title: Segmental absence of intestinal musculature -presentation in a 10-year-old boy with an extensive involved segment.
abstract: Segmental absence of the intestinal musculature is a rare cause of acute abdomen most likely associated with intestinal perforation and sepsis in neonates and adults. We present a case of a 10-year-old boy who developed acute abdomen and was treated with right hemicolectomy and partial resection of the ileum. The ileum showed a 20-cm-long stenotic segment showing prominent thinning of the intestinal wall. This case occurred in an older child, which appears to be rare when comparing the literature. The bowel defect was also the largest described to date, indicating the potential effect of an atonic segment leading to obstructive disease.
authors.affiliation: 1Federal University of Bahia , Salvador , Brazil.
authors.forename: Daniel Abensur
authors.initials: DA
authors.lastname: Athanazio
authors.forename: Mateus T Amaral
authors.initials: MT
authors.lastname: Rocha
authors.forename: Angela P
authors.initials: AP
authors.lastname: Mattos
authors.forename: Tereza Cristina Medrado
authors.initials: TC
authors.lastname: Ribeiro
authors.forename: Ney Cristian Boa
authors.initials: NC
authors.lastname: Sorte
authors.forename: Luiz Antonio R
authors.initials: LA
authors.lastname: Freitas
authors.forename: Luciana R
authors.initials: LR
authors.lastname: Silva
mesh_terms: D000006:Abdomen, Acute / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D002648:Child; D003082:Colectomy; D006801:Humans; D007082:Ileum / Q000601:surgery*; D007416:Intestinal Perforation / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery*; D008297:Male; D044342:Malnutrition / Q000150:complications; D009130:Muscle, Smooth / Q000473:pathology*; D016896:Treatment Outcome
keywords: congenital abnormalities; ileum; peritonitis