prune belly syndrome
Rare congenital disorder characterized by absent abdominal muscles, urinary tract abnormalities, and cryptorchidism.
Full Definition
Prune belly syndrome, also known as Eagle-Barrett syndrome, is a rare congenital condition characterized by the classic triad of deficient or absent abdominal wall musculature, severe urinary tract abnormalities, and bilateral cryptorchidism in males. The wrinkled appearance of the abdominal wall gives the condition its name. Urological abnormalities include massive bladder distension, tortuous dilated ureters, and hydronephrosis. Management requires multidisciplinary care with surgical reconstruction of the urinary tract and consideration of abdominal wall reconstruction.
Usage
Usage note: May also be referenced as Eagle-Barrett syndrome; describe all three components of the triad.
In Context
- "Prenatal ultrasound suggested prune belly syndrome with severe oligohydramnios and bladder distension." — Maternal-fetal medicine consultation
- "Staged urological reconstruction was planned for the newborn with prune belly syndrome." — Neonatal surgical planning