Prune belly syndrome in a neonate with severe bilateral hydronephrosis: A rare case report from Somalia.

INTRODUCTION AND IMPORTANCE

Prune belly syndrome (PBS), also known as Eagle-Barret syndrome, is a rare congenital disorder characterized by abdominal wall muscle underdevelopment, urinary system abnormalities, and cryptorchidism. This case report presents the clinical features, diagnosis, and management of PBS in a newborn. This is the first case report of prune belly syndrome in Somalia. The condition is estimated to occur in approximately 1 in 30,000 to 1 in 50,000 live births, making it a relatively uncommon presentation. Recognizing and managing this syndrome is crucial, as it can lead to significant morbidity and mortality if not addressed promptly.

CASE PRESENTATION

A term baby delivered without complications developed respiratory distress, jaundice, and urinary retention shortly after birth. Physical examination revealed abdominal distension, fluid in the abdomen, and bilateral undescended testes. Laboratory tests showed elevated bilirubin levels and abnormal blood counts. Ultrasound findings demonstrated bilateral hydroureteronephrosis and underdeveloped abdominal wall muscles. The limited resources and infrastructure in the healthcare setting in Somalia posed challenges in providing comprehensive care for this neonate.

CLINICAL DISCUSSION

PBS is a rare congenital syndrome with a higher prevalence in males. Its exact cause is not fully understood, but genetic factors may play a role. The management of PBS in resource-limited settings can be particularly challenging. The differential diagnosis included sepsis, neonatal jaundice, and posterior urethral valves. The key interventions included supportive care, such as maintaining fluid and electrolyte balance, treating infections, and addressing any urinary tract abnormalities. The limited access to specialized pediatric urology services and advanced diagnostic tools, such as magnetic resonance imaging (MRI), hindered the ability to fully characterize the extent of the urinary tract abnormalities and plan definitive surgical interventions.

CONCLUSION

Despite the constraints of the resource-limited setting, the supportive care and management strategies implemented led to an improvement in the baby's condition. This case highlights the importance of recognizing and managing Prune Belly Syndrome, even in environments with limited healthcare resources. Continued efforts to improve diagnostic capabilities and access to specialized care are crucial for optimizing the outcomes of patients with this rare and complex congenital disorder.