Medical Policy: 07.01.79
Original Effective Date: December 2018
Reviewed: December 2020
Revised: December 2020
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 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.
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.
|Syndrome||Median IRP||Esophageal contractility||Qualifications/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.
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.
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 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:
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) 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.
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.
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.
The Clinical Practice Updates Committee of the American Gastroenterological Association proposes the following recommendations:
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:
These 2020 ASGE guidelines were endorsed by the American Neurogastroenterology and Motility Society and the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES).
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.
|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.
POEM uses available laparoscopic instrumentation and, as a surgical procedure, is not subject to regulation by the U.S. Food and Drug Administration
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.
To report provider services, use appropriate CPT codes, Alpha Numeric (HCPCS level 2) codes, Revenue codes and / or diagnosis codes.
Wellmark medical policies address the complex issue of technology assessment of new and emerging treatments, devices, drugs, etc. They are developed to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. Wellmark medical policies contain only a partial, general description of plan or program benefits and do not constitute a contract. Wellmark does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Wellmark or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. Our medical policies may be updated and therefore are subject to change without notice.
*CPT® is a registered trademark of the American Medical Association.