Jejunal atresia with absent mesentery and a helical ileum

Of 59 infants with jejunoileal atresia, seven presented with absence of mesentery, the main superior mesenteric artery ceasing to exist beyond the origin of the right colic or ileocolic artery, and a helical ileum. This distinctive form of jejunal atresia has been recognized with increasing frequency in recent years. Distal to the atresia, the small bowel receives its blood supply retrograde from an artery derived from the ileocolic or right colic arcades, and the ileum coils around its nutrient artery in an "apple peel" or "Christmas tree" deformity. The first three patients in this report died. The lastion, but they recovered as their malabsorption gradually disappeared. The treatment includes resection of the dilated bowel, as in other atresias. Resection of part of the distal bowel may be required for additional atresias or for poor vascularity with questionable viability. Postoperative malabsorption generally requires intravenous hyperalimentation.