Massive hematuria due to portal hypertension in a child with bladder augmentation for vesical exstrophy

We report a case of a 17-year-old patient with severe hematuria from an ileocystoplasty-augmented bladder for vesical exstrophy, with associated cirrhosis secondary to autoimmune hepatitis, diagnosed years later. Varix development and further bleeding from ectopic varices, related to cirrhosis and portal hypertension, are a rare cause of hematuria in patients with surgically augmented bladder volume. This child was successfully treated with a transjugular intrahepatic portosystemic shunt procedure as a bridge to hepatic transplantation.