Stepwise Surgical Management of Severe Dysphagia in Multiple System Atrophy: A Case Report

Stepwise Surgical Management of Severe Dysphagia in Multiple System Atrophy: A Case Report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Stepwise Surgical Management of Severe Dysphagia in Multiple System Atrophy: A Case Report Yumiko Ogawa, Rumi Ueha, Takao Goto, Miguel Limbert Ramos, Sakutarou Koike, and 2 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8426946/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Introduction: Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disorder in which dysphagia emerges early and worsens over time. Although swallowing improvement procedures such as laryngeal suspension or cricopharyngeal myotomy can temporarily restore oral intake, surgical strategies for severe dysphagia in patients with prior swallowing surgery and reduced physiological reserve remain poorly described. Case Report: A 70-year-old man with MSA had previously undergone laryngeal suspension and tracheostomy, allowing continued oral intake. Over the following months, he experienced progressive deterioration, including dysphonia, copious secretions requiring frequent suctioning, and recurrent aspiration pneumonia and transient ventilatory dependence. Clinical assessment revealed diminished laryngeal sensation, severe saliva pooling, silent aspiration on laryngoscopy and videofluoroscopy, and impaired upper esophageal sphincter (UES) relaxation on high-resolution manometry. Given his frailty, extended spectrum beta-lactamase colonization, and a desire to resume limited oral intake, a minimally invasive yet effective approach was required. Subglottic laryngeal closure with bilateral cricopharyngeal myotomy was performed through a small operative field, achieving complete aspiration prevention and improved UES opening. Conclusion This case highlights the importance of individualized, stage-specific surgical planning for advanced MSA-related dysphagia. Careful integration of physiological findings, systemic tolerance, and patient-centered goals can optimize outcomes, even in patients with prior swallowing surgery. Multiple system atrophy dysphagia aspiration prevention surgery subglottic closure cricopharyngeal myotomy Figures Figure 1 Figure 2 Figure 3 Figure 4 Introduction Severe dysphagia in neuromuscular disorders may lead to life-threatening aspiration, and enteral feeding or tracheostomy is often required as the disease progresses [ 1 ]. While surgery was traditionally approached cautiously, recent evidence indicates that surgical treatment for dysphagia may be considered to improve quality of life and reduce aspiration risk [ 2 ]. Multiple system atrophy (MSA) is a progressive neurodegenerative disorder in which dysphagia commonly develops early and often becomes refractory as the disease advances. In Japan, aspiration prevention surgery (APS) is listed among the therapeutic options for severe dysphagia in advanced MSA according to current clinical practical guidelines [ 3 ]. Although swallowing improvement surgeries such as laryngeal suspension may temporarily restore oral intake in selected patients [ 4 ], severe dysphagia may recur with disease progression, and there is limited guidance on subsequent surgical management. In this report, we describe a patient with progressive MSA who had previously undergone laryngeal suspension and later developed advanced severe dysphagia, requiring reconsideration of surgical intervention. We discuss the clinical decision-making process and the rationale for selecting an individualized, stage-specific surgical strategy in advanced neurodegenerative dysphagia. Case Presentation A 70-year-old male with a 9-year history of MSA-cerebellar type (MSA-C) had experienced recurrent aspiration pneumonia due to progressive severe dysphagia, along with urinary tract infections. As previously reported [ 4 ], comprehensive swallowing assessments had led to swallowing improvement surgery consisting of laryngeal suspension and tracheostomy, enabling continued oral intake while preserving phonation. However, five months later, the patient demonstrated further disease progression, developing marked dysphonia, increased tracheal secretions that required frequent suctioning, and declining oral intake. At six months postoperatively, he developed aspiration pneumonia. Despite the cessation of oral intake, the patient continued to experience recurrent episodes of aspiration pneumonia, eventually necessitating ventilatory support. Following antibiotic therapy and careful systemic management, the patient was successfully weaned from mechanical ventilation. However, respiratory cultures revealed persistent colonization with extended-spectrum β-lactamase (ESBL)-producing Escherichia coli and Klebsiella pneumoniae. Given the patient’s severely compromised condition and continued risk of life-threatening aspiration, the attending neurologist requested reassessment of swallowing function to explore potential surgical options. Clinical and instrumental evaluation On physical examination, the patient was unable to speak due to severe dysarthria and respiratory difficulty, even with a speech cannula. Although tongue movement was impaired, protrusion and retraction were partially preserved. The thyroid cartilage remained elevated from the prior laryngeal suspension with no signs of local infection. He was nearly bedridden but could briefly tolerate sitting in a wheelchair at 60° recline. Although his extremities were rigid and immobile, simple communication was achieved through lip movements and eye blinking. Laryngoscopy revealed intact velopharyngeal closure, involuntary bilateral vocal fold movement, and markedly diminished laryngeal sensation. The hypopharyngeal space appeared widened secondary to the previous laryngeal suspension, but copious saliva pooling and partial tracheal penetration were observed (Fig. 1 A). Videofluoroscopic swallowing study (VFSS) using 3 mL of thickened contrast agent (100 mPa·s) demonstrated weak pharyngeal contraction, poor upper esophageal sphincter (UES) opening, and silent aspiration of the contrast agent into the airway. Even with a large volume of aspirate, no effective cough reflex was elicited (Fig. 1 B). High-resolution manometry (HRM) showed elevated resting UES