A descriptive study of prune belly in New York State, 1983 to 1989.

OBJECTIVE

To determine the prevalence and spectrum of prune belly in a defined population.

DESIGN

Population-based descriptive study using New York State's Congenital Malformations Registry.

SETTING

The Congenital Malformations Registry is a statewide registry of children diagnosed as having congenital anomalies before the age of 2 years.

PATIENTS

Infants with the diagnosis of prune-belly syndrome born during the years 1983 to 1989 to women who were New York State residents and verified by medical record review.

MAIN OUTCOME MEASURES

The live birth prevalence of prune belly for the total population and for population subgroups, such as race, sex, plurality, and maternal age. The occurrence of other malformations with prune belly.

RESULTS

Sixty cases of prune belly were ascertained (50 male and 10 female). The live birth prevalence was 3.2 per 100,000 and declined over the time period. The prevalence was higher in males, 5.1 per 100,000, than females, 1.1; and higher in blacks, 5.8, than whites, 2.6. The live birth prevalence of prune belly in twins, 12.2 per 100,000, was four times higher than that found in singletons, 3.0. More than 36 (60%) of patients died, most in the first week. Forty-two (70%) of patients had one of the commonly described associated defects; pulmonary hypoplasia was the most common. Almost one third of patients had defects other than those typically associated with prune belly.

CONCLUSIONS

Twins, blacks, and children born to younger mothers appear to be at higher risk. Mortality remains high, especially early with many deaths due to pulmonary hypoplasia. Further studies should include stillborns and terminated pregnancies.