Esophagitis dissecans superficialis (EDS) secondary to esophagogastric junction outflow obstruction (EGJOO): a case repo

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CASE REPORT

Esophagitis dissecans superficialis (EDS) secondary to esophagogastric junction outflow obstruction (EGJOO): a case report and literature review Tanya J. Olszewski1 · Shahin Ayazi1   · Katrin Schwameis1 · Stacey B. Miller2 · Kirsten Newhams1 · Blair A. Jobe1 Received: 2 June 2020 / Accepted: 17 September 2020 © Japanese Society of Gastroenterology 2020

Abstract Esophageal dissecans superficialis (EDS) is a rare disease with endoscopic findings of sloughing squamous tissue with underlying normal mucosa and had no known cause. The literature does support possible causality between the presence of an esophageal stricture and EDS however there has been no association to date between EDS and esophagogastric junction outflow obstruction (EGJOO). We present a case of newly diagnosed EGJOO in a patient with long standing gastroesophageal reflux disease who presented with dysphagia. Evaluation identified endoscopically normal mucosa and a diagnosis of esophagogastric junction outflow obstruction on high resolution impedance manometry. A month later, repeat endoscopy identified diffusely sloughing mucosa consistent with EDS. Endoscopic dilation followed by a robotic Heller myotomy with Dor fundoplication to relive the outflow obstruction resulted in resolution of EDS in this case. Keywords  Esophageal dissecans superficialis (EDS) · Esophagogastric junction outflow obstruction (EGJOO) · Gastroesophageal reflux disease (GERD) · Dysphagia · High resolution impedance manometry (HRIM)

Introduction Esophageal dissecans superficialis (EDS) is a rare, benign and poorly understood disease process of the esophagus, which has endoscopic findings of sloughing squamous tissue with normal underlying mucosa [1–3]. EDS has been linked to a wide variety of medications, infections, medical conditions and social or behavioral activities, although has also occurred as an idiopathic condition [2, 4–6]. The literature does support a relationship between the presence of an esophageal stricture and EDS, and this occurs likely to chronic esophageal retention [7]. To date, there has been no description of EDS occurring in the face of esophagogastric junction outflow obstruction (EGJOO) in the absence of stricture. We present a case of newly diagnosed EGJOO in a patient with dysphagia and long-standing gastroesophageal * Blair A. Jobe [email protected] 1



Esophageal Institute, Allegheny Health Network, 4815 Liberty Avenue, Suite 439, Pittsburgh, PA 15224, USA



Department of Pathology, Allegheny Health Network, Pittsburgh, PA, USA

2

reflux disease (GERD) who subsequently and rapidly developed EDS.

Case presentation The patient is a 73-year-old female with history of GERD who presented to the clinic with new onset dysphagia. She had symptoms of heartburn and regurgitation and had been taking proton pump inhibitors (PPI) for more than 5 years with the need for dose escalation following worsening of her symptoms. She subsequently developed cervical dysphagia and substernal burning for the 6 months prior to evaluation. An initial