This child presented with end-stage renal disease due to bilateral dysplasia. The history is notable for multiple urinary tract infections and episodes of pyelonephritis. There was one instance of urosepsis. Pulmonary problems include recurrent bronchitis; GI symptoms include chronic constipation.Challenge: What is the diagnosis?
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ReplyDeleteThis is prune-belly or Eagle-Barrett syndrome, a congenital disorder defined by a triad of abdominal muscle deficiency (giving the characteristic appearance), urinary tract abnormalities, and bilateral cryptorchidism. This syndrome is more common in males than females.
Sources: UpToDate; embryology.med.unsw.edu.au.