RESUMO
Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with multisystemic involvement, affecting central nervous system, skin, bone system and vessels, with a very heterogeneous clinical presentation. Vascular abnormalities are typically recognized in neurofibromatosis type 1 affecting cardiovascular and cerebrovascular systems. The incidence of circle of Willis anomalies in children with NF1 is twofold higher than in general population. In this paper, we report of 19-years-old female with NF1 and twig-like middle cerebral artery.
Assuntos
Neurofibromatose 1 , Adulto , Criança , Feminino , Humanos , Artéria Cerebral Média/diagnóstico por imagem , Neurofibromatose 1/complicações , Pele , Adulto JovemRESUMO
BACKGROUND AND AIMS: Conventional surgery of the thoracic aorta is frequently associated with significant mortality and morbidity. Here we present treatment of surgical pathologies of the thoracic aorta using a less invasive endovascular approach. METHODS: From January 2000 to May 2004, 51 patients (mean age 62.7 +/- 12.8 years, 36 men) underwent endovascular repair of thoracic aortic lesions in our institution. All patients underwent computed tomography scan at discharge, 6 and 12 months and annually thereafter. Clinical follow-up was carried out by outpatient clinic visit or by telephone contact with the patients or their referring physicians. Mean follow-up was 15.8 months. RESULTS: The 30-day mortality rate was 3.8%, one death in the group of chronic (1.9%) and one in the group of acute lesion (1.9%). The survival rate in the follow-up period was 92.4% at 6 months. Computed tomography angiography confirmed exclusion of the lesion in 25 out of 27 chronic patients, whereas type I and II endoleaks were detected in two patients treated with a secondary procedure. In addition, two patients with an acute type B aortic dissection presented with early endoleaks. The overall rate of complications was 10%. No other endoleaks or deaths were observed at later follow-up. CONCLUSIONS: The early and mid-term results of endovascular stent-grafting for the treatment of chronic and acute thoracic aortic diseases showed a good outcome and low rate of complications. Stent-grafting of the thoracic aorta is still an investigational procedure that needs further validation.