Wunderlich’s Syndrome, or Spontaneous Retroperitoneal Hemorrhage, in a Patient with Tuberous Sclerosis and Bilateral Renal Angiomyolipoma

摘要

Objective: Rare disease Background: 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 tuber- ous sclerosis. A case of spontaneous retroperitoneal hemorrhage in a patient with tuberous sclerosis and bi- lateral renal angiomyolipoma is presented.Case Report: A 33-year-old female Caucasian patient, with a known medical history of tuberous sclerosis, was admitted to hospital as an emergency, with right-sided abdominal pain. Abdominal computed tomography (CT) imaging showed bilateral renal tumors, consistent with bilateral renal angiomyolipoma. The larger tumor, involving the enlarged right kidney (24.0 cm in length), had a diameter of 21.0 cm and was associated with hemoperitone- um and retroperitoneal hemorrhage, and contrast ‘blush’ on CT confirmed arterial bleeding. An initial urgent exploratory laparotomy with renal packing was initially performed, but right nephrectomy was required for hemorrhage control. The patient was discharged from hospital on the 23 rd postoperative day, without further complications.Conclusions: This report describes a case of Wunderlich’s Syndrome, or spontaneous retroperitoneal hemorrhage, in a pa- tient with tuberous sclerosis and bilateral renal angiomyolipoma, presenting as an emergency. An early diag- nosis and timely treatment are important in cases of retroperitoneal hemorrhage to prevent life-threatening complications.