Laryngotracheal Amyloidosis: Amyloid Airway Center 23-Year Experience.

Objectives: Localized laryngotracheal amyloidosis (LA) is a rare disease that can impact phonation and respiration. Treatment options include observation, surgery, and radiation therapy (RT). Given the rare incidence of LA, evidence regarding optimal management and long-term outcomes is limited.

Study Design: Retrospective cross-sectional analysis.

Methods: All patients with LA presenting to an international amyloid center from 1999 to 2022 were analyzed. Patients were categorized by treatment modality: surgery, RT, or observation. Patient and disease factors including demographics, clinical presentation, and progression with need for additional treatment were evaluated.

Results: Seventy-one patients (27M:44F) with LA were treated with surgery (n = 40), RT (n = 11), and observation (n = 20). Gender distribution, age at diagnosis, and systemic workup did not differ significantly between treatment cohorts. A correlation was identified between LA location and treatment modality, with higher rate of subglottic/tracheal amyloid in RT patients vs. surgery and observation patients [(90% and 52% respectively), p < 0.005]. Surgery patients had a median of two surgeries for disease management (range: 1-32) and RT patients had median five surgeries prior to RT (range: 0-17). Six patients required tracheotomy: 3/40 surgery, 3/11 RT and 0/20 in observation cohort. Surgery and RT patients had a longer duration of follow-up (mean 6.7 and 11.7 years) compared with the observation cohort (5.7 years).

Conclusion: Laryngotracheal amyloidosis is a rare disease with variable presentation. Selective surgery of involved subsites is the primary treatment, though multiple surgeries may be needed to optimize function. Observation is appropriate for those with minimal symptoms. For recalcitrant disease, and particularly subglottic/tracheal amyloid, radiotherapy can be beneficial.

Level Of Evidence: 4 Laryngoscope, 134:1606-1613, 2024.