Cricopharyngeal Myotomy as Treatment for Dysphagia in Inclusion Body Myositis and the Patterns of Symptom Recurrence
Hazem Iaali1, Ibrahim Shammas1, Emily Hosfield1, Catherine Shonely1, Elie Naddaf1
1Neurology, Mayo Clinic
Objective:

To evaluate the effectiveness and duration of effect of cricopharyngeal (CP) myotomy in treating dysphagia among patients with inclusion body myositis (IBM).

Background:

Dysphagia is common in IBM and can drastically shape the morbidity and mortality of the disease. Obstruction by CP hypertrophy is one element in the complex etiopathogenesis of IBM dysphagia, alongside oropharyngeal muscle weakness. CP myotomy may provide short-term relief. However, limited literature is available, especially on its long-term effectiveness.

Design/Methods:

We identified patients with IBM who underwent CP myotomy through chart review. We evaluated short-term (within 6 months postoperatively) and long-term outcomes, including patient-reported symptoms, EAT-10 scores, modified barium swallow impairment profile (MBSImP) and penetration/aspiration scores (PAS). Time to progression (TTP) was defined as the first reported worsening of swallowing postoperatively and was analyzed using Kaplan-Meier method.

Results:

67 patients (31 males) were identified as surgical candidates for 70 CP myotomies, due to significant obstruction on their swallow evaluation. The median age at surgery was 72.3 years (IQR 67.8-78.7), and median duration from symptom onset was 6.4 years (3.7-10.8). In the short term, 65/70 (93%) patients reported improvement in their dysphagia, and 28/29 patients with available postoperative MBSImP/PAS scores showed improvement in one or more pharyngeal domains (obstruction, penetration/aspiration, or residue). Postoperative complications were rare. The median EAT-10 score change at first follow-up was 17.33 (12.5-23.7, p<0.001). In the long term, 45/65 (69.2%) reported return of their difficulty swallowing. The median TTP was 3.07 years (95% CI: 2.26,3.96). Only sex was significantly associated with progression, while age at myotomy and disease duration were not. Females had a longer median TTP than males (3.96 vs 1.99 years, p=0.0136).

Conclusions:

Most patients with IBM with significant obstruction experienced improvement after CP myotomy. The effect lasted an average of 3 years and persisted longer in females than in males.

10.1212/WNL.0000000000213085
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