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
Prevalence : < 10 per
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. Atypical for GERD 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 Regurgitation and heartburn Chest pain Weight loss
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

12. 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

13. Treatment Options

14. Treatment Options

15. 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

16. 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

17. Study Pt. No. Symptomatic Improvement %  LES pressure %
No. Tx session
immediate
12m
Pasricha et al 31 90 44 -
1-2
Fishman et al 60 70 36 1
D’Onofrio et al 37 84 30
Kolbasnik et al 77 65
1-3
Annese et al 38
Cuilliere et al 55 72

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

19. 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

20. Perforation risk : < 5%
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

21. Botulinum toxin vs Dilatation
Study
Design
Pt no. FU
Symptomatic remission
Perf.
Vaezi et al GUT 1999
RCT
20 Dilatation
12m
70% (P<0.05) 5%
22 Botox
32% -
Milaeli et al
APT 2001
53% (P<0.05) 0%
20 Botox
15%

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

24. Result from Laparoscopic cardiomyotomy
Study
No. FU
Relief of dysphagia
LES pressure
Patti Ann Surg 1999
133
28m
93%
30 to 9 mmHg
Tsiaoussis Am J Surg 2007 68
8 year
91%
35 to < 8 mmHg

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

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

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

28. 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

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

30. However, lack of randomized controlled trial for comparison
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

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

32. 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)

33. 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

34. Thank you