Updates on management of achalasia Hung Sze Wing Dorothy

Achalasia Achalasia is a primary esophageal motility disorder characterised by absence of esophageal peristalsis, impaired lower esophageal sphincter response Peak incidence 30-60 years old Equally common among men and women 1 in 100,000 incidence per year ACG Clinical Guideline: Diagnosis and Management of Achalasia Michael F. Vaezi , MD, PhD, MSc, FACG 1 , John E. Pandolfi no , MD, MSCI 2 and Marcelo F. Vela , MD, MSCR 3

Presenting symptoms: Dysphagia (most common) Regurgitation Chest pain Weight loss

Investigations OGD Ba swallow: bird’s beak appearance Grossly normal Tight lower esophageal sphincter (LES) Dilated sigmoid esophagus with retained food and saliva Ba swallow: bird’s beak appearance High resolution manometry (HRM) =gold standard Radiology, St Vincent’s university hospital

Normal HRM Proximal esophagus Distal esophagus Lower pressure Higher pressure The University Hospital, Cincinnati

Chicago classification Developed by investigators at Northwestern University of Chicago facilitate the diagnosis of achalasia classify achalasia Integrated relaxation pressure (IRP) = mean pressure of LES during the 4 seconds of maximal relaxation in the 10-second window beginning at UES relaxation Normal IRP ≤ 15 mmHg Achalasia = ↑ IRP + failed peristalsis or spasm  The Chicago Classification of esophageal motility disorders, v3.0 P.J. Kahrilas et al. Neurogastroenterology and motility. Dec 2014

Chicago classification Type I (“classic achalasia”): minimal pressure in esophagus Type II: pan-esophageal pressurization (most common) Type III: Spasm. At least 20% of swallows reveal rapidly propagating or spastic simultaneous contraction Type I Type II Per oral endoscopic myotomy (POEM) for all spastic esophageal disorders? Endosc Int Open 2015 Jun; 3(3): E202–E204. Type III

Prognosis Response to pneumatic dilatation or laparoscopic Heller myotomy Type I intermediate response (~81%) Type II has best response (~96%) Type III has the least favorable response (~66%) JAMA. 2015;313(18):1841. Achalasia: a systematic review

Eckardt Score for achalasia severity Sign/symptoms Score 1 2 3 Recent weight loss (kg) None <5 5-10 >10 Dysphagia Occasional Daily Each meal Chest pain Several times a day Regurgitation Score: 0-12 Eckardt AJ, Eckardt VF. Treatment and surveillance strategies in achalasia: an update. Nat Rev Gastroenterol Hepatol. 2011;8(6):311-319.

Treatment options Pharmacological Endoscopic Surgical

Pharmacological options Calcium channel blockers and nitrates Least effective Side effects e.g. dizziness, headache Symptomatic improvement 53 to 87% Achalasia: a new clinically relevant classification by high-resolution manometry. Pandolfino JE, Kwiatek MA, Nealis T, Bulsiewicz W, Post J, Kahrilas PJ. Gastroenterology. 2008 Nov; 135(5):1526-33.

Endoscopic Botulinum toxin Pneumatic dilatation Durability 6-12months 1 month response rate >75% Pneumatic dilatation Annese V, Bassotti G, Coccia G et al. A multicentre randomised study of intrasphincteric botulinum toxin in patients with oesophageal achalasia. GISMAD Achalasia Study Group. Gut 2000;46:597–600

Pneumatic dilatation Endoscopic, graded dilatation Good short term results No GA Requires repeated dilatation Risk of perforation ~5%   Pneumatic dilation versus laparoscopic Heller’s myotomy for idiopathic achalasia. Boeckxstaens GE et al. N Eng J Med, May 2011

Surgical options New options Laparoscopic Heller myotomy Per-oral endoscopic myotomy (POEM)

Heller myotomy Divide circular and longitudinal muscles Extended 6-8cm on the esophagus and 1.5-3cm on gastric cardia + Fundoplication to reduce reflux Dor/Toupet 87% success after 2 years Boeckxstaens GE, Annese V, des Varannes SB et al. Pneumatic dilation versus laparoscopic Heller’s myotomy for idiopathic achalasia. N Engl J Med 2011;364:1807–181 Surgical treatment for achalasia – GI motility online – Nature. Jedediah A. Kaufman at al

POEM Submucosal tunneling Tunneling beyond GE junction Circular muscles divided Closure of mucosal entry Per-Oral Endoscopic Myotomy: A Series of 500 Patients. H Inoue et al. Journal of the American College of Surgeons, august 2015.

POEM vs Heller: systemic review and meta-analysis Author Journal Number of patients Improvement in dysphagia GERD Schlottmann et al Annals of surgery 2018 74 studies >7000 patients POEM better than Heller More GERD in POEM Talukdar R, Inoue H et al Surgical Endoscopy 2015 19 studies >1000 patients No difference Marano L et al. Medicine (Baltimore) 2016 11 studies 486 patients Trend towards less GERD in Heller

HK data POEM vs Heller

Retrospective cohort study 2001 to 2014 33 patients POEM 23 patients laparoscopic Heller myotomy Similar post-operative dysphagia score at 4 weeks, 3 months, 6 months GERD symptoms similar (25% Heller, 15.2% POEM p = 0.311)

POEM vs Heller Similar efficacy ?more GERD post-op in POEM patients

Longer myotomy = better? Theoretical advantage of a longer myotomy in POEM = ?better outcome Incision limited superiorly by hiatus

Prognosis Response to pneumatic dilatation or laparoscopic Heller myotomy Type I intermediate prognosis(~81%) Type II has best prognosis (~96%) Type III has the least favorable response to treatment (~66%) JAMA. 2015;313(18):1841. Achalasia: a systematic review

Endoscopy International Open, Jun 2015 75 patients with type III achalasia 49 underwent POEM 26 underwent laparoscopic Heller’s myotomy POEM better response (98.0 % vs 80.8 %; P =0.01) and significantly shorter OT time

Update in American gastroenterological association (AGA) guideline 2017 If expertise available: POEM should be considered as primary therapy for type III achalasia POEM should be considered a treatment option comparable to laparoscopic Heller myotomy for any of the achalasia syndromes

Dilatation vs Heller vs POEM Pros Cons Pneumatic dilatation No GA No skin incisions Repeated dilatation Perforation Lower efficacy Heller myotomy One off procedure Good long term results Requires GA POEM One-off procedure Short term comparable to Heller ?best option for type 3 achalasia No long term results ?more GERD

Conclusion Treatment options Botox and oral medications for unfit patients Pneumatic dilatation Heller, POEM Fitness for OT Patient’s preference $ Expertise Type of achalasia

Thank you

Fit for OT Yes No Type 3 achalasia Types 1 or 2 achalasia Botulinum toxin Fail Expertise available financially accept POEM or Heller Yes No Calcium channel blockers Nitrates POEM Heller