Medical Policy: 07.01.79 

Original Effective Date: December 2018 

Reviewed: December 2020 

Revised: December 2020 

 

Notice:

This policy contains information which is clinical in nature. The policy is not medical advice. The information in this policy is used by Wellmark to make determinations whether medical treatment is covered under the terms of a Wellmark member's health benefit plan. Physicians and other health care providers are responsible for medical advice and treatment. If you have specific health care needs, you should consult an appropriate health care professional. If you would like to request an accessible version of this document, please contact customer service at 800-524-9242.

 

Benefit Application:

Benefit determinations are based on the applicable contract language in effect at the time the services were rendered. Exclusions, limitations or exceptions may apply. Benefits may vary based on contract, and individual member benefits must be verified. Wellmark determines medical necessity only if the benefit exists and no contract exclusions are applicable. This medical policy may not apply to FEP. Benefits are determined by the Federal Employee Program.

 

This Medical Policy document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy will be reviewed regularly and be updated as scientific and medical literature becomes available.

 

Description:

Achalasia

Esophageal achalasia is a primary motor disorder of the esophagus characterized by insufficient lower esophageal sphincter (LES) relaxation and loss of esophageal peristalsis. This results in patient complaints of dysphagia to solids and liquids, regurgitation, and occasional chest pain with or without weight loss.

 

The goal of treatment is to improve esophageal emptying and patient’s symptoms by relieving the functional obstruction at the level of the gastroesophageal junction. This is usually accomplished by decreasing the LES pressure to <10 mm Hg. Mechanical disruption of the LES, either via surgical myotomy or endoscopic pneumatic dilation, is the preferred treatment approach. Biochemical relaxation is also an option, although it is less effective and typically reserved for patients who cannot undergo surgery. Among the two options for mechanical LES disruption, most surgeons recommend surgical myotomy as the first-line treatment. Pneumatic dilation, while initially effective, is felt to have lower long-term success rates.

 

Diagnosis of Achalasia

Clinical Achalasia Syndromes Within and Beyond Chicago Classification, Version 3.0

SyndromeMedian IRPEsophageal contractilityQualifications/notes
Chicago Classification: type I achalasia Greater than upper limit of normal Absent contractility Most published treatment trials excluded end-stage cases
Chicago Classification: type II achalasia Greater than upper limit of normal Absent peristalsis Panesophageal pressurization with ≥20% of swallows Most common presenting achalasia subtype Often misdiagnosed before HRM because of esophageal shortening and pseudorelaxation
Chicago Classification: type III achalasia Greater than upper limit of normal Absent peristalsis Premature contractions with ≥20% of swallows Often mistaken for spasm before HRM Obstructive physiology includes the distal esophagus
Chicago Classification: EGJ outflow obstruction Greater than upper limit of normal Sufficient peristalsis to exclude types I, II or III achalasia Can be early or incomplete achalasia (12%–40%) Can resolve spontaneously Can be artifact; further imaging of EGJ may clarify diagnosis
Chicago Classification: absent contractility Less than upper limit of normal Absent contractility Abnormal functional luminal imaging probe distensibility index or esophageal pressurization with swallows or multiple repetitive swallows supports an achalasia diagnosis
Chicago Classification: distal esophageal spasm Normal or increased ≥20% premature contractions ( distal latency <4.5 s) May be evolving type III achalasia
Chicago Classification: jackhammer Normal or increased ≥20% of swallows with distal contractile integral >8000 mm Hg/s/cm May be evolving type III achalasia if distal latency <4.5 s with ≥20% swallows
Opioid effect: Greater than upper limit of normal Normal, hypercontractile, or premature Can mimic EGJ outflow obstruction, type III achalasia, distal esophageal spasm, or jackhammer
Mechanical obstruction: Normal or increased Absent, normal, or hypercontractile

 

 

*Apart from the achalasia subtypes, these syndromes are not specific for achalasia and may have distinct pathophysiology, but instances occur in which they are optimally managed as if they were achalasia.

