Raj Dalsania, MD1, Lisa S. Cassani, MD1, Shreya M. Raja, MD2
1Emory University School of Medicine, Atlanta, GA; 2Emory University School of Medicine, Decatur, GA
Introduction: Pseudoachalasia is an uncommon condition that is difficult to distinguish from primary achalasia. Pseudoachalasia is usually caused by malignancy involving the gastroesophageal junction (GEJ); however, a paraneoplastic etiology has been reported in rare cases. Our aim is to describe the diagnosis and management of a case of paraneoplastic pseudoachalasia resulting from acetylcholine receptor (AchR) ganglionic (a3) antibodies.
Case Description/Methods: A 72-year-old male with a history of lung adenocarcinoma, bladder transitional cell carcinoma and prostate adenocarcinoma presented with acute chest pain, regurgitation, and dysphagia. Positron emission tomography scan showed increased distal esophageal uptake prompting a referral for endoscopy. Endoscopy showed a tortuous, spiraling esophageal body, distal esophageal ulcer, and narrowed GEJ (Figure 1). Ulcer biopsies returned benign, and endoscopic ultrasound (EUS) was performed. EUS of the GEJ showed diffuse thickening of the muscularis mucosa with no discrete mass. Motility evaluation was performed. Barium esophagram demonstrated a corkscrew esophagus and persistently narrowed GEJ (Figure 2). Esophageal manometry was attempted, but the catheter was unable to traverse the lower esophageal sphincter. Tracings of the esophageal body revealed spastic contractions with occasional pan-esophageal pressurization suggestive of possible mixed Type II/III achalasia (Figure 3). A paraneoplastic serum autoantibody panel was obtained and returned positive with AChR a3 antibody level of 220 pmol/L (normal < 53). Evaluation for recurrent malignancy was negative. Trial of a cholinesterase inhibitor and calcium channel blocker were attempted without improvement. Intravenous immunoglobulin therapy was declined by the patient. Endoscopic botulinum toxin injection to the esophageal body and LES was performed and resulted in sustained symptom relief.
Discussion: Paraneoplastic pseudoachalasia is rare, and requires a high index of suspicion for diagnosis. It may be the initial clinical sign of malignancy, or as in our case, a finding concerning for recurrent malignancy prompting re-evaluation. Prior reports suggest AChR antibodies are associated with adenocarcinomas. In patients with known malignancy who present with achalasia-type symptoms, a paraneoplastic autoantibody panel can assist with diagnosis. Treatment of the malignancy is the primary therapy, while medications and endoscopic interventions remain anecdotal.
Citation: Raj Dalsania, MD; Lisa S. Cassani, MD; Shreya M. Raja, MD. P0318 - A SPASTIC SPIRAL: PARANEOPLASTIC PSEUDOACHALASIA IN AN ELDERLY MALE WITH MULTIPLE MALIGNANCIES. Program No. P0318. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.