View source for Prune belly syndrome

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{{Infobox_Disease |
  Name           = {{PAGENAME}} |
  Image          = |
  Caption        = |
  DiseasesDB     = 31089 |
  ICD10          = {{ICD10|Q|79|4|q|65}} |
  ICD9           = {{ICD9|756.71}} |
  ICDO           = |
  OMIM           = 100100 |
  MedlinePlus    = |
    MeshID         = D011535 |
}}
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==Overview==

'''Prune belly syndrome''' is a rare [[birth defect]] affecting about 1 in 30,000 births.<ref>{{cite journal |author=Baird PA, MacDonald EC |title=An epidemiologic study of congenital malformations of the anterior abdominal wall in more than half a million consecutive live births |journal=Am. J. Hum. Genet. |volume=33 |issue=3 |pages=470-8 |year=1981 |pmid=6454342 |doi=}}</ref>  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 [[abdomen]]s of those with the disorder.  Other names for the syndrome include Abdominal Muscle Deficiency Syndrome, Congenital Absence of the Abdominal Muscles, Eagle-Barrett Syndrome,<ref>. F. Eagle Jr, and G. S. Barrett; Jr: Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: a syndrome; Reort of nine cases. Pediatrics, Evanston, Illinois, 1950, 6 (5): 721-736.</ref> Obrinsky Syndrome,<ref>W. Obrinsky. Agenesis of abdominal muscles with associated malformations of the genitourinary tract. A clinical syndrome. American Journal of Diseases of Children, Chicago, 1949, 77: 362-373.</ref> Fröhlich Syndrome,<ref>Frolich, F. Der Mangel der Muskeln, insbesondere der Seitenbauchmuskeln. Dissertation: Wurzburg 1839.</ref> or rarely, Triad Syndrome.

==Symptoms==

* A partial or complete lack of [[Abdomen|abdominal muscle]]s.  There may be wrinkly folds of skin covering the abdomen.
* [[Undescended testicles]] in males
* Urinary tract abnormality such as unusually large [[ureter]]s, distended [[urinary bladder|bladder]], accumulation and backflow of [[urine]] from the bladder to the ureters and the [[kidney]]s
* Frequent [[urinary tract infection]]s due to the inability to properly expel urine.

==Diagnosis==

Prune belly syndrome can be diagnosed via [[medical ultrasonography|ultrasound]] while a child is still in-utero.<ref>{{WhoNamedIt|synd|1499}}</ref>  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 infection]]s 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," in which a catheter is inserted into the [[urethra]] to fill the bladder with dye.  An [[X-ray]] can detect if urine has a reverse flow ''into'' the ureters and kidneys (see [http://www.mypacs.net/cgi-bin/repos/mpv3_repo/wrm/repo-view.pl?cx_subject=281639&cx_image_only_mode=off&cx_repo=mpv3_repo&cx_from_folder= example x-ray teaching file]).
Prune belly syndrome can also 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.

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 [[wikt:discordant|discordant]].

==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==
<references/>

==External links==

* [http://www.prunebelly.org/] The Prune Belly Syndrome Network:  A support group created by and for prune-belly patients. 

{{Congenital malformations and deformations of musculoskeletal system}}
{{SIB}}
[[Category:Urology]]
[[Category:Congenital disorders]]
[[Category:Syndromes]]
[[fr:Syndrome de Prune Belly]]

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