Via Research Recognition Day 2024 VCOM-Carolinas

Clinical Case-Based Reports

Diffuse Esophageal Spasm ‘Corkscrew Esophagus’ An Alternative Treatment Approach McKenzie Allen, OMS-III, Via Edward College of Osteopathic Medicine Carolinas Campus Clinical Mentor: Wayne Frei, MD, Aiken Regional Medical Center

Report of Case

Abstract

Discussion/Conclusions

References DES is a rare esophageal motility disorder without a well-established treatment algorithm for evidence-based modalities. There are commonly delays in diagnosis, missed diagnoses, and often unpredictable outcomes for patients with DES. 1 • In patients who are not candidates for the more invasive treatment options or are intolerant to medications, endoscopic pneumatic dilation can be an effective management strategy. 11 • Barium swallow has a higher sensitivity than endoscopy for detecting strictures 4 . Taking into consideration this patient’s age, comorbidities, long standing history of GERD and dysphagia, led to the decision to use barium swallow. (Esophageal manometry is the gold standard for diagnosis of DES. 2 ) • Limited studies on the effectiveness/long term outcomes of the more invasive surgical approaches in individuals with DES. 2 • Not yet enough data to support endoscopic Botulinum toxin injections as a primary treatment modality. 5 • A previous study analyzing the long term outcomes of esophageal dilation in 51 patients with diffuse esophageal spasm revealed that 4 years after esophageal dilation, 56.25% reported improvement of their symptoms. 12 No reports of esophageal perforation. 12 This study concluded that based on their results, esophageal dilation in patients with DES is both safe and has beneficial long term effects . 12 • Key differences: previous study had a mean diagnosis age of 68.5 years 12 . Our patient was being treated for multiple other comorbidities at the time of esophageal dilation and was eventually released on hospice, limiting the ability for long term follow up. • In the elderly or immunocompromised, there is a need for less invasive treatment modalities that provide a solution , longer than the duration of action of an oral medication, like with esophageal dilation. This case adds to the literature by demonstrating the successful management of a complex case of DES with esophageal dilation alone . • In this case, classic imaging and clinical presentation along with thorough history were sufficient in allowing us to make the diagnosis of DES. More studies are needed to evaluate the treatment of DES in cases complicated by other factors such as other serious medical issues, advanced age, associated conditions such as GERD, history of smoking or alcohol use. More research on different diagnostic approaches is needed to support alternative approaches to diagnosis in certain cases. Goel, S., & Nookala, V. (2020). Diffuse Esophageal Spasm. PubMed; StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK541106/ Salvador, R., Costantini, M., Rizzetto, C., & Zaninotto, G. (2011). Diffuse esophageal spasm: the surgical approach. Diseases of the Esophagus, 25(4), 311–318. https://doi.org/10.1111/j.1442 2050.2010.01172.x Lee, E. M., Park, M. I., Moon, W., Kim, K. M., Park, S. J., & Kim, H. H. (2010). A Case of Symptomatic Diffuse Esophageal Spasm During Multiple Rapid Swallowing Test on High-Resolution Manometry. Journal of Neurogastroenterology and Motility, 16(4), 433–436. https://doi.org/10.5056/jnm.2010.16.4.433 Goyal, A., Chatterjee, K., Yadlapati, S., & Singh, S. (2017). Health-care utilization and complications of endoscopic esophageal dilation in a national population. Clinical Endoscopy, 50(4), 366– 371. https://doi.org/10.5946/ce.2016.155 Patel, D. A., Yadlapati, R., & Vaezi, M. F. (2022). Esophageal Motility Disorders: Current Approach to Diagnostics and Therapeutics. Gastroenterology, 162(6), 1617– 1634. https://doi.org/10.1053/j.gastro.2021.12.289 Sterling JL, Schey R, Malik Z. The Role of Botulinum Toxin Injections for Esophageal Motility Disorders. Curr Treat Options Gastroenterol. 2018 Dec;16(4):528-540. doi: 10.1007/s11938-018 0212-0. PMID: 30406479. Vanuytsel T, Bisschops R, Farré R, Pauwels A, Holvoet L, Arts J, Caenepeel P, De Wulf D, Mimidis K, Rommel N, Tack J. Botulinum toxin reduces Dysphagia in patients with nonachalasia primary esophageal motility disorders. Clin Gastroenterol Hepatol. 2013 Sep;11(9):1115-1121.e2. doi: 10.1016/j.cgh.2013.03.021. Epub 2013 Apr 13. PMID: 23591282. Okuda T, Higashino M, Osugi H, Maekawa N, Tanimura S, Kinoshita H, Wakasa K. [Case of diffuse esophageal spasm treated by long myotomy]. Nihon Geka Gakkai Zasshi. 1993 Oct;94(10):1159-63. Japanese. PMID: 8232189. Kuwano H, Miyazaki T, Masuda N, Kato H, Kusano M. Long myotomy of the esophagus and gastric cardia with a complete fundic patch procedure for diffuse esophageal spasm. Hepatogastroenterology. 2004 Nov-Dec;51(60):1729-31. PMID: 15532814. Wong, I., & Law, S. (2017). Peroral endoscopic myotomy (poem) for treating esophageal motility disorders. Annals of Translational Medicine, 5(8), 192–192. https://doi.org/10.21037/atm.2017.04.36 Pehlivanov, N., & Pasricha, P. J. (2006, May 16). Medical and endoscopic management of Achalasia. Nature News. https://doi.org/10.1038/gimo52 Almansa, C., Eslick, G. D., DeVault, K. R., & Achem, S. R. (2009). W1870 long term outcome of esophageal dilation in patients with diffuse esophageal spasm. Gastroenterology -Baltimore Then Philadelphia-, 136(5), 744. Arasi Thangavelu-Veluswamy, M. (2020, October 7). What is a barium swallow test? perform, side effects & prep. eMedicineHealth. https://www.emedicinehealth.com/barium_swallow/article_em.htm High-resolution manometry image. panel a represents a normal ... (n.d.). https://www.researchgate.net/figure/High-resolution-manometry-image-Panel-A-represents-a-normal-high-resolution manometry_fig1_330685157

