Treatment challenges of sigmoid-shaped esophagus and severe achalasia

BACKGROUND: Achalasia is a chronic motility disorder which may require surgical interventions to effectively manage patients’ symptoms and improve functional status. In late stage achalasia, patients may present with sigmoid-shaped esophagus which complicates traditional treatment approaches for achalasia as the esophagus is massively dilated and dysfunctional with delicate tissue integrity. Severe Achalasia with sigmoid esophagus imposes significant challenge to surgeons and treating physicians. Various assessment modalities and treatment approaches have been tried. Surgical treatment continues to be controversial. Some have argued that a less aggressive approach similar to that in early Achalasia results in satisfactory outcomes. Others have argued a more aggressive approach of esophagectomy is necessary. We present a review of the challenges encountered in each approach with recommendation for selecting the right treatment for the individual cases. CONCLUSIONS: Different treatment options for sigmoid type achalasia are available with ongoing controversy among the options. Heller myotomy with Dor fundoplication can provide satisfactory symptoms improvement and treatment outcomes.