Achalasia is a disorder of the esophagus that prevents normal swallowing to occur. Patients may feel as if they have a “lump in their throat" or have difficulty swallowing (dysphagia), particularly liquids. Typically, esophageal dysmotility develops slowly over months or even years. Achalasia is most commonly seen in the 3rd and 7th decade of life, but can occur at nearly any age and in both sexes.1
Difficulty Swallowing and Esophageal Dysmotility
The normal esophagus is a muscular organ that actively pushes solid food and liquids through to the stomach. This process, known as peristalsis, must also be coordinated with another muscle that guards the entrance to the stomach – the lower esophageal sphinctor (LES). The LES must relax at just the right time to allow passage. In latin, achalasia literally means, without relaxation and was first used to describe the condition as early as 1927.2
Symptoms of Achalasia
In addition to difficulty swallowing, those suffering form achalasia often report frequent regurgitation. Sometimes this may include undigested food. Clinically, those with achalasia will often learn to adapt to their condition by altering their eating and drinking habits to allow extra time for substances to pass. More infrequently, patients report chest pain of varying degrees associated with eating1.
What the Doctor is Thinking
Generally, when a patient comes in to see a physician with a series of symptoms, a doctor typically worries first about the worst thing that may be causing a symptom and then considers the likelihood that some other condition may be causing the problem. Likely, you are worried about the same things. Your doctor will probably want to run some tests to rule out other conditions that may be causing these symptoms. Conditions that may produce a similar presentation to achalasia include but are not limited to:
- Cancer of the esophagus (the most worrisome, but not most likely)
- Esophageal strictures
- Other mechanical motility disorders of the esophagus
- Chagas disease
Diagnosis of Achalasia
The gold standard for diagnosis of achalasia is a direct study of the pressures of the esophagus.4 This test is called esophageal manometry. Another test that helps confirm a diagnosis is endoscopy. This procedure entails a doctor placing a small camera into your upper digestive tract to look for signs of esophageal dysmotility. Both procedures are generally tolerated well with minimal discomfort.
Medical Management: Goals to Lower Resting Tension of the LES
Botulinum toxin (the same substance used in cosmetic procedures) is injected into LES. This functionally paralyzes the nerves that increase LES muscle tone. This leads to a therapeutic decrease in LES pressure.5 Although the therapy is initially as successful as other interventions, the effects can fade by as much as 41% per year. Repeated injections are usually recommended.6
Medications similar to those used for various cardiac conditions may also be used for achalasia. These medicines do not cure achalasia, but are meant to lessen the severity of symptoms. The two classes of medications that are commonly used are nitrates and calcium channel blockers. Unfortunately, many people find the side effects of these drugs difficult to tolerate. In addition, these medications are considered to be generally less effective than surgical or procedural interventions.7
Surgical Treatment: Dilation of the LES for Treatment of Achalasia
Options for surgical intervention in esophageal dysmotility include dilation, a procedure known as a Heller myotomy, and fundoplication.
This treatment for achalasia uses a flexible, soft dilator that is tapered and passed by the LES. Though often temporarily successful, it often requires repeat sessions to accomplish only limited dilation.6
This procedure consists of placing an inflatable balloon through the mouth and expanding the affected portion of the esophagus. While bougienage may be effective for smaller strictures, larger balloons may be ncessary for a controlled rupture of the muscle fibers composing the LES.1 Long term (i.e. 5-10 years) clinical response rates to balloon dilatations are reported at up to 85%, though response is highly variable and still may not approach the efficacy of a true surgical intervention.9
The Heller has long been considered the gold standard of intervention for achalasia. With the advent of minimally invasive laprotomy, the surgery can now be performed as outpatient day surgery. In a Heller myotomy, the LES is manually cut, allowing a permanent dilation of the LES to be realized.10 This procedure may be combined with another procedure called fundoplication to help reduce GERD from occurring post-operatively. GERD may develop in patients as a surgical complication in up to 30 percent of those undergoing a Heller myotomy.11
- Pohl, D, Tutuian, R, et al. Achalasia: an overview of diagnosis and treatment. Journal of Gastrointestinal and Liver Diseases, 2007; 297:302.
- Hurst A. The treatment of achalasia of the cardia: so-called ‘cardiospasm’. Lancet 1927;1:618.
- Podas T, Eaden J, Mayberry M, Mayberry J. Achalasia: a critical review of epidemiological studies. Am J Gastroenterol 1998;93:2345-2347.
- Goldenberg SP, Burrell M, Fette GG, Vos C, Traube M. Classic and vigorous achalasia: a comparison of manometric, radiographic, and clinical findings. Gastroenterology 1991;101:743-748.
- Pasricha PJ, Ravich WJ, Hendrix TR, Sostre S, Jones B, Kalloo AN. Intrasphincteric botulinum toxin for the treatment of achalasia. N Engl J Med 1995;332:774-778.
- Campos, GM, Vittinghoff, E, Rabl, C, et al. Endoscopic and surgical treatments for achalasia: a systematic review and meta-analysis. Ann Surg 2009; 249:45.
- Gelfond, M, Rozen, P, Gilat, T. Isosorbide dinitrate and nifedipine treatment of achalasia: A clinical, manometric and radionuclide evaluation. Gastroenterology 1982; 83:963.
- West, RL, Hirsch, DP, Bartelsman, JF, et al. Long term results of pneumatic dilation in achalasia followed for more than 5 years. Am J Gastroenterol 2002; 97:1346.
- Parkman HP, Reynolds JC, Ouyang A, Rosato EF, Eisenberg JM, Cohen S. Pneumatic dilatation or esophagomyotomy treatment for idiopathic achalasia: clinical outcomes and cost analysis. Dig Dis Sci 1993;38:75-85.
- Pai, GP, Ellison, RG, Rubin, JW, Moore, HV. Two decades of experience with modified Heller’s myotomy for achalasia. Ann Thorac Surg 1984; 38:201.
- Vogt, D, Curet, M, Pitcher, D, et al. Successful treatment of esophageal achalasia with laparoscopic Heller myotomy and Toupet fundoplication. Am J Surg 1997; 174:709.