S4406 Pseudoachalasia Caused by Retroperitoneal Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

Introduction: Dysphagia can be caused by a variety of esophageal motility disorders, with achalasia being 1 of them. Pseudoachalasia, often due to underlying malignancy associated with paraneoplastic activity, can mimic achalasia and present a diagnostic challenge. Here, we report a rare case of dysphagia and suspected Type III achalasia, ultimately diagnosed with pseudoachalasia secondary to chronic lymphocytic leukemia/small lymphocytic lymphoma. Case Description/Methods: A 62-year-old man with a past medical history of diabetes mellitus and aortic valve stenosis status post TAVR on Coumadin who presented with dysphagia. His initial esophagogastroduodenoscopy showed LA grade A esophagitis. A spasm was seen in the lower 3rd of the esophagus. Esophageal biopsy was unremarkable and there was no evidence of eosinophilic esophagitis. He was placed on proton pump inhibitor without improvement of his symptoms. Subsequent esophageal manometry showed findings suggestive of esophagogastric junction (EGJ) outflow obstruction; however, 3/10 supine swallows were hypercontractile, and some hypercontractile swallows were seen with the upright swallows, suggesting possibilities of Type III achalasia. The barium esophagogram showed tapering of the distal esophagus with obstruction of passage of barium tablet suggesting achalasia. Endoluminal functional lumen imaging probe (EndoFLIP™) suggested EGJ outflow obstruction concerning the achalasia variant with a diameter of 8.2 and a distensibility index of 0.7 at 60 ml. The patient was placed on a low-dose sildenafil with consideration for Peroral endoscopic myotomy. However, the patient had a motor vehicle accident and a subsequent computed tomography scan that showed a retroperitoneal 10 × 9 × 9 cm mass encasing the aorta and extending to the EGJ. The biopsy of the mass suggested chronic lymphocytic leukemia/small lymphocytic lymphoma. The patient is getting chemotherapy and continues to take sildenafil. Dysphagia symptoms are completely resolved. Discussion: This case highlights the importance of considering the possibility of pseudoachalasia in patients with esophageal dysphagia and workup suggesting achalasia. The diagnostic approach included endoscopic evaluation with biopsy, esophageal manometry, barium esophagogram and EndoFLIP™ leading to the correct diagnosis. The patient’s symptoms resolved following treatment for malignancy, which demonstrates the importance of addressing the underlying condition in dysphagia management.