Risk factors affecting late-presenting liver failure in adult patients with biliary atresia Masaki Nio ⁎ , Motoshi Wada, Hideyuki Sasaki, Hiromu Tanaka, Atsushi Okamura Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1–1 Seiryo-machi, Aoba-ku, Sedai 980–8574, Japan Received 23 August 2012; accepted 1 September 2012 Key words: Biliary atresia; Kasai operation; Long-term follow-up; Liver failure; Operative age; Cholangitis Abstract Purpose: Following the Kasai operation, a number of patients have developed liver failure, even after long-term postoperative courses. We assessed the clinical parameters to clarify the early risk factors affecting late-presenting liver failure in biliary atresia. Materials and Methods: From 1955 to 1991, 277 patients underwent a Kasai operation. Among those patients, 92 survived with their native liver for more than 20 years, and 72 continue to survive with their native liver in good condition (Group 1). In 20 patients, persistent jaundice recurred after the age of 20 years (Group 2). The postoperative courses of these patients were assessed retrospectively, and the clinical parameters, including age at the time of the Kasai operation (AGE, days), the period required for jaundice to disappear (PJD, days), and the association with early cholangitis (CG), were compared between the 2 groups. Results: Of the 20 patients in Group 2, 8 survived after a liver transplantation (LTx). Eight patients had recurrent jaundice, including 4 on the waiting list for anLTx. Additionally, 2 patients died after anLTx at the ages of 22 and 39. Another patient died of liver failure at the age of 28. One patient died of massive esophageal variceal bleeding at the age of 29. Significant differences were confirmed with respect to AGE (Group 1 b Group 2, p b .001), PJD (Group 1 b Group 2, p b .001), and CG (Group 1: Group 2 = 47 %: 75 %, p = .028). Conclusions: A considerable number of adult patients developed liver failure, even after the age of 20 years. AGE, PJD, and CG were found to be risk factors affecting late-presenting liver failure. Therefore, close patient follow-up is essential, especially for long-term survivors with a late operative age and early postoperative complications. © 2012 Elsevier Inc. All rights reserved. The Kasai operation has been a first-line modality of treatment for biliary atresia since its development in the 1950s. A considerable number of patients, however, have required liver transplant during the postoperative course. According to a recent report from the Japanese Biliary Atresia Registry, approximately 40% of patients required a liver transplant (LTx) within the 5 years following a Kasai operation [1]. The remaining patients survived for a longer period with their native livers. Among those patients, a number who were older than 20 years developed liver failure. Predicting the long-term prognosis is important to ⁎ Corresponding author. Tel.: +81 22 717 7235; fax: +81 22 717 72401. E-mail address: mnio@ped-surg.med.tohoku.ac.jp (M. Nio). www.elsevier.com/locate/jpedsurg 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.09.003 Journal of Pediatric Surgery (2012) 47, 2179–2183