Atypical presentation of esophageal Dieulafoy: diagnostic and therapeutic challenges in end stage achalasia.

Dieulafoy lesions are rare vascular anomalies that can cause life-threatening gastrointestinal bleeding, most commonly in the stomach. Esophageal Dieulafoy lesions are exceptionally uncommon, particularly in patients with end-stage achalasia. We report a 46-year-old male with a history of achalasia and prior Heller myotomy who presented with acute hematemesis and hemorrhagic shock. Initial endoscopic and angiographic interventions failed to localize or control the bleeding. Computed tomography angiography revealed active bleeding in the distal esophagus. Surgical exploration identified a Dieulafoy lesion on the right lateral wall of the distal esophagus, which was successfully oversewn. Due to a severely dilated, nonfunctional esophagus, esophagectomy was performed, followed by delayed substernal gastric pull-up and cervical esophagogastrostomy. The postoperative course was complicated by acute respiratory distress syndrome and transient vocal cord paralysis, but the patient ultimately recovered well. This case illustrates the diagnostic and therapeutic challenges of esophageal Dieulafoy lesions in the context of end-stage achalasia. Early recognition, multidisciplinary management, and staged surgical reconstruction are critical for optimal outcomes in such complex presentations.