Prune belly syndrome
ORPHA:2970· ICD-10 Q79.4
Definition
A rare lower urinary tract obstruction (LUTO) characterized by varying degrees of an enlarged urinary bladder, dilated ureters, hydronephrosis, and poorly contractile and disorganized detrusor and ureteral smooth muscle, in association with hypoplastic or absent midline abdominal skeletal musculature, and bilaterally undescended testes in males.
- Prevalence
- Unknown
- Inheritance
- Autosomal dominant, Not applicable, X-linked recessive
- Age of onset
- Antenatal, Neonatal