Treatment

Until such time as the primary cause of achalasia can be reversed, namely the loss of esophageal innervation by inhibitory neurons, treatment of achalasia must address the relief of symptoms. Since the primary disorders are aperistalsis and abnormal LES relaxation, the goal of non-invasive and invasive treatments is relief of the obstruction and its associated dysphagia. Medical treatment focuses on promoting LES relaxation, whereas endoscopic and surgical treatments address disruption of the LES muscle itself.

Medical options

Medical treatments have targeted improving LES relaxation through the use of anticholinergics, calcium channel blockers and nitrates. Anticholinergics were among the first drugs studied, but these generally showed no benefit.82-84 Of the calcium channel blockers, nifedipine has shown the most promise.85 A nifedipine dose of 10-30 mg prior to meals results in statistically significant decreases in mean LES pressure, as shown in a randomized, blinded trial by Traube et al.86 Despite these statistically significant changes, nifedipine-lowered resting LES pressure remains sufficiently elevated for subjec tive symptoms to persist; furthermore, radionu-clide transit studies fail to show significant improvement.85,86 The side-effect profile of nifedipine includes peripheral edema, headache, flushing and hypotension, which are seen relatively frequently during chronic treatment.82

Nitrates have also been tried, given the role NO seems to play in the disease process. Administered sublingually prior to a meal, isosorbide dinitrate (a long-acting version of nitroglyc-erin) will reduce mean LES pressure for approximately 60 min.87 A randomized, crossover trial evaluating sublingual isosorbide dinitrate and nifedipine showed both better subjective improvement and improved LES relaxation in the nitrate group versus the nifedipine group. However, side-effects (such as headache and hypotension) were greater, and radionuclide transport did not confirm improvement in four of eight patients reporting decreased symptoms.88 Therefore, medical treatment does seem to offer some subjective benefit to a subset of patients, but at a cost of significant side-effects, and a general progression of the disease in up to 50% of patients.82 The application of medical treatment seems relegated to those patients who are unwilling or medically unable to undergo more definitive treatment, or as a bridging measure until definitive treatment can be performed.67,73,82,85,86

Surgical options

The gold standard in treatment of achalasia is surgical esophagomyotomy - the modified Heller procedure. It is against this procedure that other surgical, and medical, treatments are compared. Surgical treatments include endoscopy with botu-linum toxin injection, forceful dilatation, open Heller myotomy and laparoscopic Heller myotomy.

Endoscopic botulinum toxin injection

With endoscopy playing an important role in the work-up of achalasia, it seems attractive to initiate therapy concurrently. Botulinum toxin (BoTox) is a potent inhibitor of presynaptic acetylcholine release, and can be injected into the LES through an endoscope. After a pilot study had demonstrated efficacy, Pasricha et al undertook a randomized, blinded and controlled study of BoTox.89 This study showed significant decreases in symptom scores, resting LES pressure and esophageal food retention. Approximately half of the injected patients, however, had no response or relapsed within 2 months of initial treatment and required either repeat injection or pneumatic dilatation. Subsequent studies in adults have shown that BoTox generally provides good initial results, but these improvements typically are not long-lasting.90-92 Follow-up showed that 10-35% of patients had no initial response, 30-40% relapsed within 4 months, and 38-67% had 'lasting' effects to an average of 1.3-2.5 years.90-92

An initial experience with BoTox in an 11-year-old child showed encouraging initial results, with repeat injection required 1 year after initial therapy.93 Two recent studies have evaluated the role of BoTox in a series of children.94,95 In both, the mean duration of symptomatic relief was short-lived (3-7 months) and required repeat injections or eventual Heller myotomy. Among the limited patients who did not progress to surgical treatment, a few showed lasting benefit over several years, and some opted for frequent repeated injections rather than undergoing surgery. While BoTox is generally considered safe, complications such as gastroesophageal reflux, esophageal inflammation and ulceration with hemorrhage have been noted.96 The consensus at this time relegates BoTox injection to patients who are unwilling or medically unsuitable for more invasive correction, or as a subsequent adjunct to patients who are symptomatic after myotomy or dilatation.67, 90,91,94,95

