A Case of Esophageal “Double Jeopardy”

Abstract

Question: A 66-year-old male outpatient was evaluated for progressive dysphagia to liquids and solids, which had slowly developed over 6 years. He also described a background of longstanding heartburn treated with proton pump inhibitor therapy for more than 10 years. In the last few years, his heartburn had largely resolved. His past medical history was significant for rheumatoid arthritis and hypercholesterolemia. Physical examination revealed no palpable lymphadenopathy, no cranial nerve deficits, and no significant cardiorespiratory or abdominal findings. A barium esophagram showed a 12-cm column of barium and no peristalsis (Figure A). High-resolution manometry revealed an absence of peristalsis, elevated basal lower esophageal sphincter pressure without relaxation, and panesophageal pressurization consistent with type II achalasia (Figure B). Endoscopy showed a fluid-filled esophagus with resistance to passage of the scope through the gastroesophageal junction. Endoscopic images are provided in Figure C. What is the diagnosis? Look on page 712 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The endoscopic images (Figure C) reveal a dilated esophagus with mucosal changes consistent with long-segment Barrett’s esophagus. Biopsies confirmed the presence of specialized intestinal metaplasia but in several biopsies high grade dysplasia and intramucosal cancer was detected (Figure D). A paraneoplastic panel, including ANNA-1/anti-Hu and calcium channel antibodies was negative. Positron emission tomography showed a tiny focus of activity in the distal esophagus. Endoscopic ultrasonography combined with endoscopic mucosal resection demonstrated a T1b stage esophageal adenocarcinoma. Achalasia and Barrett’s esophagus co-occurring in the same patient is a rare clinical scenario. The added complexity to this case was the finding of an early esophageal cancer. Theoretically, achalasia should protect against reflux, but this patient had decades of reflux before developing achalasia symptoms. In our patient, the intramucosal cancer was not the cause for dysphagia and esophageal dilation, but an incidental finding. Hence, this is not a case of pseudoachalasia owing to an obstructing adenocarcinoma or to a paraneoplastic phenomenon. Barrett’s esophagus and achalasia usually coexist several years after achalasia intervention, such as myotomy or pneumatic balloon dilation, presumably owing to the development of gastroesophageal reflux disease. In 2 studies where follow-up endoscopy was performed at intervals ranging from 5 to 9 years after myotomy, Barrett’s esophagus had developed in 7% and 8.4% of patients, respectively.1Leeuwenburgh I. Scholten P. Caljé T.J. et al.Barrett's esophagus and esophageal adenocarcinoma are common after treatment for achalasia.Dig Dis Sci. 2013; 58: 244-252Crossref PubMed Scopus (34) Google Scholar, 2Gossage J. Devitt P.G. Watson D.I. et al.Surveillance endoscopy at five or more years after cardiomyotomy for achalasia.Ann Surg. 2014; 259: 464-468Crossref PubMed Scopus (22) Google Scholar Only a handful of cases exist where Barrett’s esophagus has been noted to coexisting with achalasia before achalasia therapy, and to our knowledge this is the first reported case where intramucosal cancer was present at the index endoscopy. Furthermore, there may be an increased risk of adenocarcinoma among patients who develop Barrett’s esophagus after achalasia therapy, ranging from 0.2 to 20.1Leeuwenburgh I. Scholten P. Caljé T.J. et al.Barrett's esophagus and esophageal adenocarcinoma are common after treatment for achalasia.Dig Dis Sci. 2013; 58: 244-252Crossref PubMed Scopus (34) Google Scholar Whether the same cancer risk applies to patients with coexisting Barrett’s at the time of achalasia diagnosis is unknown. A population-based study from Sweden also suggested that achalasia is a risk factor not only for squamous cell cancer of the esophagus, but also adenocarcinoma.3Zendehdel K. Nyrén O. Edberg A. et al.Risk of esophageal adenocarcinoma in achalasia patients, a retrospective cohort study in Sweden.Am J Gastroenterol. 2011; 106: 57-61Crossref PubMed Scopus (84) Google Scholar Our case highlights that mucosal changes in an achalasia patient should not simply be attributed to esophageal stasis, and biopsies are mandatory. Our patient elected for endoscopic therapy of his early esophageal adenocarcinoma. This was achieved by endoscopic mucosal resection, after which no residual high-grade dysplasia or esophageal adenocarcinoma was identified. Attempts at achieving complete remission from Barrett’s esophagus were attempted by radiofrequency ablation, but this was complicated by considerable delayed bleeding, which responded to endoscopic therapy. At 7 months after successful endoscopic mucosal resection, the decision was made to treat his achalasia with a Heller myotomy. This procedure was successful, despite the surgeon noting extensive scarring as a result of the prior endoscopic therapies. We plan to continue surveillance of his residual nondysplastic Barrett’s esophagus at regular intervals indefinitely.