Multiple segmental absence of intestinal musculature presenting as spontaneous isolated perforation in an extremely low-birth-weight infant.

Abstract:

:Defect of the intestinal musculature is a rare condition. It may cause intestinal perforation or obstruction. It manifests itself mainly in the neonatal period and usually affects preterm infants. We describe one such case, which was first diagnosed as a spontaneous isolated intestinal perforation. Emergency laparotomy was performed and showed multiple perforations, with accompanying peritonitis and ascites. Pathologic examination showed partial or complete absence of the musculature, particularly of the inner circular layer, with fibrous tissue in the regions of missing muscle, and abnormal vasculature. The myenteric plexus was absent in areas of muscle loss but present in other sites. These findings suggest that the absence of muscle may not represent a congenital malformation but may be secondary to ischemic injury.

journal_name

J Pediatr Surg

authors

Oretti C,Bussani R,Janes A,Demarini S

doi

10.1016/j.jpedsurg.2010.05.029

subject

Has Abstract

pub_date

2010-08-01 00:00:00

pages

E25-7

issue

8

eissn

0022-3468

issn

1531-5037

pii

S0022-3468(10)00436-7

journal_volume

45

pub_type

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