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Medical Science Monitor Basic Research


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

Challenging differential diagnosis, Management of emergency care, Rare disease

Sara Catarino Santos, Liliana Duarte, Fernando Valério, Júlio Constantino, Jorge Pereira, Carlos Casimiro

Portugal Department of General Surgery, Centro Hospitalar Tondela-Viseu, Viseu, Portugal

Am J Case Rep 2017; 18:1309-1314

DOI: 10.12659/AJCR.905975

Available online: 2017-12-08

Published: 2017-12-08


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 tuberous sclerosis. A case of spontaneous retroperitoneal hemorrhage in a patient with tuberous sclerosis and bilateral 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 hemoperitoneum 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 23rd postoperative day, without further complications.
CONCLUSIONS: This report describes a case of Wunderlich’s Syndrome, or spontaneous retroperitoneal hemorrhage, in a patient with tuberous sclerosis and bilateral renal angiomyolipoma, presenting as an emergency. An early diagnosis and timely treatment are important in cases of retroperitoneal hemorrhage to prevent life-threatening complications.

Keywords: Angiomyolipoma, Hemoperitoneum, Nephrectomy, Tuberous Sclerosis