Frequency of Prune Belly Syndrome

Sabtu, 22 Januari 2011
Prune belly syndrome affects 1 per 30,000-40,000 live births. Approximately 3-4% of all prune belly syndrome cases occur in females. Twinning is associated with prune belly syndrome; 4% of all cases are products of twin pregnancies.

Prune belly syndrome is associated with trisomy 18 and 21. Patients with prune belly syndrome also have an increased incidence of tetralogy of Fallot (TF) and ventriculoseptal defects.

In 1903, Strumme proposed that prune belly syndrome may be caused by in utero bladder obstruction, stating that dilatation of the urinary tract in utero leads to secondary-pressure atrophy of the abdominal wall and the subsequent clinical findings. More recent theories focus on a functional obstruction due to prostatic hypoplasia that leads to a conformational change in the prostatic urethra during voiding, thereby causing obstruction. The most recent theories suggest a transient obstruction at the junction of the glanular and penile urethra. This would explain the high incidence of megalourethra observed in cases of prune belly syndrome.

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