1778 Too Stubborn to Contract: A Case of End Stage Achalasia Presenting as a Sigmoid Esophagus

Abstract

INTRODUCTION: Sigmoid esophagus is a form of end stage achalasia characterized by massive dilatation and tortuosity of the esophagus. It is associated with poor response to conventional treatment and poses a significant challenge in the management. We present a case of an elderly female with achalasia who presented with sigmoid esophagus and was not considered a surgical candidate due to comorbidities. CASE DESCRIPTION/METHODS: An 84-year-old female with medical history of achalasia, colon cancer, heart failure and end-stage renal disease, admitted for aspiration pneumonia and respiratory failure requiring mechanical ventilation. Her achalasia was diagnosed three years prior to the presentation and was treated with botulinum toxin injection multiple times. Physical exam showed copious secretions in the oral cavity. Laboratory data showed leukocytosis. Chest CT showed a markedly dilated and tortuous esophagus with 10.2 cm diameter in maximum dimension. Upper endoscopy revealed a dilated and patulous lower esophagus with a tight gastroesophageal junction without any obstructing mass. A large amount of food was noted in the entire esophagus which was removed by a Roth net. Achalasia was considered end stage and the patient was deemed not a candidate for surgical treatment of achalasia in view of the comorbidities. A percutaneous gastrostomy tube was placed. DISCUSSION: Achalasia is an esophageal motility disorder characterized by esophageal aperistalsis and failure of the lower esophageal sphincter to relax during deglutition. Sigmoid esophagus is a severe form of achalasia characterized by a tortuous and massively dilated esophagus more than 6 cm that resembles sigmoid colon. Despite the treatment by the conventional methods like surgical myotomy, pneumatic dilation or botulinum toxin, 5% of the patients may develop an end stage achalasia. Dysphagia, regurgitation and recurrent aspiration pneumonia are the main clinical features. Imaging and esophagogram show dilated esophagus. Endoscopy shows tortuous dilated lumen and friable mucosa with food debris. Manometry is usually not needed to diagnose sigmoid esophagus as the esophagogram and endoscopy are diagnostic. Whereas, Heller’s myotomy is the treatment of choice in achalasia, it is regarded inadequate for the treatment of sigmoid esophagus due to operative difficulty, and esophagectomy is considered as the most appropriate option. However, the esophagectomy is associated with 5-10 % mortality and is not an option for patients with multiple comorbidities.