J Korean Dysphagia Soc.  2022 Jul;12(2):134-137. 10.34160/jkds.2022.12.2.007.

Successful Management of Secondary Achalasia in a Patient with Myotonic Dystrophy

• Hong JW
• Yoon JA
• Park HE
• Shin YB

• KMID: 2532239
• DOI: http://doi.org/10.34160/jkds.2022.12.2.007

Abstract

Myotonic dystrophy (MD) is a systemic disease that affects any level of the gastrointestinal tract. Due to its rare occurrence, achalasia could be misdiagnosed due to symptoms being similar to gastroesophageal reflux (GERD). Moreover, there is no known appropriate treatment for achalasia in patients with MD. A 58-year-old man was diagnosed with type 1 MD. He complained of dyspepsia and chest pain, which showed no improvement even after administering GERD medication. Secondary achalasia was subsequently diagnosed after examining the lower esophageal sphincter by performing a video fluoroscopic-swallowing study (VFSS) and high-resolution manometry. Thereafter, the patient was successfully treated with balloon dilatation, and showed significant improvements in the symptoms. The patient remained well for 1 year. We report a rare case of secondary achalasia in an MD patient. Our study validates that VFSS might be useful for the early diagnosis of achalasia, and esophageal intervention (such as balloon dilatation) should be considered on confirmation of the affliction.

Keyword

Myotonic dystrophy; Achalasia; Dysphagia; Balloon dilatation; VFSS