Segmental absence of intestinal musculature

BACKGROUND/PURPOSE: The aim of this study was to investigate the possible etiologic factors of small bowel atresia and to detect the prognostic role of adequate resection and tapering in postoperative morbidity and mortality. METHODS: Intestinal resection specimens were obtained from 10 patients with jejunoileal atresia and 3 control subjects without any gastrointestinal disease. Intestinal specimens taken from 2-cm and 4-cm proximal sides of atresia, atretic segment, and 1-cm and 2-cm distal sides of atresia were stained with Masson trichrome and H&E...

An unusual case of segmental defect of ileal musculature with terminal ileal atresia and ventricular septal defect is reported. Clinically the patient was diagnosed as ileal atresia. On exploration, there was type I atresia at ileocaecal junction with attendant ileal dilatation and an adjoining 5 cm x 4 cm segment resembling segmental dilatation of ileum, about 15 cm proximal to ileocaecal junction. Pathological examination of segmentally dilated ileum revealed focal absence of circular and longitudinal muscle coat without any evidence of necrosis or inflammation...

Herein we describe a case of 33-year old woman repeatedly affected by incomplete ileus. Primary segmental absence of lamina muscularis propria has been found in this patient. Histological examination revealed areas of small intestine with total absence of muscularis propria followed by areas of the intestinal wall characterised by normal histological structure. No necrosis, inflammation, and fibrosis supporting the secondary origin of this lesion was found.

A case of intrauterine intestinal volvulus without malrotation is presented, and 9 other cases from the world literature are reviewed. Bloody diarrhea, bluish discoloration of the abdominal wall (Cullen's sign), and a tense abdomen in neonates suggest midgut volvulus requiring prompt surgical management. On pathological examination, the neck of the twisted bowel in this case showed a half-circle defect of the intestinal muscle. This suggests that a segmental defect of the intestinal musculature could be secondary to volvulus...

A full-term baby boy with a segmental defect of the ileal musculature associated with terminal ileal and biliary atresia is presented. The newborn had a dilated loop of the ileum 30 cm proximal to the ileal atretic site. Pathological study results showed absence of the intestinal musculature with relatively intact mucosa. Foci of recent muscular necrosis were found in the lesion. One month later, relaparotomy was performed because of persistent jaundice and hepatic duct atresia was confirmed. Segmental defect of the intestinal musculature associated with ileal atresia and biliary atresia has not been reported in the literature...

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We report a case of segmental defect of colonic muscularis propria revealed by perendoscopic perforation in a 64-year-old woman. Segmental absence of intestinal musculature is well documented in new-borns and infants and is more frequent in small bowel. It is characterized by localised absence of muscularis propria without fibrous scar. The remaining layers of the bowel wall are intact. The pathogenesis of this lesion is discussed.

Two cases of premature newborns with a segmental defect of the intestinal musculature are presented. The clinical diagnoses were intestinal obstruction and perforation, respectively. Emergency laparotomy was performed. In the first case, this showed a dilated loop of ileum and proximally dilated small bowel. The second case had ileal perforation with 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 cases...

We describe a rare case of a viable preterm infant who developed a spontaneous localized perforation of the small bowel caused by a segmental absence of the intestinal musculature and associated with vascular ectasia. The findings may support a causal relationship between absence of the intestinal muscle coat and a vascular network disorder which may be responsible for the gastroenteric defect.

Absent intestinal musculature is a rare entity of uncertain etiology. The diagnosis is confirmed histologically by segmental absence of the intestinal muscularis. The remaining layers of the bowel wall are completely intact, and notably absent are significant inflammation and hemorrhage. The authors report two cases of segmental absence of intestinal musculature presenting as perforations. There was gross or microscopic evidence of diverticula formation at sites of perforation and absent muscle. Based on the anatomic evidence in these specimens and a review of the surgical literature, we propose that the etiology is based on embryologic diverticuli...

The case of a newborn infant with small-intestine atresia coexisting with segmental absence of muscle coats leading to perforation and meconium peritonitis is presented. The theories of pathogenesis of the simultaneous occurrence of segmental absence of gut musculature and atresia are briefly reviewed. A possible etiologic role of vascular accident in utero for both conditions is discussed.

The contracted segment of terminal large intestine in both Hirschsprung's disease in humans and in the piebald-lethal strain of mice with congenital megacolon may result from muscarinic cholinergic receptor denervation supersensitivity. This hypothesis was tested by direct binding studies using the muscarinic receptor ligand, tritiated quinuclidinyl benzilate. There were no differences in either the receptor density, dissociation constant, or agonist/antagonist Hill coefficients in the proximal, middle, or distal colon of mice with congenital megacolon as compared with normal littermates...

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These studies were directed toward better characterization of the abnormalities of motor function in the large intestine of mutant mice with congenital aganglionosis and megacolon. Analysis of pressure-volume relations in the megacolon and aganglionic terminal segment showed increased intestinal wall compliance in the dilated colon and reduced wall compliance in the aganglionic region as compared to normal littermates. Migrating contractile complexes occurred spontaneously in ganglionated regions of the large intestine of both normal and mutant mice, but never propagated into the aganglionic segment of the abnormal bowel...

Three instances of segmental absence of small intestinal musculature are described. Based on their clinical and pathological features and on the 12 cases previously described in the English literature, these can be classified into two groups: primary and secondary. In the primary group, the etiology is unknown, the onset of symptoms is acute, and there are no pathologic findings in the remaining layers of the small bowel except for superimposed perforation, or intussusception. In the secondary group, there is a longer history of intestinal symptoms and of multiple surgical procedures...

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