Clinical outcome and neurological development of patients with biliary atresia associated with a bleeding tendency: a single institution experience.

Abstract

We compared the clinical features of patients with biliary atresia (BA) associated with a bleeding tendency (BT) at the time of the diagnosis with those of patients without a bleeding tendency (NBT). The patients' background characteristics, age in days at the first visit, Kasai portoenterostomy (KPE), and postoperative course were retrospectively analyzed. Nine of the 93 BA patients (9.7%) showed a BT, including 7 with intracranial hemorrhaging (ICH), 1 with gastrointestinal bleeding, and 1 with a prothrombin time (PT) of 0%. The age at the first visit was 62 ± 12days old for BT patients and 53 ± 27days old for NBT patients (p = 0.4); the age at KPE was 77 ± 9days old for BT patients and 65 ± 24days old for NBT patients (p = 0.2); the time from the first visit to surgery was 13 ± 7days for BT patients and 11 ± 10days for NBT patients (p = 0.5); and the native liver survival rate was 56% for BT patients and 58% for NBT patients (p = 1), with no significant difference in any of the parameters. The neurological outcomes of survivors of ICH were favorable. Appropriate BT correction allowed early KPE even after ICH, resulting in native liver survival rates comparable to those of NBT patients without significant neurological complications.