Does an isolated benign choledochal stricture hide a PSC?

Paola De Angelis, Renato Tambucci, Erminia Romeo, Francesca Rea, Claudia Caloisi, Tamara Caldaro, Giovanni Federici Di Abriola, Francesca Foschia, Filippo Torroni, L. Monti, Luigi Dall'Oglio

Research output: Contribution to journalArticlepeer-review

Abstract

AbstractBackground 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.

Original languageEnglish
Pages (from-to)1006-1011
Number of pages6
JournalJournal of Pediatric Surgery
Volume48
Issue number5
DOIs
Publication statusPublished - May 2013

Keywords

  • Choledochal stenosis
  • Primary sclerosing cholangitis
  • Ulcerative colitis

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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