S4408 Superior Vena Cava Syndrome: A Rare but Severe Complication of Mega-Esophagus in Achalasia

Introduction: Megaesophagus is a common complication of achalasia. It arises from mechanical obstruction at the lower esophageal sphincter, resulting in progressive dilation of the esophageal body and subsequent accumulation of food material. While typical symptoms include dysphagia and regurgitation, severe complications such as extrinsic tracheal compression and superior vena cava (SVC) syndrome are rarely reported. We present a challenging case of achalasia complicated by acute airway obstruction and SVC syndrome. Case Description/Methods: A 33-year-old man with a known history of achalasia, remotely treated with pneumatic balloon dilation, presented to the emergency department with acute-onset shortness of breath after ingesting a large food bolus. Computed tomography imaging revealed a markedly dilated, food-filled esophagus causing significant tracheal compression without perforation. His respiratory distress progressed to stridor, necessitating emergent intubation. Post-intubation, he developed facial and neck swelling with plethora and dusky discoloration, concerning for acute SVC syndrome. Bedside bronchoscopy demonstrated severe tracheal compression by the dilated esophagus, with minimal luminal patency. Post-intubation chest X-ray showed further dilation of the esophagus with increased intra-luminal air. Placement of an orogastric tube resulted in esophageal decompression and resolution of tracheal compression and SVC syndrome. Subsequent esophagogastroduodenoscopy revealed a markedly dilated, atonic, tortuous esophagus with retained food and blood, semi-circumferential ulcerations, and a widely patent lower esophageal sphincter, consistent with vigorous achalasia. Much of the food material was removed using a Roth net. A barium esophagram demonstrated a classic bird’s beak appearance with contrast passage into the stomach. Ultimately, the patient's diet was slowly advanced to a regular diet. He was discharged on proton pump inhibitor therapy with plans for further outpatient management of advanced achalasia in his home country. Discussion: This case highlights a rare but life-threatening complication of achalasia presenting as megaesophagus complicated by acute SVC syndrome, which can occur after endotracheal intubation. Prompt recognition and management, including esophageal decompression, are critical to prevent further deterioration and optimize outcomes.