Jackhammer esophagus with and without esophagogastric junction outflow obstruction demonstrates altered neural control resembling type 3 achalasia

Abstract

Background Esophageal hypercontractility can manifest with and without esophagogastric junction (EGJ) outflow obstruction. We investigated clinical presentations and motility patterns in patients with esophageal hypercontractile disorders. Methods Esophageal HRM studies fulfilling Chicago Classification 3.0 criteria for jackhammer esophagus (distal contractile integral, DCI >8000 mmHg.cm.s in ≥ 20% swallows) with (n = 30) and without (n = 83) EGJ obstruction (integrated relaxation pressure, IRP > 15 mm Hg) were retrospectively reviewed from five centers (4 in Europe, 1 in US). Single swallows (SS) and multiple rapid swallows (MRS) were analyzed using HRM software tools (IRP, DCI, distal latency, DL); MRS: SS DCI ratio >1 defined contraction reserve. Comparison groups were achalasia type 3 (n = 72, positive control for abnormal inhibition and EGJ obstruction) and healthy controls (n = 18). Symptoms, HRM metrics, and MRS contraction reserve were analyzed within jackhammer subgroups and comparison groups. Key Results The esophageal smooth muscle was excessively stimulated at baseline in jackhammer subgroups, with lack of augmentation following MRS identified more often compared with controls (P = .003) and type 3 achalasia (P = .07). Consistently abnormal inhibition was identified in type 3 achalasia (47%), and to a lower extent in jackhammer with obstruction (37%, P = .33), jackhammer esophagus (28%, P = .01), and controls (11%, P < .01 compared with type 3 achalasia). Perceptive symptoms (heartburn, chest pain) were common in jackhammer esophagus (P < .01 compared with type 3 achalasia), while transit symptoms (dysphagia) were more frequent with presence of EGJ obstruction (P ≤ .01 compared with jackhammer without obstruction). Conclusions and inferences The balance of excessive excitation and abnormal inhibition defines clinical and manometric manifestations in esophageal hypercontractile disorders.

Jackhammer esophagus: A meta‐analysis of patient demographics, disease presentation, high‐resolution manometry data, and treatment outcomes

“…The mean pooled age of patients diagnosed with JE was 60.8 years [95% CI: 57.1-64.4; Cochran's Q = 51.2, I 2 = 84.4%], 16,20-22,25,27-30 and 64.9% of diagnosed patients were female [95% CI: 57.7%-72.1%; Cochran's Q = 42.6, I 2 = 71.8%] 16,[20][21][22][23]25,26,[28][29][30][31][32][33]. …”

Hypercontractile esophagus resolved after radiofrequency catheter ablation for atrial fibrillation: About a case

“…Esophageal innervation, which is different from heart innervation, is a balance between excitatory and inhibitory fibers from the extrinsic nervous system acting on the myenteric plexuses of the esophagus. Pathophysiology of hypercontractile esophagus remains unclear but it has been recently shown that exaggerated excitation is found in patients with hypercontractile esophagus, combined with abnormal inhibitory function in case of associated eso‐gastric junction outflow obstruction 8 . In our case, unlike the case of Tolone et al , 3 the hypercontractile esophagus is resolved after RCFA.…”

The hypercontractile esophagus: Still a tough nut to crack

“…As summarized in Table 1, many publications have reported on the prevalence of symptoms in cohorts of patients with HE. 13,[18][19][20]23,24 As a group, patients with HE report a broad range of symptoms, among which dysphagia (32%-100%), chest pain (10%-52%), and reflux symptoms (17%-58%) are the most prominent. In this respect, the symptom regurgitation is a source of confusion in the literature on HE since regurgitation can be a reflux symptom as well as a symptom of impaired esophageal transit.…”

“…More recently, Quader et al evaluated 113 HE patients, of whom 30 also had EGJ outflow obstruction. In the group without outflow obstruction (n = 83), perceptive symptoms (heartburn, chest pain) were the most common (73%), while in the group with outflow obstruction (n = 30) transit symptoms (dysphagia) were reported most frequently (72%) 23 …”

“…As summarized in Table 1, many publications have reported on the prevalence of symptoms in cohorts of patients with HE 13,18–20,23,24 . As a group, patients with HE report a broad range of symptoms, among which dysphagia (32%‐100%), chest pain (10%‐52%), and reflux symptoms (17%‐58%) are the most prominent.…”