Background/purpose: Description of the long-term follow-up of 5 patients operated on for atresia of the common bile duct.
Methods: During a 25-year period (1960 to 1985) 45 infants underwent surgical exploration for biliary atresia (BA), of which, 5 (11.1%) were found to have atresia of the common bile duct. The children were followed up into adult life by pediatric surgeons, pediatricians, and later, adult hepatologists with the range of 19 to 36 years. Liver function tests, histology, complications, and somatic development (including sexual maturation and mental development) were obtained continuously.
Results: Liver function test results were normal in all but 1 patient. Repeated ultrasound scan and postoperative liver biopsies were normal in 2 patients and moderately and mildly abnormal in 3 patients, respectively, suggesting hepatic fibrosis. HIDA hepatic scans in all but 1 patient showed prompt uptake by the liver with passage into the bowel within 30 minutes. Endoscopic retrograde cholangiography (ERCP; 1 patient) and duodenography (4 patients) showed, at most, mild reflux of contrast material into the extra- or intrahepatic bile ducts. One patient with cholecysto-duodenostomy had 3 episodes of clinically proven ascending cholangitis. All 5 patients had normal physical growth and mental development, they are all age-appropriate schooled, and they are working and living a normal life.
Conclusions: Long-term favorable outcome has been suggested to be more influenced by anatomic and biological features rather than the surgical correction. The hypothesis is supported that BA is not a static congenital malformation but a progressive inflammatory panductular obliterative disease of the bile ducts starting in the antenatal period, which might cease, either early at birth or any time in infancy. The destructive inflammatory process might involve only the distal part of the extrahepatic bile duct causing obstruction and leaving the proximal ducts patent.
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