Edited by: Małgorzata Wierzbicka, Poznan University of Medical Sciences, Poland
Reviewed by: Giovanni Ruoppolo, Sapienza University of Rome, Italy; A. B. Zulkiflee, University Malaya Medical Centre, Malaysia
This article was submitted to Otorhinolaryngology - Head and Neck Surgery, a section of the journal Frontiers in Surgery
†These authors have contributed equally to this work
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Dysphagia, one of the major complications of neuromuscular diseases such as Parkinson's disease and amyotrophic lateral sclerosis (ALS), decreases quality of life and may lead to malnutrition or aspiration pneumonia. Although recent reports have suggested that surgical aspiration prevention improves quality of life and enables oral intake, the selection of appropriate aspiration prevention techniques has rarely been discussed. In this report, we present the cases of three patients with neuromuscular diseases who underwent surgical aspiration prevention; we selected the surgical techniques based on analysis of the dysphagia mechanisms, disease progression, and general condition in each case. Case 1 was a 55-year-old man with multiple system atrophy (MSA) and presented with dysphagia associated with insufficient upper esophageal sphincter (UES) relaxation. We performed central-part laryngectomy, which was able to improve UES relaxation. Case 2 was a 79-year-old man with progressive supranuclear palsy who presented with respiratory disorder and dysphagia. Glottic closure under local anesthesia was selected because he also had acute hepatobiliary dysfunction and methicillin-resistant
Dysphagia is an important complication in patients with neuromuscular disorders, including Parkinson's disease and amyotrophic lateral sclerosis (ALS); it negatively impacts patient quality of life and may lead to fatal outcome due to malnutrition and aspiration pneumonia (
A 55-year-old man developed dysarthria at age 50 years and gait disorder at age 51 years. He was diagnosed with MSA at age 53 years. At the age of 55 years, he was still able to orally intake nourishment and walk with assistance, but dyspnea suddenly appeared after an incident of vomiting. He was transferred to the Neurological Department of our institution by ambulance and developed respiratory disorder due to aspiration pneumonia. Although general treatment under tracheal intubation was performed for 1 week, extubation was considered difficult due to the large amount of aspirated saliva and sputum in the respiratory tract. Hence, otolaryngologists were consulted for the possibility of tracheostomy. The results of the blood tests were as follows: albumin, 3.0 g/dL; C-reactive protein, 3.04 mg/dL; white blood cell count, 13,800/μL; neutrophils, 77.5%; eosinophils, 2.5%; basophils, 0.5%; monocytes, 4.7%; and lymphocytes, 14.8%. Blood gas analysis revealed a pH of 7.45, carbon dioxide partial pressure of 41.1 mm Hg, and oxygen partial pressure of 134 mmHg (FiO2 50%).
Even after the tracheostomy, the patient had difficulty in swallowing saliva, resulting in discharge of copious amounts of sputum from the tracheostomy tube and the suction lumen. Laryngeal fiberoscopy showed bilateral vocal cord abduction impairment (
Clinical findings in case 1.
Reduction of the abnormally high pressure at the UES was considered important for smooth pharyngoesophageal passage in this patient. As cricopharyngeal myotomy appeared to insufficiently contribute to the improvement of UES opening during swallowing due to physical obstruction by the remaining lamina of the cricoid cartilage, modified central-part laryngectomy (
A 79-year-old man developed parkinsonism with bradykinesia at age 74 years and was diagnosed with progressive supranuclear palsy at the age of 78 years. At the age of 79 years, he became bedridden and developed speech disorder, and a gastrostomy was created in place of oral feeding for prevention of recurrent aspiration pneumonia. As the difficulty in sputum expectoration and respiratory disorder progressed, a nasal airway tube was inserted to suction pharyngeal phlegm and maintain the airway from the nose to the larynx. However, the respiratory disorder progressively became severe, and the patient was transferred to our hospital for a tracheostomy.
At the time of admission, acute exacerbation of chronic cholecystitis and pneumonia with pleural effusion occurred, and the following blood test results were obtained: aspartate aminotransferase, 88 IU/L; alanine aminotransferase, 102 IU/L; lactate dehydrogenase, 200 IU/L; γ-glutamyl transpeptidase, 546 IU/L; alkaline phosphatase, 1,453 IU/L; blood urea nitrogen, 12 mg/dL; creatinine, 0.50 mg/dL; sodium, 141 mEq/L; potassium, 4.3 mEq/L; chloride, 100 mEq/L; albumin, 2.7 g/dL; C-reactive protein, 1.52 mg/dL; white blood cell count, 3,060/μL; neutrophils, 50.0%; eosinophils, 1.0%; basophils, 1.0%; monocytes, 4.0%; and lymphocytes, 43.0%. Blood gas analysis revealed a pH of 7.49, carbon dioxide partial pressure of 47.6 mm Hg, and oxygen partial pressure of 77 mmHg (room air). Chest radiography and chest computed tomography revealed bilateral pleural effusions. Methicillin-resistant
Clinical findings in case 2.
