THE ASSOCIATION OF SYNDROMIC PAUCITY OF THE INTERLOBULAR BILE-DUCTS AND CONGENITAL MECHANICAL OBSTRUCTION OF THE SMALL-INTESTINE

Three patients with congenital mechanical intestinal obstruction and f eatures of syndromic paucity of the interlobular bile ducts (SPILBD) a re presented. Two of them have siblings with syndromic paucity of the interlobular bile ducts. Two patients had jejunoileal atresia, while t he other had a meconium plug with intestinal perforation. All subjects underwent surgery for intestinal obstruction within the first 24 h of life. Progressive disappearance of the bile ductules was observed in one case. We believe that an insult such as vascular insufficiency to both bile ductal epithelium and the small intestine may be the cause o f this congenital disorder.