 

Traditional surgical options for achalasia

Laparoscopic Heller myotomy (LHM) has been the gold standard surgical approach for the past two decades. Myotomy (eg, Heller myotomy) is a procedure that involves the incision of the muscle fibers of the lower esophageal sphincter (LES) without disrupting the mucosal lining of the esophagus. It can be performed as an open or laparoscopic procedure.

 

POEM Procedure

The Peroral Endoscopic Myotomy (POEM) procedure (sometimes grouped into the procedures labeled Natural orifice transluminal endoscopic surgery or “NOTES”) allows for performing a myotomy of the LES using endoscopy rather than laparoscopy as with laparoscopic Heller myotomy (LHM). Sometimes described as Endoscopic Heller Myotomy, POEM has been described as a less invasive alternative to laparoscopic myotomy which involves the use of an endoscope that is guided through the esophagus to make an incision in the mucosa. This reportedly creates a submucosal tunnel for access to the LES and proximal stomach where muscle fibers in the LES and proximal stomach are cut. The internal incisions are closed with clips after the myotomy is complete. POEM combines the minimally invasive benefit of endoscopy with the durability of a surgical myotomy. The POEM technique has been used to treat spastic esophageal disorders (eg, spastic achalasia, distal esophageal spasm, jackhammer esophagus, or nutcracker esophagus) and severe gastroparesis refractory to medical therapy.

 

Alternatives to POEM procedure

Alternatives include esophageal dilation, LHM, and botulinum toxin injection.

 

Esophageal dilation is performed in a graded approach, starting with a small balloon (typically 30 mm), then progressing to larger balloons (35-40 mm) 2 to 4 weeks later. The balloons are placed at the level of the gastroesophageal junction and inflated slowly, in order to tear the muscle fibers in a controlled manner. Esophageal perforations are a potential complication. Long-term studies have estimated that approximately one-third of patients may need a repeat procedure.

 

Heller laparoscopic myotomy is a minimally invasive procedure in which the thick muscle of the lower esophagus and the upper stomach is cut to open the tight LES. The procedure involves 5 small incisions to insert the camera and surgical instruments. Reported success rates are high (>90%), with a 5-year follow-up study showing an 8% rate of symptom recurrence.

 

Endoscopic botulinum toxin is injected with a sclerotherapy needle approximately 1 cm above the esophagogastric junction. The complication rate is low and approximately 80% of patients experience immediate symptom relief. The effect diminishes over time, with more than 60% of patients reporting recurrent symptoms at 1 year.

 

Review of the published studies shows some advantages of this technique but also some disadvantages: 

 

Advantages
  • No surgical incisions
  • Less postoperative discomfort
  • It has been used successfully to treat recurrent dysphagia after LHM, avoiding the need for another laparoscopic operation. 

 

Disadvantages
  • Hospitalization is actually increased, PD is an outpatient procedure and LHM usually requires a 24 hour hospitalization, after POEM, patients are often hospitalized for 2–4 days
  • The post-POEM LES pressure is usually between 15 and 20 mm Hg, a known predictor for recurrence of symptoms;
  • The incidence of abnormal reflux as proven by pH monitoring is around 9% after LHM and fundoplication it is around 40–50% after POEM.
  • But most importantly, we have only very heterogeneous short to mid-term results from non-randomized trials: although some studies have shown excellent outcomes, others have documented a failure rate of 11% at 6 months and 18% at 1 year. Therefore, in order to establish the real value of POEM in the treatment of achalasia, long-term results of prospective and randomized trials are needed. 

 

Gastroparesis

Gastroparesis is a chronic disorder of gastric motility characterized by delayed gastric emptying of solids in the absence of mechanical obstruction. It can frequently result from longstanding diabetes mellitus and vagal nerve injury, or can be idiopathic in nature. Gastroparesis leads to postprandial nausea and vomiting, bloating, early satiety and discomfort. In severe cases, nausea and vomiting may cause weight loss, dehydration, electrolyte disturbances and malnutrition due to inadequate caloric and fluid intake. Gastric per-oral endoscopic myotomy of the pylorus (G-POEM) recently has been reported as minimally invasive therapy for gastroparesis.

