History

Fact Explanation
Dysphagia Most of the patients present with dysphagia and or odynophagia. They complain of food getting stuck in the retrosternal area or in the suprasternal notch. Characteristic feature of esophageal motility disorders is that patients complain of intermittent dysphagia to both solids and liquids. In order to propel the swallowed food bolus esophageal muscle groups have to contract and relax in an organized manner ( contraction of the circular muscles should follow the contraction of longitudinal muscles of the mid-esophagus). Diffuse spasms of the body of the esophagus cause non-peritalsis and food gets stuck in the mid-esophagus, causing dysphagia. [1]
Chest pain Patients present with severe retrosternal chest pain which often radiates to the back. Esophageal dyskinesia is considered a common cause for unexplained chest pain. Patients develop chest pain due to spasmodic contractions of the esophagus as well as due to esophageal distension. [1,4]
Globus pharyngeus This refers to the sensation of a lump or foreign body in the throat, which is not painful. Motor disorders of the esophagus can cause a diffuse sensation of a foreign body in the throat. [2,3]
References
  1. BIEDERMANN LUC, GöTZE OLIVER. Nutcracker Esophagus. N Engl J Med [online] 2013 May [viewed 10 August 2014] Available from: doi:10.1056/NEJMicm1210209
  2. LEE BE, KIM GH. Globus pharyngeus: A review of its etiology, diagnosis and treatment World J Gastroenterol [online] 2012 May 28, 18(20):2462-2471 [viewed 10 August 2014] Available from: doi:10.3748/wjg.v18.i20.2462
  3. CASHMAN E. C., DONNELLY M. J.. The Natural History of Globus Pharyngeus. International Journal of Otolaryngology [online] 2010 December, 2010:1-4 [viewed 10 August 2014] Available from: doi:10.1155/2010/159630
  4. LEE EM, PARK MI, MOON W, KIM KM, PARK SJ, KIM HH. A Case of Symptomatic Diffuse Esophageal Spasm During Multiple Rapid Swallowing Test on High-Resolution Manometry J Neurogastroenterol Motil [online] 2010 Oct, 16(4):433-436 [viewed 10 August 2014] Available from: doi:10.5056/jnm.2010.16.4.433

Examination

Fact Explanation
Loss of weight Some patients may refuse meals because of the dysphagia and odynophagia.
References

Differential Diagnoses

Fact Explanation
Acute coronary syndrome Patients with myocardial ischemia present with severe retrosternal chest pain which radiates to left arm and or jaw. [2]
Achalasia Achalasia is a cause of dysphagia and odynophagia. Patients present with dysphagia, regurgitation of undigested food particles and chest pain. Increased pressure of lower esophageal sphincter and non-propagating peristaltic waves are findings of esophageal manometry. [1]
Chronic Chagas disease Megaesophagus is one of the intestinal complications of chronic Chagas disease. Patients present with dysphagia, odynophagia, regurgitation and epigastric discomfort. [3]
Esophageal carcinoma Esophageal carcinoma should be suspected in any patient presenting with progressive dysphagia.
Esophagitis Patients present with dyspepsia, chest pain, nausea and vomiting. Upper gastrointestinal endoscopy is useful in making the diagnosis. [4]
Gastroesophageal reflux disease (GERD) Patients with GERD present with burning epigastric pain and acid reflux. Patients can later develop esophageal stricture and dysphagia.
References
  1. SPILIOPOULOS DIMITRIOS, SPILIOPOULOS MICHAIL, AWALA ALERO. Esophageal Achalasia: An Uncommon Complication during Pregnancy Treated Conservatively. Case Reports in Obstetrics and Gynecology [online] 2013 December, 2013:1-4 [viewed 10 August 2014] Available from: doi:10.1155/2013/639698
  2. THYGESEN K., ALPERT J. S., WHITE H. D., JAFFE A. S., APPLE F. S., et al. Universal definition of myocardial infarction: Kristian Thygesen, Joseph S. Alpert and Harvey D. White on behalf of the Joint ESC/ACCF/AHA/WHF Task Force for the Redefinition of Myocardial Infarction. European Heart Journal [online] 2007 September, 28(20):2525-2538 [viewed 10 August 2014] Available from: doi:10.1093/eurheartj/ehm355
  3. RASSI ANIS, RASSI ANIS, MARIN-NETO JOSé ANTONIO. Chagas disease. The Lancet [online] 2010 April, 375(9723):1388-1402 [viewed 10 August 2014] Available from: doi:10.1016/S0140-6736(10)60061-X
  4. NONEVSKI I. T., DOWNS-KELLY E., FALK G. W.. Eosinophilic esophagitis: An increasingly recognized cause of dysphagia, food impaction, and refractory heartburn. Cleveland Clinic Journal of Medicine [online] 2008 September, 75(9):623-633 [viewed 10 August 2014] Available from: doi:10.3949/ccjm.75.9.623

Investigations - for Diagnosis

Fact Explanation
Upper gastrointestinal endoscopy (UGIE) UGIE shows helical folds of the esophageal lumen in nutcracker esophagus. Non-propagating and disordered esophageal contractions can be observed in some patients. [1,2]
Barium swallow test This shows the corkscrew appearance and segmentation of esophagus in nutcracker esophagus. [1]
Esophageal manometry High-resolution manometry of the esophagus shows high-amplitude pressure waves. [1]
CT scan Patients with diffuse esophageal spasm can have thickened esophageal wall (more than 3mm) due to muscular hypertrophy. [2]
Endoscopic ultrasound scan Presence of thickened esophageal muscle walls indicates the presence of esophageal spasms and hypertrophy. [2]
References
  1. BIEDERMANN LUC, GöTZE OLIVER. Nutcracker Esophagus. N Engl J Med [online] 2013 May [viewed 10 August 2014] Available from: doi:10.1056/NEJMicm1210209
  2. ROMAN S, KAHRILAS PJ. Management of Spastic Disorders of the Esophagus Gastroenterol Clin North Am [online] 2013 Mar, 42(1):27-43 [viewed 10 August 2014] Available from: doi:10.1016/j.gtc.2012.11.002

Management - Specific Treatments

Fact Explanation
Antispasmodics Antispasmodic drugs are indicated in the treatment of spastic contractions of the esophagus. Calcium channel blockers, sildenafil and nitrates are commonly used.
Endoscopic injection of botulinum toxin Local injections of botulinum toxin is also useful in treating dyskinesia of the esophagus. [1]
Balloon dilatation Balloon dilatation of the spastic segments can be done to relieve dysphagia. [1]
Surgery If the symptoms do not resolve with initial medical management myotomy is indicated which reduces the spasms and dysphagia. [1]
References
  1. KELLY EA, KOSZEWSKI IJ, JARADEH SS, MERATI AL, BLUMIN JH, BOCK JM. Botulinum Toxin Injection for the Treatment of Upper Esophageal Sphincter Dysfunction Ann Otol Rhinol Laryngol [online] 2013 Feb, 122(2):100-108 [viewed 10 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3951150