S4423 Pseudoachalasia in an Achalasia Patient: A Case of a Ticking Time Bomb

Introduction: The differential for dysphagia is broad, with dysphagia aortica being an incredibly rare cause of pseudoachalasia. In a patient with longstanding, refractory achalasia who presents with progressive dysphagia, Occam’s Razor would suggest that the underlying cause remains achalasia itself. We present a case in which a patient with known achalasia developed concurrent pseudoachalasia due to dysphagia aortica. This case underscores the diligence of her gastroenterologist in identifying an unsuspected aortic aneurysm – an entity that the cardiothoracic surgery team deemed “a ticking time bomb.” Case Description/Methods: Our patient is a 31-year-old woman with history of type II achalasia, having undergone two laparoscopic Heller myotomies (with Dor fundoplication ultimately replacing the original Toupet) and a peroral endoscopic myotomy. Following her successful peroral endoscopic myotomy, she had 4 years of symptom control before experiencing progressive dysphagia. Esophagogastroduodenoscopy revealed mid-esophageal tortuosity, concerning for either extrinsic compression or evolving post-surgical changes in the setting of achalasia. Endoscopic ultrasound showed a thin open lower esophageal sphincter, a loose fundoplication, and a markedly dilated vascular structure in the mid-thoracic esophagus, exerting a clear mass effect. A gated computed tomography angiogram confirmed a large saccular aneurysm arising from the posterior medial aortic arch. The aneurysmal sac measured 4.6 × 4.2 × 2.5 cm and caused significant compression of the esophagus. Given the aneurysm’s size and saccular nature, cardiothoracic surgery expedited intervention within 2 weeks of diagnosis. She underwent a successful open thoracoabdominal aortic aneurysm repair. Further testing revealed underlying connective tissue disease as the likely etiology. Discussion: This case illustrates a rare instance of pseudoachalasia secondary to vascular compression from an aortic aneurysm in a patient with established primary achalasia. While the differential for dysphagia is broad, in patients with a history of refractory achalasia, it can be tempting to attribute symptoms to the primary diagnosis itself. This case highlights the importance of Hickam’s Dictum, reminding us that patients may have multiple coexisting diseases simultaneously. In this case, the gastroenterologist’s careful attention to alternate possibilities and thorough workup uncovered the true etiology of the patient’s symptoms, ultimately saving her life.