Endovascular treatment of intrarenal aneurysms bleeding and angiomyolipomas in a patient with tuberous sclerosis and polycystic kidney disease

Abstract

Tuberous sclerosis complex (TSC) and autosomal dominant polycystic kidney disease (ADPKD) are conditions related to renal failure that can rarely occur in association as a contiguous gene syndrome. Angiomyolipomas (AMLs) are renal tumors strongly related to TSC that may rupture and cause life-threatening bleedings. We present a patient with TSC, ADPKD, and renal AMLs with persistent hematuria requiring blood transfusion. The persistent hematuria was successfully treated through endovascular embolization, a minimally invasive nephron sparing technique.

mTOR inhibitors are the first-choice therapy for renal angiomyolipomas secondary to tuberous sclerosis

“…We read with great interest the case report “Endovascular treatment of intrarenal aneurysm bleeding and angiomyolipomas in a patient with tuberous sclerosis and polycystic kidney disease” by Leite et al 1 , in which embolization was successfully employed. In cases of bleeding, this preemptive interventional and curative treatment is preferable to partial or total nephrectomy, since it preserves the renal parenchyma and kidney function 2 .…”

“…The case report by Leite et al highlights that less invasive, renal-sparing surgery provides effective therapy for patients with bleeding from renal AML, one of the most feared complications of tuberous sclerosis, which can result in hemorrhagic shock and death 1 , 3 . Additionally, we would like to emphasize that tuberous sclerosis patients need regular medical follow-up over lifetime.…”

Inibidores da mTOR são a primeira escolha no tratamento de angiomiolipomas renais associados à esclerose tuberosa

“…Lemos com grande interesse o relato de caso "Tratamento endovascular de sangramentos e angiomiolipomas de aneurismas intrarrenais em um paciente com esclerose tuberosa e doença renal policística" de Leite et al 1 , os quais reportam o sucesso terapêutico da embolização. Esse tratamento intervencionista preemptivo e curativo, nos casos de hemorragias, é preferencial às nefrectomias, tanto parciais quanto totais, para preservar o parênquima e a função renais 2 .…”

“…O relato de caso de Leite et al sinaliza que intervenções cirúrgicas menos invasivas, que preservam o parênquima renal, podem ser usadas com sucesso no tratamento de uma das complicações mais temidas de angiomiolipomas renais, o sangramento, que pode resultar em choque hemorrágico e óbito 1,3 . Complementarmente, gostaríamos de reforçar a necessidade de um olhar adicional no cuidado dos pacientes com…”

Endovascular Treatment of Renal Angiomyolipoma Rupture - Case Report

“…It has a great relationship with patients diagnosed with tuberous sclerosis, reaching a prevalence of up to 80% in this group. Its treatment takes into account the size of the tumor, the symptoms and the possibility of complications, with its rupture and evolution to hemorrhagic shock being the most serious complication 2,3 .…”