A Rare Case of a Life-Threatening Massive Upper Gastrointestinal Bleed and Airway Obstruction in a Patient With a Megaesophagus Secondary to Longstanding Achalasia

Achalasia is a relatively rare motor disorder characterized by esophageal aperistalsis and incomplete relaxation of the lower esophageal sphincter. In only 10% of patients, untreated or poorly managed achalasia can progress to esophageal dilation and eventual loss of total functionality resulting in a characteristic sigmoid dolichomegaesopahagus. In extremely rare instances, this sigmoid dolichomegaesopahagus can present clinically as acute airway obstruction or a fatal, life-threatening hemorrhage requiring immediate intervention. We present the case of a 65-year-old female with a past medical history of long-standing achalasia who had complaints of shortness of breath, chest pain, and two episodes of life-threatening hematemesis requiring a blood transfusion. An angiography illustrated significant distention of the esophagus occupying most of the right hemithorax and non-specific intraluminal fluid with a small amount of gas. Emergent esophagogastroduodenoscopy showed fibrosis and necrosis of the esophageal mucosa with food debris, suggesting that the bleeding was likely coming from an ulcer caused by pressure necrosis. The patient was hemodynamically unstable after the procedure and was transferred to another facility the next day for an esophagectomy. Patients with achalasia have an increased susceptibility to develop pressure ulcers due to increased shear force on the esophageal wall, increased moisture of the esophageal wall from prolonged contact of food boluses, and underlying malnutrition and weight loss from the indigestion of food causing atrophy of the mucosal barriers. The management of these ulcers is to treat and manage the underlying cause. Although there are no curative treatments for achalasia, symptomatic relief through both surgical and medical therapies are the mainstay of management, with an esophagectomy reserved for refractory cases or in patients who develop end-stage complications.