Esophageal intramural pseudodiverticulosis complicated by a pneumomediastinum

A 64-year-old man was seen at the outpatient clinic complaining about progressive dysphagia since the last week. He suffered from a pronounced intent to vomit, an excessive salivation and a vague chest pain. Physical examination was unremarkable. Prior medical history revealed intermittent dysphagia the past two years with an episode of acute dysphagia one year ago due to a food impaction that was endoscopically removed. The esophagogastroduodenoscopy (EGD) at that time showed a benign esophageal stricture in the distal esophagus. Biopsy specimens that were taken from the stricture at that time showed active esophagitis and the presence of mycosis. The patient had been successfully treated with proton pump inhibitors and antimycotics for respectively four and two weeks and there was no recurrence of severe dysphagia until today. Because of a recurrent episode of severe dysphagia today, a new EGD was performed as next step in the evaluation of this case. Endoscopic evaluation showed a stenosis of the distal esophagus that could be passed by the scope. Because of the vague chest pain already present before the procedure, we performed a standard chest X-ray that revealed free air as a sign of a pneumomediastinum (Fig. 1A). We consequently performed a computed tomography (CT) scan of the chest which confirmed the presence of a pneumomediastinum with dominant localization around the esophagus from the level of the gastroesophageal junction to the cervical base (Fig 1B). Small intramural air bubbles in the proximal part of the esophagus were seen on these CT images suggestive for intramural esophageal pseudodiverticulosis (EIPD). The next diagnostic step was to perform an X-ray esophagogram with Telebrix gastro® (joxitalamaat, meglumine) which could not reveal a leakage of contrast but revealed pathognomonic signs of EIPD. It also showed a linear to circular loss of filling probably due to the stenosis in the distal esophagus that was visualized on the EGD (Fig. 2A,B,C,D,E). We admitted the patient in the hospital for transient fasting, proton pump inhibitors and treatment with intravenous antibiotics and total parenteral nutrition. Radiographic control after one week no longer showed the presence of free air as a sign of a pneumomediastinum. Oral feeding was restarted without problems and the patient was discharged out of the hospital. Follow-up consultation was scheduled and a balloon dilatation of the stenosis will be considered. Dysphagia is a common complaint of patients seen at the outpatient clinic as well as at the emergency room. The differential diagnosis is broad and includes common causes such as gastroesophageal reflux disease, strictures, webs, tumors, foreign bodies, functional disorders etc. that are well known by physicians. We report here EIPD as a cause of dysphagia that is less known by physicians and it is rarely described in the literature. EIPD is characterized by multiple, segmental or diffuse, flask-like outpouchings in the esophageal wall corresponding to dilated and inflamed excretory ducts of the submucosal esophageal glands. The underlying etiology still remains unclear. Symptoms can be various, but the predominant symptom is dysphagia. Esophageal strictures, esophageal candidiasis and gastroesophageal reflux disease are often associated. The diagnosis can be made by upper gastrointestinal endoscopy, but barium esophagography is the modality of choice. Complications of EIPD are rare and include broncho-esophageal and esophagomediastinal fistula, pleural and pericardial effusion, abscesses, gastrointestinal bleeding from a web-like stenosis or esophageal perforation with pneumomediastinum like in our case. The treatment for EIPD should be directed towards treating underlying associated conditions and relieving symptoms rather than the pseudodiverticulosis itself.status: publishe