Prune belly syndrome is a rare, hereditary birth defect affecting approximately 1 in 40,000 births. Approximately 97 percent of the affected are male. Prune belly syndrome is a congenital disease of this urinary system, characterized by a triad of symptoms. The syndrome is named after the bulk of wrinkled skin that’s frequently (although not always) present about the gut of these with the disease.
Signs and symptoms of prune belly
Prune-belly triad consists of: Cryptorchidism, abdominal wall defects and genitourinary defects:
- A partial or complete lack of abdominal wall muscles. There may be wrinkly folds of skin covering the abdomen.
- Cryptorchidism (undescended testicles) in males
- Urinary tract abnormalities such as unusually large ureters, distended bladder, accumulation and backflow of urine from the bladder to the ureters and the kidneys (vesicoureteral reflux)
Other Symptoms include:
- Frequent urinary tract infections due to the inability to properly expel urine.
- Ventricular septal defect
- Malrotation of the gut
- Club foot
- Later in life, a common symptom is post-ejaculatory discomfort. Most likely a bladder spasm, it lasts about two hours.
- Musculoskeletal abnormalities include pectus excavatum, scoliosis, and congenital joint dislocations including the hip. Diagnosis of prune belly syndrome necessitates a thorough orthopedic evaluation because of the high prevalence of associated musculoskeletal abnormalities.
Treatment of prune belly
The kind of therapy, such as that of most ailments, depends upon the seriousness of the signs. 1 choice is to execute a vesicostomy, allowing the bladder to drain through a little hole in the gut, thus helping to prevent urinary tract infections. In the same way, consistent self-catheterization, often a few times every day, may be an effective solution to preventing illnesses. A more extreme procedure is a surgical “remodeling” of the abdominal wall and urinary tract. Boys frequently will need to experience an orchiopexy, to maneuver the testes for their appropriate location at the scrotum.
