Novel Simplified Nasal Endoscopy Grading System for Hereditary Hemorrhagic Telangiectasia Patients.

Abstract

A validated and practical grading system for endoscopic findings is crucial for both clinical care and research when evaluating patients with Hereditary Hemorrhagic Telangiectasia (HHT) who experience epistaxis and nasal complaints. This study aimed to validate a novel, simplified nasal endoscopy grading system in HHT patients. The study was conducted at a tertiary referral center for HHT patients. Twenty HHT patients were derived from an earlier double-blinded, placebo-controlled study by our group, investigating the efficacy of topical propranolol gel for treating epistaxis. Patients were examined at three time points, and nasal endoscopies were recorded. The grading system categorized findings into three grades: mild (few punctate lesions), moderate (multiple telangiectasias/large arteriovenous malformations involving the anterior nasal septum), and severe (diffuse involvement of the nasal mucosa with telangiectasias). Three experienced rhinologists, blinded to clinical data, performed the grading. Primary outcomes were the correlation of the grading system with Epistaxis Severity Score (ESS) and Quality of Life (QoL). Inter-rater reliability was assessed using the intraclass correlation coefficient (ICC). 54 nasal endoscopy scores from 20 patients were analyzed. There was a significant correlation between the grading system and ESS (r = 0.8, p = 0.006) and QoL (r = -0.42, p = 0.002). Worse ESS and QoL scores were associated with more severe nasal endoscopy grades. The ICC of 0.8 (p < 0.0001) indicated substantial agreement among the three graders. The novel nasal endoscopy grading system demonstrated a significant correlation with ESS and QoL. Further validation of this novel grading system in larger cohorts is needed.