Does hepatic hilum morphology influence long-term prognosis in type I/I cyst biliary atresia?
OBJECTIVE
Some patients with cystic biliary atresia (BA) achieve exceptionally good postoperative courses. Early differentiation of pediatric patients with the favorable-type disease can lead to beneficial long-term postoperative management. We examined whether the hepatic hilum morphology affects long-term prognosis in type I/I cyst BA, atresia of the common bile duct with/without a cyst.


METHODS
Of 253 BA patients identified since 1972, 40 were classified as having type I/I cysts and were divided into two subtypes according to hepatic duct size (subtype α: n = 18; duct diameter, ≥ 1 mm and subtype β: n = 22; duct diameter, <1 mm) to compare postoperative clinical courses.


RESULTS
In subtypes α and β, jaundice disappeared in 16 (89 %) and 19 (86 %) patients, respectively (p = 0.81), and 13 (72 %) and 12 (55 %) survived with native livers (p = 0.18) at a mean age of 23.3 and 25.5 years, respectively (p = 0.42).


CONCLUSIONS
There were no significant differences in long-term outcomes between subtypes α and β, although approximately 40 % developed liver failure during the postoperative course. Thus, close long-term follow-up is essential in type I/I cysts, regardless of the hepatic hilum morphology.
Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1, Seiryomachi, Aobaku, Sendai, 980-8574, Japan. mnio@ped-surg.med.tohoku.ac.jp.
Masaki
M
Nio
Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1, Seiryomachi, Aobaku, Sendai, 980-8574, Japan.
Motoshi
M
Wada
Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1, Seiryomachi, Aobaku, Sendai, 980-8574, Japan.
Hideyuki
H
Sasaki
Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1, Seiryomachi, Aobaku, Sendai, 980-8574, Japan.
Hiromu
H
Tanaka
D001649:Bile Duct Diseases / Q000150:complications / Q000601:surgery; D001652:Bile Ducts; D001656:Biliary Atresia / Q000150:complications / Q000601:surgery*; D002758:Cholangiography; D003560:Cysts / Q000150:complications* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000000981:diagnostic imaging*; D011379:Prognosis; D016019:Survival Analysis
Biliary atresia; Hepatic duct size; Hepatic hilum morphology; Long-term prognosis