PruneBelly.org
Title
Prune Belly Syndrome Network
Description
G e neral:
The incidence of prune belly syndrome is 1 in 40,000 live births; 95% of cases occur in males. In the past, it was suggested that the abdominal wall was deformed by pressure from a distended bladder due to bladder outlet obstruction in utero. Currently it is thought that prune belly syndrome is a multisystem disease complex which derives from a primary defect in mesodermal development.
The major prognostic factor is the degree of dilation of the urinary tract; 20% of patients are stillborn, 30% die of renal failure or urosepsis within the first two years of life, and the remaining 50% have varying degrees of urinary pathology.
Imaging:
Renal abnormalities vary widely. There may be agenesis or hypoplasia, which is rare, or hydronephrosis. On IVP, 80% of patients have evident tortuous ureters with dilatation distally. Vesicoureteral reflux is common. The bladder is generally dilated, and the trabecula are thinned. A patent urachus may allow the bladder to decompress.
Additional Information
Related Domains
- Bladder
- Conditions And Diseases
- Discussion Forum
- Eagle Barrett
- Genetic
- Genetic Disorders
- Health
- Medical Education
- Medicine
- Meso Dermal
- Non Profit
- Pediatric Urology
- Pediatrics
- Prune Belly Syndrome
- Rare Diseases
- Support Group
- Support Network
- Ureter
- Urological
- Urology
- Conditions and Diseases
- Eagle-Barrett
- MesoDermal
- Non-profit