Wunderlich's Syndrome in a Tuberous Sclerosis Patient

Spontaneous non-traumatic renal hemorrhage known as Wunderlich's syndrome is known to occur in renal angiomyolipoma and may be the first manifestation of the disease. Angiomyolipomas in tuberous sclerosis are usually bilateral and multicentric. A 25yr old female, a case of Tuberous sclerosis with bilateral renal angiomyolipomas presented with right loin pain of increasing severity. On evaluation she had acute abdomen with increasing abdominal distension. Investigations revealed large right perinephric hematoma with bilateral renal angiomyolipomas. Exploratory laparotomy and right nephrectomy was done. Spontaneous hemorrhage in renal angiomyolipoma in a case of tuberous sclerosis and management are discussed.