 

Spastic esophageal disorders (SEDs)

Spastic esophageal disorders (SEDs) are characterized by hyperactive esophageal contractions of either abnormal propagation (premature contraction) or extreme vigorous contraction. Examples of SEDs include spastic or type III achalasia, distal esophageal spasm (DES), hypercontractile (jackhammer) esophagus, and hypertensive esophageal peristalsis (nutcracker esophagus).

 

ECRI Institute Health Technology Assessment

Evidence limitations. The evidence base consists of case series, most of which are retrospective, single-center studies. This evidence is at high risk of bias because of lack randomization, control groups, and blinding. Authors of the SRs noted that "outcome measurements and follow-up duration are quite heterogeneous across all studies" and called for RCTs and long-term follow-up to assess effectiveness.

 

Practice Guidelines and Position Statements

American College of Gastroenterology

ACG Clinical Guideline: Diagnosis and Management of Achalasia (2013)
Tailored Approach to Treating Achalasia

Recommendations:

  1. Either graded pneumatic dilation (PD) or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy for the treatment of achalasia in those fit and willing to undergo surgery (strong recommendation, moderate-quality evidence).
  2. PD and surgical myotomy should be performed in high-volume centers of excellence (strong recommendation, low-quality evidence).
  3. The choice of initial therapy should be guided by patients’ age, gender, preference, and local institutional expertise (weak recommendation, low-quality evidence).
  4. Botulinum toxin therapy is recommended in patients who are not good candidates for more definitive therapy with PD or surgical myotomy (strong recommendation, moderate quality evidence).
  5. Pharmacologic therapy for achalasia is recommended for patients who are unwilling or cannot undergo definitive treatment with either PD or surgical myotomy and have failed botulinum toxin therapy (strong recommendation, low-quality evidence).

 

Peroral endoscopic myotomy was discussed as an emerging therapy and stated to have promise as an alternative to the laparoscopic approach. The guidelines further stated that randomized prospective comparison trials are needed, and the procedure should be performed in the context of clinical trials.

 

ACG Clinical Guideline: Management of Gastroparesis (2013)

Several types of surgical interventions have been tried for treatment of gastroparesis: gastrojejunostomy, pyloromyotomy, and completion or subtotal gastrectomy. G-POEM is not mentioned in this guideline.

 

American Gastroenterological Association (2017)

The Clinical Practice Updates Committee of the American Gastroenterological Association proposes the following recommendations:

  1. in determining the need for achalasia therapy, patient-specific parameters (Chicago Classification subtype, comorbidities, early vs late disease, primary or secondary causes) should be considered along with published efficacy data;
  2. given the complexity of this procedure, POEM should be performed by experienced physicians in high-volume centers because an estimated 20–40 procedures are needed to achieve competence;
  3. if the expertise is available, POEM should be considered as primary therapy for type III achalasia;
  4. if the expertise is available, POEM should be considered as treatment option comparable with laparoscopic Heller myotomy for any of the achalasia syndromes; and
  5. post-POEM patients should be considered high risk to develop reflux esophagitis and advised of the management considerations (potential indefinite proton pump inhibitor therapy and/or surveillance endoscopy) of this before undergoing the procedure. 

 

American Society of Gastrointestinal and Endoscopic Surgeons (2014)

The American Society of Gastrointestinal and Endoscopic Surgeons issued evidence-based, consensus guidelines on the use of endoscopy in the evaluation and management of dysphagia, including esophageal achalasia. The Society recommended that: "… Endoscopic and surgical treatment options for achalasia should be discussed with the patient. In patients who opt for endoscopic management and are good surgical candidates, pneumatic dilation with large-caliber balloon dilators for the endoscopic treatment of achalasia was recommended… Long-term data and randomized trials comparing peroral endoscopic myotomy to conventional modalities of management are necessary before it can be adopted into clinical practice, but the procedure is becoming more widely used in expert centers."

