The first job Archivist had in paediatrics was working as an SHO on the gastroenterology ward in a children’s hospital in Manchester. There were rows of children with biliary atresia, small for their age, jaundiced with large swollen abdomens usually post Kasai portoenterostomy (KPE) or a few waiting to go for surgery. Pale white stools, once seen, will never be forgotten. Post-surgery, we were waiting for the jaundice to clear a sign of success and a failed Kasai operation meant liver transplant.

The first liver transplant in children was in 1963 which was unfortunately unsuccessful. Fortunately, now, graft and patient survival rates are well over 90% at 1, 5 and 10 years post-surgery. Our main concern was of ascending cholangitis and we continually looked for fever and altered LFTs and had a low threshold for starting intra venous antibiotics. What are the reasons for failure of KPE? Matkovici M et al (Journal of Pediatric Gastroenterology and Nutrition 2023;76:66–71. DOI 10.1097  /MPG .0000000000003638) have published their review of single centre (Kings College Hospital, London, UK) prospective biliary atresia database which included 135 infants within a 6 year period. They defined a successful KPE as achieving a postoperative bilirubin of≤20 µmol/L. 90 (67%) of those who underwent a KPE achieved low post operative bilirubin levels. 20 (22%) underwent liver transplant and the remainder did not have a transplant. There was no difference in age at KPE, aspartate aminotransferase-to-platelet ratio associated anomalies, and cytomegalovirus status between the two groups. The two main factors, postoperatively more common in the liver transplant group was cholangitis (any episode, 18/20 (90%) vs 15/70 (21%); p<0.0001) and portal hypertension (PHT) (gastrointestinal (GI) bleed, 10/20 (50%) vs 2/70 (2.8%); p 0.0001). The authors then went on to use univariate analysis and showed that the most significant predictive values at 3 months for liver transplant by 2 years were high APRi, bilirubin, international normalised ratio, and ultrasound (US)-detected ascites. The most important reason for failure in this cohort of “successful” Kasai operation was recurrent postoperative cholangitis. Lal B et al (Journal of Paediatric Gastroenterology and Nutrition, 77 (5), e70-e70. doi: 10.1097 /MPG.0000000000003880) responded to this paper by highlighting that although there is a high incidence of early cholangitis at 67% within 3 months, 10% of episodes can occur after 1 year post KPE. This low threshold for identifying recurrent cholangitis then continues to be paramount. So, what is the definition of cholangitis in infants? It remains complex. Possibly the bilirubin has not normalised, so an increase in preexisting jaundice is the clue, reappearance of acholic stools, and other clinical and biochemical parameters seem to be the crucial components. Matkovici et al chose an adult-orientated definition based on the the Tokyo guidelines 2018 as there is no agreed objective definition of cholangitis in paediatric population. There was also a discussion about antibiotic prophylaxis once cholangitis is diagnosed, how long should the IV course be? It’s a good read.