pressure and markedly impaired UES opening during swallowing (Fig. 1 C), findings that differed from those observed after the previous laryngeal suspension described in our previous report 4) . Based on these findings, the disease progression in MSA was considered to have further impaired both sensory and motor components of swallowing, and in combination with reduced respiratory and physical function, may have limited the patient’s ability to clear aspirated material. Progressive neurodegeneration was presumed to cause spasticity-like dysfunction of the cricopharyngeal muscle, severely compromising UES relaxation. Although balloon dilation or cricopharyngeal myotomy could be considered for impaired UES opening, these interventions alone were unlikely to sufficiently reduce aspiration risk given his overall condition. Because of the severity of respiratory dysfunction, aspiration prevention was regarded as the most appropriate strategy. After thorough discussion, the patient’s family clearly preferred APS, and the patient strongly wished to resume oral intake, even in small amounts. Individualized surgical planning process In this patient with severe dysphagia due to MSA, colonization with ESBL–producing organisms, poor systemic condition, and a strong desire to resume oral intake necessitated careful selection of an appropriate APS. Potential procedures include (1) total or partial laryngectomy, (2) laryngeal closure, and (3) laryngotracheal separation or tracheoesophageal diversion (Table 1 ). Given the patient’s deteriorated systemic status, a minimally invasive approach with short operative time was preferred. Because the thyroid cartilage had been fixed to the mandible during prior laryngeal suspension, any procedure requiring re-exposure of the thyroid cartilage, such as laryngectomy or glottic closure, was considered unsuitable, particularly given the infection risk associated with ESBL colonization. Tracheoesophageal anastomosis was also excluded due to the risk of anastomotic complications. Table 1 Aspiration prevention surgery options. ≒ 2 hours: around 2 hours; UES: upper esophageal sphincter; CPM: cricopharyngeal myotomy. Aspiration prevention surgeries Operative time Amount of bleeding Exposure of thyroid cartilage UES opening effect Surgeries to remove the larynx Total laryngectomy > 2 hours large + + Central-part laryngectomy ≒ 2 hours small + + Surgeries to close the larynx Supraglottic laryngeal closure ≒ 2 hours small + - Glottic laryngeal closure ≒ 2 hours small + with CPM Subglottic laryngeal closure ≒ 2 hours small - with CPM Surgeries to change the tracheal structure Tracheoesophageal diversion > 2 hours small - - Laryngotracheal Separation ≒ 2 hours small - - Tracheal flap method ≒ 2 hours small - - Considering the patient’s desire to resume even limited oral intake, a procedure that could also improve UES opening was required. Bilateral cricopharyngeal myotomy, which facilitates UES relaxation, was therefore considered essential. After integrating clinical and anatomical factors, subglottic laryngeal closure combined with bilateral cricopharyngeal myotomy was selected as the most appropriate surgical strategy (Fig. 2 ). Surgical Procedure The surgery was performed under general anesthesia. A 4-cm midline vertical incision was made just superior to the tracheostomy site (Fig. 3 A), exposing the cricoid cartilage and the first two tracheal rings. Despite the narrow field, adequate visualization was obtained. Bilateral cricopharyngeal myotomy was performed by transecting the cricopharyngeal muscles at their origins, located lateral to the cricoid cartilage (Fig. 3 B). Subsequently, the anterior portions of the first and second tracheal rings and the anterior two-fifths of the cricoid cartilage were then resected (Fig. 3 C), followed by circumferential transection between the cricoid and trachea at the inferior border of the cricoid cartilage. The entire subglottic tissue, dissected from the cricoid cartilage along with its perichondrium, was approximated and closed with 4 − 0 absorbable sutures, elevated toward the glottis to reduce dead space, and additionally fixed to surrounding tissue. The resected anterior cricoid segment was positioned over the closure site (Fig. 3 D), and the strap muscles were approximated and sutured at the midline to reinforce it (Fig. 3 E). Following placement of a single negative-pressure drain, a permanent tracheostoma was created to complete the procedure (Fig. 3 F). A cuffed tracheostomy tube (ID 7.5 mm) was inserted. Postoperative course Postoperative nutrition was initiated via gastrostomy feeding, and the surgical drain was removed on postoperative day (POD) 4 without complications. As tracheal suctioning decreased and surgical site swelling subsided, the tracheostomy tube was replaced with an uncuffed single-lumen tube. VFSS on POD 11 showed no anastomotic leakage and markedly improved UES passage (Fig. 4 A). Postoperative HRM demonstrated a significant reduction in resting UES pressure compared with preoperative measurements (Fig. 4 B), and laryngoscopy revealed subglottic closure with connective tissue formation and enhanced UES opening (Fig. 4 C). Oral intake was resumed with jelly on POD 12, with adjustments to positioning, food consistency, and 5-mL bolus volume. By POD 18, the diet was advanced to minced and moist consistency, and the permanent tracheostoma had stabilized and maintained a stable, clean configuration (Fig. 4 D). At three months postoperatively, the patient continued partial oral intake in combination with gastrostomy feeding, without recurrence of aspiration pneumonia or ventilatory support. Both the patient and family expressed high satisfaction. Discussion This case highlights the importance of individualized surgical decision-making for patients with severe dysphagia secondary to multiple system atrophy (MSA). In the initial surgical intervention, impaired bolus passage at the pharyngoesophageal segment was attributed to reduced laryngeal elevation, and laryngeal suspension successfully restored oral intake while preserving phonation [ 5 ]. However, as MSA progressed, further decline in both sensory and motor swallowing functions, combined with upper esophageal sphincter (UES) dysfunction and systemic deterioration, resulted in recurrent