Bilateral nephron sparing surgeries for multiple renal angiomyolipomas in Bourneville's disease

Abstract

Introduction: Angiomyolipomas are the most common renal lesions in tuberous sclerosis complex. When symptomatic, the treatment includes nephron sparing surgery. This is especially challenging when the lesions are bilateral, large and multiple. CASE-HISTORY: A 22-year-old lady with bilateral multiple large symptomatic renal angiomyolipomas associated with other manifestations of tuberous sclerosis was managed with partial nephrectomies on either side. At 30 months of follow-up, the patient has normal renal functions and no evidence of recurrence.

Discussion: Intervention for renal angiomyolipomas is indicated in symptomatic tumors larger than four centimetres. Selective arterial embolisation is the therapeutic modality of choice in such cases. However, in bilateral cases when angioembolisation may not always be feasible, nephron sparing surgery maybe considered. Nephron sparing surgery of both kidneys for angiomyolipomas in association with tuberous sclerosis has only been sparingly reported in literature.