 

In 2020, ASGE issued an evidence-based guideline on the management of achalasia. The methodologic quality of systematic reviews was assessed using the Methodological Quality of Systematic Reviews-2 (AMSTAR-2) tool and the certainty of the body of evidence was rated as very low to high based on the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) framework. ASGE rated the strength of individual recommendation based on the aggregate evidence quality and an assessment of the anticipated benefits and harms. ASGE used the phrase "we suggest" to indicate weaker recommendations and "we recommend" to indicate stronger recommendations. This guideline did not include either of the 2 available RCTs of POEM. Based on their evaluation, ASGE issued the following recommendations:

  • "We suggest POEM as the preferred treatment for management of patients with type III achalasia." (Very low quality evidence)
  • "In patients with failed initial myotomy (POEM or laparoscopic Heller myotomy), we suggest pneumatic dilation or redo myotomy using either the same or an alternative myotomy technique (POEM or laparoscopic Heller myotomy)." (Very low quality evidence)
  • "We suggest that patients undergoing POEM are counseled regarding the increased risk of postprocedure reflux compared with pneumatic dilation and laparoscopic Heller myotomy. Based on patient preferences and physician expertise, postprocedure management options include objective testing for esophageal acid exposure, long-term acid suppressive therapy, and surveillance upper endoscopy." (Low quality evidence)
  • We suggest that POEM and laparoscopic Heller myotomy are comparable treatment options for management of patients with achalasia types I and II, and the treatment option should be based on shared decision-making between the patient and provider." (Low quality evidence)

 

These 2020 ASGE guidelines were endorsed by the American Neurogastroenterology and Motility Society and the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES).

 

International Society for Diseases of the Esophagus

In 2018, the International Society for Diseases of the Esophagus published guidelines on the diagnosis and management of achalasia.34, The Society convened 51 experts from 11 countries, including several from the United States, to systematically review evidence, assess recommendations using the GRADE system, and vote to integrate the recommendations into the guidelines (>80% approval required for inclusion). Table 1 summarizes POEM recommendations.

 

Table 1. Recommendations for the Treatment of Achalasia

RecommendationLORGOR
POEM is an effective therapy for achalasia both in short- and medium-term follow-up with results comparable to Heller myotomy. Conditional Very low
POEM is an effective therapy for achalasia both in short- and medium-term follow-up with results comparable to pneumatic dilations. Conditional Low
Pretreatment information on GERD, nonsurgical options (pneumatic dilation), and surgical options with lower GERD risk (Heller myotomy) should be provided to patient. Good practice NA
POEM is feasible and effective for symptom relief in patients previously treated with endoscopic therapies. Conditional Very low
POEM may be considered an option for treating recurrent symptoms after laparoscopic Heller myotomy. Conditional Low
Appropriate training (in vivo/in vitro animal model) and proctorship should be considered prior to a clinical program of POEM. Good practice NA

 

GERD: gastroesophageal reflux disease; GOR: grade of recommendation; LOR: level of recommendation; NA: not applicable; POEM: peroral endoscopic myotomy.

 

Regulatory Status

POEM uses available laparoscopic instrumentation and, as a surgical procedure, is not subject to regulation by the U.S. Food and Drug Administration

 

Prior Approval:

Not Applicable

 

Policy:

Refer to for information related to Botulinum Toxin for Achalasia Pharmacy policy Neuromuscular Blocking Agents 05.01.02
Refer to for information related to gastric electrical stimulation policy 07.01.62

 

POEM procedure (sometimes this procedure is labeled Natural orifice transluminal endoscopic surgery or “NOTES”) for all indications, including but not limited to achalasia, spastic esophageal diorders, diverticula, and gastroparesis, is considered investigational.

