Gross hematuria in a child with tuberous sclerosis complex

Abstract

Tuberous sclerosis is an under diagnosed autosomal dominant neurocutaneous syndrome characterized by the presence of benign congenital tumors in multiple organs expression and a prevalence of 1/6,000-1/12000 newborns. There are varying degrees of renal involvement in tuberous sclerosis complex, which is usually bilateral and asymptomatic. We report a case of Tuberous sclerosis with left sided renal angiomyolipoma and bilateral multiple renal cysts in a 12-year-old boy, who presented with massive hematuria warranting blood transfusion and urosepsis. There was past medical history of repeated seizures. This case highlights the need for a high index of suspicion in a child with un-explained skin lesions, recurrent seizures, mental retardation and symptoms referable to the urinary system.