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Congenital absence of intestinal smooth muscle: a case report and review of the literature

Authors
Journal
Journal of Pediatric Surgery
0022-3468
Publisher
Elsevier
Publication Date
Volume
44
Issue
11
Identifiers
DOI: 10.1016/j.jpedsurg.2009.08.008
Keywords
  • Intestinal Perforation
  • Newborn
  • Necrotizing Enterocolitis
  • Spontaneous Intestinal Perforation (Sip)
  • Congenital Absence Of Intestinal Musculature (Caim)
Disciplines
  • Biology
  • Medicine

Abstract

Abstract Herein is reported case of an otherwise healthy full-term infant girl who presented with numerous spontaneous intestinal perforations with congenital absence of intestinal muscularis mucosae and muscularis propria. Few other cases are reported in the English literature with varying presentations. We review those cases, theories of pathogenesis, embryology, and possible connections to various clinical presentations.

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