Achalasia: a disease of varied and subtle symptoms that do not correlate with radiographic findings.
OBJECTIVE
Dysphagia is the hallmark of achalasia, and when frequent or marked, it is believed to be a good predictor of the clinical and radiographic severity of disease. We aimed to evaluate the presence and severity of dysphagia and other symptoms in achalasia and to correlate these symptoms with findings on barium studies.


METHODS
The symptoms and radiographs of 38 patients with confirmed achalasia were reviewed, 20 prospectively, 18 retrospectively. The number of typical and atypical symptoms both initially and at the time of the barium esophagogram was tallied. The severity of typical symptoms at the time of the barium esophagogram was scored on a point system. A scoring system was also devised to rate the severity of the radiographic findings. Symptoms and radiographic findings were compared.


RESULTS
Dysphagia was the initial symptom in only 39% of patients, whereas heartburn, regurgitation, and slow eating occurred initially in 24%, 24%, and 16% of patients, respectively. At the time of the barium esophagogram, all symptoms were reported by a greater percentage of patients than at presentation. The median time period between initial symptoms and symptoms reported at the time of the barium esophagogram was 48 months (range 2-360 months). At the time of the barium esophagogram, the most frequently reported symptoms were slow eating and regurgitation (79% each), followed by dysphagia (76%), and stereotyped movements (including arching of the neck and shoulders, raising of the arms, standing and sitting straight, and walking) with meals (60%). The mean number (+/-SD) of initial achalasia-related symptoms per patient was 1.34 (+/-0.67) and increased to 5.50 (+/-2.05) at the time of the barium esophagogram. The mean typical symptoms score at the time of the barium esophagogram was 3.4 (+/-1.5). Most patients (97%) reported a greater number of achalasia symptoms at the time of the barium esophagogram than initially, despite a mean total radiological score of only 2.58 (out of a maximum of 10 points). There was no statistically significant relationship between the total number of symptoms at the time of the barium esophagogram and the total radiographic score (r = -0.05, and p = 0.77) and between the typical symptom score at the time of the barium esophagogram and the total radiographic score (r = -0.11, and p = 0.51) by Spearman coefficient.


CONCLUSIONS
Achalasia is a disease with many atypical and subtle symptoms, both initially and over time. Dysphagia is initially present in only 39% of patients and is not the most frequently reported symptom over time. Neither the severity nor the total number of achalasia-related symptoms correlates with the severity of radiographic findings.
Department of Medicine, Hospital of the University of Pennsylvania, University of Pennsylvania School of Medicine, Philadelphia 19104-4283, USA.
Michael E
ME
Blam
William
W
Delfyett
Marc S
MS
Levine
David C
DC
Metz
David A
DA
Katzka
D001466:Barium Sulfate / Q000008:administration & dosage; D003287:Contrast Media / Q000008:administration & dosage; D003680:Deglutition Disorders / Q000503:physiopathology; D003937:Diagnosis, Differential; D004931:Esophageal Achalasia / Q000000981:diagnostic imaging* / Q000503:physiopathology / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012189:Retrospective Studies; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric
D003287:Contrast Media; D001466:Barium Sulfate