Tu1416 Swallow-Related Axial Shortening of the Esophagus Is Diminished in Patients With Type 3 Achalasia Esophagus

Abstract

Listen

Translate article

Introduction: Achalasia is a primary esophageal motility disorder characterized by loss of esophageal peristalsis and impaired lower esophageal sphincter (LES) relaxation. Pathophysiological studies have demonstrated loss of inhibitory nitrergic innervation in the esophagus of achalasia patients. The diagnosis of achalasia is made by a characteristic pattern of LES pressure and esophageal body contractions on esophageal manometry. However, the presence of atypical achalasia cases, or variant achalasia have been previously described with only a few case reports outlining the evolution of variant achalasia to achalasia. The identification and description of achalasia in evolution (AIE) can allow for a better understanding of the pathophysiology of the disease and for establishing early diagnosis. Objectives: To describe adult (age > 18 years) patients who have shown a temporal progression from AIE to achalasia with respect to their patient demographics, clinical presentation and esophageal manometry profiles. Methods: A retrospective chart review of all the high resolution esophageal manometry studies (EMS) in a tertiary referral center from January 2008 to July 2014. Results: Five confirmed cases of achalasia patients in whom AIE was initially described were identified. The mean age of the patients is 73.4 +/11. 1 years and 60% are males. All patients presented with varying durations (6 months to 15 years) of dysphagia to liquids and solids. Three of the five patients have a history of type 2 diabetes mellitus. AIE was applied when either the esophageal body peristalsis or LES relaxation did not meet manometric diagnostic criteria for achalasia. In all AIE patients the residual LES pressure was consistently elevated (25.5 +/9.6 mm Hg, normal < 15.0 mm Hg). Four of the five patients maintained some degree of esophageal body peristalsis. The progression of AIE to achalasia occurred over a range of 6 months to 10 years. When the diagnosis of achalasia was made, 3 patients had type 2 achalasia and 2 had type 3 achalasia. At the time of diagnosis of achalasia, all 5 patients demonstrated the characteristic manometry findings including simultaneous contractions and an elevated LES residual pressure at 29.5 +/17.6mmHg (range 19.6-60.9). Conclusions: Our observation that all AIE patients, albeit, a small number, progressed to either type 2 or type 3 achalasia suggests that type 1 achalasia may represent the later or more advanced disease. Thus, the identification of patients with AIE may allow for earlier awareness of possible disease progression, thereby facilitating the initiation of timely investigations and management strategies. Larger and longer-term study is warranted to further characterize the manometric criteria of AIE.