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Background: Epistaxis is the most common symptom in patients with hereditary hemorrhagic telangiectasia (HHT), with the greatest negative impact on quality of life (QoL). Repeated intranasal submucosal bevacizumab injections (RISBI) is a relatively new treatment option for moderate or severe grades of epistaxis in HHT. However, the effect of RISBI on QoL is not fully evaluated., Study Design: Prospective, non-comparative study., Materials and Methods: Patients treated by RISBI for HHT-associated epistaxis between June 2011 and August 2013 were prospectively invited to the present study. The end of follow-up was October 2013. The patients were requested to answer QoL questionnaires before the first treatment, and 6-8 weeks after the last treatment. Three levels of QoL were assessed: Overall QoL using Cantril's Self-Anchoring Ladder; Health-related QoL using Short Form 36 (SF-36), and Disease-specific QoL. Psychological distress was measured with the Hospital Anxiety and Depression scale (HADS)., Results: Thirty-three patients were treated with RISBI during the period referred to above. Twenty-three patients completed the QoL questionnaires. The average number of treatments per patient was 2.15 ± 1.3 (Range: 1-5). The mean overall QoL improved from 6.47 ± 1.9 to 7.26 ± 1.6 (P < .05). Several dimensions measured by SF-36 were significantly improved with a medium to strong effect size. HADS demonstrated a significant decrease in psychological distress after the last treatment., Conclusion: HHT patients treated by RISBI improved in several aspects of quality of life, and psychological distress decreased. RISBI was an effective treatment option for moderate and severe grades of HHT-associated epistaxis., Level of Evidence: 4 (case series). Laryngoscope, 130:E284-E288, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives/hypothesis: Long-term follow-up of intranasal bevacizumab therapy in hereditary hemorrhagic telangiectasia (HHT)., Study Design: Prospective, noncomparative study., Methods: Patients treated for HHT-associated epistaxis by intranasal submucosal bevacizumab injections between June 2011 and August 2013 were included and followed prospectively. The effectiveness of the treatment was evaluated by the epistaxis severity score (ESS); the epistaxis intensity, frequency, and the need of blood transfusion (IFT) score; and hemoglobin levels., Results: Thirty-three patients were included. The total number of treatments with intranasal bevacizumab injection was 210. The mean number of treatments per patient was 6.2 ± 4.6 (range, 1-16), and the mean treatment and observation period was 38.8 ± 21.8 months (range, 2-66 months). Four patients showed no improvement after treatment. Eleven patients (33.3%) showed initial improvement in both ESS and IFT, but the treatment was discontinued before the end of the study because the effect became gradually shorter lasting despite repeated injections. Twelve patients (36.3%) continued to have a positive response to the treatment at the end of the study. No local adverse effects were observed, but one patient developed osteonecrosis in both knees during the treatment period., Conclusions: Intranasal bevacizumab injection is an effective treatment for most of the moderate and severe grades of HHT-associated epistaxis. The duration of the effect of the treatment was variable. Primary and late resistance phenomena to the treatment were quite common., Level of Evidence: 4. Laryngoscope, 128:2237-2244, 2018., (© 2018 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective/Hypothesis: To evaluate the effectiveness of a standardized intranasal bevacizumab injection in treating hereditary hemorrhagic telangiectasia (HHT)-associated epistaxis. Study Design: Prospective pilot study. Methods: A total dose of 100 mg bevacizumab (25 mg/mL Avastin) was injected submucosally, 50 mg on each side. A total of 0.5 mL was injected in the sphenopalatine area, upper part of bony septum, upper part of the later nasal wall, and the anterior part of nasal floor. No cauterizations or laser therapy were done during or after the procedure. The hemoglobin level and grades of epistaxis were recorded before and monthly after the procedure. The IFT grading system (intensity [I], frequency [F] of epistaxis, and the amount of blood transfusion [T]) and epistaxis severity score (ESS) for hereditary hemorrhagic telangiectasia system were used. Quality of life (QoL) was evaluated before and 4 weeks after the procedure using the Short Form-36 Health Survey questionnaire, Cantril's Self-Anchoring Ladder questionnaire, and Slotosch disease-specific QoL questionnaire. Results: A significant improvement was found in IFT grading ( P = .007), ESS grading ( P = .001), and hemoglobin level ( P = .01). The QoL differences were statistically not significant. Conclusions: The four-injection site technique of intranasal administration of bevacizumab is an effective treatment option in HHT-associated epistaxis, at least on the short-term effect. Long-term and comparative studies are needed to further evaluate the significance of this treatment modality. Laryngoscope, 2012 [ABSTRACT FROM AUTHOR]