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Prune belly syndrome

 
Medical Dictionary: abdominal muscle deficiency syndrome
 

n.

Congenital absence of abdominal muscles, in which the outline of the intestines is visible through the protruding abdominal wall and the skin in the abdominal region is wrinkled. Also called prune-belly syndrome, triad syndrome.

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Wikipedia: Prune belly syndrome
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Prune belly syndrome
Classification and external resources
Prune belly syndrome in an Egyptian child with Down syndrome.
ICD-10 Q79.4
ICD-9 756.71
OMIM 100100
DiseasesDB 31089
eMedicine med/3055  radio/575
MeSH D011535

Prune belly syndrome is a rare birth defect affecting about 1 in 40,000 births.[1] About 96% of those affected are male. Prune belly syndrome is a congenital disorder of the urinary system, characterized by a triad of symptoms. The syndrome is named for the mass of wrinkled skin that is often (but not always) present on the abdomens of those with the disorder. Other names for the syndrome include Abdominal Muscle Deficiency Syndrome, Congenital Absence of the Abdominal Muscles, Eagle-Barrett Syndrome,[2] Obrinsky Syndrome,[3] Fröhlich Syndrome,[4] or rarely, Triad Syndrome.

Contents

Symptoms

Diagnosis

Prune belly syndrome can be diagnosed via ultrasound while a child is still in-utero.[5] An abnormally large abdominal mass is the key indicator, as the abdomen swells with the pressure of accumulated urine. In young children, frequent urinary tract infections often herald prune belly syndrome, as they are normally uncommon. If a problem is suspected, doctors can perform blood tests to check kidney function. Another test that may reveal the syndrome is the voiding cystourethrogram.

A genetic predisposition has been suggested, and PBS is much more common when the baby is a twin, although all reported twin births have been discordant.

Complications

Prune belly syndrome can result in the distending and enlarging of internal organs such as the bladder and intestines. Surgery is often required to return these organs to their natural sizes.

Treatment

The type of treatment, like that of most disorders, depends on the severity of the symptoms. One option is to perform a vesicostomy, which allows the bladder to drain through a small hole in the abdomen. A more drastic procedure is a surgical "remodeling" of the abdominal wall and urinary tract. Boys may have an orchiopexy, which moves the testicles to their proper place in the scrotum.

Even with treatment, many patients experience renal failure.

References

  1. ^ Baird PA, MacDonald EC (1981). "An epidemiologic study of congenital malformations of the anterior abdominal wall in more than half a million consecutive live births". Am. J. Hum. Genet. 33 (3): 470–8. PMID 6454342. 
  2. ^ Eagle JF, Barrett GS (1950). "Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: A syndrome. Report of 9 cases". Pediatrics 6 (5): 721–36. PMID 14797335. 
  3. ^ Obrinsky W (1949). "Agenesis of abdominal muscles with associated malformation of the genitourinary tract; a clinical syndrome". Am J Dis Child 77 (3): 362–73. PMID 18116668. 
  4. ^ Frolich, F. Der Mangel der Muskeln, insbesondere der Seitenbauchmuskeln. Dissertation: Wurzburg 1839.
  5. ^ synd/1499 at Who Named It?

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Copyrights:

Medical Dictionary. The American Heritage® Stedman's Medical Dictionary Copyright © 2002, 2001, 1995 by Houghton Mifflin Company Read more
Veterinary Dictionary. Saunders Comprehensive Veterinary Dictionary 3rd Edition. Copyright © 2007 by D.C. Blood, V.P. Studdert and C.C. Gay, Elsevier. All rights reserved.  Read more
Wikipedia. This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Prune belly syndrome" Read more