Post-Surgical Abnormalities of the Duodenum Leading to Pancreaticobiliary Disease in Children

**Background:** Congenital duodenal obstruction, occurring in 1 per 5000–10,000 live births, is caused by duodenal atresia, stenosis, annular pancreas, or duodenal webs. Long-term follow-up after surgical repair has shown morphological and functional changes such as megaduodenum, duodenal diverticula, and functional obstruction. While duodenal diverticula leading to biliary disease is well-established in adults, it is poorly documented in pediatric literature. This study reports 8 pediatric patients with congenital duodenal obstruction who later developed pancreaticobiliary disease.

**Methods:** Pediatric patients (under 18 years) with a history of duodenal obstruction who subsequently developed pancreaticobiliary disease were identified at 2 large pediatric hospitals between January 1994 and December 2018. One patient was also part of the INSPPIRE cohort. Institutional review board approval was obtained at each institution.

**Key Results:** Eight patients (5 female, 3 male) were included. Median age at presentation with pancreaticobiliary complications was 6 years (mean 7.6 years). Underlying duodenal obstructions included duodenal web (2 patients), duodenal atresia (2 patients), and annular pancreas (4 patients). Seven patients underwent surgical repair in infancy (median day of life 14, range 2–365 days); one patient with annular pancreas did not undergo repair. Comorbid conditions included trisomy 21 (3 patients), prematurity (2 patients), heterotaxy/AV canal defect (1 patient), and renal dysplasia (1 patient).

Presenting symptoms included abdominal pain, jaundice, pancreatitis, and incidental findings. Laboratory abnormalities included elevated liver enzymes (AST up to 360 U/L, ALT up to 430 U/L, GGT up to 1044 U/L, conjugated bilirubin up to 7.2 mg/dL) and elevated lipase (up to 22,000 U/L) and amylase (up to 2235 U/L). Imaging findings included dilated common bile duct (CBD) (up to 1.5 cm), choledocholithiasis, intrahepatic biliary ductal dilation, pancreatic duct stones, and duodenal dilation/diverticula. Endoscopic retrograde cholangiopancreatography (ERCP) in 5 patients revealed abnormal duodenal anatomy with distorted or stretched ampulla, CBD strictures, and stones. One patient had a slit-like ampulla. One patient (patient 1) developed portal hypertension and required liver transplantation; another (patient 2) developed portal hypertension due to chronic biliary obstruction and prior chemotherapy. Patient 3 underwent cholecystectomy and choledochoduodenostomy for persistent obstruction. Two patients were lost to follow-up. Median follow-up duration was 7 years (range 1–18 years).

**Clinical Implications:** Altered duodenal anatomy after surgical repair of congenital duodenal obstruction may lead to pancreaticobiliary disease through mechanisms such as duodenal dilation causing stasis and bacterial overgrowth, leading to stone formation; tenting or stretching of the ampulla causing sphincter of Oddi dysfunction; and recurrent ascending cholangitis leading to biliary strictures. These changes can result in significant morbidity, including portal hypertension and need for liver transplantation. Clinicians should maintain a high index of suspicion for pancreaticobiliary complications in children with a history of duodenal obstruction, even years after repair. Long-term follow-up with imaging and endoscopic evaluation may be warranted. Further studies are needed to determine the true incidence and optimal surveillance strategies.