ASGE Guideline on Achalasia Management 2020 PDF

GUIDELINE ASGE guideline on the management of achalasia Mouen A. Khashab, MD,1,* Marcelo F. Vela, MD,2,* Nirav Thosani, MD,3,* Deepak Agrawal, MD, MPH, MBA,4 James L. Buxbaum, MS, FASGE,5 Syed M. Abbas Fehmi, MD, MSc, FASGE,6 Douglas S. Fishman, MD, FAAP, FASGE,7 S...

GUIDELINE ASGE guideline on the management of achalasia Mouen A. Khashab, MD,1,* Marcelo F. Vela, MD,2,* Nirav Thosani, MD,3,* Deepak Agrawal, MD, MPH, MBA,4 James L. Buxbaum, MS, FASGE,5 Syed M. Abbas Fehmi, MD, MSc, FASGE,6 Douglas S. Fishman, MD, FAAP, FASGE,7 Suryakanth R. Gurudu, MD, FASGE,2 Laith H. Jamil, MD, FASGE,8 Terry L. Jue, MD, FASGE,9 Bijun Sai Kannadath, MBBS, MS,3 Joanna K. Law, MD,10 Jeffrey K. Lee, MD, MAS,11 Mariam Naveed, MD,12 Bashar J. Qumseya, MD, MPH,13 Mandeep S. Sawhney, MD, MS, FASGE,14 Julie Yang, MD, FASGE,15 Sachin Wani, MD, ASGE Standards of Practice Committee Chair16 This document was reviewed and approved by the Governing Board of the American Society for Gastrointestinal Endoscopy (ASGE) Endorsed by the American Neurogastroenterology and Motility Society and the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES) Achalasia is a primary esophageal motor disorder of unknown etiology characterized by degeneration of the myenteric plexus, which results in impaired relaxation of the esophagogastric junction (EGJ), along with the loss of organized peristalsis in the esophageal body. The criterion standard for diagnosing achalasia is high- resolution esophageal manometry showing incomplete relaxation of the EGJ coupled with the absence of orga- nized peristalsis. Three achalasia subtypes have been defined based on high-resolution manometry findings in the esophageal body. Treatment of patients with achalasia has evolved in recent years with the introduction of peroral endoscopic myotomy. Other treatment options include botulinum toxin injection, pneumatic dilation, and Heller myotomy. This American Society for Gastrointestinal Endoscopy Standards of Practice Guideline provides evidence-based recommendations for the treatment of achalasia, based on an updated assessment of the individ- ual and comparative effectiveness, adverse effects, and cost of the 4 aforementioned achalasia therapies. (Gastro- intest Endosc 2020;91:213-27.) INTRODUCTION DIAGNOSIS OF ACHALASIA Achalasia is a primary esophageal motor disorder of Esophageal motor abnormalities in achalasia lead to unknown etiology characterized by degeneration of the symptoms of dysphagia for solids and liquids without myenteric plexus, which results in impaired relaxation oropharyngeal transfer difficulties in roughly 90% of pa- of the esophagogastric junction (EGJ), along with the tients, regurgitation in 75%, weight loss in 60%, chest loss of organized peristalsis in the esophageal body. pain in 50%, and heartburn in 40%.4 In patients with a These abnormalities typically lead to dysphagia and regur- clinical presentation suggestive of achalasia, endoscopy is gitation.1 Achalasia occurs equally in males and females. mandatory to exclude pseudoachalasia or other forms of Achalasia has traditionally been viewed as a rare disease, mechanical obstruction at the EGJ.1 Although endoscopy with a globally reported incidence varying from.03 to may often reveal esophageal dilation, retention of food 1.63 per 100,000 persons per year.2 However, most and secretions, and a “puckered” EGJ, these findings are estimates of incidence have been derived from not diagnostic of achalasia and endoscopy may be retrospective searches of hospital discharge databases, normal, especially in early stages of the disease before with the diagnosis based on older diagnostic techniques esophageal dilation ensues. Barium esophagram can be such as conventional manometry or barium very helpful, particularly when the typical “bird beak” esophagram. More recent studies incorporating state-of- appearance at the EGJ with upstream esophageal dilation the-art high-resolution manometry and data derived is found, but as with endoscopy, an esophagram may be from motility laboratory databases suggest a higher inci- unrevealing when the esophagus is not dilated. A dence of 2.92 of 100,000 in Central Chicago2 and 2.3 to modified esophagram with timed emptying of a 2.8 of 100,000 in South Australia.3 standardized barium volume, known as the “timed www.giejournal.org Volume 91, No. 2 : 2020 GASTROINTESTINAL ENDOSCOPY 213 ASGE guideline on the management of achalasia barium esophagram,” is preferable because in addition to document is to provide evidence-based recommendations aiding diagnosis, it has been shown to be useful as a for the treatment of achalasia, based on an updated assess- means to objectively document treatment outcomes and ment of the comparative effectiveness, adverse effects, predict symptom recurrence.5 and cost of achalasia therapies. The criterion standard for diagnosing achalasia is high- resolution esophageal manometry showing incomplete METHODS relaxation of the EGJ coupled with the absence of orga- nized peristalsis. Three achalasia subtypes have been Overview defined based on the high-resolution manometry findings This document was prepared by a working group of the in the esophageal body: type I or classic achalasia with low Standards of Practice Committee of the American Society intraesophageal pressure, type II with pan-esophageal for Gastrointestinal Endoscopy (ASGE). It includes a sys- pressurization, and type III with high-amplitude spastic tematic review of available literature along with guidelines contractions.6 Importantly, multiple studies have shown for the role of endoscopy in management of achalasia us- that treatment