Prune Belly Syndrome

Definition

Definition of Prune Belly Syndrome
Prune belly syndrome is a rare, genetic, birth defect affecting about 1 in 40,000 births. About 97% 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 abdomens of those with the disorder. Other names for the syndrome include Abdominal Muscle Deficiency Syndrome, Congenital Absence of the Abdominal Muscles, Eagle-Barrett Syndrome, Obrinsky Syndrome, Fröhlich Syndrome, or rarely, Triad Syndrome.

Symptoms

Symptoms of Prune Belly Syndrome

1. A partial or complete lack of abdominal muscles. There may be wrinkly folds of skin covering the abdomen.
2. Undescended testicles in males
3. Urinary tract abnormality such as unusually large ureters, distended bladder, accumulation and backflow of urine from the bladder to the ureters and the kidneys
4. Frequent urinary tract infections due to the inability to properly expel urine.
5. Later in life, a common symptom is post-ejaculatory discomfort. Most likely a bladder spasm, it lasts about two hours.

Causes

Causes of Prune Belly Syndrome
The cause of the syndrome is unknown, but researchers believe that disruption of the growth of the fetus causes the problems to develop. It is thought that something blocks a part of the fetus' urinary tract, and this causes other parts of the tract to develop abnormally. Prune Belly syndrome occurs in 1 in 40,000 live births in the U.S.

Diagnosis

Diagnosis of Prune Belly Syndrome
Prune belly syndrome can be diagnosed via ultrasound while a child is still in-utero. 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 infections 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.

Treatment

Treatment of Prune Belly Syndrome
Prune belly syndrome can be diagnosed via ultrasound while a child is still in-utero. 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 infections 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.

Prognosis

Prognosis of Prune Belly Syndrome
Prune belly syndrome can 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.

Prevention

Prevention of Prune Belly Syndrome
There is no known way to prevent this condition. If the baby is diagnosed with a urinary tract obstruction before birth, in rare cases surgery during the pregnancy may help prevent the problem from progressing to prune belly syndrome.