P1.093: Surgical Outcomes in Neonates and Infants with Choledochal Cyst Malformations: Analysis of the Pediatric Health Information System (PHIS) database

Background: An increase in antenatally diagnosed choledochal cyst malformations (CCMs) in recent years have led to the question of optimal timing for definitive cyst excision. The aim of the present study is to investigate the impact of early biliary reconstruction on outcomes in a national cohort of children underwent surgery within the first year of life.

Methods: The Pediatric Health Information System (PHIS) database was queried to identified children ≤ 1 year underwent definitive surgery for CCMs between October 2015 and September 2024. Excluded from the analysis were those having concurrent diagnostic code for biliary atresia and those who underwent only drainage procedures and or cholecystectomy without cyst excision. Preoperative characteristics and outcomes of children ≤ 3-month-old (Group A) at the time of definitive surgery were compared with those older than 3 months(Group B). Fisher exact and Mann-Whitney U tests were used to compare demographics, operative characteristics, length of hospital stay, 90-day complications and hospital readmissions between the two groups.

Results: A total of 267 patients meeting inclusion criteria were included in the analysis (N= 71 group A; N=196 group B). Overall, 21.3% were preterm and 22.5% had a confirmed diagnosis within 30 days of life. Symptoms at diagnosis are depicted in Figure 1. Younger children were more often transfer from other hospital (p=0.05) and admitted as urgent, emergent procedures (p= 0.001). Surgery was performed with an open approach more often in younger children (19.7% versus 44.9%;p < 0.001), however there were no differences in the type of surgical reconstruction between the two groups (p=0.678). Postoperative length of stay was significantly longer for Group A (median[interquartile range]:6[4-9]days and 5[4-7]days respectively; p=0.05). Overall,

there were no differences in 90 days complications (Group A: 19.7% versus Group B: 16.8%, p=0.59) and procedures for complications (9;12.7% vs 2;13.3%; p=1.00).

Overall younger children were more often readmitted in inpatient setting compared with older children (26.8% versus 11.7%,p=0.004), and the main reason for impatient readmission in younger children was postoperative cholangitis (N=9;12.7% vs 5;2.6%; p=0.003), however there were no differences in re-laparotomy for revision of bilioenteric anastomosis (N=1;1.4% vs N=4;2% p=1.000) or endoscopic biliary duct dilation (N=2;2.8% vs N=3;1.3%; p=0.611 respectively).

At median of 285(IQR:182-340) days, six patients developed portal hypertension (Group A: 5;7% and Group B: 1;0.5%; p= 0.006), two of them had preoperative ascites and confirmed cirrhosis at intraoperative biopsies and two had preoperative cholangitis and recurrent postoperative cholangitis.

Conclusion: In this dataset, early surgery is not associated with an increased risk of complications, revisions or endoscopic drainages. Definitive surgery should be considered when the diagnosis is confirmed even in asymptomatic patients.