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Congenital Abnormalities

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Prune belly sequence » Abdomen Q79.4

Definition:

A complex congenital malformation characterized by deficient abdominal muscle and urinary obstruction/distension. It can be caused by urethral obstruction secondary to posterior urethral valves or urethral atresia. In the affected fetus the deficiency of the abdominal muscle may not be evident. It can be associated with undescended testes, clubfoot, and limb deficiencies.
Notes:
It is frequently asociated with prenatal urinary tract obstuction and other disruptive events constituting the Potter sequence [Q60.6]. Yet its pathogenic cause is exclusively mechanic: distention, reduction, withered aspect, has been questioned in cases where ascitis has been surgically avoided in prenatal period, without been able to avoid Prune Belly syndrome at birth.

Notes:

Image:

Photos 1-6: Three cases with redundant and creased abdominal wall skin. Prune belly

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