2. Prune belly syndrome, aka Eagle Barrett syndrome or triad syndrome, is a rare
anomaly comprising a specific constellation of features.
3 major findings:
•gross pelvicalyceal and ureteric dilatation with renal dysplasia
•anterior abdominal wall underdevelopment (resulting in the "prune belly“
•bilateral undescended testes (cryptorchidism) in males
3. Often a/w other respiratory, gastrointestinal, musculoskeletal, and cardiovascular anomalies.
Estimated incidence is at ~1 in 35,000-50,000 live births.
It occurs almost exclusively in males (>95% ) and is seen more frequently in twin pregnancies.
5. Clinical presentation
The ureters are most dilated distally, with the renal pelvis demonstrating
disproportionately little dilatation.
The renal parenchyma is usually well maintained, and renal function is
good.
Stasis makes infections and stones the biggest problems.
6. Urinary tract abnormalities include:
•B/L hydroureteronephrosis: often with extremely dilated, tortuous ureters
•varying degrees of renal dysplasia
•enlarged urinary bladder, often with urachal diverticulum
•VUR is common
•poor bladder contractility
•dilated posterior urethra without urethral obstruction
•calyceal diverticulum
7. Pathology
Unknown cause.
Theory -mesenchymal insult to the fetus at ~6 weeks
gestation -deficient abdominal muscular development.
Secondary to chronic intrauterine abdominal distention
with subsequent pressure atrophy of abdominal muscles.
8. Prune belly syndrome occurs with variable degrees of severity.
In severe cases, renal dysplasia and oligohydramnios in utero result in pulmonary hypoplasia.
Stillborn or die shortly after birth (respiratory complications).
Less severe renal disease -may survive infancy, but recurrent UTI or progressive renal insufficiency.
Some mild cases may have little or no loss of renal function and therefore a better prognosis.
9. Plain radiograph
Bulging abdomen due to lack of abdominal wall muscles .
MCUG
Important examination for urethral assessment.
Risky for patient due to possibility of sepsis development.
2/3 of patients have VUR
10. Ultrasound
Small kidneys with abnormal dilatation of calyces and ureters bilaterally.
Large urinary bladder with thick wall and wide neck.
There can be a patent urachus or urachal diverticulum.
Ultrasound is used as a long term follow up.
11. D/D
For antenatal hydronephrosis with hydroureter, consider:
•posterior urethral valves: may show a key hole sign and no evidence of cryptorchidism
