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Prune belly syndrome in Finland - A population-based study on current epidemiology and hospital admissions
Niklas Pakkasjärvi1, Johanna Syvänen2, Asta Tauriainen2
1Department of Pediatric Surgery, Turku University Hospital and University of Turku, Kiinamyllynkatu 4-8, 20521, Turku, Finland; New Children's Hospital, Helsinki University Hospital, Stenbäckinkatu 9, 00290, Helsinki, Finland.
Insights
Prune Belly Syndrome (PBS) affects 1 in 44,000 births in Finland, with a 20% infant mortality rate. This rare condition leads to significant hospitalizations, highlighting a substantial disease burden.
Area of Science:
- Pediatric Medicine
- Medical Epidemiology
- Congenital Malformations
Background:
- Prune Belly Syndrome (PBS) is a rare multisystem disorder characterized by abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism.
- Previous prevalence estimates for PBS range from 1 in 35,000 to 50,000 live births.
Purpose of the Study:
- To determine the epidemiology and early hospital admission patterns of PBS in Finland.
- To investigate potential maternal risk factors associated with PBS through a case-control analysis.
Main Methods:
- A population-based register study linking the Finnish Register of Congenital Malformations with hospital admission data.
- Case-control analysis of maternal risk factors using data from the Birth Register and the Drugs and Pregnancy database.
Main Results:
- 31 PBS cases identified between 1993-2015 (15 live births, 16 terminations), with a total prevalence of 1 in 44,000 births.
- Infant mortality rate was 20% (3 of 15 live births).
- PBS patients experienced significantly higher hospital admissions (3.2/year) and days (10.6/year) compared to the general pediatric population (35- and 27-fold increases).
- Multiple maternal miscarriages were identified as a significant risk factor.
Conclusions:
- PBS presents a considerable disease burden in Finland, marked by high infant mortality and extensive healthcare utilization.
- The study provides contemporary epidemiological data for PBS, emphasizing its significant impact on affected individuals and healthcare systems.
- Limitations include the restricted variables available in register-based studies.
Background:
Prune belly syndrome (PBS) is a multisystem disease characterized by absent or deficient abdominal musculature with accompanying lax skin, urinary tract abnormalities, and cryptorchidism. Previous studies have estimated a birth prevalence of 1 in 35,000-50,000 live births.
Objective:
We set out to clarify the epidemiology and early hospital admissions of PBS in Finland through a population-based register study. Further, possible maternal risk factors for PBS were analyzed in a case-control setting.
Study Design:
The Finnish Register of Congenital Malformations was linked to the Care Register for Health Care, a population-based hospital admission data for PBS patients. Additionally, five matched controls were identified in the Birth Register and maternal risk factors of PBS were studied utilizing data from the Drugs and Pregnancy database.
Results:
We identified 31 cases of PBS during 1993-2015, 15 of which were live born and 16 elective terminations. The total prevalence was 1 in 44,000 births. Three patients (20%) died during infancy. On average, PBS-patients had 3.2 admissions and 10.6 hospital days per year in Finland during the study period years 1998-2015, 35- and 27-fold compared to children in Finland in general. Multiple miscarriages were significantly associated to PBS in maternal risk factor analyses.
Discussion:
The burden of disease is significant in PBS, demonstrated as a high infant mortality rate (20%), multiple hospital admissions, and inpatient care in days. The available variables are limited as a register-based study.
Conclusion:
We present data on contemporary epidemiology in a population-based study and show that the total prevalence of PBS is 1 in 44,000 in Finland. PBS entails a significant disease burden with admissions and hospital days over 35- and 27-fold compared to the general pediatric population, further aggravated by an infant mortality rate of 20%.
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