title: Does an isolated benign choledochal stricture hide a PSC?
abstract: 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.
  
  
  CONCLUSIONS
  IBCS can be successfully treated by therapeutic ERCP. The occurrence of UC could suggest that IBCS is a form of PSC.
authors.affiliation: Digestive Surgery and Endoscopy Unit, Bambino Gesù Children's Hospital, IRCCS, Rome. paoladeangelis.opbg@gmail.com
authors.forename: Paola
authors.initials: P
authors.lastname: De Angelis
authors.forename: Renato
authors.initials: R
authors.lastname: Tambucci
authors.forename: Erminia
authors.initials: E
authors.lastname: Romeo
authors.forename: Francesca
authors.initials: F
authors.lastname: Rea
authors.forename: Claudia
authors.initials: C
authors.lastname: Caloisi
authors.forename: Tamara
authors.initials: T
authors.lastname: Caldaro
authors.forename: Giovanni Federici
authors.initials: GF
authors.lastname: di Abriola
authors.forename: Francesca
authors.initials: F
authors.lastname: Foschia
authors.forename: Filippo
authors.initials: F
authors.lastname: Torroni
authors.forename: L
authors.initials: L
authors.lastname: Monti
authors.forename: Luigi
authors.initials: L
authors.lastname: Dall'Oglio
mesh_terms: D000293:Adolescent; D002648:Child; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000000981:diagnostic imaging; D015529:Choledochal Cyst / Q000150:complications / Q000000981:diagnostic imaging; D001651:Cholestasis, Extrahepatic / Q000150:complications; D003093:Colitis, Ulcerative / Q000150:complications; D003113:Colonoscopy; D003135:Common Bile Duct / Q000000981:diagnostic imaging / Q000473:pathology*; D003251:Constriction, Pathologic / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D004106:Dilatation; D019160:Endosonography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D041781:Jaundice, Obstructive / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D010195:Pancreatitis / Q000150:complications; D016717:Sphincterotomy, Endoscopic; D015607:Stents