aspiration pneumonia. These findings underscore the necessity of continuous reassessment of swallowing physiology and tailored surgical approaches according to the evolving disease stage [ 6 , 7 ]. Patients with MSA frequently present with complex pharyngolaryngeal and UES dysfunction, including reduced laryngeal elevation, impaired vocal fold motion, and poor coordination between the pharynx and esophagus [ 2 , 6 – 8 ]. In patients with clinically significant dysphagia, these abnormalities can be clearly demonstrated using high-resolution manometry (HRM) and videofluoroscopic swallowing studies (VFSS) [ 7 ]. Moreover, esophageal dysmotility is common in MSA, affecting more than 80% of patients and predisposing them to nocturnal gastroesophageal reflux and silent aspiration, which may contribute to the risk of sudden death in this population [ 9 – 11 ]. These pathophysiological features emphasize that aspiration in MSA is multifactorial, involving dysfunction of both upper and lower aerodigestive tracts. APS in patients with MSA has been reported to improve oral intake status and reduce suctioning frequency, thereby enhancing quality of life for both patients and caregivers[ 5 , 12 , 13 ]. In advanced stages of MSA, where dysphagia progresses despite conservative management, APS represents an important therapeutic option for preventing recurrent aspiration pneumonia. Various surgical techniques for aspiration prevention have been described, including total or partial laryngectomy, laryngeal closure, and laryngotracheal separation[ 14 ]. Ideally, the choice of procedure should be individualized based on the patient’s systemic condition, anatomical factors, disease progression, and personal goals. However, in clinical practice, procedure selection is sometimes determined by surgeon experience or institutional preference rather than patient-specific factors. In the present case, subglottic laryngeal closure was considered the most suitable approach because of its relatively low invasiveness, limited bleeding, and feasibility of permanent tracheostoma formation. In addition, this technique allowed simultaneous bilateral cricopharyngeal myotomy, which was essential given the patient’s significant UES dysfunction and strong desire to resume oral intake. The combined approach resulted in improved bolus passage and successful partial oral intake without recurrence of aspiration pneumonia. This case highlights the importance of individualized surgical decision-making in patients with progressive neurodegenerative diseases. Rather than applying a uniform surgical strategy, APS should be tailored to each patient’s pathophysiology, systemic condition, and treatment goals to achieve meaningful functional outcomes. Conclusion Surgical management of MSA-related dysphagia should not rely on a one-size-fits-all protocol. Instead, clinicians must integrate physiological findings, systemic conditions, and patient-centered goals to determine an individualized, safe, and functionally meaningful treatment strategy. Dissemination of this patient-specific decision-making process among multidisciplinary dysphagia care teams is essential for optimizing management of progressive neurodegenerative disorders. Declarations Fundings: None Conflicts of interest/Competing interests: On behalf of all the authors, the corresponding author states that there are no conflicts of interest. Ethics: This case report was approved by the Ethics Committee of the University of Tokyo (No. 2487, 2022179NI) and conformed with the tenets of the amended Declaration of Helsinki. Informed consent was obtained from the patient for participation in this study. Written informed consent was obtained from the patient for publication of the images. Availability of data: Data are available on a reasonable request. Author contributions: YO: contributed to patient care and drafted the manuscript. RU: took full responsibility for patient care, drew the figures, drafted the manuscript. TG: contributed to patient care and reviewed the manuscript. MLR: contributed to patient care, reviewed the manuscript, and edited English. SK: contributed to patient care and reviewed the manuscript. RI: contributed to patient care and reviewed the manuscript. YS: contributed to patient care and reviewed the manuscript. KK: contributed to patient care and critically revised the work. All authors reviewed and approved the final version of the manuscript for publication. References Ueha R, Cotaoco C, Kondo K, Yamasoba T. Management and Treatment for Dysphagia in Neurodegenerative Disorders. J Clin Med. 2023;13(1). 10.3390/jcm13010156 . Calandra-Buonaura G, Alfonsi E, Vignatelli L, Benarroch EE, Giannini G, Iranzo A, Low PA, Martinelli P, Provini F, Quinn N, Tolosa E, Wenning GK, Abbruzzese G, Bower P, Antonini A, Bhatia KP, Bonavita J, Pellecchia MT, Pizzorni N, Tison F, Ghorayeb I, Meissner WG, Ozawa T, Pacchetti C, Pozzi NG, Vicini C, Schindler A, Cortelli P, Kaufmann H. Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment. Parkinsonism Relat Disord. 2021;86:124–32. 10.1016/j.parkreldis.2021.03.027 . Japan SN. Practical Guideline for Spinocerebellar Degeneration and Multiple System Atrophy 2018. Tokyo: Nankodo Co., Ltd; 2018. Ramos ML, Ueha R, Goto T, Ichijo K, Sugita H, Shirota Y, Kondo K. A Challenging Case of Severe Dysphagia in Multiple System Atrophy. Dysphagia. 2025. 10.1007/s00455-025-10871-x . Ueha R, Nito T, Sakamoto T, Yamauchi A, Tsunoda K, Yamasoba T. Post-operative swallowing in multiple system atrophy. Eur J Neurol. 2016;23(2):393–400. 10.1111/ene.12880 . Tsuchiya K, Ueha R, Suzuki S, Goto T, Sato T, Nito T, Yamasoba T. Heightened risk of early vocal fold motion impairment onset and dysphagia in the parkinsonian variant of multiple system atrophy: a comparative study. Clin Park Relat Disord. 2020;3:100037. 10.1016/j.prdoa.2020.100037 . Ueha R, Goto T, Sato T, Nativ-Zeltzer N, Shen SC, Nito T, Belafsky PC, Yamasoba T. High Resolution Manofluorographic Study in Patients With Multiple System Atrophy: Possible Early Detection of Upper Esophageal Sphincter and Proximal Esophageal Abnormality. Front Med (Lausanne). 2018;5:286. 10.3389/fmed.2018.00286 . Do HJ, Seo HG, Lee HH, Oh BM, Kim Y, Kim A, Kim HJ, Jeon B, Han TR. Progression of Oropharyngeal Dysphagia in Patients with Multiple System Atrophy. Dysphagia. 