ORIGINAL ARTICLE Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre Li Wei Chiang, Chuan Yaw Lee, Gita Krishnaswamy, Shireen Anne Nah, Ajmal Kader, Christina Ong, Yee Low and Kong Boo Phua Department of Paediatrics, KK Women’s and Children’s Hospital, Singapore Aim: Biliary atresia (BA) has preponderance in Asian populations with Kasai portoenterostomy (KP) regarded as the first-line standard of care. Yet reports from Southeast Asia remain scant. This study reviews the demographics, short- and medium-term outcomes for our cohort, and evalu- ates prognostic factors for outcome. Methods: All patients diagnosed with BA between January 1997 and December 2013 were included. Clinical data were obtained from medical records. Jaundice clearance was defined as total bilirubin < 20 μmol/L within 6 months after KP. Two-year and 5-year native liver survival (NLS) were determined. Prognostic factors examined included gender, ethnicity, associated anomalies, age at KP, post-KP cholangitis and clearance of jaundice within 6 months. Results: Of 58 patients studied, 31(53.4%) were male. Median age at time of KP was 53 days (range: 28–127). Ethnic distribution showed 32 (55.2%) Chinese, 16 (27.6%) Malays and 10 (17.2%) others. Twenty-one (36.2%) patients achieved jaundice clearance by 6 months. Two-year NLS rate was 36 out of 50 (72%), while 5-year NLS rate was 16 out of 35 (45.7%). Only clearance of jaundice within 6 months had a significant associa- tion with NLS (P = 0.006). All other factors showed no significant impact on outcome. Conclusions: Our short- and medium-term outcomes after KP for BA are comparable with those reported by most international centres. How- ever, prognostic factors such as age at KP, cholangitis episodes and associated anomalies did not show significant correlation; only clearance of jaundice within 6 months was significantly predictive of NLS. Key words: biliary atresia; Kasai portoenterostomy; neonatal jaundice. What is already known on this topic 1 Biliary atresia is a rare disease characterised by the inflammatory obliteration of the biliary system in infants. 2 Kasai portoenterostomy (KP) is the only treatment which may arrest the disease process in some patients. 3 Many prognostic factors have been proposed including age at time of Kasai but most remain debatable. What this paper adds 1 We describe a Southeast Asian single institute experience on KP. 2 In this series, jaundice clearance after KP is the only significant prognostic factor for native liver survival (NLS). 3 In this series, age at time of KP does not demonstrate impact on NLS. Biliary atresia (BA) is a neonatal disease with an incidence vary- ing from 1:18 000 live births in European populations 1–4 to 1:3300 in the Asia Pacific region. 5–7 It is characterised by the inflammatory obliteration of the biliary tree, resulting in severe cholestasis and cirrhosis. Kasai portoenterostomy (KP) is viewed as the initial therapy of choice, with liver transplantation reserved as rescue treatment. While the KP aims to re-establish bile flow, its reported success rate is inconsistent. 3,4,8,9 Patient’s age at time of KP, anatomical pattern of the biliary remnant, surgeon’s experience with KP, occurrence of post-KP cholangitis and corticosteroid administration have all been suggested as factors influencing the outcome of KP. The purpose of this study is to describe the clinical features of BA and the short- to medium-term results after KP for BA in a single Southeast Asian paediatric institution. We also aim to determine the impact of various prognostic factors on the out- come of our BA children. Methods Institutional ethical board approval was obtained prior to com- mencement of this retrospective study. Between January 1997 and December 2013, we reviewed 73 consecutive children in our institution who were diagnosed with BA either by laparotomy or by intra-operative cholangiogram. We excluded those who did not undergo KP and those with incomplete medical records. Data pertaining to demographics, clinical characteristics, operative and Correspondence: Dr Kong Boo Phua, Department of Paediatrics, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, 229899 Singapore. Fax: +65 62917923; email: phua.kong.boo.kkh@gmail.com Conflict of interest: None declared. Accepted for publication 2 September 2016. doi:10.1111/jpc.13379 Journal of Paediatrics and Child Health (2016) © 2016 Paediatrics and Child Health Division (The Royal Australasian College of Physicians) 1