Microsurgical Treatment of ADPKD Patients with Intracranial Aneurysms: clinical experience of a tertiary vascular neurosurgery center

Introdução: A doença renal policística autossômica dominante (DRPAD) é uma das doenças genéticas mais comuns com uma taxa de prevalência de aproximadamente 1 em 400 a 1 em 1000 indivíduos. Os aneurismas intracranianos consistem em uma das anomalias vasculares mais frequentes na DRPAD e ainda uma importante causa de morbidade e mortalidade nesses pacientes. Métodos: Foi realizada uma investigação observacional retrospectiva em dois centros terciários de neurocirurgia vascular de referência brasileira, de janeiro de 2015 a março de 2018. Resultados: Dezesseis pacientes foram operados, 13 deles eletivamente e 3 de urgência, e submetidos ao tratamento de vinte aneurismas cerebrais, sendo que doze eram do sexo feminino (75%) e média de idade de 58,3 anos (47-73 anos). A maioria da localização dos aneurismas foi a circulação anterior, com 40% na artéria cerebral média (ACM) e 35% na artéria comunicante anterior (ACoA). Quinze pacientes (93,7%) foram submetidos à clipagem cirúrgica isolada e um paciente foi submetido à clipagem cirúrgica mais tratamento endovascular. No grupo eletivo, 30% dos pacientes tiveram complicações hemorrágicas pós-operatórias e 2 pacientes morreram, enquanto no outro grupo 2 pacientes tiveram boa evolução e um faleceu. Conclusão: Os aneurismas intracranianos são muito comuns em pacientes com DRPAD e uma importante causa de morbidade e mortalidade para eles, principalmente nos casos de hemorragia subaracnoidea. O tratamento desses pacientes quando bem planejado é seguro e essencial para manter sua qualidade de vida e evitar a temida ruptura do aneurisma e as complicações da hemorragia subaracnoidea.

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common genetic disorders with a prevalence rate of approximately 1 in 400 to 1 in 1000 individuals. Intracranial aneurysms are one of the most frequent vascular anomalies in ADPKD and still an important cause of morbidity and mortality in these patients. Methods: A retrospective observational investigation was carried out in two Brazilian tertiary referral vascular neurosurgery center, from January 2015 to March 2018. Results: Sixteen patients, 13 of them electively and 3 as emergency, and underwent treatment for twenty brain aneurysms were operated on. Twelve of them were female (75%) and the mean age was 58.3 years (47-73 years). The majority of aneurysms location was the anterior circulation, with 40% in the middle cerebral artery (MCA) and 35% in anterior communicating artery (ACoA). Fifteen patients (93.7%) underwent surgical clipping alone and one patient undergone surgical clipping plus endovascular treatment. In the elective group, 30% of the patients had postoperative hemorrhagic complications and 2 patients died, while in the other group, 2 patients had a good outcome and one died. Conclusion: Intracranial aneurysms are very common in patients with ADPKD and an important cause of morbidity and mortality for them, especially in cases with subarachnoid hemorrhage. If well-planned these patients treatment is safe and essential to maintain their quality of life and avoid the dreaded rupture of the aneurysm and the complications of subarachnoid hemorrhage.

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