Treatment Options for Upper Esophageal Sphincter (UES) Dysfunction Post-cerebral Vascular Accident (CVA); A Case Report

Case Diagnosis: 43-year-old male with upper esophageal sphincter dysfunction due to acute lateral medullary infarct (Wallenberg syndrome)

Case Description or Program Description: This 43-year-old male required gastrostomy-jejunostomy tube for severe dysphagia after lateral medullary infarct. Modified barium swallow study (MBSS) demonstrated increased UES tone causing deep laryngeal penetration with nectar consistency liquids. Otorhinolaryngology and gastroenterology recommended no surgical dilation or botulinum toxin (BTX) injection at the time.

Setting: Inpatient Rehabilitation Facility (IRF)

Assessment/Results: Baclofen was investigated as an option for modifying UES tone, however literature review showed that baclofen has no significant effect on decreasing UES tone.

Discussion (relevance): Due to a large majority of swallowing coordination centers located in the lateral medulla, lateral medullary infarcts commonly result in dysphagia and difficulty in UES relaxation. Literature review was conducted to explore potential pharmaceutical treatments to decrease UES tone to improve dysphagia. However, literature showed baclofen paradoxically increases lower esophageal sphincter tone, and offered no effect on UES pressure compared to placebo.
Due to the severity of dysphagia and the significant impairments to quality of life, we found it important to discuss treatment options and address the need for further investigation.
Other treatments include balloon catheterization, BTX injection, and surgical myotomy. The efficacy of balloon dilation has mixed results. Balloon dilation combined with speech therapy showed improvement in post-treatment UES residual pressure. BTX injections have been shown to relieve symptoms and reduce UES pressure for approximately 12 weeks with some long term functional improvements reported. Although cricopharyngeal myotomy is an effective treatment for anatomic sphincter constriction, studies indicate limited success in cases of neurogenic dysphagia.

Conclusions: There are limited treatment options available for neurogenic dysphagia due to UES dysfunction. While balloon dilation combined with speech therapy demonstrates the greatest improvement, there is a need for further investigation of pharmaceutical and interventional treatments.