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Functional Esophagogastric Junction Obstruction with Intact Peristalsis: A Heterogeneous Syndrome Sometimes Akin to Achalasia

  • John R. Scherer
  • Monika A. Kwiatek
  • Nathanial J. Soper
  • John E. Pandolfino
  • Peter James KahrilasEmail author
Original Article

Abstract

Background

Some patients with suspected achalasia are found on manometry to have preserved peristalsis, thereby excluding that diagnosis. This study evaluated a series of such patients with functional esophagogastric junction (EGJ) obstruction.

Methods

Among 1,000 consecutive high-resolution manometry studies, 16 patients had functional EGJ obstruction characterized by impaired EGJ relaxation and intact peristalsis. Eight patients with post-fundoplication dysphagia and similarly impaired EGJ relaxation were studied as a comparator group with mechanical obstruction. Intrabolus pressure (IBP) was measured 1 cm proximal to the EGJ. Sixty-eight normal controls were used to define normal IBP. Patients’ clinical features were evaluated.

Results

Functional EGJ obstruction patients presented with dysphagia (96%) and/or chest pain (42%). IBP was significantly elevated in idiopathic and post-fundoplication dysphagia patients versus controls. Among the idiopathic EGJ obstruction group treated with pneumatic dilation, BoToxTM, or Heller myotomy, only the three treated with Heller myotomy responded well. Among the post-fundoplication dysphagia patients, three of four responded well to redo operations.

Conclusion

Functional EGJ obstruction is characterized by pressure topography metrics demonstrating EGJ outflow obstruction of magnitude comparable to that seen with post-fundoplication dysphagia. Affected patients experience dysphagia and/or chest pain. In some cases, functional EGJ obstruction may represent an incomplete achalasia syndrome.

Keywords

Esophagus Achalasia Dysphagia Manometry 

Notes

Acknowledgment

This work was supported by R01 DC00646 (PJK & JEP) from the Public Health Service.

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Copyright information

© The Society for Surgery of the Alimentary Tract 2009

Authors and Affiliations

  • John R. Scherer
    • 1
  • Monika A. Kwiatek
    • 1
  • Nathanial J. Soper
    • 2
  • John E. Pandolfino
    • 1
  • Peter James Kahrilas
    • 1
    Email author
  1. 1.Department of Medicine, Division of Gastroenterology, Feinberg School of MedicineNorthwestern UniversityChicagoUSA
  2. 2.Department of Surgery, Feinberg School of MedicineNorthwestern UniversityChicagoUSA

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