SEGMENTAL DEFECT OF THE INTESTINAL MUSCULATURE OF A NEWBORN - EVIDENCE OF ACQUIRED PATHOGENESIS

Two cases of premature newborns with a segmental defect of the intesti nal musculature are presented. The clinical diagnoses were intestinal obstruction and perforation, respectively. Emergency laparotomy was pe rformed. In the first case, this showed a dilated loop of ileum and pr oximally dilated small bowel. The second case had ileal perforation wi th marked pneumoperitoneum. Pathological examination showed multifocal partial or complete absence of the muscularis propria, with relative preservation of the remaining components of the bowel wall in both cas es. In addition, foci of recent muscular necrosis was noted in case 1, and early replacement fibrosis in case 2. These findings suggest that the muscle defect was secondary to muscle injury rather than a primar y absence of muscle. The different theories of pathogenesis are discus sed. (C) 1996 by W.B. Saunders Company