Abstract
A clinicopathologic study of 20 cases of the purne belly synrome was performed. Ninetten were male infants, and the twentieth is the first female infant reported with both deficient abdominal musculature and severe urinary tract anomalies. Eight patients died (ages 5 hours to 3 months), and postmortem examinations were performed. Of these, five demonstrated anatomic uretheral obstruction and advanced bilateral renal dysplasia. Of 12 living patients (age 2 months to 25 years) only two (age 1 and 21/2 years) have anatomic urethral obstruction. However, each had a patent urachus (not present in those who died), and urinary diversion was performed shortly after birth. From the present series and a review of the literature it appears that the presence of an anatomic urethral obstruction defines a lethal variant of this syndrome.
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