title: Muscle layer histopathology and manometry pattern of primary esophageal motility disorders including achalasia.
abstract: BACKGROUND
  Histopathology of muscularis externa in primary esophageal motility disorders has been characterized previously. We aimed to correlate the results of high-resolution manometry with those of histopathology.
  
  
  METHODS
  During peroral endoscopic myotomy, peroral esophageal muscle biopsy was performed in patients with primary esophageal motility disorders. Immunohistochemical staining for c-kit was performed to assess the interstitial cells of Cajal (ICCs). Hematoxylin Eosin and Azan-Mallory staining were used to detect muscle atrophy, inflammation, and fibrosis, respectively.
  
  
  RESULTS
  Slides from 30 patients with the following motility disorders were analyzed: achalasia (type I: 14, type II: 5, type III: 3), one diffuse esophageal spasm (DES), two outflow obstruction (OO), four jackhammer esophagus (JE), and one nutcracker esophagus (NE). ICCs were preserved in high numbers in type III achalasia (n=9.4±1.2 cells/high power field [HPF]), compared to types I (n=3.7±0.3 cells/HPF) and II (n=3.5±1.0 cells/HPF). Moreover, severe fibrosis was only observed in type I achalasia and not in other types of achalasia, OO, or DES. Four of five patients with JE and NE had severe inflammation with eosinophilic infiltration of the esophageal muscle layer (73.8±50.3 eosinophils/HPF) with no epithelial eosinophils. One patient with JE showed a visceral myopathy pattern.
  
  
  CONCLUSIONS
  Compared to types I and II, type III achalasia showed preserved ICCs, with variable data regarding DES and OO. In disorders considered as primary esophageal motility disorders, a disease category exists, which shows eosinophilic infiltration in the esophageal muscle layer with no eosinophils in the epithelium.
authors.affiliation: Division of Gastroenterology and Hepatology, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Nakajima
authors.affiliation: Division of Gastroenterology and Hepatology, Niigata University Medical and Dental Hospital, Niigata, Japan.|Division of Gastroenterology and Hepatology, Saiseikai Niigata Daini Hospital, Niigata, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Sato
authors.affiliation: Division of Gastroenterology and Hepatology, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Takahashi
authors.affiliation: Division of Cellular and Molecular Pathology, Department of Cellular Function, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
authors.forename: G
authors.initials: G
authors.lastname: Hasegawa
authors.affiliation: Division of Gastroenterology and Hepatology, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Mizuno
authors.affiliation: Division of Gastroenterology and Hepatology, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Hashimoto
authors.affiliation: Division of Gastroenterology and Hepatology, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Sato
authors.affiliation: Division of Gastroenterology and Hepatology, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Terai
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004931:Esophageal Achalasia / Q000473:pathology / Q000503:physiopathology; D015154:Esophageal Motility Disorders / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008365:Manometry / Q000379:methods*; D008875:Middle Aged; D009130:Muscle, Smooth / Q000473:pathology* / Q000503:physiopathology*; D000074433:Myotomy / Q000379:methods
keywords: achalasia; high-resolution manometry; interstitial cells of Cajal; peroral endoscopic myotomy; primary esophageal motility disorders