Nasal Septal Perforation and Hereditary Hemorrhagic Telangiectasia: Evolution in Management

To review septal perforation management and outcomes in patients with hereditary hemorrhagic telangiectasia (HHT).

Collection and presentation of patient demographic, perforation size, and prior perforation and HHT treatment data over a 20-year period. Symptom and quality of life treatment outcomes were determined using the Nasal Obstruction Symptom Evaluation (NOSE)-Perf scale and the 5-Factor Glasgow Inventory (GBI-5F), respectively, in patients treated since 2017.

Nine patients met study criteria. Mean (range) age was 64.4 (52-80) years and seven were biological males. Mean (range) perforation length and height were 1.9 (1.2-2.6) and 1.4 (1.0-1.8) cm. Four patients were treated with a customized septal button, one with posterior septal resection, and four with endonasal bilateral mucosal flap repair supported with an interposition graft. The button prostheses were well tolerated and the surgical closures successful at 8-60 months postoperatively. Subjective responses in the first three patients, and NOSE-Perf with GBI-5F scores in the latter six patients, demonstrated symptom and quality of life improvement for all patients. Perforation repair patients demonstrated substantial improvement in mean GBI-5F subdomain scores for quality of life, self-confidence, and social involvement and all have undergone sodium tetradecyl sclerotherapy injection postoperatively without re-perforation.

Symptom and quality of life improvement can be achieved in HHT patients with a perforation using customized septal buttons, posterior septal resection, or bilateral flap surgical repair. Surgical closure combined with injection sclerotherapy is feasible with a low risk of re-perforation and represents an effective treatment strategy in selected patients.

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