The Prune Belly Syndrome involves a congenital defect (absence, hypoplasia) of the abdominal musculature together with urogenital disease. The disease can be classified based on the severity of the findings, which correlates with survival.
Gender distribution: almost always male
Urogenital abnormalities:
(1) renal dysplasia
(2) dilation of urinary excretory system (hydronephrosis, hydroureter, hypotonic bladder)
(3) bilateral cryptorchidism
Findings |
Category I |
Category II |
Category III |
renal dysplasia |
bilateral and severe |
unilateral or focal |
none to minimal |
dilatation of the urinary tract |
present |
present |
none to mild |
urine output |
scant and dilute if any |
present but may be reduced |
normal |
pulmonary hypoplasia |
present |
absent |
absent |
oligohydramnios |
present |
absent |
absent |
other anomalies |
patent urachus, complete urethral obstruction, cardiac, GI, orthopedic |
abdominal distention |
may be present but usually mild |
survival |
death during neonatal period or infancy common |
usually survive infancy; may have problems with renal failure and recurrent UTIs |
usually good, may have problems from recurrent UTI |
Specialty: Nephrology, Clinical Laboratory, Pedatrics