Esophageal dilatation

In contrast to the above-described treatments, esophageal dilatation addresses achalasia through forceful disruption of the LES muscle fibers. The largest experience with dilatation has been in adults, and current therapy involves the use of balloon dilators. The balloons are placed across the LES, rapidly inflated and then deflated after a period of 30 s to several minutes, depending on patient tolerance. As the procedure is performed under fluoroscopy, the dilatations are repeated until the waist of the LES is obliterated by the balloon. 'Excellent' to 'good' results, based on patient survey, are obtained in 65-93% of patients (this higher number was based on pooling results from multiple, graded dilatations).82,97-99 With long-term follow-up, trends were seen in patients who either failed initial treatment or quickly relapsed, with the most significant in younger age (under 45 years).73,100 While dilatation is relatively safe, early and late complications do occur. The most significant early complication is perforation (0-12%), which if recognized early can be treated conservatively with good effect.73,82,101

One of the major benefits of esophageal dilatations in adults is avoidance of general anesthesia, which is generally lost when treating children, making it a less attractive option. Despite showing some degree of success in a small series,102 the typical experience is poor long-term response requiring frequent re-dilatation, and eventually surgery, particularly in younger children.71,95,99,103 This requirement for multiple dilatations in children, as well as the need for monitored general anesthesia, also decrease the proposed benefit of lower hospital costs.104 Given these results, balloon dilatation plays a minimal role in the treatment of children with achalasia. Balloon dilatation should be reserved for patients unwilling to undergo surgery, or as a treatment adjunct in patients with residual symptoms after surgery.

Esophagomyotomy

Surgical esophagomyotomy for achalasia was first performed in Germany by Ernest Heller, and involved a laparotomy with both an anterior and a posterior esophagomyotomy.105 The procedure was modified by Zaaijer in 1923 to utilize only an anterior esophagomyotomy;106 all surgery for acha-lasia now employs a variant of this procedure.107 An example of the affected esophageal segment pre- and post-myotomy is shown in Figure 5.2. A consensus on the optimal surgical technique remains to be resolved. Traditional approaches have been through a standard midline laparotomy or a left thoracotomy, with equally good results.108-111 A review of the literature by Ferguson showed that, regardless of operative approach, an open Heller procedure resulted in symptomatic improvement in 89% of patients, a mortality rate of 0.3%, a reoperative rate of 2.9% and a postoperative GERD incidence of 10%.73

The benefits of minimally invasive surgery, decreased pain, shorter hospital stay, and improved cosmesis have prompted surgeons to apply this approach to esophagomyotomy. As with open surgery, both thoracoscopic and laparoscopic approaches are in current use, and by nature of similar results to the open surgery, minimally invasive procedures are becoming the standard.112-118 In addition to controversy over whether an abdominal or thoracic approach is better, there is no

Figure 5.2 (a) Transition between dilated (normal) and narrowed (abnormal) segment of esophageal achalasia prior to myotomy. (b) Performing myotomy with an endoscopic spreader. (c) Myotomy completed to the gastroesophageal junction. (d) Completed myotomy including dissection onto the lesser curvature of the stomach (circularis muscle seen distally).

Figure 5.2 (a) Transition between dilated (normal) and narrowed (abnormal) segment of esophageal achalasia prior to myotomy. (b) Performing myotomy with an endoscopic spreader. (c) Myotomy completed to the gastroesophageal junction. (d) Completed myotomy including dissection onto the lesser curvature of the stomach (circularis muscle seen distally).

consensus on the benefit of performing a simultaneous fundoplication. For example, at our institution we do not routinely perform concomitant fundoplication. Proponents of a fundoplication cite increased risk of late GERD, whereas opponents state that limiting the gastric portion of the esophagomyotomy to less than 1 cm limits the incidence of GERD and that a fundoplication may actually result in pseudoachalasia from making an over-tight wrap.73,119-124

The complications of Heller myotomy include intraoperative perforation (treated by over-sewing the tear, and buttressing with gastric fundus), recurrence of symptoms (typically the result of incomplete myotomy or addition of a fund-oplication that is too tight) and esophageal leak.67,70,109,125 Concern exists about performing a Heller myotomy after previous non-surgical therapies, as this may incur a higher complication rate. While previous treatment by balloon dilatations or BoTox injection seems to result in scar forma tion,126 it does not seem to affect subsequent esophagomyotomy.127,128

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