RESUMO
Cystic malformations of the bile ducts are rare congenital disorders, with an incidence of 1/2000000 live births. Complication including the angiocholitis, chronic pancreatitis, progressive biliary cirrhosis, portal hypertension or gallbladder lithiases can reveal severe disorder. Spontaneous perforation is one of the rare complications described for the first time in 1934 by Weber. We report the case of an 18-month old baby admitted with subocclusive syndrome with biliary peritonitis. Ultrasound was performed showing abdominal effusion with cystic formation communicating with the bile ducts associated with subcapsular effusion of the liver confirmed by a scanner. Treatment was based on peritoneal toilet with redon drain at the level of the perforation and subhepatic drain without cyst excision. The patient was re-admitted 6 months after this incident to be definitively treated.
