Eosinophilic Esophagitis


Brief History:

In 1977 the first published case of eosinophilic esophagitis (EE) was a 51 year old male. From 1978-1990 there were several cases reported with symptoms of dysphagia, heartburn, vomiting (which we define as feeling nauseated before gastric contents are expelled as opposed to regurgitation) and chest pain. In 1995 13 males with EE responded to steroids. Another report in the same year consisted of 10 children with EE that were treated with gastroesophageal reflux therapy but only responded later to an amino acid formula diet. In 1998 20 children with EE were treated with oral steroids and all 20 were resolved in 1 month.

Epidemiology:

The epidemiology of EE is still obscure. In children with gastroesophageal reflux symptoms, 68-94% are unresponsive to PPI prescription therapy. The prevalence of EE is unknown. However, in one Australian community with a population of 198,000, 19 cases of EE were diagnosed in a 21 month period.(Croese, J et al.: Gastroentest Endosc 2003;58:516-522.) Male predominance in EE was reported in 2004 by Arora and Yamazaki of Mayo Clinic College of Medicine. They reviewed reports of male predominance from eight study locations. Male predominance in children was noted in 85 of 117 (73%) and in adults 70 of 94 (75%). At the Joy McCann Culverhouse Center for Esophageal & Swallowing Disorders male predominance has been noted in 42 of 62 (68%). Furthermore, atopic conditions (Asthma, Dermatitis, Food Allergy) are known to be frequently associated with EE in children and adults.

Clinical Presentation:

Clinical presentation for children includes vomiting, regurgitation, abdominal pain, dysphagia, solid food sitophobia and is more common in white males. Clinical presentation for adults includes dysphagia, food impactions, GERD symptoms with or without heartburn, vague high epigastric/chest pain, nausea, vomiting, solid food sitophobia, and a history of slow eating since childhood. Adults are usually white males under 40 years old.

Endoscopy:

In many cases EE will appear with ringed strictures. During upper endoscopy the endoscope should pass easily, however the mucosal lining is extremely fragile in the ringed or narrowed sections. The ringed strictures may appear at any level, but primarily in the mid esophagus as shown in the following figure.

Figure 1.Ringed strictures of EE. The white spots or patches indicate concentrations of eosinophils on the mucosal surface.

Figure 2.The esophagus will have an irregular mucosal pattern that appears as railroad track-like linear creases running perpendicular to the ringed strictures. This finding in EE is accentuated by chromoendoscopy using methylene blue dye to mark the creases.

Pathology:
Biopsies should be taken at multiple levels, mid, proximal, and distal with 2 specimens taken at each level since eosinophils may be patchy. The stomach and duodenum should be biopsied to examine for the possibility of associated eosinophilic gastroenteritis. Bouin's fixative should not be used as it makes eosinophil definition difficult. Pathologists should be requested to count eosinophils under a high powered field (hpf) - 400X. Diagnosis of EE is confirmed when 2 fields average >15eosinophils/hpf. Eosinophils are orange-red in color with a black nucleus, as shown below.

Figure 3. Biopsy of EE in distal esophagus. Arrows indicate a few of the many eosinophils in this slide.

Figure 4. Biopsy of EE in a ringed stricture of the mid esophagus. Arrows indicate a few of the many eosinophils in this slide.

Strictures with EE:
One third of our patients presented with EE/ringed stricture in the age group of 21-30. Age distribution is shown in Figure 5.

Figure 5. Number of patients with EE/ringed stricture in each age group at the for Esophageal & Swallowing Disorders.

Dilation:
Savary dilators were the initial selection for severe strictures, but Maloney dilators were also used under flouroscopy in some cases. Hydrostatic TTS dilators are not used for this type of stricture since the operator is unable to sense resistance of the stricture to stretching. The initial lumen dilation is performed in 2 increments of 1mm maximum, for each session, every 3 to 6 weeks with a goal of 16mm (final diameter) over a 4 to 6 month span. The average number of sessions to complete the goal was 4 and the average maximum dilation achieved was 46Fr. Slow dilation progression is considered safe and least likely to produce pain or major injury after dilation.

Treatment:
Although there is no single specific therapy for treatment of EE, there are several that have proven effective. PPI therapy is considered first as adjunctive therapy since acid reflux is known to occur in some patients. For patients with a food specific allergy, dietary elimination has produced variable results, but elemental (amino acid) diets, although difficult to tolerate because of taste, have been excellent in diminishing the number of eosinophils. For patients with non-specific food allergies, systemic and topical steroids are helpful. Dilation should be considered for severe strictures. Some helpful tips for the patient include modifying food consistency, careful chewing, liquid flushing and avoidance of voluntary regurgitation for food impactions, as this may cause perforation. Continued follow-up with repeat endoscopy and biopies are essential. EE is a chronic condition that requires long term therapy, consequently patients should be treated toward histologic normalcy keeping in mind that therapy may not cure the condition but may decrease complications.