Presentation Description
Institution: ENT department, Royal Prince Alfred Hospital - New South Wales, Australia
Aims
Hereditary Haemorrhagic Telangiectasia (HHT) is a multisystem disorder characterized by mucocutaneous telangiectasias and arteriovenous malformations (AVM) [1]. Epistaxis is common and variable in severity. We report our experience managing epistaxis at a specialized multidisciplinary HHT clinic.
Methodology
A retrospective cohort study was performed identifying patients treated through the HHT clinic at Royal Prince Alfred Hospital (RPA).
Our treatment paradigms included a stepwise escalation starting with medical treatment (topical agents) and good blood pressure control. Ongoing bleeding despite medical management was controlled with coblation ± KTP laser ablation. Severe cases were treated with systemic bevacizumab.
Results
62 patients (39 females) were identified with mean age of 52.5 ± 17.4 years. Epistaxis was the primary complaint in 56 patients (90.3%). The most common genetic mutations were ACVRL1 (43.5%) and ENG (29.0%) with no differences in baseline epistaxis severity between the two groups. ENG patients had more lung AVMs (70.6% v 8.7%, p < 0.001), whereas ACVRL1 patients had more hepatic AVMs (42.9% v 0.0%, p = 0.029).
Complete data were available for 31 patients (21 females). Of those, 14 patients (45%) underwent medical treatment alone, and 13 (42%) required the addition of coblation. Four patients (13%) with severe disease were commenced on Avastin. Patients who were treated with coblation showed a significant reduction in mean ESS of 3.81 ± 0.81 (p < 0.001). Those treated with Avastin also reported a significant reduction of 4.75 ± 1.03 (p = 0.019).
Conclusion
This study highlights the benefits of our treatment paradigm in the management of patients with HHT. The data showed a significant improvement in patients’ self-reported outcomes.
References
1.Faughnan, M.E., et al., Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Ann Intern Med, 2020. 173(12): p. 989-1001.
Speakers
Authors
Authors
Dr Niridu Peiris - , Dr Raymond Hayler - , Kathleen Le Marquand - , A/Prof Edmund Lau - , A/Prof Hubert Low -