A new endoscopic staging system for hereditary hemorrhagic telangiectasia

Background: Hereditary hemorrhagic telangiectasia (HHT) is predominantly characterized by nasal telangiectases that cause severe epistaxis. Recently, the Epistaxis Severity Score (ESS) was developed and validated as a standardized measurement to evaluate epistaxis treatment efficacy. We propose a new endoscopic staging system to characterize nasal findings in HHT patients and correlate this to the ESS. Methods: This is a prospective cohort study. A total of 33 individuals with HHT confirmed by Curaçao criteria were recruited and evaluated by a single otolaryngologist between August 2010 and February 2013. Endoscopic parameters including patterns and sites of telangiectases and degree of nasal crusting were used to construct an endoscopy score for each subject. Multiple linear regression models were used to correlate this endoscopy score to the ESS. Results: A total of 33 subjects completed the study. The mean ± standard deviation (SD) age was 50.3 ± 13.2 years, and 20 (60.6%) were female. In the cohort, mean ± SD ESS was 4.05 ± 2.13 (range, 0.50 to 8.22). Most subjects (53.1%) had more than 4 nasal sites involved and (56.2%) had punctate telangiectases; 30.3% had mild crusting and 21.2% had moderate/severe crusting. These endoscopic findings were weighted by their correlation coefficients against epistaxis severity and normalized to create a new endoscopy score. After adjusting for confounding variables, the HHT Endoscopy Score (HES) was strongly associated with the ESS (r = 0.79, p < 0.001). Conclusion: The HES correlates highly with patient-reported epistaxis severity and may provide a useful outcome measure in future studies.