Signs and symptoms

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Because the incidence of achalasia in children is so low, the majority of information regarding the diagnosis comes from adults. The earliest, and most common, symptom of achalasia is dysphagia. This dysphagia initially is for solids, but frequently progresses to dysphagia for liquids by the time treatment is sought.65-67 The next most common symptom in adults is regurgitation, which is non-acidic and non-bilious, owing to the contracted LES, and most often occurs right after eating or during sleep. Patients often find ways to accommodate these dysfunctions, and will ameliorate symptoms by such methods as drinking large volumes of water with meals, holding their arms above the head or performing a Valsalva maneu-ver.67-69 A summary of the most common presenting symptoms is provided in Table 5.1.

Table 5.1 Symptoms of achalasia (from reference 67)

Symptoms

Number of patients

Mean (%)

Mean range (%)

Dysphagia

1930

97

82-100

Regurgitation

1892

75

56-97

Weight loss

1675

58

30-91

Chest pain

1894

43

17-95

Heartburn

127

36

27-42

Cough

732

30

11-46

The presenting symptoms in children tend to correlate with patient age and can mimic those seen in adults, or they can be more vague, thus requiring a high degree of suspicion for diagnosis. Symptoms in older children (more than 7 years) tend to parallel those seen in adults, so dysphagia and regurgitation predominate, but with subster-nal chest pain and burning also appearing in about half the patients.70 Children aged less than 6 years, particularly infants, more commonly present with respiratory symptoms, complaints, similar to those of gastroesophageal reflux disease (GERD), occasional emesis and failure to thrive.12,71 Regurgitation and dysphagia are the most common symptoms, present in 83% and 71-80% of patients, respectively, followed by failure to thrive in 54-70%.70,72 Especially in non-verbal children, a diagnosis of achalasia should be entertained when presented with a patient experiencing significant chronic respiratory symptoms, such as choking, recurrent pneumonia, severe asthma, chronic bronchitis or chronic cough, because these are seen in 25-100% of patients.12,70 The physical examination tends to be unremarkable, but can reveal findings secondary to dysphagia (and a resultant decrease in oral intake) and food stasis, such as weight loss, malnutrition and halitosis.67

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