Choledochal malformations: Lessons learnt in Johannesburg

Abstract

Background. Choledochal malformation (CM) is a well-described and relatively rare condition. CMs may present on antenatal ultrasound screening, through childhood and into adulthood. The aetiology is not well understood but the association with a pancreaticobiliary malunion predisposes to the development of CMs.Objectives. To review the experience of CMs in the Department of Paediatric Surgery, University of the Witwatersrand, Johannesburg to improve our patient care for this population.Methods. After institutional ethics approval, a retrospective record review was conducted of patients presenting with CM to the Department of Paediatric Surgery, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg. All patients managed between January 2010 and May 2017 were included.Results. A total of 35 patients underwent surgery for CMs and 2 patients were excluded from the study owing to incomplete records. Most of our patients (83%) presented with jaundice and a median (interquartile range) bilirubin level of 167 (32 - 234) mmol/L. In our cohort of patients those with type IV lesions presented at a younger age and with higher bilirubin and gamma-glutamyl transferase levels, although this finding was not statistically significant.Conclusion. Although uncommon, CMs may lead to significant morbidity and malignancy. Specialised care is necessary to improve longterm outcomes in these patients