Achalasia
may exacerbate symptoms. Regurgitation of undigested food is also a common complaint. Other patients may present with respiratory symptoms secondary to chronic regurgitation and subsequent aspiration; up to 10% of patients may develop significant pulmonary complications. Patient symptomatology ranges from sub-clinical to severe dysphagia, chest pain, and heartburn (some patients may be initially misdiagnosed with acid reflux). Heartburn, in the setting of achalasia, usually is the result of fermentation of undigested food in the lower esophagus rather than actual gastric reflux itself. This emphasizes the importance of pre-operative esophageal motility studies in patients with symptoms of gastroesophageal reflux. Patients may also present with weight loss; however, significant weight loss in an elderly patient should raise the suspicion of malignancy.10,13,14
Important physiologic features caused by this disorder include: •
• • •
failure of the LES to relax, either partially or completely; elevation of LES pressure in some patients; and
loss of the coordination of LES relaxation in response to swallowing and esophageal contraction.
Diagnosis
Diagnosis of achalasia relies on having a high clinical suspicion of the disorder. Once suspected clinically, the following tests can help confirm clinical suspicion and identify the extent of the disorder (see Table 1).
Achalasia occurs most often between age 25 and 60 years.9 Initial
symptoms are often subtle and, as such, lead to delay in the clinical diagnosis. A detailed history plays an important role in the diagnosis of achalasia. The most common initial presenting complaint is progressive dysphagia of solids (99%), liquids (95%) or both. Other associated symptoms may include heartburn, chest pain, regurgitation, cough, globus sensation, hiccups, weight loss, and choking spells. In addition, patients typically cope with the dysphagia by changing positions and swallowing with the help of water.33 Dysphagia may be progressive or may stabilize at a certain severity. Complications of a retained food bolus in the esophagus may lead to aspiration, pneumonia, or lung abscess.
Barium esophogram is usually the first diagnostic modality performed. A classic radiological finding of achalasia is the “bird’s beak” appearance at the LES (from a dilated proximal esophagus and an abrupt tapering at the LES). Manometry is the most sensitive tool for the diagnosis of achalasia. The three characteristic manometric findings in achalasia are elevated resting LES pressure (usually > 45mmHg), incomplete LES relaxation, and aperistalsis in the smooth muscle portion of the body of the esophagus (see Figures 2 and 3). The esophagus may not contract or have simultaneous contractions. The term pseudoachalasia is used to describe patients with clinical symptoms or radiologic findings of achalasia who have normal manometric findings.
US GASTROENTEROLOGY & HEPATOLOGY REVIEW
Table 1: Diagnostic Tests, Their Utilities and Findings Diagnostic Test Utility
Findings Barium esophagram
First diagnostic test based on clinical
Dilated esophagus, failure of contrast to empty into
impression of achalasia stomach, “bird beak sign” May show lack of peristalisis Antegrade and retrograde motion of barium
Dilation of the esophageal body Endoscopy
May rule out malignancy Confirm the lack of peristalsis potential. For therapeutic intervention (using botulinum or balloon
and failure of LES to relax May feel a pop when going through the LES
possible loss of peristalsis in the distal smooth muscle segment of the esophagus; contractions occur, but they are weak simultaneous uncoordinated and, therefore, non-propulsive;
dilation). Endoscopic ultrasound: used as an adjunct to endoscopy for evaluating the LES length and contraction
Manometry
Most sensitive test for the diagnosis of achalasia
Elevated resting LES pressure (usually > 45mmHg),
incomplete LES relaxation, and aperistalsis in the smooth muscle portion
of the body of the esophagus High-resolution manometry has identified three subtypes of achalasia.36 All
three subtypes exhibit incomplete LES relaxation but can be distinguished by swallow-induced pressure response in the body of the esophagus.
• •
Type I achalasia – swallowing results in no significant change in esophageal pressure.
Type II achalasia – swallowing results in moderate simultaneous pressure that spans the entire length of the esophagus.
• Type III achalasia – swallowing results in abnormal lumen-obliterating contractions (spasms).
Clinically, this subclassification allows physicians to estimate a patient’s response to treatment (e.g. type II patients are more likely to respond to therapy than type III patients). In addition to the above described types, manometry may also identify “vigorous achalasia”, which is characterized by simultaneous severe contractions (>60mmHg).9
Upper endoscopy may confirm the lack of peristalsis and failure of LES to relax. A “pop” may be encountered when passing the scope through the LES. Endoscopy can also rule out malignancy and other etiologies of dysphagia. Endoscopic ultrasound (EUS) may be used as an adjunct to evaluate the LES length and contraction.
Management
Treatment is multi-modal including lifestyle changes, medical treatment, and surgical management.
Lifestyle changes may require the patient to eat slowly, chew thoroughly, and avoid eating too close to bedtime. Raising the head of the bed or sleeping with a pillow wedge promotes emptying of the esophagus by gravity.
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