A case of Jackhammer esophagus caused by eosinophilic esophagitis in which per-oral endoscopic myotomy resulted in symptom improvement

Abstract

A 73-year-old female with a 6-month history of progressive dysphagia and chest pain was referred to our hospital. She underwent esophagogastroduodenoscopy, which revealed abnormally strong contractions in the distal esophagus. Esophageal biopsy specimens showed massive eosinophil infiltration into the epithelium, and high-resolution manometry (HRM) also demonstrated abnormally strong contractions in the distal esophagus. Based on these results, she was diagnosed with Jackhammer esophagus (JHE) due to eosinophilic esophagitis (EoE). Treatment was started with 5 mg/day of prednisolone (PSL), and the number of peripheral blood eosinophils quickly decreased without any improvement in the patient's dysphagia. Esophageal biopsy specimens obtained after the PSL treatment showed the disappearance of eosinophils from the epithelium. However, abnormally strong contractions were still detected on HRM. Per-oral endoscopic myotomy (POEM) was performed to treat the JHE. Interestingly, the intraoperative esophageal muscle biopsy sample demonstrated massive eosinophil infiltration into the muscle layer. After the POEM, the patient's symptoms improved, and abnormal contractions were no longer detected on HRM. The current case suggests that when EoE combined with an esophageal motility disorder are refractory to steroid therapy, clinicians should be aware that motility disorders can develop due to eosinophil infiltration deep into the esophageal muscularis propria.

Markedly Effective Steroid Treatment of Three Patients with Allergy-related Jackhammer Esophagus

“…Furthermore, the submucosal thickening of the esophagus that was seen on EUS decreased, and the esophageal peristalsis in HRM normalized. Thus, the pathological condition was assumed to be the same as that reported by Tanaka et al (17). In addition, Tang et al (18) performed POEM for JHE triggered by EoEM and reported long-term symptom improvement.…”

“…Tanaka et al (17) reported a case of JHE caused by EoE. A 73-year-old woman had a 6-month history of dysphagia and chest pain, and EUS revealed thickening of the submucosal layer of the esophagus.…”

Manometrically jackhammer esophagus with fluoroscopically/endoscopically distal esophageal spasm: a case report

“…Mallet et al reported that 54.3 % of these patients had gastro-esophageal reflux disease (GERD), while an upper GI endoscopy showed non-erosive to mild reflux esophagitis (Los Angeles grade A) [ 9 ]. A case report by Tanaka et al revealed abnormally strong contractions in the distal esophagus on upper GI endoscopy and pathological results of the esophageal biopsy specimens showed massive eosinophil infiltration into the epithelium, which were diagnosed as JH esophagus due to eosinophilic esophagitis [ 10 ]. In another study by Clément et al found that most of them were normal, about one-third who had abnormal findings during endoscopy included hiatal hernia, longitudinal striae, impression of esophageal dilatations, and increased LES tone.…”

“…Currently, the standard treatment of this disease is not well defined. Pharmacologic treatment should be selected as the first choice, includes smooth muscle relaxants such as calcium channel blockers, nitrates, or phosphodiesterase-5 inhibitors, which reduce both LES pressure and esophageal contraction amplitude [ 10 ]. Another option which can relieve chest pain symptoms without improvement in motility function is low-dose antidepressants [ 12 ].…”

Oesophageal eosinophilia and oesophageal diseases in children: are the limits clear?

“… 44 45 Improvement of oesophageal eosinophilia has also been described with the use of steroids in JO. 48 49 …”