2020;35(1):24–31. 10.1007/s00455-019-09990-z . Ueha R, Sato T, Goto T, Yamauchi A, Nativ-Zeltzer N, Mitsui J, Belafsky PC, Yamasoba T. Esophageal Dysmotility is Common in Patients With Multiple System Atrophy. Laryngoscope. 2021;131(4):832–8. 10.1002/lary.28852 . Taniguchi H, Nakayama H, Hori K, Nishizawa M, Inoue M, Shimohata T. Esophageal Involvement in Multiple System Atrophy. Dysphagia. 2015;30(6):669–73. 10.1007/s00455-015-9641-2 . Ono Y, Kunieda K, Takada J, Shimohata T. Distal oesophageal spasm in a patient with multiple system atrophy: A case report. eNeurologicalSci. 2024;35:100500. 10.1016/j.ensci.2024.100500 . Katoh M, Ueha R, Sato T, Sugasawa S, Goto T, Yamauchi A, Yamasoba T. Choice of Aspiration Prevention Surgery for Patients With Neuromuscular Disorders: Report of Three Cases. Front Surg. 2019;6:66. 10.3389/fsurg.2019.00066 . Koyama M, Ueha R, Sato T, Goto T, Yamauchi A, Kaneoka A, Suzuki S, Nito T, Yamasoba T. Aspiration Prevention Surgery: Clinical Factors Associated With Improvements in Oral Status Intake and Suction Frequency. Otolaryngol Head Neck Surg. 2023;168(5):1146–55. 10.1002/ohn.183 . Ueha R, Magdayao RB, Koyama M, Sato T, Goto T, Yamasoba T. Aspiration prevention surgeries: a review. Respir Res. 2023;24(1):43. 10.1186/s12931-023-02354-0 . Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-8426946","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":587579502,"identity":"4507ec69-1659-412e-b1da-bc382bf4af7b","order_by":0,"name":"Yumiko Ogawa","email":"","orcid":"","institution":"The University of Tokyo","correspondingAuthor":false,"prefix":"","firstName":"Yumiko","middleName":"","lastName":"Ogawa","suffix":""},{"id":587579503,"identity":"84ab3a2f-6d85-4b17-abe4-d98f7850c2a1","order_by":1,"name":"Rumi Ueha","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABAklEQVRIiWNgGAWjYBACg/tnDA7/YLCp72fvgYhIQGWYcWq5wWP4mIchjUey5wyYT5QWY2MehsM8BjdyULXgdtgNHjNpsJabbw8w8zD8kZOckXyA4UcNA7s5Tr/0f5P+AdJyOy8BqMXAWFoiLYGx5xgDs2UDHlse/gNpyTFg5v1nkDhPIseAgbeBgdngAE4t5r8NwA47YwCyBayF8S9+LWbSEmDv80C0zJYAWYdHi+QMsPdBgZxjcHAOg7GxZM+zhMMyxyRw+oVf/gxIiw0PP/sZwwdvGOTkJI4nH3z4psYmGVeIoQCISwQSQAyJZANitEAthui0I0HLKBgFo2AUDG8AALq7UyKNvaDEAAAAAElFTkSuQmCC","orcid":"","institution":"The University of Tokyo","correspondingAuthor":true,"prefix":"","firstName":"Rumi","middleName":"","lastName":"Ueha","suffix":""},{"id":587579504,"identity":"2482a923-b7e9-4aad-ab70-d82da49752cf","order_by":2,"name":"Takao Goto","email":"","orcid":"","institution":"The University of Tokyo","correspondingAuthor":false,"prefix":"","firstName":"Takao","middleName":"","lastName":"Goto","suffix":""},{"id":587579505,"identity":"c0642e4f-0815-4cd2-80fe-5a5939087f11","order_by":3,"name":"Miguel Limbert Ramos","email":"","orcid":"","institution":"Bataan General Hospital and Medical Center","correspondingAuthor":false,"prefix":"","firstName":"Miguel","middleName":"Limbert","lastName":"Ramos","suffix":""},{"id":587579506,"identity":"0e5d1246-5b70-470b-89df-46efa6da87d9","order_by":4,"name":"Sakutarou Koike","email":"","orcid":"","institution":"The University of Tokyo","correspondingAuthor":false,"prefix":"","firstName":"Sakutarou","middleName":"","lastName":"Koike","suffix":""},{"id":587579507,"identity":"870070b2-ce5c-46f9-96e7-5e3951ba6a19","order_by":5,"name":"Ryota Ishizuka","email":"","orcid":"","institution":"The University of Tokyo","correspondingAuthor":false,"prefix":"","firstName":"Ryota","middleName":"","lastName":"Ishizuka","suffix":""},{"id":587579508,"identity":"146cae76-5fc8-4caf-abfd-8e1682d206db","order_by":6,"name":"Yuichiro Shirota","email":"","orcid":"","institution":"The University of Tokyo","correspondingAuthor":false,"prefix":"","firstName":"Yuichiro","middleName":"","lastName":"Shirota","suffix":""}],"badges":[],"createdAt":"2025-12-22 16:23:42","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-8426946/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-8426946/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":102337221,"identity":"5a6cb386-9270-4384-b637-883180071f07","added_by":"auto","created_at":"2026-02-10 16:11:59","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":3597918,"visible":true,"origin":"","legend":"\u003cp\u003ePreoperative examinations.\u003c/p\u003e\n\u003cp\u003e(A) laryngoscopy revealed saliva pooling in the hypopharynx and entry of saliva into the trachea. (B) videoflouroscopic swallowing study with 3 ml thickened contrast agent showed silent aspiration into the airway. (C) high-resolution manometry (HRM) tracing obtained during swallowing (yellow arrow). UES: upper esophageal sphincter\u003c/p\u003e","description":"","filename":"Figure1.png","url":"https://assets-eu.researchsquare.com/files/rs-8426946/v1/7c95b72128d07e2be4c12069.png"},{"id":102337335,"identity":"e3e877e3-83ca-4250-8f13-fa432fb790d3","added_by":"auto","created_at":"2026-02-10 16:12:24","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":219989,"visible":true,"origin":"","legend":"\u003cp\u003eSchematic diagram of subglottic laryngeal closure with bilateral cricopharyngeal myotomy. UES: upper esophageal sphincter; CPM: cricopharyngeal myotomy.\u003c/p\u003e","description":"","filename":"Figure2.png","url":"https://assets-eu.researchsquare.com/files/rs-8426946/v1/9d30d29d432ff6890868e8d8.png"},{"id":102337318,"identity":"3da0193d-9860-4c58-9940-6fc91dbe60c7","added_by":"auto","created_at":"2026-02-10 16:12:15","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":16339042,"visible":true,"origin":"","legend":"\u003cp\u003eIntraoperative views.\u003c/p\u003e\n\u003cp\u003e(A) skin incision line. (B) the front parts of the cricoid cartilage and first two tracheal rings were resected, and both cricopharyngeal muscles were cut at their lateral attachments (star marks). (C) circumferential transection between the cricoid cartilage and the trachea along the inferior border of the cricoid cartilage (arrows). (D) reinforcement of the subglottic closure using a fragment of the resected cricoid cartilage (arrowheads). (E) filling and reinforcement of the subglottic space with the strap muscles. (F) creation of a permanent tracheostoma.