ABDOMINAL MUSCLES, ABSENCE OF, WITH URINARY TRACT ABNORMALITY AND CRYPTORCHIDISM
Victor A. McKusick, Johns Hopkins University, September 6, 2007
[for Professionals mainly]
Alternative titles; symbols
PRUNE BELLY SYNDROME
This condition was first described by Frolich (1839). The appellation "prune belly syndrome" is descriptive because the intestinal pattern is evident through the thin, lax, protruding abdominal wall in the infant ( Osler, 1901 ). ( Osler did not use the term "prune belly" ... "In the summer of 1897 a case of remarkable distension of the abdomen was admitted to the wards, with greatly distended bladder, and on my return in September, Dr. Futcher, knowing that I would be interested in it, sent for the child".) The full syndrome probably occurs only in males ... Multiple cases (of the full syndrome) in families have rarely been reported, ... In British Columbia, Baird and MacDonald (1981) found a frequency of 1 in 29,231 live births ... similar to Poland syndrome (173800) in being rather consistently reproduced in many cases but having no clearly demonstrable mendelian basis ...

Prune Belly Syndrome
Pedsurology.com
[for Professionals mainly] [Support Groups] [Illustrated]
Management ... has posed a significant amount of trouble to the pediatric urologist ... calls for conservative management of the urinary tract in these boys by some authors while others have advocated an aggressive approach ... operating on them at ten days of life. At the present time, no definitive timing for therapy has been substantiated one way or the other ... The management of the abdominal wall abnormalities has several camps with some going for no surgical reconstruction of the abdominal wall while others advocate surgical reconstruction and this group being divided amongst two separate factions advocating different techniques of abdominal wall reconstruction ... Obstruction and/or upper urinary tract dilatation is not a rare occurrence in these children ...
The management of the undescended testis in the patient with prune belly syndrome is some thing that should be left to the experienced pediatric urologist ...
Abdominal wall reconstruction is performed in most boys to improve respiratory function as well as for cosmetic reasons ...
Obstruction at the uretero - pelvic junction has been observed in patients with prune belly syndrome ... Infravesical obstruction or obstruction at the prostatic urethra was originally thought to be due to a Type I valve. This theory has been replaced by a new thinking in which the obstruction is felt to be caused by severe angulation at the prostatic and membranous urethral junction. This may be possibly due to a lack of striated muscle in the membranous urethra or the urogenital diaphragm, or a ring of obstructive tissue acting as a flap valve due to hypoplasia of the prostate, creating a ballooning of the prostatic urethra. In these patient's, studies have shown also that there is a reduction in the smooth muscle in the prostate with an increase in the connective tissue content which has been thought to lead to a functional obstruction ... A patent urachus can be frequently found in patients with urethral obstruction ... Posterior urethral valves are occasionally seen in patients with prune belly syndrome ...
The prune belly syndrome is characterized by elongated, dilated and tortuous megaureters ... ureteral abnormalities ...
Megacystis in prune belly patients is typically described. The bladder is routinely enlarged, although trabeculations are rarely present and muscular hypertrohyy is inconsistent ...
Pre operative view prior to Abdominal plication!
Post operative results after Abdominal wall plication.

Prune Belly Syndrome
National Organization for Rare Disorders, Inc. (NORD), November 5, 2007
Abdominal Muscle Deficiency Syndrome
Congenital Absence of the Abdominal Muscles
Eagle - Barrett Syndrome
Obrinsky Syndrome

Prune Belly Syndrome
United States National Library of Medicine, December 12, 2006
prune belly syndrome ( PBS )
Synonyms
Eagle - Barrett syndrome
Fröhlich syndrome
Obrinsky syndrome
abdominal muscle deficiency anomalad
abdominal muscle deficiency syndrome
abdominal musculature aplasia syndrome
absence of abdominal muscle syndrome
aplastic abdominal muscle syndrome
defective abdominal wall syndrome

Prune Belly Syndrome
I.B.I.S. Birth Defects, 2002
[Ukrainian]
Two fact sheets for specialists in Ukrainian.
Synonyms ... Main diagnostic criteria ... Clinical features ... Association of symptoms ... Etiology ... Pathogenesis ... Treatment ...

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Last Updated: 2007/11/08

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