Donato Mariana, Pimentel João, Cabral Rui, Escada Pedro
Otolaryngology Department. Hospital de Egas Moniz. Centro Hospitalar Lisboa Ocidental. Lisbon. Portugal.
Departamento de Otorrinolaringologia. Hospital de Egas Moniz. Centro Hospitalar Lisboa Ocidental. Lisboa. Portugal.
Acta Med Port. 2018 Jan 31;31(1):63-66. doi: 10.20344/amp.8802.
Hereditary hemorrhagic telangiectasia is a rare multi-systemic autosomal dominant disorder characterized by dysplasia of the vascular connective tissue and recurrent bleeding tendency. Epistaxis is the most common and earliest symptom. It is usually mild to moderate, however, in some patients it can be severe with significant interference in quality of life. We report a case of a patient with hereditary hemorrhagic telangiectasia, in which an episode of refractory epistaxis was treated with radiofrequency and fibrin sealant. Control of acute bleeding and elimination of telangiectasia was possible with this technique. After six months follow-up, there was no epistaxis recurrence. Various treatments for epistaxis in this disease have been described in the literature, but there is no consensus about the gold-standard procedure. Radiofrequency ablation of telangiectasia is a recent technique that has shown to be safe, effective and well tolerated, even in patients who underwent other previous treatments.
遗传性出血性毛细血管扩张症是一种罕见的多系统常染色体显性疾病，其特征为血管结缔组织发育异常和反复出血倾向。鼻出血是最常见且最早出现的症状。通常为轻度至中度，然而，在一些患者中可能较为严重，对生活质量造成显著干扰。我们报告一例遗传性出血性毛细血管扩张症患者，其中一次难治性鼻出血采用射频和纤维蛋白封闭剂进行治疗。通过该技术能够控制急性出血并消除毛细血管扩张。经过六个月的随访，鼻出血未复发。文献中已描述了针对该疾病鼻出血的各种治疗方法，但对于金标准手术尚无共识。毛细血管扩张症的射频消融是一项最近的技术，已证明即使在先前接受过其他治疗的患者中也是安全、有效且耐受性良好的。
