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Management of Achalasia
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  1. Management of Achalasia Joint Hospital Surgical Grand Round Dennis KY Ngo Department of Surgery Prince of Wales Hospital

  2. Background • Greek term : failure to relax • One of esophageal motility abnormalities • Characterized by • Incomplete relaxation of the lower esophageal sphincter (LES ) • Aperistalsis of the body of esophagus • Simultaneous low amplitudes esophageal contraction • No apparent esophageal contraction

  3. Due to degeneration of inhibitory neurones in the wall of esophagus, preferentially nitric oxide producing. • Cause is unknown • ? Viral infection (VZV or HSV-1) • ? Immune-mediated • Class II HLA antigen – DQw1 • Epidemiology • Incidence : 0.5 per 100 000 • Prevalence : < 10 per 100 000 • No sex predilection • Age ~ 20-50 Kraichely et al Disease of the Esophagus 2006

  4. Case • F/45 • Good past health • Presented with acid regurgitation for 5 years • Initially treated as gastroesophageal reflux disease ( GERD ) • Refer to us for surgical treatment of GERD • Further questioning : dysphagia symptoms with hold up sensation at lower chest level

  5. F/45 • Good past health • Presented with acid regurgitation for 5 years • Initially treated as gastroesophageal reflux disease ( GERD ) • Refer to us for surgical treatment of GERD • Further questioning : dysphagia symptoms with hold up sensation at lower chest level Atypical for GERD

  6. Symptoms • Dysphagia • Both solid and liquid • Regurgitation and heartburn • A common presentation • Often misdiagnosed as GERD, esp. early achalasia • Delayed clearance – generate lactic acid from retained food residue • Howard et al Gut 1992 • Chest pain • Weight loss

  7. Investigation

  8. Upper Endoscopy (esophagogastroduodenoscopy) • First choice of investigation of dysphagia • Mechanical obstruction • Malignancy, esp around the lower esophageal sphincter ( pseudoachalasia ) • Cues for achalasia • Esophageal dilatation • Presence of food residue inside the esophagus

  9. Radiology ( Barium swallow ) • Features on Fluoroscopic Barium swallow • “Bird beak” like OGJ • Esophageal dilatation • Non-peristaltic esophagus • Signs of aspiration pneumonia

  10. Manometry • Diagnostic for achalasia • Diagnostic features : • Incomplete relaxation of LES • Normally – to a level < 8 mmHg above the gastric pressure • Aperistalsis of esophagus • Other characteristic features: • Elevated resting LES ( > 26 mmHg ) • Pressurization of esophagus • resting pressure in the esophagus exceeds the resting pressure in the stomach Spechler et al Gut 2001

  11. Aim of management • Cannot reverse the underlying the pathogenesis • Focused on reducing the LES pressure • Facilitate the emptying of esophageal content by gravity Symptomatic control and prevention of end organ damage

  12. Treatment Options

  13. Treatment Options

  14. Pharmacologic therapy • Commonly calcium channel blocker and nitrates • Poor results, effects diminish with time • Significant side effects of hypotension, headache and peripheral edema • NOT Applicable in clinical setting now Lake et al Alimentary Pharmacology & Therapeutics 2006

  15. Botulinum toxin injection • Potent inhibitor of the release of Acetylcholine • Excitatory influence of LES tone • Balance the action between excitation and inhibition neurons • Injection to LES • Four quadrant manner • Total 100 U

  16. Endoscopic dilatation • Different size of balloon • 30mm, 35mm and 40mm • Rigiflex balloon dilator

  17. Long term follow-up result • 2 large scale long term FU results • Retrospective study on 66 patients • Success rate : 85.7% ( 12 weeks after procedure ) • Cumulative success rate : 74% (5 years), 62%(10 years) • 21% requiring second dilatation • Perforation rate : 4.5 % ( all managed conservatively ) • Chan et al Endoscopy 2004 • Prospective study on 54 patients • 40% (5 years) and 36% (10 years) • One patient with perforation, managed conservatively • Eckardt et al Gut 2004

  18. Predictors of success • Older age • Decrease in LES pressure > 50% after dilatation • Perforation risk : < 5% • Risk of gastroesophageal reflux symptoms ~ 4-16%, can be managed by medical therapy Ghoshal et al Am J Gastroenterol 2004 Eckardt et al Gut 2004

  19. Botulinum toxin vs Dilatation

  20. Cardiomyotomy • Heller’s myotomy • 1914 • Original description • Anterior and posterior myotomy • Currently • Less length of myotomy • Only done anteriorly • Open ( transabdominal or transthoracic ) • Laparoscopic transabdominal

  21. Result from Laparoscopic cardiomyotomy

  22. Controversy 1 • ? Antireflux surgery is needed for cardiomyotomy • Variable incidence of reflux symptoms after cardiomyotomy

  23. Richards et al Ann Surg 2004 LES pressure was similar : 13.7mmHg vs 13.9 mmHg

  24. Controversy 2 • Antireflux surgery is needed in myotomy • ? Total or partial

  25. Choice of antireflux surgery • Total vs partial • Retard the esophageal clearance in a aperistaltic esophagus • Not enough pressure for food propagation • Progressive dilatation of the esophagus, result in dysphagia again • Favour partial fundoplication

  26. Controvery 3 • Partial fundoplication for myotomy ? Anterior Partial ( Dor ) ? Posterior Partial ( Toupet )

  27. Studies on individual performance for laparoscopic Heller myotomy + Dor or Toupet fundoplication • Both have good dysphagia relief together with reflux control • However, lack of randomized controlled trial for comparison • The choice is based on the surgeon’s belief and expertise

  28. Treatment options remaining : • Laparoscopic cardiomyotomy with partial fundoplication • Endoscopic balloon dilatation

  29. Lap myotomy vs Diltation • One randomized controlled trial recently • Kostic et al World J Surg 2007 • 51 patients • 25 Laparoscopic myotomy + Toupet fundoplication • 26 Dilatation • FU for 12 months • Results : • Symptomatic relief • 96% (Surgery) • 77% (Dilatation)

  30. Conclusion • Achalasia sometimes mixed up with gastroesophageal reflux disease • High index of suspicion is needed • Manometry is gold standard for Diagnosis of Achalasia • Treatment options available • Surgery vs endoscopic balloon dilatation • Trend more towards to Surgery in good operative risk in view of excellent and durable symptomatic risk with low complication rate

  31. Thank you