Case report: successful resection of a leiomyoma causing pseudoachalasia at the esophagogastric junction by tunnel endoscopy

Deng et al. BMC Gastroenterology (2016) 16:24 DOI 10.1186/s12876-016-0445-0 CASE REPORT Open Access Case report: successful resection of a leiomyoma causing pseudoachalasia at the esophagogastric junction by tunnel endoscopy Bin Deng1, Xue-Feng Gao1, Yun-Yun Sun1, Yuan-Zhi Wang1, Da-Cheng Wu1, Wei-Ming Xiao1, Jian Wu1 and Yan-Bing Ding1,2* Abstract Background: Pseudoachalasia is a rare disorder whose presentation strongly resembles idiopathic achalasia. Case presentation: Here, we present a case of a 42-year-old female patient with esophageal leiomyoma who was initially diagnosed with achalasia. On endoscopical investigation, however, it became apparent that she had pseudoachalasia as consequence of a leiomyoma at the esophagogastric junction (EGJ). The condition was successfully treated through submucosal tunneling endoscopic resection. Conclusion: This case suggests that submucosal tunneling endoscopic resection is a therapeutic u option for the treatment of pseudoachalasia caused by leiomyoma of EGJ. Keywords: Pseudoachalasia, Esophagogastric junction, Leiomyoma Background Achalasia is the most common functional gastrointestinal disorder. It is characterized by aberrant upper gastrointestinal motility due to loss of peristalsis in the lower two-thirds of the esophagus and impaired relaxation of the lower esophageal sphincter [1, 2]. Achalasia can sometimes be difficult to distinguish from pseudoachalasia, a term that covers a spectrum of disorders that can mimic the clinical and investigational presentation of idiopathic achalasia [3]. In the herein presented case, general clinical assessment as well as endoscopic, manometric, and radiologic studies suggested a diagnosis of achalasia, and as a consequence the patient was scheduled to undergo peroral endoscopic myotomy (POEM). However, on performing the endoscopy for POEM, a leiomyoma originating from the muscularis propria (MP) layer and located at the esophagogastric junction was found. A revised * Correspondence: 1 Department of Gastroenterology, the Second Clinical College of Yangzhou University, Yangzhou, Jiangsu 225001, China 2 Department of Gastroenterology, Yangzhou No. 1 People’s Hospital, 368# of HanJiang middle road, Yangzhou, Jiangsu 225001, China diagnosis of pseudoachalasia was made. As a consequence the patient was subsequently treated with submucosal tunneling endoscopic resection (STER). Case presentation A 42-year-old female patient was referred to the Department of Gastroenterology at the Second Clinical College of Yangzhou University, for long-standing complaints of solid and liquid dysphagia accompanied by episodes of regurgitation. She underwent two upper digestive endoscopies, on which the presence of food residues in the esophageal lumen and rebound passage through the gastroesophageal junction was observed. Gastroesophageal contrast radiography showed slight dilatation of the esophagus, an absence of primary peristalsis but presence of aperistaltic waves, and a narrow distal esophagus with a ‘bird’s beak’ aspect (Fig. 1). A CT scan was performed, but was unremarkable and did not reveal any esophageal lesion. Esophageal manometry demonstrated a hypertensive lower esophageal sphincter pressure in conjunction with incomplete relaxation and isobaric aperistaltic pressurizations, all consistent with © 2016 Deng et al. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Deng et al. BMC Gastroenterology (2016) 16:24 Fig. 1 Radiograph of the barium esophageal transit examination. The findings have characteristic appearance of achalasia type II achalasia (Fig. 2). The patient was counseled regarding the diagnosis of achalasia and scheduled for POEM. Informed patient consent was obtained before the procedure. The patient was intubated and brought under general anesthesia. Subsequently, upper digestive endoscopy was performed by using a conventional endoscope. Carbon dioxide insufflation was used throughout the procedure. At the esophageal mucosa, 10 cm proximal to the gastroesophageal junction, a 2 cm longitudinal mucosal Fig. 2 Esophageal high-resolution manometry of our patient. The results show that lower esophageal sphincter pressure is reduced, with concomitant relaxation and passage of lower esophageal sphincter seen upon swallowing. The results are concordant with achalasia type II Page 2 of 4 incision was made using a Hook knife to serve as the entry point for the tunnel in the planned POEM procedure. The mucosa was then separated from muscular layer starting at beginning of the tunnel and the endoscope was then used to enlarge the submucosal tunnel by further separation of mucosa and muscularis. When the channel reached the proximity of the esophagogastric junction, however, a white band of potential tumor tissue became apparent. On encountering this structure, it was decided to resect the putative tumor by separating it from the surrounding muscular layer under direct endoscopic view using an insulated-tip knife. The then mobilized tumor was excised out of the tunnel using a snare through the mucosal entry. The endoscope was then withdrawn from the submucosal tunnel and was capable of passing the gastroesophageal junction without apparent difficulty, thus the myotomy was aborted. Several metal clips were employed to close the mucosal incision (Fig. 3). Subsequent histological evaluation combined with relevant immunohistochemistry produced a definitive diagnosis of leiomyoma (Fig. 4). Now, 10 months postoperatively the patient is largely asymptomatic. Conclusions Achalasia is a primary esophageal motility disorder, which manifests itself through dysphagia to liquids and solid foods. Support for a diagnosis of achalasia can be obtained by endoscopic and radiological studies, but the mainstay of the diagnostic process consists of the manometric findings. Classically, three characteristic manometric features are present in achalasia: an elevated resting lower esophageal sphincter pressure, incomplete lower esophageal sphincter relaxation, and esophageal aperistalsis [4]. Pseudoachalasia is characterized by achalasia-like symptoms caused by secondary etiologies. Clinical, radiological, and endoscopic findings closely resemble those of achalasia, but the treatment and associated prognosis are markedly different [5]. Hence, correct discrimination between achalasia and pseudoachalasia is important, but it is frequently complicated by the absence of specific (...truncated)