\u003c/p\u003e","description":"","filename":"Figure3.png","url":"https://assets-eu.researchsquare.com/files/rs-8426946/v1/dea76475ee0eed3dff2957bf.png"},{"id":102337320,"identity":"47a24833-2ffa-4fac-9b36-1d99cf873003","added_by":"auto","created_at":"2026-02-10 16:12:16","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":5755801,"visible":true,"origin":"","legend":"\u003cp\u003ePostoperative examinations.\u003c/p\u003e\n\u003cp\u003e(A) videoflouroscopic swallowing study using 5 mL of thin liquid contrast agent at 60° postural angle. (B) postoperative high-resolution manometry revealed sustained opening of the UES. (C) visualization of the upper esophageal sphincter (UES, yellow arrows) in flexible laryngoscopy. (D) postoperative view of the permanent tracheostoma and surgical site.\u003c/p\u003e","description":"","filename":"Figure4.png","url":"https://assets-eu.researchsquare.com/files/rs-8426946/v1/5d0b431903a6db0286289d7c.png"},{"id":105728208,"identity":"33162217-05ee-47e7-9e79-edf02df8db6f","added_by":"auto","created_at":"2026-03-30 11:10:54","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":24831882,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8426946/v1/99137b31-8ba8-4941-94f4-bbd00ad95685.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Stepwise Surgical Management of Severe Dysphagia in Multiple System Atrophy: A Case Report","fulltext":[{"header":"Introduction","content":"\u003cp\u003eSevere dysphagia in neuromuscular disorders may lead to life-threatening aspiration, and enteral feeding or tracheostomy is often required as the disease progresses [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. While surgery was traditionally approached cautiously, recent evidence indicates that surgical treatment for dysphagia may be considered to improve quality of life and reduce aspiration risk [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eMultiple system atrophy (MSA) is a progressive neurodegenerative disorder in which dysphagia commonly develops early and often becomes refractory as the disease advances. In Japan, aspiration prevention surgery (APS) is listed among the therapeutic options for severe dysphagia in advanced MSA according to current clinical practical guidelines [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. Although swallowing improvement surgeries such as laryngeal suspension may temporarily restore oral intake in selected patients [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e], severe dysphagia may recur with disease progression, and there is limited guidance on subsequent surgical management.\u003c/p\u003e \u003cp\u003eIn this report, we describe a patient with progressive MSA who had previously undergone laryngeal suspension and later developed advanced severe dysphagia, requiring reconsideration of surgical intervention. We discuss the clinical decision-making process and the rationale for selecting an individualized, stage-specific surgical strategy in advanced neurodegenerative dysphagia.\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cp\u003eA 70-year-old male with a 9-year history of MSA-cerebellar type (MSA-C) had experienced recurrent aspiration pneumonia due to progressive severe dysphagia, along with urinary tract infections. As previously reported [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e], comprehensive swallowing assessments had led to swallowing improvement surgery consisting of laryngeal suspension and tracheostomy, enabling continued oral intake while preserving phonation.\u003c/p\u003e \u003cp\u003eHowever, five months later, the patient demonstrated further disease progression, developing marked dysphonia, increased tracheal secretions that required frequent suctioning, and declining oral intake. At six months postoperatively, he developed aspiration pneumonia. Despite the cessation of oral intake, the patient continued to experience recurrent episodes of aspiration pneumonia, eventually necessitating ventilatory support.\u003c/p\u003e \u003cp\u003eFollowing antibiotic therapy and careful systemic management, the patient was successfully weaned from mechanical ventilation. However, respiratory cultures revealed persistent colonization with extended-spectrum β-lactamase (ESBL)-producing Escherichia coli and Klebsiella pneumoniae. Given the patient\u0026rsquo;s severely compromised condition and continued risk of life-threatening aspiration, the attending neurologist requested reassessment of swallowing function to explore potential surgical options.\u003c/p\u003e \u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eClinical and instrumental evaluation\u003c/h2\u003e \u003cp\u003eOn physical examination, the patient was unable to speak due to severe dysarthria and respiratory difficulty, even with a speech cannula. Although tongue movement was impaired, protrusion and retraction were partially preserved. The thyroid cartilage remained elevated from the prior laryngeal suspension with no signs of local infection. He was nearly bedridden but could briefly tolerate sitting in a wheelchair at 60\u0026deg; recline. Although his extremities were rigid and immobile, simple communication was achieved through lip movements and eye blinking.\u003c/p\u003e \u003cp\u003eLaryngoscopy revealed intact velopharyngeal closure, involuntary bilateral vocal fold movement, and markedly diminished laryngeal sensation. The hypopharyngeal space appeared widened secondary to the previous laryngeal suspension, but copious saliva pooling and partial tracheal penetration were observed (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eA). Videofluoroscopic swallowing study (VFSS) using 3 mL of thickened contrast agent (100 mPa\u0026middot;s) demonstrated weak pharyngeal contraction, poor upper esophageal sphincter (UES) opening, and silent aspiration of the contrast agent into the airway. Even with a large volume of aspirate, no effective cough reflex was elicited (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eB). High-resolution manometry (HRM) showed elevated resting UES pressure and markedly impaired UES opening during swallowing (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003eC), findings that differed from those observed after the previous laryngeal suspension described in our previous report\u003csup\u003e4)\u003c/sup\u003e.