T1 - Long-term outcome in type I biliary atresia
AU - Nio, Masaki
AU - Sano, Nobuyuki
AU - Ishii, Tomohiro
AU - Sasaki, Hideyuki
AU - Hayashi, Yutaka
AU - Ohi, Ryoji
PY - 2006/12
Y1 - 2006/12
N2 - Purpose: 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.
AB - Purpose: 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.
KW - Biliary atresia
KW - Long-term follow-up
KW - Type I
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U2 - 10.1016/j.jpedsurg.2006.08.019
DO - 10.1016/j.jpedsurg.2006.08.019
M3 - Article
C2 - 17161184
AN - SCOPUS:33845191517
VL - 41
SP - 1973
EP - 1975
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 12
ER -