Definitions of the levels of evidence (+,++,+++,++++) and the grades of the recommendations (weak or strong) are provided at the end of the "Major Recommendations" field.
Diagnosis and Preoperative Workup
Patients with suspected achalasia should undergo a barium esophagram, an upper endoscopy, and esophageal manometry to confirm the diagnosis (+++, strong).
Pharmacotherapy plays a very limited role in the treatment of achalastic patients and should be used in very early stages of the disease, temporarily prior to more definitive treatments, or for patients who fail or are not candidates for other treatment modalities (++++, strong).
Botulinum Toxin Injections
Botulinum toxin injection can be administered safely, but its effectiveness is limited especially in the long term. It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++, strong).
Among nonoperative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications. It should be considered in selected patients who refuse surgery or are poor operative candidates (++++, strong).
The use of esophageal stents cannot be recommended for the treatment of achalasia (++, strong).
Surgical Treatment of Achalasia
Laparoscopic myotomy can be performed safely and with minimal morbidity in appropriately selected patients by appropriately trained surgeons and leads to dysphagia control and improved quality of life in the majority of patients (++++, strong). A relatively small proportion of patients, however, will experience recurrent symptoms in the long term often associated with postoperative reflux.
Effect of Prior Endoscopic Treatments on Myotomy Outcomes
Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity, but the literature is inconclusive. A careful approach by an experienced team is advisable (++, strong).
Myotomy Versus Endoscopic Treatment
Laparoscopic myotomy with partial fundoplication provides superior and longer-lasting symptom relief with low morbidity for patients with achalasia compared with other treatment modalities and should be considered the procedure of choice to treat achalasia (++++, strong).
Type of Surgical Approach
Transabdominal is superior to transthoracic esophageal myotomy due to improved postoperative reflux control by the addition of an antireflux procedure, performed only when the myotomy is done transabdominally. Laparoscopic myotomy offers advantages regarding postoperative pain, length of stay, and morbidity compared to open myotomy. The laparoscopic approach also allows routine incorporation of an antireflux procedure after myotomy, and is associated with the lowest patient morbidity, and therefore, is the procedure of choice for the surgical treatment of achalasia in most patients (+++, strong).
Compared with laparoscopy, robotic assistance has been demonstrated to decrease the rate of intraoperative esophageal mucosal perforations (++, weak), but no clear differences in postoperative morbidity, symptom relief, or long-term outcomes have been described. Further study is necessary to better establish the role of robotic myotomy.
Role of Fundoplication After Myotomy
Patients who undergo a myotomy should also have a fundoplication to prevent postoperative reflux and minimize treatment failures (++++, strong).
The optimal type of fundoplication is debated (posterior vs. anterior), but partial fundoplication should be favored over total fundoplication, as it is associated with decreased dysphagia rates and similar reflux control (++, weak). Additional evidence is needed to determine which partial fundoplication provides the best reflux control after myotomy.
Length of Myotomy
The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2 cm on the stomach (+, weak).
Treatment Options After Failed Myotomy
Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++, weak), but endoscopic balloon dilation after myotomy is currently considered hazardous by most experts and should be avoided (++, weak).
Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++, strong).
Esophagectomy should be considered in appropriately selected patients after myotomy failure (+, weak).
Epiphrenic diverticula should be treated surgically when symptomatic. Given their frequent association with achalasia, esophageal manometry should be pursued to confirm the diagnosis of achalasia when they are identified. A myotomy at the opposite side of the diverticulum that goes beyond the distal extent of the diverticulum should be performed when achalasia is present. In this situation, concomitant diverticulectomy may be indicated based on the size of the diverticulum. When diverticula are not resected, endoscopic surveillance is advised. The optimal approach for their treatment needs further study, and surgeons should be aware of the relatively high incidence of postoperative leaks (+, weak).
Quality of Evidence
Both the quality of the evidence and the strength of the recommendation for each of the guidelines were assessed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. There is a 4-tiered system for quality of evidence: very low (+), low (++), moderate (+++), or high (++++).
Strength of Recommendations
Both the quality of the evidence and the strength of the recommendation for each of the guidelines were assessed according to the GRADE system. There is a 2-tiered system for strength of recommendation (weak or strong).