Abstract:

Tuberous sclerosis is a multisystem neurocutaneous genetic disease that might be seen in one live birth in 6,000-10,000. Angiomyolipomas (AML) are the most common renal lesions that could be seen in 80% of tuberous sclerosis patients. Nephron-sparing surgery or selective arterial embolization are methods that could be used in the treatment of AML. Here in we present the case who has been admitted to our emergency department with retroperitoneal bleeding due to bilateral giant AML and treated with the endovascular method. A 55-year-old woman, who has been followed regularly in our hospital since 2016 with the diagnosis of tuberous sclerosis. The patient was admitted to the emergency department of our hospital with complaints of right upper quadrant pain and dizziness. On computed tomography, 4 cm of free fluid was observed at the posterior part of the right kidney posterior, consistent with hemorrhage. On renal angiography, selective arterial angioembolization (SAE) was performed on the inferior segmental artery, which is feeding the AML. Surgical intervention was not considered in the foreground because the patient has bilateral giant AML and would not be anephric. It was decided to begin the patient on everolimus (mTOR inhibitor) treatment. In the patient’s first year follow-up, imaging was performed with non-contrast computed tomograpy. Computed tomograpy showed no size change in giant AML in both kidneys. Although the patient’s creatinine levels increased to 3.04 mg/dL and urea to 148 mg/dL during the follow-ups, she did not need hemodialysis. AML, which is seen as a part of tuberous sclerosis, is one of the important causes of mortality in tuberous sclerosis, which causes life-threatening bleeding and requires surgical or endovascular treatments. Nephron-sparing surgery could be difficult in bilateral cases. Therefore, SAE should be considered an important treatment option in emergencies.

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