Appeal type: Experimental/Investigational
Decision: Overturned

Diagnosis: Achalasia
Treatment: Per oral endoscopic myotomy
The insurer denied the Per oral endoscopic myotomy.
The denial is overturned. 

The patient is a male with hypertension, hyperlipidemia, elevated body mass index (32.1), sleep apnea (on CPAP [continuous positive airway pressure]),  paroxysmal atrial fibrillation, possible hepatitis (remote), status post septoplasty, shoulder surgery, and left wrist repair.  

Pertinent to this review, the patient has a three-year history of symptomatic achalasia. This has manifest with dysphagia to both solids and liquids, frequent food regurgitation, weight loss, as well as nocturnal heartburn, and vomiting three times per week (telemedicine visit).  Achalasia with predominantly type II features has been confirmed by upper endoscopy, barium swallow/UGI (upper gastrointestinal series), and esophageal manometry. 

In treatment of achalasia, the patient has declined Heller myotomy, in lieu of which POEM (per oral endoscopic myotomy) (CPT 43499) has been advised, and is scheduled to be performed.

Yes, the health plan should cover the proposed treatment.

Achalasia is a rare motility disorder of the esophagus characterized by the absence of peristalsis and a defective relaxation of the LES (lower esophageal sphincter) resulting in an impaired bolus transport and stasis of food in the esophagus.[Boeckxstaens] Because its exact etiology still is unknown, current treatment options of achalasia are directed only at relieving the functional obstruction at the level of the LES (lower esophageal sphincters). Three manometric subtypes are identified based on the residual esophageal wave pattern: type I in which the esophageal body shows minimal contractility; type II, in which there is no peristalsis but intermittent periods of compartmentalized esophageal pressurization; and type III, in which there are spastic contractions in the distal esophagus. (1)

Rohof et al evaluated response in "patients treated with either pneumatic dilation (PD) or laparoscopic Heller myotomy (LHM).  Manometric tracings were classified according to the three Chicago subtypes. Forty-four patients had achalasia type I (25%), 114 patients had achalasia type II (65%), and 18 patients had achalasia type III (10%). After a minimum follow-up period of two years, success rates were significantly higher among patients with type II achalasia (96%) than type I achalasia (81%) or type III achalasia (66%). The success rate of PD (pneumatic dilation) was significantly higher than that of LHM (laparoscopic Heller myotomy) for patients with type II achalasia (100% vs 93%), but LHM (laparoscopic Heller myotomy) had a higher success rate than PD (pneumatic dilation) for patients with type III achalasia (86% vs 40%, difference was not statistically significant because of the small number of patients). For type I achalasia, LHM (laparoscopic Heller myotomy) and PD (pneumatic dilation) had similar rates of success (81% vs 85%)." (2)

Recommendations for treatment from the ACG (American College of Gastroenterology) for achalasia, include: 
1. Initial treatment: either graded pneumatic dilation or laparoscopic surgical myotomy with a partial fundoplication
2. Pneumatic dilation (PD) and laparoscopic Heller myotomy (LHM) should be performed in high-volume centers of excellence
3. choice of initial therapy should be guided by patients' age, gender, preference, and local institutional expertise
4. Botulinum toxin therapy is recommended in patients who are not good candidates for, or are unwilling or cannot undergo, more definitive therapy with pneumatic dilation (PD) or surgical myotomy 
5. Esophagectomy should be reserved for patients who have failed pneumatic dilation (PD) and/or myotomy and who are good candidates for surgery.

The ACG (American College of Gastroenterology) opines: "the efficacy of both PD (pneumatic dilation) and Heller myotomy decreases over time. Repeat treatment will be required by a good proportion of patients after 5 years." (3)

In the patientt's case, available records support that his achalasia is symptomatic but no therapeutics have yet been attempted.  To underscore the benefit of POEM (per oral endoscopic myotomy), Ngamruengphong et al. (10) assessed POEM per oral endoscopic myotomy) in patients with Heller myotomy-refractory achalasia. In a retrospective cohort study the clinical outcome data on 180 patients who had underwent POEM (per oral endoscopic myotomy) at 13 tertiary centers were divided into those who had undergone prior HM (Heller myotomy) or matched controls (non-HM). A total of 90 patients in each group were analyzed for technical success (length of myotomy, orientation of myotomy), clinical response (decrease in Eckardt score to less than (<) three), manometric data, procedure-related adverse events, and post-procedural symptoms. The median duration of symptom recurrence was five years before rescue POEM (per oral endoscopic myotomy). The most common reason for failed HM (Heller Myotmoy) was thought to be incomplete prior myotomy. The HM (Heller myotomy) group had a higher proportion of patients with prior pneumatic dilation (44% vs 26%). Technical success of POEM (per oral endoscopic myotomy) in postfailed (Heller myotomy) HM or no HM (Hellec mytomy) patients was comparable (98% vs 100%), with two failures in the HM (Heller myotomy) group attributed to severe submucosal fibrosis precluding submucosal tunneling. Clinical response was lower in patients with prior HM (Heller mytomy) (81%) compared with the non-HM (Heller myotomy) group (94%), with median follow-up of nine months. There were no significant differences in adverse events or rate of post-procedure reflux between the HM (Heller myotomy) and the non-HM (Heller myotomy) groups, indicating that POEM per oral endoscopic myotomy) is safe in patients with prior HM (Heller myotomy).

The cited literature supports that POEM (per oral endoscopic myotomy) is not investigational, and is an excellent option, for the patients indication. POEM (per oral endoscopic myotomy) is medically indicated (with greater likelihood of benefit than available standard approaches) for treatment of the patient's condition.