An Unusual Cause of Recurrent Dysphagia.

Question: A 40-year-old woman, G2P0 at 11+6 gestational age, presented to the emergency department with a 1-week history of severe nausea and bilious vomiting with an inability to tolerate oral intake. She had been treated in the emergency department on 2 prior occasions for similar symptoms with intravenous (IV) fluids and anti-emetics. The patient had a history of achalasia diagnosed at the age of 34. She was initially managed conservatively but underwent a Heller myotomy and Dor fundoplication in September 2012 owing to worsening dysphagia. Two weeks postoperatively, she experienced intermittent dysphagia felt to be secondary to the fundoplication. This was treated with pneumatic balloon dilation with good effect. She underwent a second dilation in January 2013 for recurrent dysphagia and had been free of symptoms since that time. She was admitted to the gynecology service and treatment for hyperemesis gravidarum was initiated with IV fluids and anti-emetics. The gastroenterology service was consulted on admission for possible upper gastrointestinal (GI) bleeding in the context of coffee ground emesis. Her hemoglobin remained stable and she was conservatively managed with IV proton pump inhibitor for a possible Mallory-Weiss tear. Physical examination on presentation was unremarkable. On admission day 3, she began to complain of dysphagia. She developed nonbilious regurgitation of oral intake and an inability to manage secretions. This was accompanied by right upper quadrant and epigastric pain. She underwent a gastroscopy with an intention of performing pneumatic dilation for possible recurrent achalasia. Gastroscopy revealed a massively dilated esophagus with copious retained secretions and absence of peristaltic contractions. Within the distal esophagus, there was a large, round intraluminal esophageal mass that was compressible with biopsy forceps (Figure A). The 9.2-mm scope was easily passed through the gastroesophageal junction without resistance. Retroflexed view of the fundus and cardia revealed a tight gastroesophageal junction felt to be reflective of the previous fundoplication. A nasojejunal feeding tube was placed endoscopically for nutritional support. 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. A computed tomography scan of the thorax was arranged to further characterize the esophageal lesion, which demonstrated retrograde gastroesophageal intussusception (RGEI) with a large portion of the gastric fundus and body as well as omental fat intussuscepted into the dilated distal esophagus (Figure B, C). Because of the lack of improvement in her symptoms and based on the computed tomography finding, the patient underwent laparoscopic repair of the RGEI. A large portion of the stomach was found incarcerated in the distal esophagus, which was laparoscopically reduced followed by a redo fundoplication and anterior gastropexy. A small distal esophageal perforation was also repaired. The patient made an uneventful postoperative recovery with complete resolution of her dysphagia. She was tolerating a regular diet at the time of discharge. RGEI is an extremely rare condition that occurs when there is retrograde prolapse of all stomach layers (mucosa, muscularis, and serosa) into the esophagus, usually as a result of severe vomiting/retching in the context of an incompetent gastroesophageal sphincter mechanism.1Ujiki M.B. Hirano I. Blum M.G. Retrograde gastric intussusception after myotomy for achalasia.Ann Thorac Surg. 2006; 81: 1134-1136Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar This is distinguished from the considerably more common phenomenon of gastric prolapse, which involves invagination of only the mucosal layer. Increased intra-abdominal pressure such as that related to vomiting in pregnancy with hyperemesis gravidarum, eating disorders, gastric outlet obstruction, and even physical exertion increase the risk of developing RGEI.2Gowen G.F. Stoldt H.S. Rosato F.E. Five risk factors identify patients with gastroesophageal intussusception.Arch Surg. 1999; 134: 1394-1397Crossref PubMed Scopus (22) Google Scholar Hiatal hernias and increased gastric mobility owing to laxity of gastric attachments or previous surgeries resulting in the absence of gastric attachments also are significant risk factors.2Gowen G.F. Stoldt H.S. Rosato F.E. Five risk factors identify patients with gastroesophageal intussusception.Arch Surg. 1999; 134: 1394-1397Crossref PubMed Scopus (22) Google Scholar, 3Lukish J.R. Eichelberger M.R. Henry L. et al.Gastroesophageal intussusception: a new cause of acute esophageal obstruction in children.J Pediatr Surg. 2004; 39: 1125-1127Abstract Full Text Full Text PDF PubMed Scopus (13) Google Scholar Four cases of RGEI have been reported previously in patients with achalasia after myotomy and fundoplication.1Ujiki M.B. Hirano I. Blum M.G. Retrograde gastric intussusception after myotomy for achalasia.Ann Thorac Surg. 2006; 81: 1134-1136Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar This patient had several unique factors that likely led to the development of RGEI, including a massively dilated esophagus, incompetent gastroesophageal sphincter owing to previous myotomy and dilation, excessive gastric mobility owing to previous surgical intervention, and persistent violent vomiting and retching in pregnancy.