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Updates on the management of Achalasia. Joint Hospital Surgical Grand Round 21 July 2012 Lok Hon Ting (NDH). Pathophysiology. Motor disorder of the esophagus characterized by: Incomplete or absent relaxation of LES Aperistalsis of esophageal body
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Updates on the management of Achalasia Joint Hospital Surgical Grand Round 21 July 2012 Lok Hon Ting (NDH)
Pathophysiology • Motor disorder of the esophagus characterized by: • Incomplete or absent relaxation of LES • Aperistalsis of esophageal body • Destruction of ganglion cells present in the esophageal wall and LES • > Impaired relaxation of LES • Cause unknown, proposed etiology: • Viral hypothesis (VZV, HSV-1) • Jones DB. J Clin Pathol 1983. Robertson CS. Gut 1993 • Autoimmune hypothesis
Clinical manifestation • Epidemiology • Prevalence 1 per 100,000 • No gender predilection • Sadowski DC et al. Neurogastroenterol Motil 2010 • Symptoms: • Dysphagia – Both liquids and solids • Regurgitation +/- Pulmonary Aspiration • Chest pain / Heartburn in ~50% patient • Spechler SJ et al. Gut 1995 • Weight Loss • 16-fold increased risk of Ca Esophagus • Sandler RS et al. JAMA 1995
Investigation • OGD • tight cardia and food residual in esophgaus • Barium Swallow - Sensitivity 95% • Ott DJ et al. AJR Am J Roentgenol 1987 • Esophageal manometry • absence of any esophageal peristaltic contractions • failure of the LES to relax to less than 8 mm Hg • Gideon RM. Gastrointest Endosc Clin N Am 2005
Pharmacological treatment • Nitrates, Calcium channel blockers • Evidence: • Conclusion: Ineffective
Botulinum toxin injection • Endoscopic injection at 4 quadrants of LES • Inhibit release of acetylcholine in muscle synapse • First used by Pasricha in 1993
Botulinum toxin injection • Promising short term effect • Symptoms recurrence beyond 6 months follow up • 76% response to 2nd injection, but not to further injection Farnoosh Farrokhi etal. Orphanet Journal of Rare Diseases 2007
Botulinum toxin injection • Side effects 0 – 33% • Chest pain, reflux symptoms and rash • D Gui. Aliment Pharmacol Ther 2003 • Subsequent myotomy more difficult • Pehlivanov N. Neurogastroenterol Motil 2006 • Conclusion: • Safe and effective in short term symptoms relief • For elderly or frail patient only
Pneumatic dilatation • To disrupt circular muscle fiber of LES without full thickness perforation • First used by Sir Thomas Willis since the condition was first recognized • Rigiflex Polyethylene balloon (30, 35, 40mm diameter)
Pneumatic dilatation Guilherme M. Campos et al. Annals of Surgery 2009
Pneumatic dilatation • A pool of 1065 patients in 15 controlled series • Mean follow-up 30.8 months (6 – 111 months) • Rate of symptom improvement decreases with FU duration • Perforation rate: 1.6% (0 – 8%) • Subsequent treatment after index dilatation: • Repeated dilatation 25% • Myotomy 5%
Heller’s myotomy • First described by Ernest Heller in 1914 • Cutting the anterior and posterior aspect of LES • Current practice: myotomy over anterior aspect only • Minimally invasive approach 1990s • Thoracoscopic versus laparoscopic • Laparoscopic approach: less morbidity and quicker recovery • Richter JE. Gastroenterol hepatol 2008 • > standard approach
Heller’s myotomy Bresadola et al. Surg Laparosc Endoscc Percutan Tech 2012
Heller myotomy • A pool of 1708 patients in 19 publications • Follow-up duration: 4.78 year (range: 0.5 -11.2 years) • Symptom response rate: 79.3% (range: 47 – 97%) • GERD: • With fundoplication: 15.2% (range: 0 – 44%) • Without fundoplication: 37% (range: 11 – 60%) • Response rates decreased in patients with longer FU • > 7 years: 80% > 10 years: 74% > 20 years 65% Csendes. Ann Surg 2006
Heller’s myotomy and anti reflux surgery • Conclusion: • Heller’s myotomy with concomitant Dor’s fundoplication is the procedure of choice
Pneumatic Dilatation versus Heller’s Myotomy A Csendes et al. Guts 1989 Randomized controlled trial Subjects: Pneumatic dilatation (n = 39) Open Heller’s myotomy + Dor’s fundoplication (n =42) Conclusion: The study shows that surgical treatment offers a better final clinical result than pneumatic dilatation with the Mosher bag
Pneumatic Dilatation versus Lap Heller’s Myotomy S Kostic et al. World J Surg 2006 Randomized controlled trial Subjects: Graded pneumatic dilatation (n = 26) Heller’s myotomy + toupet’s fundoplication (n =25) Primary outcome: Treatment failure rate 2 Perforations after pneumatic dilatation
Pneumatic Dilatation versus Lap Heller’s Myotomy • Lopushinsky SR et al. JAMA 2006 • Retrospective longitudinal study • Subjects: Pneumatic dilatation 1181 (80.8%) • Surgical myotomy 280 (19.2%) • Primary outcome: use of subsequent intervention • Differences in risk were observed only when subsequent pneumatic dilatation was included as an adverse outcome
Pneumatic Dilatation versus Heller’s Myotomy • Emerging evidence showing comparable result between pneumatic dilatation and Heller’s Myotomy • Improvement of dilatation devices and technique • Definition of treatment failure • Some of the latest studies accept repeated dilatation as part of the dilatation program, instead of treatment failure • Both pneumatic dilatation and Heller’s Myotomy are reasonable choices of treatment if patients accept repeated dilatation
Per Oral Endoscopic Myotomy • Natural orifice transluminal endoscopic surgery -> Novel approach for Achalasia • The concept of Submucosal tunneling and procedure was described by Samiyama K in 2007 • Endoscopic myotomy was first reported by Pasricha et al. in a porcine model • Endoscopy 2007
Per Oral Endoscopic Myotomy • First series of 17 patients with achalasia treated by P.O.E.M., reported by Inoue et al • Endoscopy 2010
Per Oral Endoscopic Myotomy • 17 patients • seven women, ten men • mean age 41.4 years, range 18–62 • Long submucosal tunnel created (mean 12.4cm) • Mean myotomy length = 8.1cm • Dysphagia symptoms score: 10 1.3 (p = 0.0003) • LES pressure: 52.4mmHg 19.8mmHg (p = 0.0001)
Per Oral Endoscopic Myotomy • Experience from various centers
Conclusion • Laparoscopic cardiomyotomy + partial fundoplication is the standard treatment for achalasia • Pneumatic dilatation is reasonable alternative if patient accepts risk of repeated dilatation • Botox injection is only recommended for elderly and frail patients
Conclusion • POEM is a novel approach showing promising short term results • Long term follow up needed • rate of symptoms recurrence • need for subsequent intervention • incidence of GERD • complication profile