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eBased on these findings, the disease progression in MSA was considered to have further impaired both sensory and motor components of swallowing, and in combination with reduced respiratory and physical function, may have limited the patient\u0026rsquo;s ability to clear aspirated material. Progressive neurodegeneration was presumed to cause spasticity-like dysfunction of the cricopharyngeal muscle, severely compromising UES relaxation. Although balloon dilation or cricopharyngeal myotomy could be considered for impaired UES opening, these interventions alone were unlikely to sufficiently reduce aspiration risk given his overall condition. Because of the severity of respiratory dysfunction, aspiration prevention was regarded as the most appropriate strategy. After thorough discussion, the patient\u0026rsquo;s family clearly preferred APS, and the patient strongly wished to resume oral intake, even in small amounts.\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003eIndividualized surgical planning process\u003c/h3\u003e\n\u003cp\u003eIn this patient with severe dysphagia due to MSA, colonization with ESBL\u0026ndash;producing organisms, poor systemic condition, and a strong desire to resume oral intake necessitated careful selection of an appropriate APS. Potential procedures include (1) total or partial laryngectomy, (2) laryngeal closure, and (3) laryngotracheal separation or tracheoesophageal diversion (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). Given the patient\u0026rsquo;s deteriorated systemic status, a minimally invasive approach with short operative time was preferred. Because the thyroid cartilage had been fixed to the mandible during prior laryngeal suspension, any procedure requiring re-exposure of the thyroid cartilage, such as laryngectomy or glottic closure, was considered unsuitable, particularly given the infection risk associated with ESBL colonization. Tracheoesophageal anastomosis was also excluded due to the risk of anastomotic complications.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eAspiration prevention surgery options. ≒ 2 hours: around 2 hours; UES: upper esophageal sphincter; CPM: cricopharyngeal myotomy.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"6\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e \u003cp\u003eAspiration prevention surgeries\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eOperative time\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eAmount of bleeding\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eExposure of\u003c/p\u003e \u003cp\u003ethyroid cartilage\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003eUES opening effect\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e\u003cb\u003eSurgeries to \u003c/b\u003e\u003c/p\u003e \u003cp\u003e\u003cb\u003eremove the larynx\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eTotal laryngectomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e\u0026gt;\u0026thinsp;2 hours\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003elarge\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e+\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCentral-part laryngectomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e≒ 2 hours\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003esmall\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e+\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003e\u003cb\u003eSurgeries to \u003c/b\u003e\u003c/p\u003e \u003cp\u003e\u003cb\u003eclose the larynx\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSupraglottic laryngeal closure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e≒ 2 hours\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003esmall\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eGlottic laryngeal closure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e≒ 2 hours\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003esmall\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e+\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003ewith CPM\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSubglottic laryngeal closure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e≒ 2 hours\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003esmall\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003ewith CPM\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"2\" rowspan=\"3\"\u003e \u003cp\u003e\u003cb\u003eSurgeries to change \u003c/b\u003e\u003c/p\u003e \u003cp\u003e\u003cb\u003ethe tracheal structure\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eTracheoesophageal diversion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e\u0026gt;\u0026thinsp;2 hours\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003esmall\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eLaryngotracheal Separation\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e≒ 2 hours\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003esmall\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eTracheal flap method\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e≒ 2 hours\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003esmall\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eConsidering the patient\u0026rsquo;s desire to resume even limited oral intake, a procedure that could also improve UES opening was required. Bilateral cricopharyngeal myotomy, which facilitates UES relaxation, was therefore considered essential. After integrating clinical and anatomical factors, subglottic laryngeal closure combined with bilateral cricopharyngeal myotomy was selected as the most appropriate surgical strategy (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e\n\u003ch3\u003eSurgical Procedure\u003c/h3\u003e\n\u003cp\u003eThe surgery was performed under general anesthesia. A 4-cm midline vertical incision was made just superior to the tracheostomy site (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eA), exposing the cricoid cartilage and the first two tracheal rings. Despite the narrow field, adequate visualization was obtained. Bilateral cricopharyngeal myotomy was performed by transecting the cricopharyngeal muscles at their origins, located lateral to the cricoid cartilage (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eB). Subsequently, the anterior portions of the first and second tracheal rings and the anterior two-fifths of the cricoid cartilage were