What is the likely diagnosis?
a) eosinophilic oesophagitis
b) oesophageal candiasis
d) diffuse oesophageal spasm
e) oesophageal atony
In the video clip you will note "trachealisation" of the oesophagus with longitudinal furrows (in the clip this is seen best at the squamo-columnar junction) and white spots, due to eosinophilic micro-abscesses. The patient had a history of "allergies to seafood" and had to be careful not too eat too fast due to the risk of vomiting. However, in the last 10 years he had only required endoscopic removal of a food bolus on three occasions.
Whilst children often present with abdominal pain, vomiting and dysphagia, adults may also complain of dysphagia, heartburn, or a food bolus impaction. Patients frequently have a history of allergy (asthma, hay fever, eczema) and there is a male predominance (70% in adults).
Diagnosist criteria includes (1) clinical symptoms of esophageal dysfunction and (2) >15 eosinophils/HPF. As eosinophils may also be found in reflux oesophagitis, a lack of responsiveness to high-dose PPI is added to the list above by some authorities.
Althought the above criteria may seem straight forward, there are challenges such as standardisation of oesophageal biopsy protocols, uncertainty in how eosinophil counts are determined and variability in the size of microscope high-power fields. My personal preference is to take 2 biopsies at three levels in the oesophagus.
Histologically, the eosinophilic infiltrate can be surprisingly patchy. In addition, there may be eosinophilic micro-abscesses (easily seen as white spots in the video). In addition, up to one third of patients have a dense fibrotic response in the submucosa. This may be evident as "easy ripping" of the surface mucosa at intubation or after dilatation.
The condition is thought to be due to aberrant responses to environmental antigens (mainly food). There appears to be an antigen-driven hypersensitivity reaction characterized by a mixed IgE-dependent/delayed-type reaction at least partially attributable to IL-13 (a TH2 cytokine).
Treatment options include elimination diets or elemental diet as well as repeated short courses of topical fluticasone propionate. Those who believe in an overlap between reflux disease and eosinophilic oesophagitis would also advocate PPI therapy. Strictures can be dilated and there is evidence that symptoms then improve for at least a year.
There have also been some reports of improvement with the leukotriene receptor antagonist montelukast and promising early results with mepolizumab, a monoclonal antibody against interleukin-5.