The patient was diagnosed with severe oropharyngeal dysphagia related to respiratory impairment, and then aspiration prevention surgery and permanent-tracheostoma creation were proposed to prevent airway obstruction and aspiration pneumonia, which are common causes of progressive supranuclear palsy death (
A 75-year-old man had experienced stumbling while walking and difficulty in arm raising at age 73 years. He was diagnosed with flail arm ALS and developed respiratory disorder at the age of 74 years, for which treatment with non-invasive positive pressure ventilation started at that time. As mechanical ventilatory support was required due to deterioration of respiratory function after pneumonia, the neurologists considered tracheostomy and/or surgical aspiration prevention, which is recommended in the Japanese guidelines if patients wish to intake food orally (
At the first visit at our department, he was wheelchair bound, used non-invasive positive pressure ventilation, and could eat normally with a little difficulty. The blood tests revealed no considerable issues. Blood gas analysis revealed a pH of 7.386, carbon dioxide partial pressure of 50.7 mm Hg, and oxygen partial pressure of 81.8 mm Hg (2 L/min of oxygen). Vital capacity was 2.18 L (%vital capacity, 58.0%) as shown by spirometry tests. VFSS revealed that there was no obvious aspiration during liquid swallowing, except for short duration of velopharyngeal closure and small volumes of pharyngeal residue (
Clinical findings in case 3.
Tracheostomy and simultaneous aspiration prevention surgery were planned because the swallowing function was expected to worsen with the tracheostomy and with ALS progression and the patient strongly desired to be treated with aspiration prevention surgery. Subglottic closure with total cricoidectomy (
We presented the cases of three patients with neuromuscular disorders who underwent aspiration prevention surgery that was individually selected for each patient. To select a proper surgical procedure for aspiration prevention, we preoperatively attempt to perform detailed examinations for swallowing function including VFSS, HRMF (if possible), and physical examinations such as cardiac or respiratory studies. We believe that the appropriate surgical procedures for aspiration prevention should be selected with consideration of the patient's physical status, prognosis, expected time course, and the characteristics of the dysphagia such as impaired UES relaxation during swallowing.
Patients with neuromuscular disorders, such as Parkinson's disease, MSA, and ALS often develop dysphagia as the diseases progress (
Respiratory dysfunction is also caused by neuromuscular disorders, and tracheostomy is needed to manage artificial ventilation. As is well-known, tracheostomy can impair swallowing function (
Recently, aspiration prevention surgery has been considered to be a better option for postoperative feeding and quality of life in patients with irreversible severe dysphagia and impaired articulation or vocal function in comparison with only tracheostomy (
Characteristics of aspiration prevention surgeries.
Total laryngectomy ( |
General > local | 154–615 | 60–550 | Improved | Possible |
Central-part laryngectomy ( |
General | 70–150 | <50 | Improved | Possible |
Laryngotracheal diversion ( |
General or local | 55–228 | 2–193 | Unchanged | Dependent on cases |
Laryngotracheal separation ( |
General or local | 83–210 | 9–184 | Unchanged | Dependent on cases |
Laryngeal closure | |||||
Glottic closure ( |
Local > general | 76–176 | 1–20 | Unchanged | Impossible |
Subglottic closure (SubC) ( |
Local > general | 85–290 | 10–120 | Unchanged | Impossible |
SubC with cricoidectomy ( |
General or local | 125 | Minor | Improved | Possible |
It is often the case that aspiration prevention surgeries deprive the patients of their voice, and substitute speech techniques, such as esophageal voice or artificial larynx, are recommended. However, prosthetic voice restoration after total laryngectomy has recently become possible. As it is considered that a voice prosthesis can be placed not only after total, but also after central-part, laryngectomy, and laryngeal closure with total cricoidectomy (
Dysphagia in neuromuscular disorders such as in Parkinson's disease, MSA, and ALS is caused by various mechanisms. Aspiration prevention for patients with neuromuscular disorders eliminates the possibility of aspiration pneumonia and airway obstruction and can prolong life expectancy. Appropriate choice of the surgical procedure for aspiration prevention under detailed preoperative examination, such as evaluation of UES relaxation during swallowing by HRMF, should be performed with consideration to patient physical status, expected time course, and the characteristics of the dysphagia.
The datasets generated for this study are available on request to the corresponding author.
The studies involving human participants were reviewed and approved by the Human Ethics Committee of the University of Tokyo (No. 2487) and complied with the amended Declaration of Helsinki. Written informed consent was obtained from all patients in this study. The patients/participants provided their written informed consent to participate in this study.
MK was involved in patient care and surgery, collected information, and drafted the manuscript. RU conceived the study, was involved in patient care, surgery, follow-up, and preparation of the imaging, and drafted the manuscript. SS, TS, TG, and AY were involved in patient care and surgery and critically revised the manuscript. TY was involved in patient care and critically revised the manuscript.
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
Abnormal deglutitive proximal esophageal contraction
Amyotrophic lateral sclerosis
High-resolution manofluorography
Multiple system atrophy
Upper esophageal sphincter
Videofluoroscopic swallowing study.