Abstract
Background: Esophageal manometry is the gold standard for diagnosing achalasia. Typical findings are absent peristalsis and incomplete relaxation of the LES (integrated relaxation pressure (IRP) .15 mmHg). However, in a subgroup of patients with typical symptoms of achalasia, stasis on barium esophagogram and absent peristalsis onmanometry, LES relaxation is not impaired. The aim of our study was to further characterize these patients using distensibility measurements of the esophagogastric junction (EGJ) and to study the effect of treatment. Methods: Consecutive patients with typical symptoms of achalasia, no abnormalities on upper endoscopy, significant stasis on barium esophagogram, absent peristalsis but normal IRP were included. Distensibility of the EGJ was measured using impedance planimetry (EndoFLIP). Distensibility was defined as the minimal cross-sectional area (CSA) of the EGJ divided by balloon pressure at volumes of 20, 30, 40 and 50 ml (mm2/mmHg) and was compared to previously established data of 15 healthy controls. The cut-off for normality was determined at the lower 90th percentile of the EGJ distensibility at 50 ml in these controls. Symptom severity was assessed using the Eckardt score, a score ,4 was considered as treatment success. Measurements of EGJ distensibility and Eckardt score were repeated .3 months after treatment. Results: We included 9 patients (5 male; age 21-59 years) with typical symptoms of achalasia, Eckardt score 6 (5-7) (median (IQR)). On esophageal manometry failed contractions were observed in 5 patients, panesophageal pressurization in 3 patients and spastic contractions in 1 patient. The median IRP was 9.3 mmHg (3.712), baseline LES pressure was 8.6 mmHg (4.5-11.9). Distensibility of the EGJ was significantly reduced in patients compared to controls at all balloon volumes: 20 ml (1.97 ± 0.16 vs 2.46 ± 0.54 mm2/mmHg, P ,.05 (mean ± SEM)), 30 ml (1.81 ± 0.08 vs 2.67 ± 0.36 mm2/mmHg, P ,.0001), 40 ml (1.08 ± 0.12 vs 5.02 ± 0.58 mm2/mmHg, P ,.0001) and 50 ml (1.08 ± 0.11 vs 6.28 ± 0.65 mm2/mmHg, P ,.0001). All patients exhibited EGJ distensibility below the cut-off value set for normality (2.9 mm2/mmHg). Treatment was performed in 6 patients (4 pneumodilation, 2 Heller myotomy). Post-treatment, in all of these patients symptomatic improvement was seen (Eckardt 2 (1-2)) and a substantial increase in EGJ distensibility, to a value within the normal range (5.32 ± 0.9 mm2/mmHg) was observed. Conclusions: A subgroup of patients with typical symptoms of achalasia, significant esophageal stasis, absent peristalsis but no impaired LES relaxation on esophageal manometry can have impaired EGJ distensibility at impedance planimetry. These patients can be regarded as having achalasia and respond favorably to achalasia treatment.
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