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Oculopharyngeal muscular dystrophy (OPMD) is a rare autosomal dominant, progressive degenerative muscle disorder featuring dysphagia with limited therapeutic options. The aim of this study was to evaluate the safety and efficacy of repeated endoscopic dilatation for OPMD over a 15-year period. All patients seen at our Regional Swallowing Clinic with OPMD confirmed by genetic analysis were included. Cricopharyngeal dilatation was performed as an outpatient procedure using a wire-guided 18-mm (54 Fr) Savary-Gilliard bougie with the patient under sedation. Patients were offered repeat endoscopic dilatation when symptoms recurred. Symptom severity prior to initial dilatation and at follow-up was evaluated using the Sydney Swallow Questionnaire (SSQ). Nine patients (7 female, 2 male) were included for analysis. Median total treatment period was 13 years (range = 3-15), median number of dilatations per patient was 7.2 (range = 1-16), and median interval between treatments was 15 months (range = 4.5-45). All patients recorded sustained symptom improvement. Mean SSQ score (out of 1,700) was 1,108.11 (SD ± 272.85) prior to first dilatation and 297.78 (SD ± 189.14) at last follow-up, representing a 73% decrease (95% CI = 52-94) in degree of dysphagia symptoms (paired t-test, P = 0.0001). All mean scores for individual questions also showed significant improvement (P < 0.05). No adverse events were reported with all patients maintaining oral feeding at last follow-up. Repeated cricopharyngeal dilatation is a safe, effective, well-tolerated, and long-lasting treatment for dysphagia in OPMD.

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Objective: Oncogenic human papillomavirus (HPV) has been established as a causative agent for 25% of head and neck squamous cell cancer (HNSCC), including laryngeal squamous cell cancer (LSCC). HPV-positive oropharyngeal patients have improved prognosis. We determined outcomes in LSCC in the context of HPV infection.Method: Seventy-nine primary LSCC were examined for 21 risk factors. HPV status was determined using real-time quantitative PCR. Chi-square/Fisher exact test were used to test association of HPV-positive with the other risk factors. Kaplan-Meier and log rank test were used to study the risk of HPV-positive status for overall survival.Results: HPV-16 was detected in 21 out of 77 (27%) primary LSCC, 16% (5/32) African American (AA) and 33% (15/45) Caucasian American (CA). Significance between race and HPV status was marginal (P = .058). HPV-positive status was significantly associated with male gender (P = .017) and insurance type (P = .001). HPV-positive LSCC had longer median survival (6.64 years) when compared with HPV-negative (6.25 years), but was not significant. Conclusion:We found high prevalence of HPV in males vs females and lower prevalence of HPV infection in AA vs CA. Larger multi-ethnic LSCC cohorts are needed to more clearly delineate HPV-related survival outcomes across ethnicities. Laryngology/Broncho-Esophagology long-Term RepeatedCricopharyngeal Dilatation for OPMD Joseph G. Manjaly, MD (presenter); Jonathan C. Corlett; Oliver Dale; Peter G. Vaughan-Shaw; Roger FrostObjective: Oculopharyngeal muscular dystrophy (OPMD) is a rare, autosomal dominant, progressive degenerative muscle disorder featuring dysphagia with limited therapeutic options. Cricopharyngeal myotomy improves symptoms, yet dysphagia frequently recurs, failing to prevent aspiration and enteral feeding. The objective is to evaluate safety and efficacy of repeated endoscopic dilatation for OPMD over a 15-year period.Method: All patients at our unit with genetically confirmed OPMD were included. Cricopharyngeal dilatation was performed with a wire-guided 18 mm Savary-Gilliard bougie. Repeat dilatation was offered when symptoms recurred. Symptom severity prior to initial dilatation and at follow-up was evaluated using the Sydney Swallow Questionnaire (SSQ).Results: Nine patients (7 female, 2 male) were included. Median total treatment period was 13 years (range, 3-15 years), median number of dilatations per patient was 7.2 (1-16), and median interval between treatments was 15 months (range, 4.5-45 months). All patients recorded sustained symptom improvement. Mean SSQ score was 1108 out of 1700 (SD ± 272.9) prior to first dilatation and 298 out of 1700 (SD ± 189.1) at last follow-up, representing a 73% decrease (95% CI 52-94) in degree of dysphagia symptoms (Paired t test, P = .0001). All mean scores for individual questions also showed significant improvement (P < .05). No adverse events were reported with all patients maintaining oral feeding at last follow-up. Conclusion:Repeated cricopharyngeal dilatation is a safe, effe...