Hepatobiliary malformations: proposed updation of classification system, clinicopathological profile and a report of largest pediatric giant choledochal cyst

Background: The present study was aimed to update the classification of hepatobiliary malformations and study the clinicopathological profile of pediatric choledochal cyst (CDC) and pediatric giant chol Methods: We have retrospectively analysed the data of 57 consecutive cases of CDCs in paediatric patients from a time period spanning from 2016 to 2020. Results: Revised classification of hepatobiliary malformations was proposed and these were divided into 2 broad headings, choledochal (congenital and acquired) and extra-choledochal spectrum. 57 pediatric patients were diagnosed as having CDC with average age 4.615 years and female to male ratio of 3.7:1. We have also reported one of the largest GCC measuring 23 x 10 x 9 cm size. The classical triad was known to be more common and seen in 60% GCCs as opposed to 14.5% in CDCs. Values of serum bilirubin, SGOT, SGPT, PT/INR were elevated in CDC series and normal in GCC patients. 55 patients (96.5%) underwent cyst excision and Roux-en-Y hepaticojejunostomy. Mortality was seen in 7.01% patients (n = 4) following surgery. Conclusion: Simplified and broader classification system for CDCs has been proposed. Clinical studies found that GCC differs considerably from classical CDCs.