Published in:
01-08-2018 | Case Report
Per-oral endoscopic myotomy for esophageal achalasia in a case of Allgrove syndrome
Authors:
Jun Nakamura, Takuto Hikichi, Haruhiro Inoue, Ko Watanabe, Hitomi Kikuchi, Tadayuki Takagi, Rei Suzuki, Mitsuru Sugimoto, Naoki Konno, Yuichi Waragai, Hiroyuki Asama, Mika Takasumi, Yuki Sato, Hiroki Irie, Katsutoshi Obara, Hiromasa Ohira
Published in:
Clinical Journal of Gastroenterology
|
Issue 4/2018
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Abstract
Allgrove syndrome, also known as Triple A syndrome, is a rare autosomal recessive genetic disease characterized by three signs: esophageal achalasia, adrenocorticotropic hormone refractoriness, and alacrima. A 31-year-old male presented to our hospital for treatment of difficulty swallowing caused by esophageal achalasia. Because he had complicating alacrima, a neurologic disease, and a family history of consanguineous marriage, a genetic neurologic disease was suspected. Then, a mutation in the achalasia–addisonianism–alacrima syndrome gene was identified. With the diagnosis of Allgrove syndrome, a per-oral endoscopic myotomy (POEM) was performed for esophageal achalasia. After the POEM, the symptoms and the esophageal pressure findings ameliorated quickly, with no recurrence noted throughout a follow-up of more than 1 year. Here, we report the first case of POEM performed for esophageal achalasia in Allgrove syndrome.