title: Spontaneous perforation of the biliary tract in infancy: a series of 11 cases.
abstract: Eleven patients presenting with spontaneous perforation of the biliary tract were treated at Bicêtre Hospital between 1971 and 1993. Three groups were individualised, each with a different pattern of local presentation: generalised biliary peritonitis (n = 2), localised biliary peritonitis (n = 4), secondary biliary stenosis (n = 5). In each case, cholestatic jaundice developed after a postnatal symptom-free interval. Ten patients were operated on. Perforation was located in the cystic duct (n = 2), at the junction of the cystic and hepatic ducts (n = 4), in the common hepatic duct (n = 1) or common bile duct (n = 1). The site of perforation was no longer identifiable in two cases with stenosis. A cholecystectomy was performed in the 2 cases with cystic duct perforation; in the cases of lesions of the main duct, either simple external biliary drainage (n = 3) or biliary reconstruction (n = 5) was carried out. Postoperative complications included bile leak (n = 2), ascending cholangitis (n = 1), portal vein thrombosis (n = 2). Five patients were submitted to further surgery including biliary revision (n = 3), porto-systemic shunt (n = 1), and other procedures (n = 2). One infant died from postoperative sepsis; 2 were lost to follow-up, one of which probably did not survive; 4 are alive and well. Late sequelae are present in 4 children: portal hypertension (n = 1), mild residual bile duct dilatation without cholestasis (n = 1), and mild to moderate liver fibrosis (n = 2). Prompt diagnosis and appropriate treatment should improve the prognosis of this rare condition.
authors.affiliation: Surgical Unit, Department of Paediatrics of Bicêtre Hôpital, Le Kremlin-Bicêtre, France.
authors.forename: C
authors.initials: C
authors.lastname: Chardot
authors.forename: F
authors.initials: F
authors.lastname: Iskandarani
authors.forename: O
authors.initials: O
authors.lastname: De Dreuzy
authors.forename: B
authors.initials: B
authors.lastname: Duquesne
authors.forename: D
authors.initials: D
authors.lastname: Pariente
authors.forename: O
authors.initials: O
authors.lastname: Bernard
authors.forename: F
authors.initials: F
authors.lastname: Gauthier
authors.forename: J
authors.initials: J
authors.lastname: Valayer
mesh_terms: D001649:Bile Duct Diseases / Q000000981:diagnostic imaging / Q000601:surgery*; D017734:Bile Ducts, Extrahepatic / Q000000981:diagnostic imaging / Q000601:surgery*; D002758:Cholangiography; D001651:Cholestasis, Extrahepatic / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010538:Peritonitis / Q000000981:diagnostic imaging / Q000601:surgery*; D011183:Postoperative Complications / Q000000981:diagnostic imaging; D012422:Rupture, Spontaneous; D016896:Treatment Outcome