91-year-old female with multiple comorbidities including C. difficile colitis, post-COVID pneumonia, septic shock, hyperchloremic metabolic acidosis, a history of smoking, COPD, and congestive heart failure presented with progressive dysphagia to solids. Physical exam revealed a cachectic and malnourished female with a BMI of 16. The patient presented to the emergency department with persistent diarrhea secondary to Clostridium difficile colitis. Her diarrhea resolved with a 10 day course of fidaxomicin. The patient continued to have worsening leukocytosis throughout her hospital course. Her sputum culture was positive for Kleibsiella pneumoniae , indicating aspiration pneumonia likely secondary to swallowing difficulties. Treatment with meropenem was initiated. Differential diagnoses at the time included esophageal strictures secondary to history of gastroesophageal reflux disease (GERD), esophageal cancer, esophagitis and esophageal motility disorder . Barium swallow revealed lack of the primary stripping wave with to and fro motion within the esophagus with corkscrew appearance of the esophagus as seen in Figure 1. She was not a candidate for surgical intervention. Pharmacologic treatment with metoclopramide before meals and nifedipine was initiated. The patient continued to progress to dysphagia with liquids and solids. The patient was then evaluated by surgery. During her initial evaluation, she reported swallowing issues over the last few years and increasing chest discomfort. Gastroenterology was consulted. Upper gastrointestinal endoscopy with esophageal balloon dilation was performed. Sublingual nitroglycerin was ordered post procedure for pain secondary to esophageal spasm. In the following days, swallowing improved, and oral intake increased. Management of the patient’s other comorbid conditions was continued for several more weeks until the patient was released to home on hospice.

