Esophagectomy for Management of End-Stage Achalasia With Mega-Esophagus: A Case Report

Abstract

As therapies for achalasia have evolved over the past few decades, end-stage achalasia and its management are seen less in the developed world due to the success of pneumatic dilation, surgical myotomies, and endoscopic myotomy. We present two patients with end-stage achalasia and their subsequent surgical management. We report two patients with end-stage achalasia who underwent esophagectomy for mega-esophagus. The first patient is a 71-year-old male who presented with symptoms concerning for aspiration pneumonia. Subsequent CT demonstrated a mega-esophagus with a substantial food impaction. Imaging revealed a similar appearance on x-ray 10 years prior. He underwent endoscopic disimpaction and LES botulinum toxin injection in preparation for definitive treatment via McKeown esophagectomy one month later. The second patient is a 79-year-old male with known history of type 2 achalasia, who presented with two weeks of progressive dysphagia to both solids and liquids. He had undergone both pneumatic dilations and botulinum toxin injections in the past. Endoscopy demonstrated a large amount of impacted food and a 1 cm intrinsic stenosis at the esophageal cardia. His symptoms remained refractory to endoscopic interventions, and the patient ultimately underwent esophagectomy the following month. The optimal management of advanced or end-stage achalasia is not well defined. Esophagectomy is safe and effective but is generally reserved for patients who fail prior interventions to include pneumatic dilation, Botox injections, and surgical or endoscopic myotomies. Our first patient underwent minimal treatment prior to presentation with a profound, long-standing mega-esophagus with respiratory compromise. As a result, esophagectomy was selected as an initial definitive therapy. The second patient underwent multiple interventions, with esophagectomy chosen only once he became refractory to dilations. While end-stage achalasia is increasingly less common in the developed world due to success rates of pneumatic dilation and myotomies, there is still a role for esophagectomy, as demonstrated in these two patients. The current indication for esophagectomy is either disabling symptoms refractory to other interventions or radiographic evidence of sigmoid or mega-esophagus. Each indication is separately represented in these two patient cases. In both cases, the surgery was performed electively, and the treatment approach was tailored to disease extent and comorbidities.1715 Figure 1. First patient's chest CT demonstrating mega-esophagus