A syndrome of multiple gastrointestinal atresias with intraluminal calcification. A report of a case and a review of the literature.

An infant with multiple gastrointestinal atresias from the stomach to the rectum is reported and the literature concerning this syndrome is reviewed. The syndrome has been reported, to date, exclusively in infants born to families with a French-Canadian background. The radiological hallmark of this syndrome is extensive calcification of intraluminal content between the areas of atresia which appears as rounded or oval homogeneous radiopacities on abdominal radiographs. Inheritance is thought to be autosomal recessive and the etiology is uncertain. All previously reported cases have died. The significance of biliary gas, seen in postoperative films, is discussed.