 

Randomized trials are required to confirm its superiority over the currently established treatment modalities. Currently evidence does show that the incidence of abnormal reflux is increased in POEM versus laparoscopic Heller myotomy. The need for inpatient stay with this procedure continues to be extended versus established treatment modalities. The long-term results on health outcomes remain unknown at this time for both POEM, Z-POEM, D-POEM, and G-POEM procedures. The level of evidence for the POEM procedure at this time is of low-to very-low quality.

 

Procedure Codes and Billing Guidelines:

To report provider services, use appropriate CPT codes, Alpha Numeric (HCPCS level 2) codes, Revenue codes and / or diagnosis codes.

  • 43499 Unlisted procedure, esophagus
  • 43999 Unlisted procedure, stomach

 

Selected References:

  • Kahrilas, Peter J et al. “Clinical Practice Update: The Use of Per-Oral Endoscopic Myotomy in Achalasia: Expert Review and Best Practice Advice From the AGA Institute” Gastroenterology vol. 153,5 (2017): 1205-1211.
  • Khashab MA. K. Peroral endoscopic myotomy (POEM). UpToDate.
  • Pasha SF, Acosta RD, Chandrasekhara V, et al. The role of endoscopy in the evaluation and management of dysphagia. Gastrointest Endosc. Feb 2014;79(2):191-201. PMID 24332405
  • Vaezi MF, Pandolfino JE, Vela MF. ACG clinical guideline: diagnosis and management of achalasia. Am J Gastroenterol. Aug 2013;108(8):1238-1249; quiz 1250. PMID 23877351
  • Stefanidis D, Richardson W, Farrell TM, et al. SAGES guidelines for the surgical treatment of esophageal achalasia. Surg Endosc. Feb 2012;26(2):296-311. PMID 22044977
  • Inoue H, Minami H, Kobayashi Y et al. Peroral endoscopic myotomy (POEM) for esophageal achalasia. Endoscopy 2010; 42: 265–271.
  • Onimaru M, Inoue H, Ikeda H et al. Peroral endoscopic myotomy is a viable option for failed surgical esophagocardiomyotomy instead of redo surgical Heller myotomy: a single center prospective study. J Am Coll Surg 2013; 217: 598–605.
  • Bhayani NH, Kurian AA, Dunst CM et al. A Comparative Study on Comprehensive, Objective Outcomes of Laparoscopic Heller Myotomy With Per-Oral Endoscopic Myotomy (POEM) for Achalasia. Ann Surg 2014; 259: 1098–1103.
  • Hungness ES, Teitelbaum EN, Santos BF et al. Comparison of perioperative outcomes between peroral esophageal myotomy (POEM) and laparoscopic Heller myotomy. J Gastrointest Surg 2013; 17: 228–235.
  • Von Renteln D, Fuchs KH, Fockens P et al. Peroral endoscopic myotomy for the treatment of achalasia: an international prospective multicenter trial. Gastroenterology 2013; 145: 309–311.
  • Familiari P, Gigante G, Marchese M et al. Peroral endoscopic myotomy for esophageal achalasia. Outcomes of the first 100 patients with short term follow-up. Ann Surg 2014; e-pub ahead of print 30 October 2014.
  • Boeckxstaens GE, Annese V, des Varannes SB et al. Pneumatic dilatation versus laparoscopic Heller myotomy for idiopathic achalasia. N Engl J Med 2011; 12: 1807–1816.
  • Campos G M, Vittinghoff E, Rabl C et al. Endoscopic and surgical treatments for achalasia: a systematic review and meta-analysis. Ann Surg 2009; 249: 45–57.
  • Ramirez, M, Patti, M. Changes in the diagnosis and treatment of achalasia. Clinical and Translational Gastroenterology (2015) 6, e87; doi:10.1038/ctg.2015.8
