Prune belly syndrome, or Eagle-Barrett syndrome, is a condition that occurs when a blockage along the urinary tract keeps a fetus' bladder from properly draining while in utero, causing the fetal bladder to swell with fluid and bulge into the abdominal cavity. It also represents a collection of congenital defects that are characterized by three chief problems:
• Poorly developed abdominal muscles that make the stomach appear wrinkled like the skin of a prune
• Urinary tract abnormalities, such as an enlarged bladder
• Undescended testicles (the male reproductive organs are located in the abdomen rather than the scrotum, or they are completely absent)
These anomalies can give way to a host of urinary tract complications, including hydronephrosis, vesicoureteral reflux, hyrdoureter, and persistent urinary tract infections (UTIs). The syndrome can also be accompanied by defects involving the heart, intestines and skeletal system, making a one-size-fits-all treatment plan virtually impossible.
Considerations before Treatment of Prune Belly Syndrome
When treating prune belly syndrome, a number of factors need to first be considered, including:
• The child’s age, current health state, and other complicating conditions in addition to prune belly syndrome
• The severity of the disease. For example, the physical symptoms of the wrinkled belly may not be as severe as a malfunctioning bladder that can destroy healthy kidney tissue.
• The child’s prognosis given his or her condition
• The caregivers’ personal wishes for treatment
• The child's ability to tolerate certain procedures, medications, or therapies caregivers’ personal wishes for treatment
Medication for Prune Belly Syndrome
Prophylactic antibiotics are often prescribed in children with prune belly syndrome since the major defects associated with the condition cause urinary tract complications that often prevent urine from properly exiting the body. If urine builds up, bacteria can grow, leading to a urinary tract infection. The antibiotics prevent an infection from occurring, or work to kill bacteria and halt their growth.
Surgery for Prune Belly Syndrome
When treating the condition’s three chief problems, the following may be performed:
Abdominal Wall Reconstruction
Children with prune belly syndrome often have weakened abdominal muscles, which affect the child’s ability to sit up properly, an important phase in growth and development. For this reason, a physician may recommend abdominal wall reconstruction to remove excess abdominal tissue while also tightening the stomach muscles. This procedure helps to reduce the physical symptoms of prune belly syndrome while enhancing a child’s ability to sit and hold himself up.
Undescended Testicle Treatments
In some babies, undescended testicles will drop down with time. For this reason, a physician may recommend waiting to see if a child’s testicles will drop. If they do not after six months, surgical repair may be recommended to prevent inguinal hernias, tumors, testicular torsion, and infertility.
Called orchiopexy, the procedure involves making one or more small incisions in the scrotum, groin, or abdomen so that the testicle can be accessed and pulled down into the scrotum--it is then stitched into place. This can be performed either laparoscopically or via open surgery.
Vesicostomy
An enlarged bladder may not empty properly because the bladder muscles are too stretched out to properly push urine out. One of the most common treatments is known as a vesicostomy, whereby a small cut is made in the baby’s skin, typically just below the belly button. Once the bladder wall is reached, a portion of it is flipped inside out and attached to the opening made in the abdomen. This allows urine to drain out freely into a diaper. This approach prevents kidney damage that can become life-threatening if left untreated. The option is not permanent and can be closed when the child is older.
Urinary Reconstruction Surgeries
If a child’s prune belly syndrome results in severe urinary deformities, more intensive urinary reconstruction may be required. One example is ureteral reimplantation. This surgery is necessary when a child’s ureter -- the tube that drains urine from the bladder to the urethra -- is blocked or in the wrong position. Ureteral reimplantation surgery involves making a small incision into an area below the belly button to access the ureter. A physician can cut the ureter from its incorrect position and re-implant it to the bladder, attaching it with small stitches that will dissolve over time.
Prognosis for Prune Belly Syndrome
The prognosis for prune belly syndrome is dependent upon how severe the condition affects the child. For example, some babies with prune belly syndrome are stillborn. However, others may experience only a small amount of abdominal muscle laxity, which may not impact development. An estimated 30 percent of prune belly syndrome patients require a kidney transplant as they get older.
While some patients with prune belly syndrome live long, full lives, they require consistent and life-long follow up to ensure the urinary tract is working properly so as to minimize potential kidney damage.
References
Singh J., Storm D., Patel A. & Jayanthi V. Prune Belly Syndrome. Pediatric Urology Book.
