[Management of biliary tract malformations in childhood--long-term survival and reoperation]

During the past 32 years, 67 of 145 cases of biliary tract malformations survived for 8-30 years as the result of various types of management from childhood. Controversies regarding management were analyzed. The majority of the patients with biliary tract malformation required radical operation initially. Regarding postoperative complications, however, non-surgical managements; PTCD, endoscopic lithotomy for remaining calculi, endoscopic injection sclerotherapy for esophageal varices or PSE (partial splenic embolization) for portal hypertension, are preferred. The optimal time to treat the patients; biliary atresia--within 2 mo. for Kasai operation, choledochal cyst depends on the patient's symptom-onset, but extrahepatic biliary tract perforations may occasionally occur in infants, then antenatal ultrasonography (US) is necessary. Five infants out of 8 patients with choledochal dilatation due to congenital duodenal obstruction and annular pancreas were successfully treated following duodenal surgery alone, however a 18 yr-old female with this lesion underwent biliary tract reconstruction because of irreversible biliary tract dilatation. The preferred management we recommend; 1) pre-or intraoperative diagnosis using US, cholangiography or intraoperative direct probe survey of the biliary or pancreatic duct, 2) accurate surgery with complete excision of the cyst or biliary stenosis plasty with adequate anastomosis, 3) a sustained long-term follow-up study utilizing US.