  • Xu J, Chen T, Elkholy S, et al. Gastric Peroral Endoscopic Myotomy (G-POEM) as a Treatment for Refractory Gastroparesis: Long-Term Outcomes. Can J Gastroenterol Hepatol. 2018;2018:6409698. Published 2018 Oct 22. doi:10.1155/2018/6409698
  • Camilleri, M., Parkman, H. P., Shafi, M. A., Abell, T. L., Gerson, L., American College of Gastroenterology (2012). Clinical guideline: management of gastroparesis. The American journal of gastroenterology, 108(1), 18-37; quiz 38.
  • ECRI Institute Peroral Endoscopic Myotomy for Treating Achalasia (2018)
  • ECRI Institute HybridKnife (Erbe USA, Inc.) for Peroral Endoscopic Myotomy (2017) 
  • Marano L, Pallabazzer G, Solito B, et al. Surgery or Peroral Esophageal Myotomy for Achalasia: A Systematic Review and Meta-Analysis. Medicine (Baltimore). 2016;95(10):e3001.
  • Inoue H, Sato H, Ikeda H, et al. Per-oral endoscopic myotomy: a series of 500 patients. J Am Coll Surg 2015; 221:256–264.
  • Francis DL, Katzka DA. Achalasia: update on the disease and its treatment. Gastroenterology 2010; 139:369–374.
  • Lee Y, Brar K, Doumouras AG, Hong D4. Peroral endoscopic myotomy (POEM) for the treatment of pediatric achalasia: a systematic review and meta-analysis. Surg Endosc. 2019 Jun;33(6):1710-1720.
  • Evensen H, Kristensen V, Larssen L, Sandstad O, Hauge T, Medhus AW. Outcome of peroral endoscopic myotomy (POEM) in treatment-naive patients. A systematic review. Scand J Gastroenterol. 2019 Jan;54(1):1-7
  • Zaninotto G, Bennett C, Boeckxstaens G, et al. The 2018 ISDE achalasia guidelines. Dis Esophagus. Aug 30 2018. PMID 30169645
  • Roman S, Kahrilas PJ. Management of spastic disorders of the esophagus. Gastroenterol Clin North Am 2013; 42:27.
  • Yang J, Novak S, Ujiki M, et al. An international study on the use of peroral endoscopic myotomy in the management of Zenker's diverticulum. Gastrointest Endosc 2019.
  • Yang J, Zeng X, Yuan X, et al. An international study on the use of peroral endoscopic myotomy (POEM) in the management of esophageal diverticula: the first multicenter D-POEM experience. Endoscopy 2019; 51:346.
  • ECRI Institute: Gastric Peroral Endoscopic Myotomy for Treating Gastropareisis, Health Technology Assessment.
  • Aiolfi A, Bona D, Riva CG, et al. Systematic Review and Bayesian Network Meta-Analysis Comparing Laparoscopic Heller Myotomy, Pneumatic Dilatation, and Peroral Endoscopic Myotomy for Esophageal Achalasia. J Laparoendosc Adv Surg Tech A. Feb 2020; 30(2): 147-155. PMID 31364910
  • Martins RK, Ribeiro IB, DE Moura DTH, et al. PERORAL (POEM) OR SURGICAL MYOTOMY FOR THE TREATMENT OF ACHALASIA: A SYSTEMATIC REVIEW AND META-ANALYSIS. Arq Gastroenterol. Jan-Mar 2020; 57(1): 79-86. PMID 32294740
  • Khashab MA, Vela MF, Thosani N, et al. ASGE guideline on the management of achalasia. Gastrointest Endosc. Feb 2020; 91(2): 213-227.e6. PMID 31839408
  • Zhong C, Tan S, Huang S, et al. Peroral endoscopic myotomy versus pneumatic dilation for achalasia: a systematic review and meta-analysis. Eur J Gastroenterol Hepatol. Nov 2020; 32(11): 1413-1421. PMID 32516175

 

Policy History:

  • December 2020 - Annual Review, Policy Revised
  • December 2019 - Annual Review, Policy Revised
  • December 2018 - New Policy

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