The extremely narrow-caliber esophagus is a treatment-resistant subphenotype of eosinophilic esophagitis

Abstract

Background and aims Some patients with eosinophilic esophagitis (EoE) have an extremely narrowed esophagus, but the characteristics of this group have not been extensively described. We aimed to characterize the narrow-caliber phenotype of EoE, determine associated risk factors, and identify differences in treatment response in this sub-group of patients. Methods This retrospective cohort study from 2001 to 2014 included subjects with a new diagnosis of EoE per consensus guidelines. Demographic, endoscopic, histologic, and treatment response data were extracted from medical records. An “extreme narrow-caliber esophagus” was defined when the neonatal endoscope was required to traverse the esophagus due to inability to pass an adult endoscope. Cases with and without the extreme narrow-caliber esophagus were compared. Multivariable logistical regression was performed to assess treatment outcomes. Results Of 513 patients with EoE, 46 (9%) had an extreme narrow-caliber esophagus. These cases were older (33 vs 22 years; p<0.01), had longer symptom duration (11 vs 3 years; p<0.01), more dysphagia (98% vs 66%; p<0.01), and food impactions (53% vs 31%; p<0.01). Dilation was more common with extreme narrowing (69% vs 17%; p<0.01). Narrow-caliber patients were more refractory to steroid treatment, with lower symptom (56% vs 85%), endoscopic (52% vs 76%), and histologic (33% vs 63%) responses (p<0.01 for all), and these differences persisted after multivariate analysis. Conclusion The extreme narrow-caliber esophagus is a more treatment-resistant sub-phenotype of EoE and is characterized by longer symptom duration and requirement for multiple dilations. Recognition at diagnosis of EoE can provide important prognostic information.

Esophageal dilation with either bougie or balloon technique as a treatment for eosinophilic esophagitis: a systematic review and meta-analysis

“…In fact, none of the reported perforations occurred in patients younger than 17 years of age, 41 which could reflect the inverse relationship of esophageal compliance with duration of uncontrolled disease activity. 7 Regarding dilator type, our review was limited by study heterogeneity and incomplete procedure-level data. The majority of balloon dilations in particular (681/837 procedures), were contributed by only 6 centers, and may not be generalizable to providers less experienced with this technique.…”

“…1, 2 Prominent presenting symptoms in adults and adolescents include dysphagia and food impaction, 3 and are often a consequence of esophageal strictures or narrowing. 4–7 Although medical and dietary therapies can decrease esophageal eosinophilia and microscopic evidence of fibrosis, 8 they may not resolve overt strictures or narrowing and therefore symptoms may not improve in the setting of a severe fibrostenotic phenotype. 7, 9, 10 In addition, a long duration of symptoms before diagnosis is associated with development of esophageal remodeling.…”

“…4–7 Although medical and dietary therapies can decrease esophageal eosinophilia and microscopic evidence of fibrosis, 8 they may not resolve overt strictures or narrowing and therefore symptoms may not improve in the setting of a severe fibrostenotic phenotype. 7, 9, 10 In addition, a long duration of symptoms before diagnosis is associated with development of esophageal remodeling. 5, 11–15 In these cases esophageal dilation remains a mainstay of therapy, both with and without concomitant medical therapy.…”

Causes and Outcomes of Esophageal Perforation in Eosinophilic Esophagitis

“…In certain individuals, this process can deteriorate into a severe phenotype in which the esophagus is narrowed along its entire length. 417, 9, 30, 42–45 Third, patients with EoE often have a long duration of symptoms prior to diagnosis 15, 17, 30, 46–48 and modify their eating behaviors to minimize symptoms. Because they may be used to transient impactions, they may not seek care rapidly, which might lead to esophageal injury from the impacted food.…”

Narrow-caliber esophagus of eosinophilic esophagitis: difficult to define, resistant to remedy

“…In the present issue, Eluri et al 9 performed a retrospective, case-control study of patients with EoE and an “extreme narrow-caliber” esophagus defined by requiring a neonatal upper endoscope (outer diameter < 6 mm) to traverse the esophagus because of esophageal stenosis. As expected, patients with an extreme narrow-caliber esophagus more commonly had dysphagia, food impaction, and required dilations compared with EoE patients with “regular-caliber” esophagus (defined by allowing passage of an adult upper endoscope).…”