Ileal dysgenesis in infants and children

Ileal dysgenesis describes the segmental dilatation of the terminal ileum treated in seven patients presenting from birth to 15 yr. Manifestations included saccular segmental ileal dilatation adherent to the peritoneal surface of an omphalocele sac in two neonates, a radiographically delineated enterolith in a child studied for an unrelated disorder, and a syndrome including pallor, fatigue, and anemia in four children aged 10 to 15 yr. Literature review uncovered 11 similar patients to 38 yr of age. Other symptoms in that group included intestinal obstruction and recurrent abdominal pain. At laparotomy all patients were found to have segmental saccular or tubular dilatation of the terminal ileum. The dilated segments varied in length from 8 to 25 cm. Meckel's diverticula were present in two patients. Segmental resection and anastomosis was performed in all patients. No lumenal stenosis was present in any specimen to account for the dilatation. Previous reports have referred to this abnormality as "giant Meckel's diverticulum" or "segmental ileal dilatation." Evaluation of these 18 patients suggests that this entity is an intrinsic abnormality of ileal development at the morphologically active site of juncture with the yolk stalk.