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

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

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

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

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

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

Investigation

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

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

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

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

Treatment Options

Treatment Options

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

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

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

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

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

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

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%

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

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

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

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

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

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

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

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

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

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)

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

Thank you