then resected (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eC), followed by circumferential transection between the cricoid and trachea at the inferior border of the cricoid cartilage. The entire subglottic tissue, dissected from the cricoid cartilage along with its perichondrium, was approximated and closed with 4\u0026thinsp;\u0026minus;\u0026thinsp;0 absorbable sutures, elevated toward the glottis to reduce dead space, and additionally fixed to surrounding tissue. The resected anterior cricoid segment was positioned over the closure site (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eD), and the strap muscles were approximated and sutured at the midline to reinforce it (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eE). Following placement of a single negative-pressure drain, a permanent tracheostoma was created to complete the procedure (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eF). A cuffed tracheostomy tube (ID 7.5 mm) was inserted.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e\n\u003ch3\u003ePostoperative course\u003c/h3\u003e\n\u003cp\u003ePostoperative nutrition was initiated via gastrostomy feeding, and the surgical drain was removed on postoperative day (POD) 4 without complications. As tracheal suctioning decreased and surgical site swelling subsided, the tracheostomy tube was replaced with an uncuffed single-lumen tube. VFSS on POD 11 showed no anastomotic leakage and markedly improved UES passage (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003eA). Postoperative HRM demonstrated a significant reduction in resting UES pressure compared with preoperative measurements (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003eB), and laryngoscopy revealed subglottic closure with connective tissue formation and enhanced UES opening (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003eC).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eOral intake was resumed with jelly on POD 12, with adjustments to positioning, food consistency, and 5-mL bolus volume. By POD 18, the diet was advanced to minced and moist consistency, and the permanent tracheostoma had stabilized and maintained a stable, clean configuration (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003eD).\u003c/p\u003e \u003cp\u003eAt three months postoperatively, the patient continued partial oral intake in combination with gastrostomy feeding, without recurrence of aspiration pneumonia or ventilatory support. Both the patient and family expressed high satisfaction.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eThis case highlights the importance of individualized surgical decision-making for patients with severe dysphagia secondary to multiple system atrophy (MSA). In the initial surgical intervention, impaired bolus passage at the pharyngoesophageal segment was attributed to reduced laryngeal elevation, and laryngeal suspension successfully restored oral intake while preserving phonation [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. However, as MSA progressed, further decline in both sensory and motor swallowing functions, combined with upper esophageal sphincter (UES) dysfunction and systemic deterioration, resulted in recurrent aspiration pneumonia. These findings underscore the necessity of continuous reassessment of swallowing physiology and tailored surgical approaches according to the evolving disease stage [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e \u003cp\u003ePatients with MSA frequently present with complex pharyngolaryngeal and UES dysfunction, including reduced laryngeal elevation, impaired vocal fold motion, and poor coordination between the pharynx and esophagus [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan additionalcitationids=\"CR7\" citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. In patients with clinically significant dysphagia, these abnormalities can be clearly demonstrated using high-resolution manometry (HRM) and videofluoroscopic swallowing studies (VFSS) [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Moreover, esophageal dysmotility is common in MSA, affecting more than 80% of patients and predisposing them to nocturnal gastroesophageal reflux and silent aspiration, which may contribute to the risk of sudden death in this population [\u003cspan additionalcitationids=\"CR10\" citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. These pathophysiological features emphasize that aspiration in MSA is multifactorial, involving dysfunction of both upper and lower aerodigestive tracts.\u003c/p\u003e \u003cp\u003eAPS in patients with MSA has been reported to improve oral intake status and reduce suctioning frequency, thereby enhancing quality of life for both patients and caregivers[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. In advanced stages of MSA, where dysphagia progresses despite conservative management, APS represents an important therapeutic option for preventing recurrent aspiration pneumonia. Various surgical techniques for aspiration prevention have been described, including total or partial laryngectomy, laryngeal closure, and laryngotracheal separation[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. Ideally, the choice of procedure should be individualized based on the patient\u0026rsquo;s systemic condition, anatomical factors, disease progression, and personal goals. However, in clinical practice, procedure selection is sometimes determined by surgeon experience or institutional preference rather than patient-specific factors. In the present case, subglottic laryngeal closure was considered the most suitable approach because of its relatively low invasiveness, limited bleeding, and feasibility of permanent tracheostoma formation. In addition, this technique allowed simultaneous bilateral cricopharyngeal myotomy, which was essential given the patient\u0026rsquo;s significant UES dysfunction and strong desire to resume oral intake. The combined approach resulted in improved bolus passage and successful partial oral intake without recurrence of aspiration pneumonia.\u003c/p\u003e \u003cp\u003eThis case highlights the importance of individualized surgical decision-making in patients with progressive neurodegenerative diseases. Rather than applying a uniform surgical strategy, APS should be tailored to each patient\u0026rsquo;s pathophysiology, systemic condition, and treatment goals to achieve meaningful functional outcomes.