Congenital biliary tract dilatation in the pediatric population is a very rare condition. The signs and symptoms are jaundice, abdominal pain, fever, abdominal mass, sepsis. The frequency in the American population is 1/15,000 and in Japan is 1/1,000. It is more frequent in women and during the neonatal period. 2/3 parts of the CBTD present in children less than 10 years old. No etiology has been identified, the afectation of the embrionary primordium has been postulated, as well as the abnormal union of the choledoco with the pancreatic duct. There are different anatomic forms that include intra and extra hepatic compromise (Todani Classification).

Patients and Methods : 29 cases were evaluated in the Gastroenterology Service since July 1984 to July 1997. Diagnosis was done by ultrasound in 100% and by TAC in 10%. 25/29 were operated and the diagnosis was confirmed by intraoperatory colangiography. Results: 26/29 were girls, time before diagnosis was 2 weeks to 8 months in patients whose clinical manifestations began before 3 months, and between 1 week to 8 years in older patients. Clinical : Cholangitis or sepsis 8/29, jaundice 24/29, abdominal mass 9/29, ascitis 3/29, biliary cirrosis by biopsy 5/15, 2/29 active bleeding and portal hipertension. Clasification : 22/29 Type I, 2/29 for types II, IV, V : two each one.