An Unusual Appearing Esophagus

Question: A 57-year-old woman with a history of intermittent episodes of mild dysphagia and chest pain without previous evaluations underwent a computed tomography scan of chest, abdomen, and pelvis with oral and intravenous contrast for staging of her recently diagnosed endometrial cancer. Although there were no signs of metastatic disease, she was found to have an unusual finding in her chest involving her esophagus. The axial view (Figure A) showed 2 distinct lumens in her esophagus with clear borders. The sagittal view (Figure B) clearly demonstrated 2 columns of tubular esophagus both communicating with the stomach distally at the gastroesophageal junction. Upper endoscopy (Figure C) revealed an opening along the edge of the gastroesophageal/fundic junction extending superiorly, but no openings were identified in the proximal esophagus. What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The patient was diagnosed with a long tubular duplication of her distal esophagus. Because she only had mild symptoms that were stable throughout her life, she was conservatively managed on proton pump inhibitors with plans to follow-up after treatment of her endometrial cancer. Esophageal duplication is a congenital anomaly occurring in 1 in 8000 births, and is believed to be due to aberrant separation of the primitive foregut tube into the esophagus and tracheobronchial tube between the fourth and fifth weeks of life.1Trappey A.F. Hirose S. Esophageal duplication and congenital esophageal stenosis.Semin Pediatr Surg. 2017; 26: 78-86Crossref PubMed Scopus (14) Google Scholar An esophageal duplication is composed of a well-developed coat of smooth muscle, an epithelial lining, and an attachment to the esophagus often via muscular attachment. There are 2 main types of duplications: cystic or tubular. There also is a third type called the diverticular form, which is observed rarely.1Trappey A.F. Hirose S. Esophageal duplication and congenital esophageal stenosis.Semin Pediatr Surg. 2017; 26: 78-86Crossref PubMed Scopus (14) Google Scholar Cystic duplications account for 80% of esophageal duplications, and usually appear as a mediastinal mass or a submucosal lesion on imaging. Tubular duplications are far less common, accounting for 20% of esophageal duplications. Tubular duplications usually communicate with the true lumen at either or both ends, and can share a long portion of the wall with the native esophagus.2Saha A.K. Kundu A.K. Tubular duplication of the oesophagus presenting with dysphagia.Singapore Med J. 2014; 55: e90-e92Crossref PubMed Scopus (7) Google Scholar Although tubular duplications can cause symptoms such as dysphagia, regurgitation, and chest pain, some patients can be asymptomatic or have very mild symptoms and be diagnosed incidentally. Patients with significant symptoms may need reconstructive surgery. In patients who are not ideal candidates for surgery, there have been case reports of a completely endoscopic management with success.3Familiari P. Landi R. Mangiola F. et al.Endoscopic management of a tubular esophageal duplication in a young adult.VideoGIE. 2020; 5: 455-457Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar