title: Spontaneous biliary perforation in infancy: Management strategies and outcomes.
abstract: OBJECTIVE
  Infantile spontaneous biliary perforation is rare with variable management strategies ranging from nonoperative treatment to complex operations such as biliary-enteric reconstruction. Biliary fistula and portal vein thrombosis are known complications, though outcomes are poorly defined.
  
  
  METHODS
  We assessed the incidence of spontaneous biliary perforation in infants <1 year old using a population database. Next, we describe 4 patients treated at our institution and review all reported cases within the past 25 years.
  
  
  RESULTS
  The incidence of spontaneous biliary perforation is 1.5 in 1,000,000 live births. Over the past 25 years, 90 cases were reported, over half of which were initially managed with a surgical drainage procedure. The most common reason for failure of this strategy was CBD obstruction. Our 4 patients were successfully managed without biliary reconstruction despite 2 presenting with CBD obstruction. Reported complications occurred in 22% of patients, most frequently biliary fistula requiring delayed biliary reconstruction.
  
  
  CONCLUSIONS
  Surgical drainage is an effective method for treatment of infantile spontaneous biliary perforation; however a persistent biliary fistula should prompt evaluation for distal CBD obstruction. Though biliary-enteric anastomosis is the historic procedure of choice for persistent fistula, with improvements in endoscopic and percutaneous treatment, extensive biliary reconstruction may be avoided in the future.
authors.affiliation: University of California, San Francisco, Campus Box 0570, 513 Parnassus Avenue, San Francisco, CA 94143-0570, United States. Electronic address: Cerine.Jeanty@ucsfmedctr.org.
authors.forename: Cerine
authors.initials: C
authors.lastname: Jeanty
authors.affiliation: University of California, San Francisco, Campus Box 0570, 513 Parnassus Avenue, San Francisco, CA 94143-0570, United States.
authors.forename: S Christopher
authors.initials: SC
authors.lastname: Derderian
authors.affiliation: University of California, San Francisco, Campus Box 0570, 513 Parnassus Avenue, San Francisco, CA 94143-0570, United States.
authors.forename: Shinjiro
authors.initials: S
authors.lastname: Hirose
authors.affiliation: University of California, San Francisco, Campus Box 0570, 513 Parnassus Avenue, San Francisco, CA 94143-0570, United States.
authors.forename: Hanmin
authors.initials: H
authors.lastname: Lee
authors.affiliation: University of California, San Francisco, Campus Box 0570, 513 Parnassus Avenue, San Francisco, CA 94143-0570, United States.
authors.forename: Benjamin E
authors.initials: BE
authors.lastname: Padilla
mesh_terms: D001658:Biliary Fistula / Q000209:etiology; D001660:Biliary Tract Diseases / Q000453:epidemiology* / Q000601:surgery; D001662:Biliary Tract Surgical Procedures; D002779:Cholestasis / Q000209:etiology / Q000601:surgery; D004322:Drainage / Q000379:methods; D016099:Endoscopy, Gastrointestinal; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D011169:Portal Vein; D012422:Rupture, Spontaneous / Q000453:epidemiology / Q000601:surgery; D020246:Venous Thrombosis / Q000209:etiology
keywords: Common bile duct perforation; Gallbladder perforation; Spontaneous biliary perforation in infants