prune belly syndrome (Eagle-Barrett syndrome)

Introduction

Abdominal muscle deficiency syndrome.

Epidemiology

• occurs in ~1/40,000 births

Clinical manifestations

• deficient abdominal muscles
• cryptorchidism (undescended testes)
• urinary tract abnormalities probably from severe urethral obstruction in fetal life
  • ureteral dilatation
  • urethral obstruction
  • vesicoureteral reflux
  • large bladder
• scoliosis
• renal dysplasia
• pulmonary hypoplasia
• oligohydramnios

More general terms

• developmental disorder syndrome (multisystem disorder)