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A descriptive study of prune belly in New York State, 1983 to 1989
1New York State Department of Health, School of Public Health.
Insights
The prevalence of prune belly syndrome was 3.2 per 100,000 live births, with higher rates in males, Black infants, and twins. High mortality, particularly from pulmonary hypoplasia, remains a significant concern.
Area of Science:
- Pediatrics
- Medical Genetics
- Epidemiology
Background:
- Prune belly syndrome is a rare congenital anomaly characterized by a triad of abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism.
- Understanding the epidemiological characteristics of prune belly syndrome is crucial for public health initiatives and clinical management.
Purpose of the Study:
- To determine the live birth prevalence and spectrum of prune belly syndrome in a defined population.
- To identify demographic and clinical factors associated with prune belly syndrome.
Main Methods:
- Population-based descriptive study utilizing data from the New York State Congenital Malformations Registry.
- Inclusion criteria: infants diagnosed with prune belly syndrome born between 1983 and 1989 to New York State residents.
- Outcome measures: live birth prevalence by subgroups (race, sex, plurality, maternal age) and co-occurring malformations.
Main Results:
- Sixty cases of prune belly syndrome were identified, with a live birth prevalence of 3.2 per 100,000 births.
- Prevalence was significantly higher in males (5.1/100,000) compared to females (1.1/100,000), Black infants (5.8/100,000) compared to White infants (2.6/100,000), and twins (12.2/100,000) compared to singletons (3.0/100,000).
- Mortality was high (60%), with most deaths occurring within the first week, often due to pulmonary hypoplasia. Pulmonary hypoplasia was the most common associated defect (70%).
Conclusions:
- Twins, Black infants, and infants born to younger mothers appear to be at increased risk for prune belly syndrome.
- High early mortality, primarily due to pulmonary hypoplasia, underscores the severity of this condition.
- Future research should consider including stillborns and terminated pregnancies to provide a more comprehensive understanding of prune belly syndrome's epidemiology.
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.