WUNDERLICH'S SYNDROME IN A PATIENT WITH ADVANCED TUBEROUS SCLEROSIS COMPLEX WITH LARGE ANGIOMYOLIPOMA

Abstract

Wunderlich's syndrome, or spontaneous non-traumatic retroperitoneal hemorrhage, can be a life-threatening event. Renal angiomyolipoma is a rare benign tumor that can occur sporadically, or in association with tuberous sclerosis. A case of spontaneous retroperitoneal hemorrhage in a patient with tuberous sclerosis and renal angiomyolipoma is presented. Tuberous sclerosis complex is a rare symptom complex and may have varied presentations.We present a case of hypovolemic shock due to spontaneous rupture of large angiomyolipoma in a 24-year-old male patient with tuberous sclerosis complex (TSC).The hemodynamic instability of our patient leads to an urgent surgery and right nephrectomy was done for the angiomyolipomas. Spontaneous hemorrhage in renal angiomyolipoma in a case of tuberous sclerosis and management are discussed.