History

Fact Explanation
Dysphagia - Both solids and fluids are equally difficult from the onset. Long standing but intermittent Due to absent peristalsis of the esophagus and impaired relaxation of the lower esophageal sphinter [1] [2]
Regurgitation - particularly at night: Aspiration pneumonia may occur due to this. Regurgitation of food from a dilated esophagus [1]
Chest pain Due to esophageal spasm [1],[3]
Weight loss - generally not marked Due to the dysphagia causing reduced nutritional intake [1]
Past history of Varicella zoster, measles infection. Varicella zoster DNA and increase in measles antibody titres have been demonstrated in some patients with Achalasia. However poor evidence is an issue. Therefore infection with the above viruses are not a definite cause of Achalasia unlike infection with Chagas disease [4]
References
  1. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012
  2. TALLEY NJ. O’CONNOR S. Examination Medicine, 7th Edition. 2013. Sydney. Churchill-Livingstone-Elsevier.
  3. VAEZI MF, PANDOLFINO JE, VELA MF. Diagnosis and Management of Achalasia. American Journal of Gastroenterology 2013; 108:1238–1249. published online 23 July 2013. doi:10.1038/ajg.2013.196
  4. GHOSHAL UC, DASCHAKRABORTY SB, SINGH R. Pathogenesis of achalasia cardia. World journal of Gastroenterology. June 28, 2012. 18(24) :3050-3057. NCBI. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3386318/

Examination

Fact Explanation
Reduced body mass index Due to chronic weight loss [1]
Reduced breath sounds on Left or Right lung - rare Grossly dilated esophagus causing lung compression [2]
Dull percussion note on left or right lung - rare Grossly dilated esophagus causing lung compression [2]
Signs of pneumonia Commonly in right lung following aspiration[3]
References
  1. LONGO DL, FAUCI AS , KASPER DL, HAUSER SL, JAMESON JL, LOSCALZO J. Harrison's principles of internal medicine, 18th Edition, United States of America, McGraw- Hill companies Inc, 2012
  2. AHMAD Z, BHARGAVA R, PANDEY DK. An Uncommon Presentation of Achalasia Cardia. Journal, Indian Academy of Clinical Medicine 2004; 5(2): 147-8
  3. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012

Differential Diagnoses

Fact Explanation
Gastro-Esophageal reflux disease Due to regurgitation. However in achalasia dysphagia for both solids and fluids predominate [1]
Chronic Chagas Disease Damage to neural plexus of the gut. However cardiac manifestations will also be present [2][3]
Diffuse esophageal spasm Differentiated by barium swallow that demonstrates "cork screw" esophagus [2]
Para neoplastic syndrome may have other manifestations of para neoplastic syndrome or presence of malignancy [4]
Scleroderma Patient may have other features of CREST syndrome - Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia [2]
References
  1. UPADHYAYA VD, GANGOPADHYAYA AN, GUPTA DK, et al. Esophageal achalasia of unknown etiology in infants. World Journal of Pediatrics 2008;4(1):63-65.
  2. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012
  3. BERN C. Antitrypanosomal Therapy for Chronic Chagas' Disease. The New England Journal of Medicine 2011; 364:2527-2534 June 30, 2011 DOI: 10.1056/NEJMct1014204
  4. BHALME MD, LEVISON SE, BANAIT GS. Paraneoplastic esophageal dysmotility-renal cell carcinoma presenting as dysphagia: a case report. Cases Journal 2009, 2:8170 doi:10.4076/1757-1626-2-8170

Investigations - for Diagnosis

Fact Explanation
Esophageal manometry Absent peristalsis and impaired relaxation of lower esophageal sphincter [1],[2]
Barium swallow Lack of peristalsis of esophagus. Lower end showing "bird's beak" appearance due to failure of relaxation of lower esophageal sphincter [1], [2]
References
  1. VAEZI MF , PANDOLFI JE, VELA MF. ACG Clinical Guideline: Diagnosis and Management of Achalasia. American Journal of Gastroenterology, advance online publication, 23 July 2013 doi: 10.1038/ajg.2013.196
  2. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012

Investigations - Fitness for Management

Fact Explanation
Coagulation studies Surgery may be a therapeutic option [1]
References
  1. VAEZI MF , PANDOLFI JE, VELA MF. ACG Clinical Guideline: Diagnosis and Management of Achalasia. American Journal of Gastroenterology, advance online publication, 23 July 2013 doi: 10.1038/ajg.2013.196

Investigations - Followup

Fact Explanation
Upper GI endoscopy Risk of developing squamous carcinoma of esophagus in patients with achalasia is increased by 33 fold [1]
References
  1. MEIJSSEN MA, TILANUS HW, VAN BLANKENSTEIN M, et al. Achalasia complicated by oesophageal squamous cell carcinoma: a prospective study in 195 patients. Gut, An international journal of Gastroenterology and Hepatology. Feb 1992; 33(2): 155–158. NCBI. Available from : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1373921/

Investigations - Screening/Staging

Fact Explanation
Chest X-ray Dilated esophagus, occasional showing fluid level behind the cardiac shadow. Fundal gas shadow is absent. [1][2]
Upper GI endoscopy To exclude pseudo achalasia [3]
CT scan - Thorax and Abdomen To exclude distal esophageal cancer [1]
References
  1. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012
  2. LONGO DL, FAUCI AS , KASPER DL, HAUSER SL, JAMESON JL, LOSCALZO J. Harrison's principles of internal medicine, 18th Edition, United States of America, McGraw- Hill companies Inc, 2012
  3. VAEZI MF , PANDOLFI JE, VELA MF. ACG Clinical Guideline: Diagnosis and Management of Achalasia. American Journal of Gastroenterology, advance online publication, 23 July 2013 doi: 10.1038/ajg.2013.196

Management - General Measures

Fact Explanation
Pharmacological therapy : calcium channel blockers, long acting nitrates, Sildenafil (Phosphodiesterase - inhibitor) Transient reduction of LES pressure by smooth muscle relaxation, thereby facilitating esophageal emptying [1]
References
  1. VAEZI MF , PANDOLFI JE, VELA MF. ACG Clinical Guideline: Diagnosis and Management of Achalasia. American Journal of Gastroenterology, advance online publication, 23 July 2013 doi: 10.1038/ajg.2013.196

Management - Specific Treatments

Fact Explanation
Intrasphinteric injection botulinum toxin via upper GI endscope Potent inhibition of acetylcholine release from nerve endings that reduce lower esophageal sphincter tone and causes sphincter relaxation [1][2]
Pneumatic dilatation via endoscope Dilatation and disruption of circular muscle fibers of lower esophageal sphincter [2]
Myotomy performed laparascopically or endoscopically Incision of the circular muscle layer of the lower esophageal sphincter [3]
Partial fundoplication - sometimes performed with surgical myotomy To lessen postoperative reflux associated with surgical myotomy [3]
References
  1. VAEZI MF , PANDOLFI JE, VELA MF. ACG Clinical Guideline: Diagnosis and Management of Achalasia. American Journal of Gastroenterology, advance online publication, 23 July 2013 doi: 10.1038/ajg.2013.196
  2. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012
  3. LONGO DL, FAUCI AS , KASPER DL, HAUSER SL, JAMESON JL, LOSCALZO J. Harrison's principles of internal medicine, 18th Edition, United States of America, McGraw- Hill companies Inc, 2012