A Case of Eosinophilic Esophagitis With Achalasia Requiring Myotomy

Abstract

Introduction: Eosinophilic esophagitis (EoE) is a clinicopathological disorder defined by esophageal dysfunction and eosinophilic infiltration of the esophagus with more than 15 eosinophils per high power field (hpf) on microscopy after ruling out other causes of eosinophilia. Dysphagia is the most common symptom first described in a 1978 case report. Esophageal dysmotility on esophageal manometry has been described in EoE patients. Only one case report documented successful management of EoE with concomitant achalasia via Heller’s myotomy. Our patient demonstrates EoE in the presence achalasia with persistent symptoms despite medical management requiring Heller myotomy for symptomatic improvement. A 48-year-old female presented to clinic with difficult, painful swallowing and food regurgitation. She was diagnosed with EoE one year prior and treated with PPI followed by swallowed fluticasone, but did not achieve reduction in eosinophil count until she started a six food elimination diet. Despite histologic improvement, her symptoms persisted. She underwent upper endoscopy at our institution revealing a narrow-caliber esophagus that was subsequently dilated. Mucosal features were consistent with EoE. Biopsies showed up to 18 intraepithelial eosinophils per hpf from the proximal esophagus. She subsequently underwent high resolution esophageal manometry (EM) revealing achalasia. The esophagus exhibited failed peristalsis without relaxation of the lower esophageal sphincter (LES). The patient underwent laparoscopic Heller myotomy with Nissen fundoplication providing nearly complete resolution of symptoms. There are few reports of achalasia in the setting of EoE since the 1978 case. One case series reported most patients with EoE have abnormalities on EM. Only one patient had achalasia, which resolved with inhaled fluticasone. Another case series identified that 4 of 512 cases of achalasia had concomitant EoE. Two patients were treated for achalasia with Heller myotomy, but symptoms recurred. A recent study evaluating EoE with high resolution EM shows that esophageal body motility abnormalities, namely weak or failed peristalsis, are most common. It is unclear if our patient has type 1 or type 2 achalasia as stratified by the Chicago Classification since panesophageal pressurization may be seen in EoE alone. EoE with achalasia may be resistant to medical management and require myotomy for symptom improvement. EoE patients may benefit from early EM studies to identity concurrent esophageal motility abnormalities.