outcomes are dependent on achalasia ing criteria highlighted in Table 1.15 After evidence subtype, and this information can guide the choice of synthesis, recommendations were drafted by the full therapy.7-9 Based on available data, pneumatic dilation, panel during a face-to-face meeting on March 16, 2018, laparoscopic Heller myotomy, and peroral endoscopic my- and approved by the Standards of Practice committee otomy (POEM) are all believed to be efficacious for acha- members and the ASGE Governing Board. lasia types I and II, whereas POEM has emerged as the preferred treatment for achalasia type III.10 Panel composition and conflict of interest The endoluminal functional lumen imaging probe (En- management doFLIP, Crospon, Galway, Ireland) is a new technology The panel consisted of 2 content experts (M.A.K., that enables assessment of the mechanical properties of M.F.V.), a committee member with expertise in systematic the esophagus and EGJ, using impedance planimetry mea- reviews and meta-analysis (N.T.), the committee chair surements of luminal cross-sectional area, along with pres- (S.W.), and other committee members. All panel members sure changes during volume-controlled distension.11 were required to disclose potential financial and intellec- Studies using EndoFLIP have shown that EGJ tual conflicts of interest, which were addressed according distensibility is reduced in achalasia patients,12 and to ASGE policies (see ASGE Conflict of Interest and Reso- symptomatic failure after treatment is associated with lution Policy at https://www.asge.org/docs/default-source/ persistently low distensibility.13 Furthermore, a recent about-asge/mission-and-governance/asge-conflict-of-inter- small study showed that achalasia could be diagnosed by est-and-disclosure-policy.pdf?sfvrsnZ2; the committee EndoFLIP in a subset of achalasia patients in whom high- member Conflict of Interest disclosure in the Conflict of In- resolution manometry revealed normal EGJ relaxation.14 terest Principles for ASGE Publication and Educational Although this technique is new and our understanding of Product Development Excluding Gastrointestinal Endos- its role in achalasia is evolving, it appears that EndoFLIP copy and CME Activity at https://www.asge.org/docs/ provides additional and complementary information in default-source/about-asge/mission-and-governance/doc- the evaluation and management of achalasia patients. asge-publications-coipolicy_2009.pdf?sfvrsnZ6). AIM AND SCOPE Formulation of clinical questions For all clinical questions, potentially relevant patient- In the last decade, there have been considerable ad- important outcomes were identified a priori and rated vances in the evaluation and management of achalasia. from “not important” to “critical” through a consensus pro- From a diagnostic perspective, high-resolution manometry cess. Relevant clinical outcomes included (1) clinical suc- has become the criterion standard, leading to the definition cess as defined by Eckardt score 3; (2) rate and severity of 3 achalasia subtypes that have confirmed implications for of adverse events; (3) length of hospital stay; (4) recur- response to and choice of therapeutic modality. Further- rence rate during long-term follow-up; and (5) rate of more, EndoFLIP is emerging as a useful technique for diag- GERD with pH studies, rate of erosive esophagitis, and nosis and objective assessment after therapy. Although proton pump inhibitor use. botulinum toxin injection, pneumatic dilation, and laparo- scopic Heller myotomy have been available for many years, Literature search and study selection criteria the treatment of achalasia has been revolutionized with the Separate literature searches were conducted for botuli- advent of POEM, which has become a routine procedure in num toxin injection, pneumatic dilation, and myotomy many centers around the world. A wealth of data examining (laparoscopic Heller myotomy and POEM) in the treatment the effectiveness of POEM has become available over the last of achalasia. A medical librarian performed a comprehen- few years, including several meta-analyses. The aim of this sive literature search from inception to October 17, 2017, 214 GASTROINTESTINAL ENDOSCOPY Volume 91, No. 2 : 2020 www.giejournal.org  ASGE guideline on the management of achalasia TABLE 1. System for rating the quality of evidence for guidelines Quality of evidence Definition Symbol High quality We are very confident that the true effect lies close to that of the estimate 4444 of effect. Moderate quality We are moderately confident in the effect estimate: the true effect is likely to be 444 close to the estimate of effect, but there is a possibility that it is substantially different. Low quality Our confidence in the effect estimate is limited: the true effect may be 44 substantially different from the estimate of effect. Very low quality We have very little confidence in the effect estimate: the true effect is likely 4 to be substantially different from the estimate of effect. Adapted from Guyatt et al.15 in the following databases: Ovid Medline(R) epub Ahead of tified outcomes of interest. For outcomes with limited or Print, In-Process & Other Non-Indexed Citations, Ovid no available direct comparisons, indirect comparisons Medline(R) Daily, Ovid Medline and Versions(R); Embase were used to estimate the magnitude and direction of ef- (Elsevier); and Wiley Cochrane Library. The searches fect. Heterogeneity was assessed using the I2 and Q statis- were limited to English language articles with animal tic. Significant heterogeneity was defined at I2 > 50% and studies excluded. No date limits were applied. Combina- significant P value (