Post written by Zaheer Nabi, MD, DNB, from the Asian Institute of Gastroenterology, Hyderabad, India.

In this video, we performed peroral endoscopic myotomy in a young man diagnosed with sigmoid achalasia based on endoscopy, esophageal manometry, and barium swallow. During submucosal tunneling, a submucosal lesion was identified. Despite extensive dissection, the margins of the submucosal lesion could not be delineated. At this point, we performed endoscopic ultrasound which revealed a large, circumferential lesion arising from muscularis propria. Snare biopsy was obtained from the lesion and POEM aborted. Histopathology revealed a diagnosis of leiomyoma. Esophagectomy was performed in this case due to diffuse involvement of lower esophagus.  

The diagnosis of achalasia is established by classical findings on esophageal manometry. In our case, manometry suggested type I achalasia. However, submucosal tunneling and intra-operative endosonography revealed a large leiomyoma. Analyzing retrospectively, minimal resistance at gastroesophageal junction and circumferential bulge at lower end of esophagus were not typical of achalasia cardia in our case. Leiomyomatosis with diffuse involvement of esophagus and leading to sigmoidization of esophagus is extremely rare. Therefore, these cases may be  misdiagnosed as achalasia.

Sigmoidization of esophagus due to non-achalasia causes should be borne in mind especially in cases with atypical findings on endoscopy. A low threshold for imaging like contrast enhanced CT or EUS may be fruitful in these cases.

Coexistence of submucosal lesions (usually leiomyomas) with achalasia has been described in the literature. Small leiomyomas can be managed concomitantly with index POEM performed for achalasia. However, diffuse involvement of esophagus or leiomyomatosis leading to sigmoidization is extremely rare. Cross-sectional imaging shows diffuse esophageal thickening in these cases.

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