Medical management of 'failing' Kasai portoenterostomy

Abstract

The only effective treatment for 'failing' Kasai portoenterostomy is liver transplantation (LT). However, to maximise a patient's chances to achieve the proclaimed >95% survival with sequential surgical management, medical follow-up and treatment must be planned carefully. This includes routine fat-soluble vitamin supplementation with choleretics, aggressive nutritional support, regular ultrasonography, optimal general paediatric care, and psychological support for the family once complications arise. Careful timing of LT is of critical importance, although recent trends include earlier consideration of LT in children with biliary atresia. This management can only be offered through centralised, specialised national services. Due to its ramifications in paediatric surgery, dietetics, metabolic, social, adolescent and transplantation medicine, paediatric hepatology is a fine example of patient care that is genuinely multidisciplinary.