Esophageal achalasia
Esophageal cardiospasm

Symptoms:
• Dysphagia to both solid and liquid
• Chest pain and vomiting of undigested food
• Symptoms of aspiration such as nocturnal cough; possible dyspnea and pneumonia
Physical findings:
• If severe and prolonged, then possible weight loss
• Focal lung examination abnormalities and wheezing also possible
ETIOLOGY
• Etiology is incompletely understood.
• This motility disorder is likely due to viral or autoimmune degeneration of the esophageal myenteric plexus.
• Herpes zoster and measles virus have been implicated.
• Association with the HLA class II antigen, DQw1, has been noted.

DIFFERENTIAL DIAGNOSIS
• Angina
• Bulimia
• Anorexia nervosa
• Gastric bezoar
• Gastritis
• Peptic ulcer disease
• Esophageal disease:
GERD
Sarcoidosis
Amyloidosis
Esophageal stricture
Esophageal webs and rings
Scleroderma
Barrett’s esophagus
Lymphoma
Chagas’ disease
Esophagitis
Diffuse esophageal spasm
Infiltrating gastric cancer
Postvagotomy dysmotility

WORKUP
• Physical examination and laboratory analyses to rule out other causes and assess complications
• Imaging studies and manometry for diagnosis
LABORATORY TESTS
• Assessment of nutritional status with albumin and prealbumin if indicated
• CBC, ECG, stress test, stool and emesis for occult blood if diagnosis is in doubt
IMAGING STUDIES
Barium swallow with fluoroscopy may demonstrate the following findings:
• Uncoordinated or absent esophageal contractions
• An acutely tapered contrast column (“bird’s beak,” Fig. 1–9 )
• Dilation of the distal (smooth muscle portion) esophagus
• Esophageal air fluid level
Manometry may be indicated if barium swallow is inconclusive. Characteristic abnormalities are as follows:
• Low-amplitude disorganized contractions
• High intraesophageal resting pressure
• High LES pressure
• Inadequate LES relaxation after swallow
Direct visualization by endoscopy can rule out other causes of dysphagia.

Three modalities of treatment:
• Medical:
Smooth muscle relaxants including nitrates and calcium channel blockers are effective in up to 70% of patients.

Botulinum toxin injection will benefit up to 90% of patients but will require repeat injections.

• Mechanical dilation:
Fixed or pneumatic dilators may benefit up to 90%. Esophageal rupture or perforation is a rare complication that can be managed conservatively in some stable patients.
• Surgical
Open and thoracoscopic esophagomyotomy are available and effective (90%). This approach currently offers the most durable symptom relief. About 10% of patients undergoing surgery will have symptomatic reflux disease.

Prognosis is excellent in patients who respond to therapy. In long-standing disease or inadequately treated disease, there is an increased risk of squamous cell carcinoma. Chronic GERD, as a result of treatment, may be complicated by Barrett’s esophagus and malignant transformation.

Choice of and response to therapy will determine referral. Some surgeons may not be facile with thoracoscopic procedures.

Harris AM et al: Achalasia management, outcome and surveillance in a specialist unit, Br J Surg 87(3):364, 2000.

Spiess AE, Kahrilas PJ: Treating achalasia, JAMA 280:638, 1998.

Vaezi MF, Richter JE: Practice guidelines: diagnosis and management of achalasia, Am J Gastroenterol 94(12):3406, 1999.

Vaezi MF et al: Botulinum toxin versus pneumatic dilatation in the treatment of achalasia: a randomized trial, Gut 44:231, 1999.