Fact-sheet: Prune Belly syndrome
Updated on 08/04/2017 at 8:36 AM
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Definition
Aplasia or severe hypoplasia of the anterior Abdominal wall muscles.
Distension of the urinary tract.
Malpositioned testis.
Marked male predominance +++.
Prognosis related to renal involvement.
Clinical features
Hypoplasia of the Abdominal wall muscles: "prune-belly" appearance.
Malpositioned testis.
Radiography
Plain abdominal radiograph: distended, flaccid appearance of the abdomen.
Retrograde urethrocystography: assessment of reflux.
Ultrasound
Hypoplasia or aplasia of the anterior Abdominal wall muscles.
Distension of the urinary tract: ureterohydronephrosis, megaureters, megabladder.
NB: role of antenatal ultrasound in the diagnosis of congenital uropathies.
CT
No indication, except possibly in the setting of renal complications.
MRI
No indication except in the setting of renal complications.
Vascular intervention
No indication.
Nuclear medicine
No indication.
Management
Abdominoplasty.
Orchiopexy.
Surgery for urinary tract malformations.
Classification
Classification of ureteral reflux.