Prune Belly Syndrome Associated with Congenital Right Bronchogenic Cyst in a Term Female Neonate in Calabar, Cross Rivers State, Southern Nigeria: A Case Report

Background: Prune Belly Syndrome (PBS), commonly found in males, is characterised by abdominal muscle aplasia/hypoplasia, urological abnormalities, and bilateral cryptorchidism. Pulmonary hypoplasia is the predominant pulmonary abnormality in PBS. Because of its rarity and loco-regional lack of data, we report a case of a term female neonate with prune belly syndrome and bronchogenic cysts. Case presentation: A three-day-old female neonate of the Efik ethnic group in Nigeria presented with respiratory distress noticed 12 hours after birth. The child was noted to have a prune-like abdomen and diagnosed with PBS (category 3) and Bronchogenic cyst, making it an interesting clinical scenario. Unfortunately, the baby passed away on the 6th day of life due to respiratory compromise, 16 hours after undergoing cyst excision surgery. Conclusion: Although pulmonary hypoplasia is the most commonly documented pulmonary abnormality (58%), limited data on pulmonary abnormalities in PBS hampers evidence-based documentation. Adopting a multidisciplinary approach in the management of patients with PBS is essential. Genetic counselling and family support are crucial in cases where there is no family history of genetic or congenital anomalies, such as the index case. Early diagnosis and close monitoring for potential complications are recommended for better outcomes.