Segmental Absence of Intestinal Musculature -Presentation in a 10-Year-Old Boy with an Extensive Involved Segment

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...

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Bibliographic Details
Published in:Fetal and pediatric pathology 2015-04, Vol.34 (2), p.128-132
Main Authors: Athanazio, Daniel Abensur, Rocha, Mateus T. Amaral, Mattos, Angela P., Ribeiro, Tereza Cristina Medrado, Sorte, Ney Cristian Boa, Freitas, Luiz Antonio R., Silva, Luciana R.
Format: Article
Language:English
Subjects:
Abdomen, Acute - diagnosis
Abdomen, Acute - pathology
Abdomen, Acute - surgery
Child
Colectomy
congenital abnormalities
Humans
ileum
Ileum - surgery
Intestinal Perforation - diagnosis
Intestinal Perforation - pathology
Intestinal Perforation - surgery
Male
Malnutrition - complications
Muscle, Smooth - pathology
peritonitis
Treatment Outcome
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Summary: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.
ISSN:1551-3815
1551-3823
DOI:10.3109/15513815.2014.987934