Prune Belly Syndrome in a Nigerian infant: a case of inevitable or preventable death?

Abstract

Background: Prune Belly Syndrome is a rare congenital disorder comprising deficient anterior abdominal musculature, undescended testes and urinary tract abnormalities. In Nigeria, the incidence is unknown but majority of the few reported cases were seen in the South west region of the country. Despite its poor prognosis, a coordinated multidisciplinary management may prevent early mortality.
Case Report: We report a 6-week old male infant brought to our health facility with the complaints of abdominal distension and abnormal feet since birth. Physical examination revealed a lax anterior abdominal wall, undescended testes, as well as bilateral talipes equinovarus. Radiologic evaluation showed urinary tract abnormalities such as dilated left ureter and pelvis and right multi-cystic, dysplastic kidney. The patient was treated with the appropriate antibiotic for culture-proven urinary tract infection after which he was referred to the Paediatric Surgeons. Few weeks after his discharge by the Paediatric Surgeons for subsequent follow-up in their clinic, the patient’s sudden demise at home was reported to us.
Conclusion: The sudden mortality of this infant has brought to the fore the gaps in treatment which could have affected his longevity. This report underscores the need for a coordinated multidisciplinary approach in the management of this rare congenital anomaly in order to improve survival outcome in a resource-limited setting.