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prune belly syndrome (Eagle-Barrett syndrome)
Abdominal muscle deficiency syndrome.
Epidemiology:
-> occurs in ~1/40,000 births
Clinical manifestations:
1) deficient abdominal muscles
2) cryptorchidism (undescended testes)
3) urinary tract abnormalities probably from severe urethral obstruction in fetal life
a) ureteral dilatation
b) urethral obstruction
c) vesicoureteral reflux
d) large bladder
4) scoliosis
5) renal dysplasia
6) pulmonary hypoplasia
7) oligohydramnios
General
developmental disorder syndrome (multisystem disorder)
References
Nelson Textbook of Pediatrics. Behrman ed. W.B. Saunders,
14th edition