Type IV-A choledochal cyst (CDC) has been considered to have a poor prognosis due to the high incidence of postoperative anastomotic strictures and intrahepatic stones. This study aimed to evaluate the surgical outcomes of children with type IV-A CDC and to provide insights for clinical diagnosis and treatment. The study retrospectively analyzed patients from June 2015 to December 2018 at our center, 76 children were diagnosed with type IV-A CDC. All patients underwent single-incision laparoscopic choledochal cyst excision and Roux-en-Y hepaticojejunostomy. The decision to perform ductoplasty was made by comprehensively considering the intrahepatic duct dilatation (IHDD) and stricture. All patients were followed up 1, 3, and 6 months postoperatively, and then every 1 year thereafter. Patients were categorized into two groups based on IHDD changes postoperatively: the long-term group (LTG), with IHDD persisting for over a year, and the short-term group (STG), where IHDD normalized within a year. Single/multiple factor logistic regression was used to analyse the factors influencing postoperative IHDD. The median follow-up period was 80 months, with a range from 64 to 101 months. The decrease in postoperative liver function parameters, compared to preoperative levels, was statistically significant. Two patients (2.63%) developed bile leaks. One patient (1.32%) developed anastomotic stricture. All patients' IHDD returned to normal size. Fifty-six (73.7%) patients showed normalization of IHDD within one-year postoperatively. The median recovery time for IHDD in patients was 1.65 months, ranging from 3 days to 74 months postoperatively. There were significant differences in intrahepatic biliary sludge and stones and maximum diameter of IHDD between STG and LTG. Logistic regression was used to analyse the factors and found that intrahepatic biliary sludge and a wider maximum diameter of IHDD were risk factors for postoperative long-term IHDD in patients. Single-incision laparoscopic hepaticojejunostomy with selective ductoplasty are safe and effective for children with Type IV-A CDC. Long-term follow-up is recommended for children presenting with intrahepatic biliary sludge and larger IHDD.
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