Peroral endoscopic myotomy for hypercontractile (Jackhammer) esophagus: A retrospective multicenter series with long-term follow-up

Abstract

Introduction: Long-term outcome data are limited for non-achalasia esophageal motility disorders treated by peroral endoscopy myotomy (POEM) as a separate group. We investigated a subset of symptomatic patients with hypercontractile esophagus (Jackhammer esophagus).

Methods: Forty two patients (mean age 60.9 years; 57% female, mean Eckardt score 6.2 ± 2.1) treated by primary peroral myotomy for symptomatic Jackhammer esophagus 2012-2018 in seven European centers were retrospectively analyzed; myotomy included the lower esophageal sphincter but did not extend more than 1 cm into the cardia in contrast to POEM for achalasia. Manometry data were re-reviewed by an independent expert. The main outcome was the failure rate defined by retreatment or an Eckardt score >3 after at least two years following POEM.

Results: Despite 100% technical success (mean intervention time 107 ± 48.9 min, mean myotomy length 16.2 ± 3.7 cm), the 2-year success rate was 64.3% in the entire group. In a subgroup analysis, POEM failure rates were significantly different between Jackhammer-patients without (n = 22), and with esophagogastric junction outflow obstruction (EGJOO, n = 20) (13.6% % vs. 60%, p = 0.003) at a follow-up of 46.5 ± 19.0 months. Adverse events occurred in nine cases (21.4%). 14 (33.3%) patients were retreated, two with surgical fundoplication due to reflux. Including retreatments, an improvement in symptom severity was found in 33 (78.6%) at the end of follow-up (Eckardt score ≤3, mean Eckardt change 4.34, p < 0.001). EGJOO (p = 0.01) and frequency of hypercontractile swallows (p = 0.02) were predictors of POEM failure. The development of a pseudodiverticulum was observed in four cases within the subgroup of EGJOO.

Conclusions: Patients with symptomatic Jackhammer without EGJOO benefit from POEM in long-term follow-up. Treatment of Jackhammer with EGJOO, however, remains challenging and probably requires full sphincter myotomy and future studies which should address the pathogenesis of this variant and alternative strategies.

Keywords: Eckardt score; Jackhammer esophagus; adverse events; esophagogastric junction outflow obstruction (EGJOO); failure; lower esophageal sphincter (LES); motility; peroral endoscopic myotomy (POEM).

FIGURE 1

Comparison of the two groups of Jackhammer esophagus without and with EGJOO: Failure rates at mean follow‐up (46.5 ± 19.0 months) (a) and Eckardt‐symptom‐score over time among the study groups (b). EGJOO, esophagogastric outflow obstruction.

FIGURE 2

Development of a pseudodoverticulum following POEM in a Jackhammer patient with EGJOO. (a) In contrast to pre‐POEM, the HRM control at 3 month FU that shows residual contractility above the area of myotomy and in the distal part corresponding to an incomplete myotomy. (b) The esophagus was undilated at baseline, whereas the 3 month control suggests a slight edge in the distal esophagus. (c) Endoscopy and esophagogram clearly show the progression of the pseudodiverticulum or blown‐out myotomy at 12 month follow‐up. This patient underwent repeat POEM. EGJOO, esophagogastric junction outflow obstruction; FU, follow‐up; HRM, high‐resolution manometry; POEM, peroral endoscopic myotomy.