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Abstract Details

Cricopharyngeal Myotomy as Treatment for Dysphagia in Inclusion Body Myositis and the Patterns of Symptom Recurrence
Neuromuscular and Clinical Neurophysiology (EMG)
S7 - Updates on Muscle Disorders (3:54 PM-4:06 PM)
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To evaluate the effectiveness and duration of effect of cricopharyngeal (CP) myotomy in treating dysphagia among patients with inclusion body myositis (IBM).

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.

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.

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).

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.

Authors/Disclosures
Hazem Iaali, MD
PRESENTER
Dr. Iaali has nothing to disclose.
Ibrahim Shammas, MD Dr. Shammas has nothing to disclose.
Emily A. Hosfield, MS, CCC-SLP, BCS-S Miss Hosfield has nothing to disclose.
Catherine A. Shonley, MA, CCC-SLP Ms. Shonley has nothing to disclose.
Elie Naddaf, MD (Mayo Clinic) Dr. Naddaf has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Expert Connect. Dr. Naddaf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Klick, Inc. Dr. Naddaf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for WebMD. Dr. Naddaf has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson and Johnson. Dr. Naddaf has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arcellx. The institution of Dr. Naddaf has received research support from NIAMS. The institution of Dr. Naddaf has received research support from Fulcrum therapeutics. The institution of Dr. Naddaf has received research support from Abcuro. The institution of Dr. Naddaf has received research support from Cabaletta . The institution of Dr. Naddaf has received research support from Arcellx.