Prune Belly Syndrome: A Ten-Year Single Tertiary Centre Experience in South-South, Nigeria

Abstract

Background: Prune belly syndrome (PBS) is a congenital anomaly that consists of a triad of abdominal wall defect, bilateral cryptorchidism, and urinary tract dilation. The disease is of varying severity. This study aims to highlight the challenges and peculiarities in the management of PBS in a resource-poor setting. Materials and Methods: This is a ten-year retrospective study conducted at the University of Port Harcourt Teaching Hospital. Ethical approval for the study was sought and gotten from the hospital's ethical committee. The information gotten included history, duration of symptoms, examination findings, age of the patient, category of disease, and intraoperative findings. The data from the folders were collected and evaluated. Frequencies, percentages, the mean and standard deviation were used to summarize the data as appropriate. Results: Fifteen patients were included in the study. The hospital incidence of PBS was 112/100,000, twelve males and three females. The age range was from 1 day to 15 years, mean age was 14 months ± 2.3 months. Most patients presented between 3 months and 2 years and 11 months. Twelve patients had category three PBS and five patients had associated anomalies. Eleven male patients died after 5 years of follow-up from progressive renal deterioration. The female patient fared better than the males. Conclusion: PBS is rare, most patients with the condition present late. The most common cause of mortality was progressive renal deterioration.