Post written by Hirofumi Abe, MD, PhD, from the Division of Gastroenterology, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan.

We present an extremely rare case that was highly suspicious for congenital esophageal stenosis (CES) and successfully treated with modified peroral endoscopic myotomy (POEM). The patient was a 5-year-old girl with trisomy 21 and noticed dysphagia after starting solid food. We diagnosed achalasia because of preoperative endoscopic and radiographic findings, and POEM was scheduled.

However, once the procedure was started, the submucosal layer gradually became narrower as we approached the esophagogastric junction. The network of muscle fibers became dense and intricate, and separating the muscle layer from the mucosa became difficult. These features are very atypical for achalasia.

Thus, we became suspicious of fibromuscular thickening—type CES at this point. We converted to a modified approach during the procedure and successfully performed treatment.

POEM is becoming one of the main treatment options for pediatric achalasia. An increase in similar situations is anticipated as pediatric POEM becomes widespread.

Distinguishing CES from achalasia before POEM is sometimes difficult. Therefore, it is important to recognize the possible need for conversion to a modified approach when the aforementioned intraprocedural findings are observed during pediatric POEM.

A, Narrowed submucosal layer and intricate muscle fibers at the stenotic area. B, Myotomy at the stenotic area.

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