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eSurgical management of MSA-related dysphagia should not rely on a one-size-fits-all protocol. Instead, clinicians must integrate physiological findings, systemic conditions, and patient-centered goals to determine an individualized, safe, and functionally meaningful treatment strategy. Dissemination of this patient-specific decision-making process among multidisciplinary dysphagia care teams is essential for optimizing management of progressive neurodegenerative disorders.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cul class=\"decimal_type\"\u003e\n \u003cli\u003eFundings: None\u003c/li\u003e\n \u003cli\u003eConflicts of interest/Competing interests: On behalf of all the authors, the corresponding author states that there are no conflicts of interest.\u003c/li\u003e\n \u003cli\u003eEthics: This case report was approved by the Ethics Committee of the University of Tokyo (No. 2487, 2022179NI)\u0026nbsp;and conformed with the tenets of the amended Declaration of Helsinki.\u003c/li\u003e\n \u003cli\u003eInformed consent was obtained from the patient for participation in this study. Written informed consent was obtained from the patient for publication of the images.\u003c/li\u003e\n \u003cli\u003eAvailability of data: Data are available on a reasonable request.\u003c/li\u003e\n \u003cli\u003eAuthor contributions:\u003c/li\u003e\n\u003c/ul\u003e\n\u003cp\u003eYO: contributed to patient care and drafted the manuscript.\u003c/p\u003e\n\u003cp\u003eRU: took full responsibility for patient care, drew the figures, drafted the manuscript.\u003c/p\u003e\n\u003cp\u003eTG: contributed to patient care and reviewed the manuscript.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eMLR: contributed to patient care, reviewed the manuscript, and edited English.\u003c/p\u003e\n\u003cp\u003eSK: contributed to patient care and reviewed the manuscript.\u003c/p\u003e\n\u003cp\u003eRI: contributed to patient care and reviewed the manuscript.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eYS: contributed to patient care and reviewed the manuscript.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eKK: contributed to patient care and critically revised the work.\u003c/p\u003e\n\u003cp\u003eAll authors reviewed and approved the final version of the manuscript for publication.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eUeha R, Cotaoco C, Kondo K, Yamasoba T. Management and Treatment for Dysphagia in Neurodegenerative Disorders. J Clin Med. 2023;13(1). \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3390/jcm13010156\u003c/span\u003e\u003cspan address=\"10.3390/jcm13010156\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eCalandra-Buonaura G, Alfonsi E, Vignatelli L, Benarroch EE, Giannini G, Iranzo A, Low PA, Martinelli P, Provini F, Quinn N, Tolosa E, Wenning GK, Abbruzzese G, Bower P, Antonini A, Bhatia KP, Bonavita J, Pellecchia MT, Pizzorni N, Tison F, Ghorayeb I, Meissner WG, Ozawa T, Pacchetti C, Pozzi NG, Vicini C, Schindler A, Cortelli P, Kaufmann H. Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment. 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Respir Res. 2023;24(1):43. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1186/s12931-023-02354-0\u003c/span\u003e\u003cspan address=\"10.1186/s12931-023-02354-0\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Multiple system atrophy, dysphagia, aspiration prevention surgery, subglottic closure, cricopharyngeal myotomy","lastPublishedDoi":"10.21203/rs.3.rs-8426946/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8426946/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eIntroduction:\u003c/h2\u003e \u003cp\u003eMultiple system atrophy (MSA) is a rapidly progressive neurodegenerative disorder in which dysphagia emerges early and worsens over time. Although swallowing improvement procedures such as laryngeal suspension or cricopharyngeal myotomy can temporarily restore oral intake, surgical strategies for severe dysphagia in patients with prior swallowing surgery and reduced physiological reserve remain poorly described.\u003c/p\u003e\u003ch2\u003eCase Report:\u003c/h2\u003e \u003cp\u003eA 70-year-old man with MSA had previously undergone laryngeal suspension and tracheostomy, allowing continued oral intake. Over the following months, he experienced progressive deterioration, including dysphonia, copious secretions requiring frequent suctioning, and recurrent aspiration pneumonia and transient ventilatory dependence. Clinical assessment revealed diminished laryngeal sensation, severe saliva pooling, silent aspiration on laryngoscopy and videofluoroscopy, and impaired upper esophageal sphincter (UES) relaxation on high-resolution manometry. Given his frailty, extended spectrum beta-lactamase colonization, and a desire to resume limited oral intake, a minimally invasive yet effective approach was required. Subglottic laryngeal closure with bilateral cricopharyngeal myotomy was performed through a small operative field, achieving complete aspiration prevention and improved UES opening.\u003c/p\u003e\u003ch2\u003eConclusion\u003c/h2\u003e \u003cp\u003eThis case highlights the importance of individualized, stage-specific surgical planning for advanced MSA-related dysphagia. Careful integration of physiological findings, systemic tolerance, and patient-centered goals can optimize outcomes, even in patients with prior swallowing surgery.\u003c/p\u003e","manuscriptTitle":"Stepwise Surgical Management of Severe Dysphagia in Multiple System Atrophy: A Case Report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2026-02-10 16:11:11","doi":"10.21203/rs.3.rs-8426946/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"d700455b-7f1f-4775-a5b4-a94feddab937","owner":[],"postedDate":"February 10th, 2026","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2026-03-25T07:57:26+00:00","versionOfRecord":[],"versionCreatedAt":"2026-02-10 16:11:11","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8426946","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8426946","identity":"rs-8426946","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}