The ringed esophagus: histological features of GERD

Abstract

OBJECTIVE: The “ringed” or “corrugated” esophagus is a cause of chronic dysphagia and recurrent food impactions in young men. It was previously believed to be a congenital condition, but recent case series have documented histological esophagitis in these patients. We have treated 19 patients with a ringed esophagus and are impressed that this represents an acquired condition with gastroesophageal reflux disease (GERD) as its etiology. Our goals are to present the largest case series to date of ringed esophagus, discuss the evidence for GERD, and suggest a strategy for its diagnosis and management. METHODS: The charts of 19 patients with a ringed esophagus were reviewed. A single pathologist interpreted all available esophageal biopsy specimens and graded them for the presence of GERD-related abnormalities. Phone interviews were conducted to assess response to therapy and confirm historical features obtained from medical records. RESULTS: The typical patient is a young man (median age 35, M:F 17:2) with long-standing dysphagia and multiple food impactions. Endoscopy revealed multiple concentric rings that persisted despite air insufflation and caused significant stenosis. Of the 11 patients with available histology, all had basal cell hyperplasia, papillomatosis, and an increased number of intraepithelial eosinophils. Other clinical features of GERD such as heartburn, endoscopic esophagitis, and hiatal hernia were often absent. Response to dilation and acid suppression was good. CONCLUSIONS: The uniform presence of histological esophagitis with intraepithelial eosinophils, basal cell hyperplasia, and papillary lengthening strongly implicates GERD in the pathogenesis of the adult ringed esophagus. In addition to a series of gradual esophageal dilations, we suggest using a proton pump inhibitor to provide acid suppression.