BACKGROUND:

Wunderlich syndrome, also called spontaneous retroperitoneal hemorrhage, is a very rare clinical entity. Its primary kidney tumor etiology is angiomyolipoma and accounts for 60% of the cases.

CLINICAL CASE:

A 22-year-old woman presented with sudden onset of pain in the right renal fossa. Upon hospital admission, the patient was conscious, lucid, and had mild hypotension. Physical examination revealed signs and symptoms of tuberous sclerosis, a soft, depressible abdomen that was painful upon palpation, a palpable mass in the right renal fossa, and no signs of peritoneal irritation. A tomography scan identified a right well-defined subcapsular hematoma with multiple bilateral angiomyolipomas. Wunderlich syndrome was diagnosed and tuberous sclerosis was confirmed through major criteria. Because the patient was hemodynamically stable she was managed conservatively, with serial hematocrit surveillance, intravenous fluids, and complete bedrest. The patient had satisfactory progression.

CONCLUSION:

Treatment of Wunderlich syndrome should begin with an evaluation of the hemodynamic status of the patient. In case of instability, the indication is urgent surgery. We present herein the first report in the medical literature of an exceptional form of Wunderlich syndrome, concomitant with tuberous sclerosis, that was managed conservatively.