The first autopsy case of a 13q- syndrome with extensive intestinal atresia, viz., absence of almost the entire jejunum and ileum, is reported. The absence of the mesentery and the histological properties of the intraperitoneal string-structures composed of some muscle layers resembling the tunica muscularis of the alimentary tract suggest that one of the causative agents of the atresia may be abnormalities of mesenteric vessels.
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