Prune belly syndrome (PBS) also known as Eagle Barret syndrome is a rare congenital disorder that is characterized by the complete or partial absence of abdominal muscles, urinary tract malformations and bilateral cryptorchidism.¹ PBS effects 1 in 40,000 births with a mortality of 20% before birth and 30% within the first two years of life.² The mortality is increased secondary to the absence of abdominal muscles contributing to pulmonary hypoplasia.³ Prune belly syndrome contributes to the 22% of leading causes of end stage renal disease being classified with the congenital disorders.¹ This rare disorder is more common in males with 95% of the cases being male and more common in blacks than whites.²
This article will briefly describe the clinical outcome of baby DJ born with prune belly syndrome. The article is not to debate the clinical diagnosis nor the treatments but, to show the prognosis of early diagnosis and renal management.
The patient in the case report was born with end stage renal disease requiring peritoneal dialysis at 8 weeks old. His clinical course consisted of multiple surgeries and revisions until he received a kidney transplantation at 11 months old from a living donor.
Thomas Z. Prune belly syndrome: A Case report. Lynchburg Journal of Medical Science. 2019; 1(3).
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