Esophageal Motility Disorders Treatment & Management

Gastroenterology consultation should be obtained for any patient with a suspected esophageal motility disorder for proper evaluation.

Transfer

Patients with achalasia, DES, or other motility disorders who need aggressive endoscopic or surgical interventions should be referred to centers with experienced gastrointestinal and surgical services.

Gastroenterologists dealing with such disease processes should be familiar with performance and interpretation of diagnostic and therapeutic procedures. This also is true for surgeons, who need to be familiar with the surgical intervention involved.

Outpatient monitoring

Patients require outpatient follow-up care. Changes in medical therapy often are needed, and, with progression of disease, alternative endoscopic or surgical interventions might be needed.

Good planning and awareness of complications mandate multidisciplinary follow-up care, involving primary care, gastroenterology, dietary, and surgery services as needed.

The primary underlying neuropathology process in patients with achalasia cannot be cured; therefore, the primary goal of treatment is symptomatic relief. ^[12] As the myenteric nerves do not regenerate, treatment goals are directed at relieving the physiologic obstruction at the level of the LES by surgical or endoscopic balloon disruption of the LES muscle or, less effectively, through medications that relax the LES smooth muscle. In the spastic motility disorders, relaxation of the esophageal body and LES smooth muscle lends some relief of dysphagia and atypical chest pain. In patients with scleroderma esophagus, treatment is more targeted, involving aggressive antireflux therapy and management of reflux complications (eg, stricture dilation).

Pharmacologic therapy

Current pharmacologic therapy can provide some symptomatic relief to patients in the early stages of achalasia when disease activity is mild or moderate. More definitive therapy is spared for patients with advanced disease. In patients with spastic esophageal motility disorders, current pharmacologic therapy provides varying degrees of symptomatic relief.

Smooth muscle relaxants, including nitrates and calcium channel blockers, were the first medications to be used in all patients with esophageal motility disorders. The greatest experience has been with isosorbide dinitrate and nifedipine. The efficacy of these agents was demonstrated in case reports and retrospective studies primarily. Other drugs used less extensively include different types of anticholinergics, amyl nitrite, nitroglycerin, theophylline, beta2-agonists, and, recently, phosphodiesterase inhibitors. The experience with these drugs is limited.

Noncardiac chest pain in the setting of spastic esophageal dysmotility often shows good response to antireflux therapy, even in the absence of typical gastroesophageal reflux symptoms. Reassurance and control of anxiety is extremely important in this setting. If antireflux therapy fails, alternatives include different classes of muscle relaxants mentioned above and pain modulators such as tricyclic antidepressants (TCAs), selective serotonin reuptake inhibitors (SSRIs), and trazodone. Some studies report positive results from behavior modification programs and biofeedback.

Chronic pain management with TCAs is effective in managing noncardiac chest pain that is resistant to other therapies.

Botulinum toxin injection

Botulinum toxin injections into the LES have been used in treating patients with achalasia. Botulinum toxin is a potent inhibitor of acetylcholine release from nerve terminals. A sclerotherapy needle is used to inject 80-100 units of botulinum toxin into the 4 quadrants of the LES.

This therapy may be a good alternative for poor surgical candidates, such as elderly patients or frail individuals. The major disadvantages are the high cost and the need for repeated therapeutic sessions.

Some data suggest efficacy in patients with DES and hypertensive LES dysfunction, especially focusing on symptomatic relief of pain, dysphagia, and regurgitation. More studies are needed to prove this effect.

Endoscopic therapy

Note the following:

• Esophagogastroduodenoscopy (EGD) with pneumatic dilation is the standard endoscopic therapy for patients with achalasia and can be performed on an outpatient basis. Forceful distension of the LES to 3 cm with disruption of the circular muscle layer is needed for symptomatic relief. This distension and disruption cannot be achieved with the standard bougie (to 56F), which provides temporary benefit at best. Balloon dilators specifically designed to treat patients with achalasia achieve adequate diameter for lasting effectiveness. Achalasia dilators are long, noncompliant, cylindrical balloons that are positioned fluoroscopically or endoscopically across the LES and then inflated to a certain characteristic diameter: 30 mm, 35 mm, or 40 mm.

• Technique details are variable among different practitioners.

• Response rates vary from 50-93%. If no response is seen after 2 successive balloon dilations, it is unlikely to work.

• Chest pain is felt during inflation, even under sedation. The major complication is perforation, which occurs at a rate as high as 8%. Patients should be observed for 3-6 hours before discharge, or, alternatively, a water-soluble contrast esophagram should be performed postdilation.

