Long-term outcomes after surgery to prevent aspiration for patients with amyotrophic lateral sclerosis

Abstract

Background Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects motor neurons selectively. In particular, weakness in respiratory and swallowing muscles occasionally causes aspiration pneumonia and choking, which can be lethal. Surgery to prevent aspiration, which separates the trachea and esophagus, can reduce the associated risks. Central-part laryngectomy (CPL) is a relatively minimally invasive surgery to prevent aspiration. No studies have been conducted on the long-term outcomes of surgery to prevent aspiration in patients with ALS. This case series aimed to determine the long-term outcomes of surgery to prevent aspiration and the use of a continuous low-pressure aspirator in patients with ALS by evaluating the frequency of intratracheal sputum suctions performed per day, intra- and postoperative complications, oral intake data, and satisfaction of patients and their primary caregiver to predict improvement in patients’ quality of life (QOL). Methods We report a case series of six patients with ALS who underwent CPL along with tracheostomy to prevent aspiration between January 2015 and November 2018. We evaluated their pre- and postoperative status and administered questionnaires at the time of last admission to the patients and their primary caregivers. Results The mean follow-up period after CPL was 33.5 months. Aerophagia was a common postoperative complication. The use of a continuous low-pressure aspirator resulted in reduced frequency of intratracheal sputum suctions. All cases avoided aspiration pneumonia. Oral intake was continued for 2–4 years after the tracheostomy and CPL. The satisfaction levels of the patient and primary caregiver were high. Conclusion Our case series suggests that the use of a continuous low-pressure aspirator in patients undergoing CPL improves oral intake and reduces the frequency of intratracheal sputum suctions, which improves the QOL of patients with ALS and their families and caregivers. CPL and continuous low-pressure aspiration should be considered as a management option for ALS with significant bulbar and respiratory muscle weakness/dysfunction.

Aspiration prevention surgeries: a review

“…It is less invasive than total laryngectomy and involves the removal of the cricoid cartilage with the glottis, cutting the cricopharyngeal muscle, but preserving the epiglottis, the entire hypopharyngeal mucosa and major vessels and nerves running into the larynx [20]. CPL was proven to be effective in preventing aspiration pneumonia and may improve oral nutrition by reducing the resting upper esophageal sphincter (UES) pressure and prolonging UES relaxation during swallowing [21][22][23][24]. CPL is an effective procedure for patients with diseases such as amyotrophic lateral sclerosis, which is characterized by reduced general muscle strength or pharyngeal contraction during swallowing that makes passage of food boluses from the pharynx to the esophagus difficult.…”

Esophageal Speech for a Patient with Amyotrophic Lateral Sclerosis Who Underwent a Central-part Laryngectomy to Prevent Aspiration: A Case Report

“…Belching is the audible escape of air from the esophagus or stomach into the pharynx, 23) affecting more than 50% of patients who undergo laryngectomy 24,25) including CPL. 26) Removing the whole cricoid cartilage in total laryngectomy or CPL facilitates UES relaxation and decreases UES pressure, leading to an influx of air to the esophagus or stomach (aerophagia). 10) Esophageal dysmotility may further contribute to the retention of air in the esophagus.…”

“…No increase in UES pressure at the initiation of swallowing was detected. These postoperative changes may have resulted in the influx of air during meals 26) to the esophagus and stomach, allowing the patient to use the air as a sound source for esophageal speech without extensive training. Therefore, patients who undergo total laryngectomy or CPL and have esophageal dysmotility may learn esophageal speech with minimal time and effort 27) because injecting air into the esophagus is the first and most challenging step in esophageal speech.…”

The Current State and Future Directions of Swallowing Care in Amyotrophic Lateral Sclerosis

“…Surgery may relieve discomfort and distress associated with aspiration for some individuals. Additionally, surgery may allow individuals to maintain oral intake for an average of 1 to 2 years and reduce the need for sputum suctioning [34]. However, although surgery prevents aspiration, it does not address the underlying mechanisms of swallowing impairment; thus, deficits in swallowing efficiency may persist [31,34].…”

“…Additionally, surgery may allow individuals to maintain oral intake for an average of 1 to 2 years and reduce the need for sputum suctioning [34]. However, although surgery prevents aspiration, it does not address the underlying mechanisms of swallowing impairment; thus, deficits in swallowing efficiency may persist [31,34]. Additionally, due to the redirection of air from the vocal folds, individuals lose the ability to voice and communicate verbally through the glottis.…”