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

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.