Long-Term Morbidity of Choledochal Malformations in Children.

Objective To assess long-term morbidity in children operated for choledochal malformation (CM) by relating clinical complications to liver histopathology, follow-up imaging, liver stiffness and biochemistry. Methods A single-center retrospective follow-up study including all CM patients (n = 55, 71% females) treated during 1976-2018 was performed. Mann Whitney U test and Spearman rank correlation were used for statistical analyses. Results During median follow-up of 5.8 (interquartile range, 2.5-12) years, one patient was lost to follow-up while all survived. Intraoperative liver biopsies showed fibrosis in 32%, and patients with Metavir stage ≥2 were younger at surgery [0.36 (0.11-1.9) years vs 3.8 (0.72-10.5) years, p = 0.024] than those without fibrosis. Overall, 21% had long-term complications including cholangitis in 9 (>2 episodes in 5) patients, anastomotic stricture in two referred patients and adhesive volvulus or hepatocellular carcinoma in one each. Anastomotic strictures were successfully managed nonoperatively and hepatocellular carcinoma with thermoablation. In postoperative MRCP performed 6.4 (3.6-16) years after hepatico-jejunostomy, diameters of both main intrahepatic ducts had decreased significantly to 3.0 (2.5-3.5) mm (p = 0.0001), but a distal cyst stump was remaining in 30% with a length of 6.0 (4.0-20) mm that associated with operation age (r = 0.71, p = 0.015) and fusiform CM type. Follow-up ultrasound revealed mild dilation of intrahepatic bile ducts in 6.3% and mildly to moderately elevated liver biochemistry in 23%, and liver stiffness (>7 kPa) in 22%. Conclusions Whilst cholangitis was the most common postoperative problem, individual patients experienced other more significant complications and one quarter of patients showed evidence of underlying liver dysfunction.