Prune belly syndrome with pouch colon and absent dermatome

Prune belly syndrome (PBS) is a rare congenital constellation of defects in pediatric surgical practice. Although anorectal anomalies have been reported in association with PBS, only few case of pouch colon with PBS has been reported. [1] In addition, our patient had deficient abdominal wall with ab...

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Bibliographic Details
Published in:African journal of paediatric surgery 2010-01, Vol.7 (1), p.25-27
Main Authors: Baba, Aejaz A, Hussain, Syed A, Shera, Altaf H, Patnaik, Rekha
Format: Article
Language:English
Subjects:
Abdominal Wall - abnormalities
Anal Canal - abnormalities
anorectal malformation
Care and treatment
Case studies
Colon - abnormalities
Colon - surgery
Colorectal diseases
Colostomy
Complications and side effects
Cystostomy
Diagnosis
Female
Follow-Up Studies
Humans
Infant, Newborn
Male
Musculoskeletal system
pouch colon
Prune belly
Prune belly syndrome
Prune Belly Syndrome - complications
Prune Belly Syndrome - surgery
Rectum - abnormalities
Risk factors
Surgery
Treatment Outcome
Urinary Fistula - surgery
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Description
Summary:Prune belly syndrome (PBS) is a rare congenital constellation of defects in pediatric surgical practice. Although anorectal anomalies have been reported in association with PBS, only few case of pouch colon with PBS has been reported. [1] In addition, our patient had deficient abdominal wall with absent dermatome in left upper quadrant, which has never been reported in the English literature. This association with abdominal wall deficiency and absent dermatome not only strengthens the theory of mesodermal arrest in the etiology of PBS but also points towards a defect in the ectodermal development.
ISSN:0189-6725
0974-5998
DOI:10.4103/0189-6725.59356