Korean J Gastroenterol.  2019 Aug;74(2):110-114. 10.4166/kjg.2019.74.2.110.

Early Phase of Achalasia Manifested as an Esophageal Subepithelial Tumor

  • 1Division of Gastroenterology, Department of Internal Medicine, Presbyterian Medical Center, Jeonju, Korea. rocephine@naver.com
  • 2Department of General Surgery, Presbyterian Medical Center, Jeonju, Korea.
  • 3Department of Pathology, Presbyterian Medical Center, Jeonju, Korea.


The Chicago classification (CC) defines an esophagogastric junction outflow obstruction (EGJOO) as the presence of several instances of intact or weak peristalsis, elevated median integrated relaxation pressure above 15 mmHg, and a discrepancy from the criteria of achalasia. The revised CC addresses the potential etiology of EGJOO, including the early forms of achalasia, mechanical obstruction, esophageal wall stiffness, or manifestation of hiatal hernia. A 58-year-old woman visited the Presbyterian Medical Center with swallowing difficulty. The patient underwent a high resolution manometry (HRM) examination and was diagnosed with EGJOO. Chest CT was performed to exclude a mechanical obstruction as a cause, and CT revealed a subepithelial tumor (SET) at the upper part of the esophagogastric junction. Therefore, laparoscopic surgery was performed and eccentric muscular hypertrophy of the distal esophagus was observed. Longitudinal myotomy and Dor fundoplication were also performed. The histology findings of the surgical specimens were consistent with achalasia. This paper reports a case of early achalasia that was finally diagnosed by the histology findings, but was initially diagnosed as EGJOO using HRM and misdiagnosed as SET in the image study.


Esophagogastric junction outflow obstruction; Esophageal achalasia; Subepithelial tumor

MeSH Terms

Esophageal Achalasia*
Esophagogastric Junction
Hernia, Hiatal
Middle Aged
Tomography, X-Ray Computed


  • Fig. 1 (A) Barium esophagography showed a dilated esophagus with pooling and stasis of contrast and narrowing of distal esophageal segment. (B) Esophagogastroscopy showed a tight esophagogastric junction, but no abnormal esophageal lesion was seen.

  • Fig. 2 Topography of high resolution manometry showed elevated integrated relaxation pressure with intact or weak peristalsis; wet swallow number 7 and 8 (Solar GI HRM, Medical Measurement Systems, Enschede, Netherlands).

  • Fig. 3 Chest computed tomography scan to exclude the cause of mechanical obstruction revealed a suspicious subepithelial tumor at the upper distal esophagus of the esophagogastric junction, estimated to be approximately 20 mm in diameter (blue arrow).

  • Fig. 4 (A) Surgical biopsy specimen showing that normal muscles of the esophagus were hypertrophied with lymphocyte infiltrate around the tissue (H&E, ×100). (B) The normal nerve trunk was destroyed (S-100, ×100).

  • Fig. 5 Barium esophagography after surgery showed normal peristaltic activity and passing.


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