Diffuse Esophageal Spasm (DES) (also referred as corkscrew esophagus) is a rare esophageal motility disorder that occurs when abnormal contractions of the esophagus lead to curling of the esophagus. The classic finding on barium swallow study resembles that of the turns of a corkscrew. This case reports reviews a case of DES with impressive imaging and an alternative treatment approach. This case report presents a 91-year-old cachectic female with DES in the context of various comorbidities. The patient was not a candidate for surgical intervention. Pharmacologic treatment was unsuccessful. Upper gastrointestinal endoscopy with pneumatic esophageal dilation was performed. The patient’s swallowing improved in the days following endoscopic dilation. There are no well established guidelines for the treatment of DES. Treatment options include surgical approaches, medications, esophageal dilation and newer approaches such as endoscopic botulinum toxin injections. This case is unique in demonstrating the successful management of a rare esophageal motility disorder, in a high risk patient using a more traditional, less invasive treatment approach. • DES is a rare esophageal motility disorder that presents as progressive dysphagia. 1 • Characterized by uncoordinated or rapidly occurring contractions of the smooth muscle of the esophagus. 1 The contractions are simultaneous and alternate with normal peristalsis. 2 • DES occurs 1 case in 100,000 population per year. 1 Common in White females. 1 Risk increases with age. 1 • Definitive etiology of DES is unknown . One theory indicates it may be the result of disrupted balance between inhibitory and excitatory postganglionic pathways. 1 • Gold standard for diagnosis: esophageal manometry. 3 • Diagnostic Criteria normal IRP with distal latency of less than 4.5 s in greater than or equal to 20% swallows and/or at least 2 premature contractions with a distal latency of less than 4.5 seconds in at least 20 percent of wet swallows with the assumption of normal lower esophageal sphincter tone. 3 • Differential diagnoses of DES includes angina, achalasia, scleroderma, esophageal cancer, gastroesophageal reflux disease, esophageal diverticula, other esophageal motility disorder, esophageal webs and rings and esophagitis. 1 • Treatment: first line: calcium channel blockers and nitrates. 1 second line: endoscopic botulinum toxin injections and pneumatic dilation. 1 • Predictors of a good response to pneumatic dilation: age > 45 years, female sex, or non dilated esophagus. 5 • Esophageal botulinum injections are beneficial in improving dysphagia in elderly and individuals with multiple comorbidities. 6,7 (studies are small, and primarily retrospective. 6, 7 )Symptoms resolved for an average of 6 months. 6,7 • Surgical approaches: Heller myotomy and peroral endoscopic myotomy (POEM). • Peroral endoscopic myotomy (POEM) was first performed in 2010 10 in the treatment of achalasia. It is a less invasive approach that has seen an exponential increase and an extension of its use into other esophageal motility disorders. 10 Randomized control trial comparing POEM to the gold standard surgical approach in achalasia, Heller myotomy, is ongoing. 10 • DES is often associated with increased lower esophageal sphincter pressure, which is a predictor of good responses to pneumatic dilation. 5,11 • Complete symptom resolution with the use of pneumatic dilation, have been recorded as long as 10 to 25 years. 11 (includes non-follow ups in the results. 11 ) • Most studies conclude the symptoms most often recur with time in 50% or more of patients. 4,5,11 Introduction

Figure 2. Example of primary stripping wave initiated by swallow, traversing the entire length of the esophagus on barium swallow

Figure 1. Barium swallow of 91-year-old female with diffuse esophageal spasm (DES). Notice the ‘corkscrew’ or ‘rosary bead’ appearance of the esophagus.

Figure 4. High-resolution manometry image in a patient with DES. Abnormal high pressures mid to distal esophagus in several swallows. Simultaneous contractions in at least 20% of swallows, accompanied by normal peristalsis.

Figure 3. Normal high-resolution manometry image. Time is on the horizontal axis, length along the esophagus is along the vertical axis. High pressure areas are seen in red.

Acknowledgements

Clinical Mentor: Wayne Frei, MD, Aiken Regional Medical Center

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