Prune Belly Syndrome

Case
A 2-day-old boy was admitted. Gestational age was 34 weeks, with no fetal
distress or asphyxia. The birth was by Cesarean section. Apgar scores were 8 at 1
minute and 9 at 5 minutes. Birth weight was 2700. The infant was born to a
19-year-old uncomplicated mother, Gravida 1 Para 1. Prenatal sonography
showed an intra-abdominal cystic collection in the fetus at 28 weeks. There was
no prenatal intervention. After admission, physical examination showed bulging
flanks, especially the right side, thin flabby, wrinkled skin, and bilateral testes
impalpable within the scrota. There were no other issues, and urine output was
normal.
PBS is presents with a triad of syndromes.

The wrinkled, “prune-like” abdominal skin due to an abdominal wall muscle defect
is a characteristic manifestation.

Urinary tract anomalies

Bilateral cryptorchidism.

*A child with this syndrome often can't fully empty his or her bladder due to many
abnormalities. This can cause serious bladder, ureter, and kidney problems.
Diagnosis and Investigations
Diagnosis of prune-belly syndrome is often made during routine prenatal
ultrasonography.

After diagnosis

Blood tests
Intravenous pyelogram
Voiding cystourethrogram
Renal ultrasound
Treatment
Treatment will depend on child’s symptoms, age, and general health and severity.

Refferal to a urologist.

Antibiotics to prevent urinary tract infections.

Some children may need surgery. A vesicostomy is surgery to make a small

opening in the bladder through the belly to empty the bladder of urine.

Other children may need to have surgery to rebuild the belly wall and urinary tract.

In boys, a surgery to advance the testes into the scrotum may be done. This
surgery is called orchiopexy.