Management of Hypermotility Disorders Achalasia

Achalasia is the hallmark hypermotility disorder, wherein dysmotility results from death of intramural inhibitory neurons. The presentation typically includes dysphagia, with regurgitation and chest pain being reported by large subsets of patients. Management is palliative, as no treatment can reverse the neural loss (Dunaway and Wong, 2001). The neural defect leads to both aperistalsis and obstruction at the level of the nonrelaxing LES. Reversing obstruction is key to treatment, as aperistalsis is less relevant to the symptomatic presentation—just as in severe hypomotility.

Treatments are divided into those producing muscle relaxation and those producing muscle disruption. There is a separate chapter on achalasia management (see Chapter 19, "Management of Achalasia"). Relaxation of the LES can be accomplished independent of inhibitory nerves with calcium channel blockers and nitrates (Hoogerwerf and

Pasricha, 2001). Nifedipine 10 mg orally or isosorbide dini-trite 5 mg sublingually immediately before meals will transiently reduce LES pressure and improve esophageal symptoms in 70% of patients. Benefits per dose are brief; sustained responses over time occur in a minority of acha-lasia patients. Side effects of headache and orthostasis interfere with use of these medications. Consequently, smooth-muscle relaxants typically are used for short term gains while planning a more durable management approach.

Botulinum toxin injection, 80 to 100 units at the level of the LES, produces sphincteric relaxation by interfering with cholinergic innervation (Hoogerwerf and Pasricha, 2001; Gui et al, 2003). Initial symptomatic improvement occurs in > 80% of patients, but duration of response to a single injection averages < 8 months. As-needed injection for symptomatic relapses can keep nearly 60% of patients satisfied for up to 2 years, but 1 to 3 injections per year may be required to achieve this outcome. Neither smooth-muscle relaxants nor botulinum toxin injection improve emptying of the esophagus as much as they improve symptoms. Good esophageal emptying may be needed to prevent long term complications associated with achalasia (aspiration pneumonia, carcinoma). Consequently, botu-linum toxin also has been reserved for patients in whom more durable treatments are unacceptable or in whom its short term gains would be satisfactory.

Pneumatic dilatation with a 30 to 40 mm diameter balloon positioned across the LES remains the most common treatment worldwide for achalasia. A step-wise dilatation program beginning with the 30 mm balloon and progressing to larger balloons as needed for unsatisfactory outcome has become popular (Kadakia and Wong, 2001). This approach may reduce the risk of perforation, which approximates 3%. Perforation caused by pneumatic dilatation can be managed nonoperatively in selected cases, but thoracotomy is required in the majority. Surgical myotomy often can be performed at the time of thoracotomy. On average, two-thirds of achalasia patients can be managed with pneumatic dilatation, but an objective test of emptying as well as symptoms may be required to establish adequacy of treatment effect (Vaezi, 2001). Risk of perforation and subsequent thoracotomy remains the largest deterrent to this approach both by physicians and by patients and has helped shift the emphasis toward minimally invasive surgery in centers where the expertise is available.

Laparoscopic myotomy is now the preferred primary surgical treatment (Balaji and Peters, 2002). A fundoplication is performed at the time of the myotomy. Results 5 years following this procedure suggest that its outcome parallels that from the transthoracic approach. Nearly 90% of patients report at least a very good outcome; recurring dysphagia and the occurrence of new reflux symptoms remain the most important contributors to deterioration in long term gains. Anatomical distortion in the distal esophagus can persist following myotomy or worsen as the esophagus dilates over time. Emptying can progressively worsen such that nothing short of esophagectomy will produce the desired outcome. Results from esophagectomy for advanced achalasia are very good.

Achalasia does not mandate intervention for the patient with limited symptoms or in whom the diagnosis is made incidentally during investigation of unrelated complaints. Progressive dilatation of the esophageal body, however, can place the patient at risk for pulmonary complications and possibly the development of esophageal squamous cell carcinoma. Monitoring for progressive dilatation is recommended with barium studies initially at one- to two-year intervals if observation is the opted approach. Chest pain in achalasia may not respond to interventions directed at reducing LES pressure, improving emptying, and decreasing symptoms more directly related to impaired transit (dys-phagia, regurgitation). A trial of PPIs can be offered to patients with persisting chest pain after an otherwise adequate intervention has been accomplished. Exclusion of Candida esophagitis is recommended. Preliminary data indicate that low dose tricyclic antidepressant (TCA) regimens, as used for spastic disorders and unexplained chest pain, also are beneficial for chest pain management in achalasia.

Constipation Prescription

Constipation Prescription

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