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Within 4 years, a 58 (to 62)-year-old man attended the emergency department five times because of acute oesophageal food impaction. Other diagnoses included alcohol and tobacco abuse, arterial hypertension, and coronary sclerosis with cardiac infarction and coronary bypass.
On each presentation, the patient complained about mild retrosternal pain and an inability to swallow anything including his own saliva. Vital signs were normal, abdominal examination was unremarkable. Routine laboratory parameters were normal. At first presentation, electrocardiography and serum troponin were normal; chest X-ray suggested moderate lung oedema and no signs of aspiration.
Each time, wedged food was removed endoscopically from various locations in the upper, middle and lower oesophagi within hours after admission. One day after the first bolus removal, double-contrast oesophagography was performed (see figure 1). In endoscopies after subsequent episodes, there was no apparent discrete stricture, but the lumen appeared narrow and rigid. The mucosa demonstrated multiple rings with apparently generous calibre, and small, longitudinally aligned openings in the oesophageal wall (see figure 2). Oesophagoscopy after the third episode suggested candidiasis, which was confirmed histologically and successfully treated with an azole. Other times, histology showed inflammation, but no fungus.
What was the diagnosis?
Diffuse intramural pseudodiverticulosis is a chronic inflammation of the oesophagus characterised by the dilatation of submucosal glands.1 It preferably affects middle aged men. Alcohol and tobacco abuse are known risk factors, but additional pathogenetic mechanisms are suspected to exist. Recurrent mucosal candidiasis or eosinophilic esophagitis might be involved here, but many patients only show transitional candida colonisation and no raised eosinophils.2 ,2 3
Clinical signs are chronic dysphagia and repeated food impactions. Typical signs in double-contrast oesophagography are multiple, small, flask-like outpouchings corresponding to dilated ducts of intramural glands (see figure 3).1 4 High-resolution endoscopy will show these as small openings in the oesophageal wall. The rigid, narrow appearance with multiple non-stenosing rings is also referred to as ‘trachealisation’. Mucosa shows either a pink tint formed by multiple, very small red dots or a dull-white glassy swelling (see figure 4).5 Histology of mucosal biopsies will show uncharacteristic inflammation with a mixed cell-type infiltrate (see figure 5).
There is no known causal therapy. Proton pump inhibitors have little effect; treating candida, if present, can attenuate symptoms.
All procedures reported in this case were in accordance with the standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.
Contributors FH cared for the patient, performed gastroscopies and wrote the manuscript. AGS assessed radiological findings, researched literature and cowrote the manuscript. SL cared for the patient, researched literature and edited the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, conduct, reporting or dissemination plans of this research.
Provenance and peer review Not commissioned; externally peer reviewed.