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遗传性出血性毛细血管扩张症鼻血管模式的新分类

New classification of nasal vasculature patterns in hereditary hemorrhagic telangiectasia.

作者信息

Mahoney Elizabeth J, Shapshay Stanley M

机构信息

Department of Otolaryngology, Boston University Medical Center, Boston, Massachusetts, USA.

出版信息

Am J Rhinol. 2006 Jan-Feb;20(1):87-90.

Abstract

BACKGROUND

Hereditary hemorrhagic telangiectasia (HHT) is a disorder characterized by the triad of recurrent epistaxis, telangiectasia, and a family history of the disease. Management of recalcitrant epistaxis in HHT remains a challenging problem for otolaryngologists. The precise coagulation of telangiectasias with the Nd-YAG laser has shown efficacy in the treatment:of HHT-associated epistaxis, but results can be variable and patient selection is critical in ensuring a successful outcome. We propose a new classification of nasal vasculature patterns in HHT as a means for selecting the Nd-YAG laser for photocoagulation treatment.

METHODS

The records of 40 patients who underwent Nd-YAG laser photocoagulation for HHT were reviewed retrospectively. Outcomes after Nd-YAG laser treatment were correlated with three observed nasal vasculature patterns: (I) isolated punctate telangiectasias or individual small arteriovenous malformation; (II) diffuse interconnecting vasculature with "feeder" vessels; and (III) large solitary arteriovenous malformation, which may be associated with scattered telangiectasia.

RESULTS

Types I and II were the most common vasculature patterns seen in this patient population. Patients with patterns I and III showed greater improvement in epistaxis after Nd-YAG laser photocoagulation. Patients with pattern II fared better with septodermoplasty.

CONCLUSION

These findings suggest that analysis of nasal vasculature patterns can improve therapeutic stratification of.patients with HHT. Proper patient selection using this new classification scheme may improve the management of epistaxis in patients with HHT.

摘要

背景

遗传性出血性毛细血管扩张症(HHT)是一种以反复鼻出血、毛细血管扩张和家族病史三联征为特征的疾病。对于耳鼻喉科医生来说,HHT中顽固性鼻出血的治疗仍然是一个具有挑战性的问题。用Nd:YAG激光精确凝固毛细血管扩张症已显示出对HHT相关性鼻出血的治疗效果,但结果可能存在差异,患者选择对于确保成功治疗至关重要。我们提出一种HHT鼻腔血管模式的新分类方法,作为选择Nd:YAG激光进行光凝治疗的一种手段。

方法

回顾性分析40例接受Nd:YAG激光光凝治疗HHT患者的记录。Nd:YAG激光治疗后的结果与观察到的三种鼻腔血管模式相关:(I)孤立的点状毛细血管扩张或单个小动静脉畸形;(II)伴有“供血”血管的弥漫性相互连接的血管;(III)大的孤立动静脉畸形,可能伴有散在的毛细血管扩张。

结果

I型和II型是该患者群体中最常见的血管模式。I型和III型患者在Nd:YAG激光光凝治疗后鼻出血改善更明显。II型患者行鼻中隔成形术效果更好。

结论

这些发现表明,分析鼻腔血管模式可改善HHT患者的治疗分层。使用这种新的分类方案进行适当的患者选择可能会改善HHT患者鼻出血的治疗。

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