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Updated: May 11, 2026

Laparoscopic Choledochal Cyst Excision and Roux-en-Y Choledochojejunostomy in Adults
Published on: February 28, 2025
Does an isolated benign choledochal stricture hide a PSC?
Paola De Angelis1, Renato Tambucci, Erminia Romeo
1Digestive Surgery and Endoscopy Unit, Bambino Gesù Children's Hospital, IRCCS, Rome. paoladeangelis.opbg@gmail.com
Insights
Isolated benign choledochal strictures (IBCS) in children are rare but treatable with endoscopic retrograde cholangiopancreatography (ERCP). Some cases may indicate a link between IBCS and primary sclerosing cholangitis (PSC) or ulcerative colitis (UC).
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Interventional Endoscopy
Background:
- Extra-hepatic biliary tree strictures in children are uncommon, often stemming from benign inflammatory causes or idiopathic fibrosing pancreatitis.
- Primary sclerosing cholangitis (PSC) can present as single or multiple biliary strictures.
- This study focuses on isolated benign choledochal stricture (IBCS) in pediatric patients.
Purpose of the Study:
- To describe the clinical presentation, treatment strategies, and outcomes for children diagnosed with IBCS.
- To evaluate the efficacy of endoscopic retrograde cholangiopancreatography (ERCP) in managing IBCS.
- To explore potential associations between IBCS and other inflammatory conditions like ulcerative colitis (UC) and PSC.
Main Methods:
- Magnetic resonance cholangiography (MRC) for diagnosis in all six patients.
- Diagnostic and therapeutic ERCP, including sphincterotomy, stricture dilation, and stenting, performed in five patients.
- Intra-choledochal mini-probe endoscopic ultrasound (EUS) with biopsy in four patients; colonoscopy for suspected UC.
Main Results:
- Six pediatric patients (mean age 12.1 years for males, 14.2 for females) with IBCS were identified.
- Presentations included acute biliary pancreatitis, obstructive jaundice, cholestasis, and pancreatitis.
- All patients were asymptomatic post-treatment (mean follow-up 21 months); however, four developed UC and one developed PSC.
Conclusions:
- Therapeutic ERCP is an effective treatment for IBCS in children.
- The development of UC in some patients suggests a potential link between IBCS and PSC, warranting further investigation.
Background:
Strictures of the extra-hepatic biliary tree are rare in children and have a benign non-traumatic inflammatory origin or are related to idiopathic fibrosing pancreatitis. Primary sclerosing cholangitis (PSC) can manifest as multiple biliary strictures or as a single dominant stricture. We describe the presentation, treatment, and outcome of six cases of isolated benign choledochal stricture (IBCS).
Methods:
All patients underwent magnetic resonance cholangiography (MRC). Five patients underwent diagnostic and therapeutic ERCP, and 4 patients underwent intra-choledochal mini-probe EUS and biopsy. Colonoscopy was performed in suspected ulcerative colitis (UC).
Results:
We report 6 patients (mean age at diagnosis: four males, 12.1 years; two females, 14.2 years) with IBCS. Clinical onset included 3 cases of acute biliary pancreatitis and obstructive jaundice, one obstructive jaundice, one cholestasis, and one pancreatitis. At diagnosis, MRC confirmed IBCS in all patients. Biliary sphincterotomy, stricture dilation, and stenting were performed in 4 patients. One child underwent hepaticojejunostomy for a type I choledocal cyst. During follow-up (mean: 21 months; range: 1-3 years), all patients were asymptomatic. Four patients developed UC (three pancolitis, one descending colitis). One child developed PSC.
Conclusion:
IBCS can be successfully treated by therapeutic ERCP. The occurrence of UC could suggest that IBCS is a form of PSC.
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