Abstract
Introduction: Spontaneous extrahepatic bile duct perforation is rare in newborns. It is a surgical cause of jaundice in this period and the acute presentation is unusual.
Objective: Present the case of spontaneous bile duct perforation in a newborn due to its serious complications if an early and timely diagnosis is not performed.
Clinical Case: An 10-day-old newborn who developed food rejection, fever and abdominal distension without jaundice, acolia, or coluria two days prior of admison. The laboratory tests revealed leukopenia, thrombocytosis, and elevation of C-reactive protein, with normal hepatic function. The abdominal x-ray showed pneumoperitoneum, and the diagnosis of necrotizing enterocolitis was made. Laparotomy was performed; extrahepatic bile duct perforation and biliary peritonitis were noted. Intraoperative cholangiography demonstrated rescatable proximal bile duct and dilated cystic duct. Hepatic-jejunostomy was performed with Roux-en-Y and cholecystectomy. In the postoperative study portal thrombosis was found, so he received anticoagulant treatment. At 8 months of age, the patient had enteral feeding tolerance and adequate weight gain.
Conclusions: Biliary perforation is a rare entity and more in the neonatal period, a condition that makes it a diagnostic and therapeutic challenge. The prognosis will depend on early intervention and intraoperative findings.

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