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.
Abstract

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