Hyperammonemic encephalopathy: a complication associated with the prune belly syndrome

Hyperammonemic encephalopathy in the urological patient is a rare but grave condition. In the pediatric urological population it has been associated with massively dilated upper tracts and urinary infection with urea-splitting organisms. We report 2 cases of hyperammonemic encephalopathy in association with the prune belly syndrome. Both patients presented comatose with markedly elevated serum ammonia levels and Proteus mirabilis urinary tract infection. Intravenous antibiotics and catheter drainage resulted in dramatic reversal of the encephalopathy. The pathophysiology of hyperammonemic encephalopathy in association with the prune belly syndrome and a review of the literature are presented.