Prune Belly Syndrome

Etiology:
— Leading theory is abnormality in development of mesoderm early in gestation resulting in abundance of fibrous tissue with lack of musculature and this lack of musculature affects abdominal wall and ureters and bladder and urethra to variable degree
— Lack of abdominal musculature may also account for failure of testes to descend
Imaging US Prenatal:
— Protuberant abdomen
— Severe urinary tract dilation
— Variable degree of pulmonary hypoplasia
— Variable degree of oligohydramnios
Imaging Radiograph:
— Bulging flanks
— Variable degree of pulmonary hypoplasia
Imaging US:
— Large bladder which may be thick walled when empty
— Dilated ureters (distal much more than proximal)
— Atrophic or dysplastic kidneys from vesicoureteral reflux
VCUG:
— Capacious urinary bladder that is not hypertrabeculated
— Markedly dilated and tortuous ureters
— Patent urachus may be present
— Megalourethra frequently seen (especially prostatic urethra)
— Vesicoureteral reflux in 85% of cases
— Impaired clearance from ureters
DDX: Posterior urethral valves
Complications:
Treatment:
Clinical:
— Almost exclusively in males
— Classic triad: Abdominal muscle hypoplasia, urinary tract dilation without obstruction presenting as flank masses, bilateral cryptoorchidism

Radiology Cases of Prune Belly Syndrome

US and VCUG of prune belly syndrome — Sagittal US of the right (above left) and left (above right) kidneys shows them to be small in size and echogenic in appearance and to be hydronephrotic. US of the right ureter (below left) showed it to be extremely dilated and to be similar in appearance to the left ureter (not provided). Oblique view of the bladder during the voiding phase of a voiding cystourethrogram (below right) shows a large capacity bladder, a normal appearing urethra, and grade I vesicoureteral reflux.

AXR of peripherally inserted central catheter / PICC in the renal vein in a patient with gastroschisis — AXR AP and lateral shows a right lower extremity PICC coursing across the midline with its tip projecting over the left renal vein. The abdomen appears to be bulging and protruberant in both medial-lateral and anterior-posterior dimensions. The tip of the bladder catheter projects high within the pelvis within a dilated bladder

AXR of prune belly syndrome — AXR AP shows bulging flanks bilaterally and low lung volumes bilaterally.

Clinical Cases of Prune Belly Syndrome

Clinical image of prune belly syndrome / Eagle Barrett syndrome — Clinical image shows a small thorax, a bulging abdomen due to hypoplastic abdominal wall musculature, and bilateral undescended testicles.

Clinical image of prune belly syndrome — Clinical image shows hypoplastic abdominal wall musculature. The bladder drainage catheter (center of image) was used to decompress the patient’s dilated genitourinary system in-utero. The patient also had a small thorax and bilateral undescended testicles.