title: Esophagogastric junction distensibility identifies achalasia subgroup with manometrically normal esophagogastric junction relaxation.
abstract: BACKGROUND
  Manometric criteria to diagnose achalasia are absent peristalsis and incomplete relaxation of the esophagogastric junction (EGJ), determined by an integrated relaxation pressure (IRP) >15 mm Hg. However, EGJ relaxation seems normal in a subgroup of patients with typical symptoms of achalasia, no endoscopic abnormalities, stasis on timed barium esophagogram (TBE), and absent peristalsis on high-resolution manometry (HRM). The aim of our study was to further characterize these patients by measuring EGJ distensibility and assessing the effect of achalasia treatment.
  
  
  METHODS
  Impedance planimetry (EndoFLIP) was used to measure EGJ distensibility and compared to previous established data of 15 healthy subjects. In case the EGJ distensibility was impaired, achalasia treatment followed. Eckardt score, HRM, TBE, and EGJ distensibility measurements were repeated >3 months after treatment.
  
  
  RESULTS
  We included 13 patients (5 male; age 19-59 years) with typical symptoms of achalasia, Eckardt score of 7 (5-7). High-resolution manometry showed absent peristalsis with low basal EGJ pressure of 10 (5.8-12.9) mm Hg and IRP of 9.3 (6.1-12) mm Hg. Esophageal stasis was 4.6 (2.7-6.9) cm after 5 minutes. Esophagogastric junction distensibility was significantly reduced in patients compared to healthy subjects (0.8 [0.7-1.2] mm2/mm Hg vs 6.3 [3.8-8.7] mm2/mm Hg). Treatment significantly improved the Eckardt score (7 [5-7] to 2 [1-3.5]) and EGJ distensibility (0.8 [0.7-1.2] mm2/mm Hg to 3.5 [1.5-6.1] mm2/mm Hg).
  
  
  CONCLUSIONS
  A subgroup of patients with clinical and radiological features of achalasia but manometrically normal EGJ relaxation has an impaired EGJ distensibility and responds favorably to achalasia treatment. Our data suggest that this condition can be considered as achalasia and treated as such.
authors.affiliation: Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: F A
authors.initials: FA
authors.lastname: Ponds
authors.affiliation: Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: A J
authors.initials: AJ
authors.lastname: Bredenoord
authors.affiliation: Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: B F
authors.initials: BF
authors.lastname: Kessing
authors.affiliation: Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: A J P M
authors.initials: AJ
authors.lastname: Smout
mesh_terms: D000328:Adult; D015331:Cohort Studies; D004931:Esophageal Achalasia / Q000175:diagnosis* / Q000503:physiopathology*; D004943:Esophagogastric Junction / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008365:Manometry / Q000379:methods*; D008875:Middle Aged; D009126:Muscle Relaxation / Q000502:physiology*
keywords: EGJ distensibility; atypical achalasia; endoFLIP; high-resolution manometry; integrated relaxation pressure