Achalasia cardia - Clinicals, Diagnosis, and Management

Upper GI

Clinicals - 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 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
Regurgitation - particularly at night: Aspiration pneumonia may occur due to this. Regurgitation of food from a dilated esophagus Regurgitation - particularly at night: Aspiration pneumonia may occur due to this.
Regurgitation of food from a dilated esophagus
Chest pain Due to esophageal spasm , Chest pain
Due to esophageal spasm ,
Weight loss - generally not marked Due to the dysphagia causing reduced nutritional intake Weight loss - generally not marked
Due to the dysphagia causing reduced nutritional intake
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 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

Clinicals - Examination

Fact Explanation
Reduced body mass index Due to chronic weight loss Reduced body mass index
Due to chronic weight loss
Reduced breath sounds on Left or Right lung - rare Grossly dilated esophagus causing lung compression Reduced breath sounds on Left or Right lung - rare
Grossly dilated esophagus causing lung compression
Dull percussion note on left or right lung - rare Grossly dilated esophagus causing lung compression Dull percussion note on left or right lung - rare
Grossly dilated esophagus causing lung compression
Signs of pneumonia Commonly in right lung following aspiration Signs of pneumonia
Commonly in right lung following aspiration

Investigations - Diagnosis

Fact Explanation
Esophageal manometry Absent peristalsis and impaired relaxation of lower esophageal sphincter , Esophageal manometry
Absent peristalsis and impaired relaxation of lower esophageal sphincter ,
Barium swallow Lack of peristalsis of esophagus.
Lower end showing "bird's beak" appearance due to failure of relaxation of lower esophageal sphincter ,
Barium swallow
Lack of peristalsis of esophagus.
Lower end showing "bird's beak" appearance due to failure of relaxation of lower esophageal sphincter ,

Investigations - Management

Fact Explanation
Upper GI endoscopy Risk of developing squamous carcinoma of esophagus in patients with achalasia is increased by 33 fold Upper GI endoscopy
Risk of developing squamous carcinoma of esophagus in patients with achalasia is increased by 33 fold
Coagulation studies Surgery may be a therapeutic option Coagulation studies
Surgery may be a therapeutic option
Chest X-ray Dilated esophagus, occasional showing fluid level behind the cardiac shadow.
Fundal gas shadow is absent.
Chest X-ray
Dilated esophagus, occasional showing fluid level behind the cardiac shadow.
Fundal gas shadow is absent.
Upper GI endoscopy To exclude pseudo achalasia Upper GI endoscopy
To exclude pseudo achalasia
CT scan - Thorax and Abdomen To exclude distal esophageal cancer CT scan - Thorax and Abdomen
To exclude distal esophageal cancer

Management - Supportive

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 Pharmacological therapy : calcium channel blockers, long acting nitrates, Sildenafil (Phosphodiesterase - inhibitor)
Transient reduction of LES pressure by smooth muscle relaxation, thereby facilitating esophageal emptying

Management - Specific

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 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
Pneumatic dilatation via endoscope Dilatation and disruption of circular muscle fibers of lower esophageal sphincter Pneumatic dilatation via endoscope
Dilatation and disruption of circular muscle fibers of lower esophageal sphincter
Myotomy performed laparascopically or endoscopically Incision of the circular muscle layer of the lower esophageal sphincter Myotomy performed laparascopically or endoscopically
Incision of the circular muscle layer of the lower esophageal sphincter
Partial fundoplication - sometimes performed with surgical myotomy To lessen postoperative reflux associated with surgical myotomy Partial fundoplication - sometimes performed with surgical myotomy
To lessen postoperative reflux associated with surgical myotomy

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  1. AHMAD Z, BHARGAVA R, PANDEY DK. An Uncommon Presentation of Achalasia Cardia. Journal, Indian Academy of Clinical Medicine 2004; 5(2): 147-8
  2. 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/
  3. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012
  4. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012
  5. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012
  6. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012
  7. KUMAR P, CLARK M. Clinical medicine, 8th Edition, London, Elsevier, 2012
  8. 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
  9. 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
  10. 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
  11. 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/
  12. TALLEY NJ. O’CONNOR S. Examination Medicine, 7th Edition. 2013. Sydney. Churchill-Livingstone-Elsevier.
  13. 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
  14. 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
  15. 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
  16. 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
  17. 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
  18. 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