Oregon Sinus Center, Department of Otolaryngology-Head and Neck Surgery, Oregon Health and Science University, Portland, OR 97239, USA.
Int Forum Allergy Rhinol. 2012 Sep-Oct;2(5):422-31. doi: 10.1002/alr.21046. Epub 2012 May 7.
Epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (HHT), affecting approximately 90% of patients at some point during their lifetime. Bleeding is chronic and varies from mild, self-limited episodes to severe, transfusion-dependent or life-threatening epistaxis. Treatment options vary from conservative, nonsurgical management to more aggressive surgical approaches. A number of treatment options have been introduced in recent years. There is little consensus in the literature regarding treatment algorithms. The objective of this investigation was to provide a contemporary review of HHT-related epistaxis, including pathophysiology, disease manifestations, and state-of-the-art treatment modalities.
A systematic review of the literature for HHT-related epistaxis was performed using the search terms "hereditary hemorrhagic telangiectasia" and "epistaxis." Additional literature search regarding current recommendations for HHT evaluation and recent developments in genetic mechanisms, pathophysiology, and treatment of HHT was also performed.
A total of 308 articles were identified and reviewed for appropriateness of inclusion whereas 64 articles met inclusion criteria. Treatment options range from topical and hormonal therapy to more aggressive surgical modalities. Most treatment descriptions are case series, with few randomized controlled trials. A number of new and novel therapies have been introduced in recent years.
HHT is a heterogeneous disease requiring multidisciplinary evaluation and treatment. Therapeutic options for HHT-related epistaxis vary from conservative, nonsurgical measures to more aggressive surgical treatments. A graduated treatment plan is recommended. Patients present with a wide degree in variation of severity of epistaxis, and treatment is best tailored to the individual patient.
鼻出血是遗传性出血性毛细血管扩张症（HHT）最常见的表现，大约 90%的患者在其一生中的某个阶段会出现鼻出血。出血是慢性的，从轻度、自限性发作到严重的、需要输血或危及生命的鼻出血不等。治疗方案从保守的非手术治疗到更积极的手术治疗不等。近年来，已经引入了许多治疗方案。文献中对于治疗方案的选择并没有达成共识。本研究的目的是对 HHT 相关的鼻出血进行现代综述，包括发病机制、疾病表现和最新的治疗方法。
通过使用搜索词“遗传性出血性毛细血管扩张症”和“鼻出血”对 HHT 相关的鼻出血进行了系统的文献回顾。还对 HHT 评估的当前建议以及 HHT 的遗传机制、发病机制和治疗的最新进展进行了额外的文献检索。
共确定了 308 篇文章，并对其纳入的适宜性进行了评估，其中 64 篇文章符合纳入标准。治疗方案从局部和激素治疗到更积极的手术方式不等。大多数治疗描述都是病例系列，随机对照试验较少。近年来已经引入了许多新的和新颖的治疗方法。
HHT 是一种异质性疾病，需要多学科的评估和治疗。HHT 相关鼻出血的治疗方案从保守的非手术措施到更积极的手术治疗不等。建议采用分级治疗方案。患者的鼻出血严重程度差异很大，治疗最好根据个体患者的情况量身定制。
