Awareness, referral and age at Kasai surgery for biliary atresia in Europe: A survey of the Quality‐of‐Care Task Force of ESPGHAN

Abstract

ObjectivesTo identify infants with biliary atresia (BA), European Society of Paediatric Gastroenteroloy and Nutrition (ESPGHAN)/North American Society of Pediatric Gastroenteroloy and Nutrition (NASPGHAN) guidelines recommend measurement of conjugated/direct bilirubin in infants with prolonged jaundice and using a stool colour card (SCC). The ‘Quality of Care’ Task Force of ESPGHAN performed two surveys to assess current case finding for BA and age at Kasai portoenterostomy (KPE).MethodsThe first survey approached 26 European hepatology centres to report age at referral and age at KPE of all infants diagnosed with BA from 2015 to 2019. The second survey targeted paediatricians in France to assess awareness and compliance with the recently introduced SCC.ResultsData from 785 patients with BA from 18 centres in 15 countries revealed a mean age at referral to tertiary centre of 55 days (median 53, IQR 48–60) (n = 636). The mean age at KPE was 61 days (median 60; IQR 54–67) (n = 772). For 6% of patients, cirrhosis was too advanced for surgery.Of 392 paediatricians answering the second survey, 53% felt familiar with the target diseases, 80% correctly identified cholestasis and 59% always inquired about the infant's stool colour. If abnormal, 93% would order blood tests and 85% call for advice. The SCC screening was considered helpful for case finding and improving knowledge of cholestatic diseases by 62% and 45% paediatricians, respectively.ConclusionsReferral of infants for KPE remains late, indicating low adherence to search for cholestasis in icteric infants by age 2−3 weeks. Knowledge and structures need improvement to allow earlier guideline conform case finding, diagnosis and therapy.