A Family Study of Seven Index Patients

The type of jejunal atresia with agenesis of the mesentery, which has been variously described as the 'Christmas tree', 'maypole', or 'apple peel' deformity, consists of a duodenal or high jejunal atresia associated with absence of the small bowel mesentery and pre-arterial arcades of the superior mesenteric artery. The resulting appearance is of distal small bowel coming straight off the caecum and twisted around a marginal artery (Fig.), suggesting to different observers the designations quoted. The affection has been described in isolated cases (Santulli and Blanc, 1961; Jiminez and Reiner, 1961; Weitzman and Vanderhoof, 1966) but not, as far as the authors know, in more than one member of a sibship. The present paper records a family study of 7 index patients with this rare malformation, belonging to 6 families.