• If no perforation is noted, the patient's diet can be advanced slowly over a few days. Acid suppression should be used after vigorous dilation with significant mucosal tears, as they are at risk for subsequent gastroesophageal reflux. If perforation complicates a pneumatic dilation, intervention varies according to severity. Small or intramural perforations can be managed with a conservative approach, including IV antibiotics, nothing by mouth, and observation; large perforations or progression of symptoms requires surgical repair, which carries favorable prognosis if performed early. At the time of surgery, a myotomy can be coupled with a repair operation.

• After pneumatic dilation, the best outcome predictor is measuring the residual LES pressure. If the pressure is less than 10 mm Hg, the outcome usually is excellent. If the LES pressure is more than 20 mm Hg postdilation, the clinical outcome usually is poor. The dilation can be repeated, although poor initial results and rapid recurrence postdilation indicate a poor response to repeat dilation. The overall success rate with endoscopic dilation is reportedly 30-98% in different series. This therapy has no effect on future Heller myotomy if surgery ultimately is needed.

• Pneumatic dilation showed positive results in a subset of patients with DES and LES dysfunction. This is based mainly on case reports and case series. Further verification of its role in this setting is needed.

Similar to endoscopic balloon dilation for achalasia, surgical treatment targets to disrupt the LES (myotomy) and, therefore, to relieve the high pressures at the gastroesophageal junction. ^[13] Since the development of laparoscopic technique for LES myotomy, a trend has emerged to treat younger and older patients with myotomy who are deemed good surgical candidates. Excellent results have been reported in 80-100% of patients. In advanced cases, when the esophageal anatomy is markedly distorted or when malignancy develops, esophagectomy becomes the surgical procedure of choice. In patients with other esophageal motility disorders, surgery rarely is indicated because these disorders generally are tolerable and do not cause malnutrition or dehydration.

Heller myotomy

Heller myotomy is the operative procedure of choice in patients with achalasia.Technically, an approximate 3- to 4-cm incision is made from the distal esophagus, across the GEJ, and onto the cardia and fundus of the stomach. The incisional depth is through both layers of the muscularis propria but not through the muscularis mucosa.

Myotomy of the LES decreases the pressure across the gastroesophageal junction and eliminates dysphagia. Unfortunately, myotomy may lead to gastroesophageal reflux; therefore, fundoplication may be coupled with the myotomy at the time of the surgery.

Myotomy can be performed through either transthoracic or transabdominal approaches; however, a transabdominal technique is favored. The laparoscopy and thoracoscopy techniques decrease perioperative morbidity and hospital stay.

Efficacy is 60-100% in different series and is found to have a longer duration of efficacy compared to endoscopic balloon disruption.

The major postoperative complication of Heller myotomy is postmyotomy reflux. Reflux symptoms tend to be particularly severe due to aperistalsis in the esophageal body preventing proper acid clearance from the esophagus. Severe esophagitis, stricturing, and Barrett changes are observed in approximately 15% of patients postoperatively. Adenocarcinoma of the esophagus also is described.

Another significant complication is recurrent or persistent dysphagia, which is observed in 10-20% of patients postoperatively. Mortality is less than 0.2%.

In the European Achalasia Trial, treatment with pneumatic dilation or laparoscopic Heller myotomy was successful in a higher percentage of patients with type II achalasia than in patients with the type I or III form of the disease. Although success rates were high for both approaches in type II achalasia, the success rate was significantly higher in the group treated with pneumatic dilation. ^[14] However, relapse is common after pneumatic dilation; for good long-term outcomes, close follow-up and repeat dilation are required. ^[15]

Esophagectomy with gastric pull up or intestinal interposition

In patients with extremely advanced disease or refractory cases, 2 surgical options are esophagectomy with gastric pull up or intestinal interposition operation.

Other indications for esophagectomy are unresolved obstructive symptoms, carcinoma, or perforation during dilation. Perioperative mortality for this surgery is 4%.

Extended Heller myotomy

Unlike achalasia, where surgical myotomy is considered early in management, in patients with refractory DES, surgical therapy is the last resort when dysphagia or pain is severe.

The operative procedure involves an extended Heller myotomy of the LES, with proximal extension to the thoracic inlet. Compared to standard myotomy, this surgery is more complex and aggressive and should be used cautiously since the symptoms are frequently not relieved.

Dietary considerations

Dietary and eating habits should to be adjusted. Smaller, more frequent meals are preferred over the larger meals of the traditional 3 meals per day pattern. Liquid foods will ultimately pass down the esophagus and through the LES with less resistance.

Patients should be instructed to take smaller bites of food, with thorough chewing and slow swallowing. Patients should follow food boluses with liquids frequently.

Instruct patients to remain in the upright posture during and for some time after mealtime (at least 2 h) to enhance esophageal emptying. Straightening of the back, raising arms above the head, and standing can increase intraesophageal pressure and enhance esophageal clearance.

Activity

Avoiding exercise and avoiding lying down during and after mealtime can enhance esophageal emptying and improve symptoms.