Prune belly syndrome

Sabtu, 22 Januari 2011
Prune belly syndrome is also called Eagle-Barrett syndrome and triad syndrome.

Prune belly syndrome is also called Eagle-Barrett syndrome and triad syndrome. The treatment of prune belly syndrome poses a significant problem to pediatric urologists. Some authors call for conservative management of the urinary tract in boys with prune belly syndrome, while others advocate an aggressive approach, operating on patients aged 10 days. No definitive timing for treatment of prune belly syndrome has been substantiated. Pediatric urologists have observed that boys with prune belly syndrome can present with a spectrum of abnormalities. At one end of the spectrum, the condition may cause severe urogenital and pulmonary problems incompatible with life (resulting in stillbirth); at the other end of the spectrum, the condition may cause few, if any, urological abnormalities that require no treatment other than orchidopexy to correct the undescended testis. The decision to aggressively treat the urinary tract in these children should be based on the clinical presentation and not solely on radiographic appearance. 

Problem
Children with prune belly syndrome can present with myriad renal, ureteral, and urethral abnormalities. Obstruction and/or upper urinary tract dilatation is not unusual in these children. The site of obstruction can vary from as high as the pelviureteral junction to as low as the prostatic membranous urethra.
A lack of abdominal muscles leads to a poor cough mechanism, which, in turn, leads to increased pulmonary secretions. Weak abdominal muscles lead to constipation because of an inability to perform the Valsalva maneuver, which helps push the stool out of the rectum during defecation.
The mortality rate associated with prune belly syndrome is 20%.

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