内镜血管分级指导遗传性出血性毛细血管扩张的分步治疗。

IF 1.7 4区医学 Q2 OTORHINOLARYNGOLOGY

Laryngoscope Investigative Otolaryngology Pub Date : 2025-10-07 DOI:10.1002/lio2.70272

Hong-Gang Duan, Fang Ji, Hui Yuan, Lin-Rong Li, Ke-Jia Yu, Zhen-Yuan Wei

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引用次数: 0

摘要

目的：建立预测遗传性出血性毛细血管扩张（HHT）严重程度的鼻内镜血管分级系统，指导分步治疗。方法：回顾性研究来自22个家庭的32例HHT患者，通过1080p内窥镜将其分为3个级别：I级：稀疏点状毛细血管扩张；II级：密集汇合血管；III级：突出性血管瘤。分析了人口统计学、鼻出血严重程度评分（ESS）、血红蛋白水平（Hb）、全身血管病变和ENG/ACVRL1突变。各年级比较治疗结果。结果：ⅰ级占18.8%，ⅱ级占34.4%，ⅲ级占46.9%。III级患者ESS最高（6.31±2.13 vs. 1.21±0.92），p p p p结论：本研究初步证实鼻内镜下血管形态与HHT鼻出血严重程度有显著相关性，可指导疾病评估和治疗方案。证据等级：3。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

Endoscopic Vascular Grading Directs Stepwise Therapy in Hereditary Hemorrhagic Telangiectasia

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Endoscopic Vascular Grading Directs Stepwise Therapy in Hereditary Hemorrhagic Telangiectasia

Objective

To establish a nasal endoscopic vascular grading system for predicting epistaxis severity and guiding stepwise therapy in hereditary hemorrhagic telangiectasia (HHT).

Methods

A retrospective cohort of 32 HHT patients from 22 families was classified via 1080p endoscopy into three grades: Grade I: Sparse punctate telangiectases; Grade II: Dense confluent vessels; Grade III: Protruding hemangiomas. Demographics, Epistaxis Severity Score (ESS), hemoglobin levels (Hb), systemic vascular lesions, and ENG/ACVRL1 mutations were analyzed. Treatment outcomes were compared across grades.

Results

Grade I accounted for 18.8%, Grade II for 34.4%, and Grade III for 46.9%. Grade III patients showed the highest ESS (6.31 ± 2.13 vs. 1.21 ± 0.92 for Grade I, p < 0.01) and the lowest Hb (97.2 ± 22.4 vs. 129.5 ± 4.9 g/L for Grade I, p < 0.05), and had more systemic lesions (80.0% of Grade III patients had hand/oral/visceral hemangiomas vs. 16.7% in Grade I, p < 0.01). Local therapies or tranexamic acid treatment sufficed for Grade I/II patients, who maintained stable ESS. Among Grade III patients who showed no improvement after treatment with tranexamic acid, bevacizumab (administered at a dose of 5 mg/kg every 3–6 months) improved the ESS and increased the Hb in 62.5% of the patients (5 out of 8), and no severe adverse events occurred.

Conclusion

This study preliminarily confirms a significant correlation between nasal endoscopic vascular morphology and HHT epistaxis severity, guiding disease assessment and treatment planning.