Spontaneous perforation of the extrahepatic bile duct in infancy: report of two cases and literature review

Abstract

Background: Spontaneous perforation of the bile duct (SPBD) is a rare disease in infancy. The pathogenesis, diagnostic modalities and treatment options for this condition are reviewed and discussed.

Methods: The authors report 2 new observations of SPBD in 2 male newborns aged respectively 27 and 21 days.

Results: The 2 newborns presented with cholestatic jaundice. Abdominal sonography showed an extrahepatic mass. Exploratory laparotomy revealed that the perforation was located in the cystic duct in the first case and in the common bile duct (CBD) in the second. The site of perforation was repaired and both patients underwent simple external biliary drainage. The postoperative course was uneventful for both patients with follow-up ranging from 2 to 4 years.

Conclusions: SPBD is a rare, but important cause of surgical jaundice in infants. The pathogenesis of SPBD is unknown and multifactorial and diagnosis is often problematic. Surgical management is always required and a conservative approach is usually recommended. The prognosis is good with early surgical management.