Unilateral agenesis of the abdominal wall musculature: An early muscle deficiency

Prune‐belly sequence (PBS) usually results from early urethral obstruction. In rare cases, PBS seems to be due to a faulty primary development of the parietal mesenchyme leading to underdevelopment of the abdominal wall musculature, and disorganization of the smooth muscles in the urinary tract. We...

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Bibliographic Details
Published in:American journal of medical genetics. Part A 2010-11, Vol.152A (11), p.2870-2874
Main Authors: Gerard‐Blanluet, Marion, Port‐Lis, Marylin, Baumann, Clarisse, Perrin‐Sabourin, Laurence, Ebrad, Patrick, Audry, Georges, Delezoide, Anne‐Lise, Verloes, Alain
Format: Article
Language:English
Subjects:
Abdominal Muscles - abnormalities
Abdominal Muscles - diagnostic imaging
Abdominal Wall - abnormalities
Child
Female
Humans
Infant
Infant, Newborn
Male
mesenchyme development
Pregnancy
prune belly sequence
Radiography
unilateral
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Summary:Prune‐belly sequence (PBS) usually results from early urethral obstruction. In rare cases, PBS seems to be due to a faulty primary development of the parietal mesenchyme leading to underdevelopment of the abdominal wall musculature, and disorganization of the smooth muscles in the urinary tract. We report on two patients with segmental, unilateral wall musculature deficiency associated with homolateral agenesis of ribs. One patient also had hemivertebrae and the other one ipsilateral diaphragmatic eventration and aplasia cutis. This combination of anomalies may represent a localized deficiency in the development of somitic mesoderm mesenchyme during early embryogenesis. © 2010 Wiley‐Liss, Inc.
ISSN:1552-4825
1552-4833
1552-4833
DOI:10.1002/ajmg.a.33708