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

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.

Frequent discrepancies among diagnostic tests for detecting lower esophageal sphincter-related obstruction.

BACKGROUND: There are frequent discrepancies among high-resolution manometry (HRM), functional lumen imaging probe (FLIP), and esophagram in identifying lower esophageal sphincter (LES)-related obstruction. We aimed to determine the frequency of those discrepancies and how they influenced clinical treatment/outcomes. METHODS: We identified patients who had all three tests (HRM, FLIP, and esophagram) and endoscopy performed for evaluation of esophageal symptoms in our Center for Esophageal Diseases. Discrepancies among the tests for the presence of LES obstruction were noted, and the performance of individual tests was compared against a consensus opinion rendered by a panel of esophagologists. Binary logistical regression was performed, and ROC curves were generated for prediction of the consensus clinical diagnosis of LES obstruction. KEY RESULTS: A total of 126 patients (mean age 57.9 ± 17.0 years; 67% female) met inclusion criteria. All three tests agreed on the presence or absence of LES obstruction in only 72 (57%) patients [no LES obstruction in 57 (45%), LES obstruction in 15 (12%)]. Thirteen patients (10%) had a change in management based on additional findings on FLIP +/- esophagram not seen on HRM with 69% having symptomatic improvement after LES-directed intervention. FLIP was the strongest predictor of a consensus diagnosis of LES obstruction by logistic regression and ROC (OR 23.36, AUC 0.796), followed by HRM (OR 15.41, AUC 0.764). CONCLUSIONS & INFERENCE: High-resolution manometry, functional lumen imaging probe, and esophagram each have considerable limitations for identifying LES obstruction, and discrepancies among these tests occur frequently. Multimodal testing is often required for adequate evaluation of LES-related obstruction.

Peroral Endoscopic Myotomy (POEM) and Laparoscopic Heller Myotomy with Dor Fundoplication for Esophagogastric Junction Outflow Obstruction (EGJOO): a Comparison of Outcomes and Impact on Physiology.

INTRODUCTION: Esophagogastric junction outflow obstruction (EGJOO) is an esophageal motility disorder characterized by failure of lower esophageal sphincter (LES) relaxation with preserved peristalsis. Studies have shown that Heller myotomy with Dor fundoplication (HMD) and per oral endoscopic myotomy (POEM) are effective treatments for EGJOO. However, there is paucity of data comparing the efficacy and impact of these two procedures. Therefore, the aim of this study was to compare outcomes and impact on esophageal physiology in patients undergoing HMD or POEM for primary EGJOO. METHODS: This was a retrospective review of patients who underwent either HMD or POEM for primary EGJOO at our institution between 2013 and 2021. Favorable outcome was defined as an Eckardt score ≤ 3 at 1 year after surgery. GERD-HRQL questionnaire, endoscopy, pH monitoring, and high-resolution manometry (HRM) results at baseline and 1 year after surgery were compared pre- and post-surgery and between groups. Objective GERD was defined as DeMeester score > 14.7 or LA grade C/D esophagitis. RESULTS: The final study population consisted of 52 patients who underwent HMD (n = 35) or POEM (n = 17) for EGJOO. At a mean (SD) follow-up of 24.6 (15.3) months, favorable outcome was achieved by 30 (85.7%) patients after HMD and 14 (82.4%) after POEM (p = 0.753). After HMD, there was a decrease GERD-HRQL total score (31 (22-45) to 4 (0-19); p < 0.001), and objective reflux (54.2 to 25.9%; p = 0.033). On manometry, there was a decrease in LES resting pressure (48 (34-59) to 13 (8-17); p < 0.001) and IRP (22 (17-28) to 8 (3-11); p < 0.001), but esophageal body characteristics did not change (p > 0.05). Incomplete bolus clearance improved (70% (10-90) to 10% (0-40); p = 0.010). After POEM, there was no change in the GERD-HRQL total score (p = 0.854), but objective reflux significantly increased (0 to 62%; p < 0.001). On manometry, there was a decrease in LES resting pressure (43 (30-68) to 31 (5-34); p = 0.042) and IRP (23 (18-33) to 12 (10-32); p = 0.048), DCI (1920 (1600-5500) to 0 (0-814); p = 0.035), with increased failed swallows (0% (0-30) to 100% (10-100); p = 0.032). Bolus clearance did not improve (p = 0.539). Compared to HMD, POEM had a longer esophageal myotomy length (11 (7-15)-vs-5 (5-6); p = 0.001), more objective reflux (p = 0.041), lower DCI (0 (0-814)-vs-1695 (929-3101); p = 0.004), and intact swallows (90 (70-100)-vs-0 (0-40); p = 0.006), but more failed swallows (100 (10-100); p = 0.018) and incomplete bolus clearance (90 (90-100)-vs-10 (0-40); p = 0.004). CONCLUSION: Peroral endoscopic myotomy and Heller myotomy with Dor fundoplication are equally effective at relieving EGJOO symptoms. However, POEM causes worse reflux and near complete loss of esophageal body function.

Multifactorial dysphagia: Azygos vein aneurysm (AVA) and esophagogastric junction outflow obstruction (EGJOO).

INTRODUCTION: Vascular impingement of the esophagus is a rare cause of dysphagia, and is most commonly due to aortic arch anomalies such as arterial lusoria. Dysphagia resultant from venous compression is even further less likely. PRESENTATION OF CASE: We present a highly unusual case of dysphagia secondary to a large aneurysm of the azygous vein near its confluence with the superior vena cava, which was managed with endovascular modalities. Despite initial treatment success, patient reported some intermittent solid food dysphagia, and was also found to have esophagogastric junction outflow obstruction (EGJOO) on high resolution impedance manometry (HRIM) which was successfully managed with surgical myotomy and partial fundoplication. DISCUSSION: The azygos vein has an intimate anatomic relationship with the esophagus as it traverses the posterior mediastinum. Because of this anatomic association, the azygos vein may present a point of esophageal obstruction in the setting of significant pathology. CONCLUSION: This case highlights the possibility of multifactorial causes of dysphagia, and that HRIM is a key aspect of this workup. Additionally we discuss the pertinent anatomy, diagnosis, and treatments for azygos vein aneurysm and EGJOO.

Esophagitis dissecans superficialis (EDS) secondary to esophagogastric junction outflow obstruction (EGJOO): a case report and literature review.

Esophageal dissecans superficialis (EDS) is a rare disease with endoscopic findings of sloughing squamous tissue with underlying normal mucosa and had no known cause. The literature does support possible causality between the presence of an esophageal stricture and EDS however there has been no association to date between EDS and esophagogastric junction outflow obstruction (EGJOO). We present a case of newly diagnosed EGJOO in a patient with long standing gastroesophageal reflux disease who presented with dysphagia. Evaluation identified endoscopically normal mucosa and a diagnosis of esophagogastric junction outflow obstruction on high resolution impedance manometry. A month later, repeat endoscopy identified diffusely sloughing mucosa consistent with EDS. Endoscopic dilation followed by a robotic Heller myotomy with Dor fundoplication to relive the outflow obstruction resulted in resolution of EDS in this case.

Upper esophageal sphincter (UES) metrics on high-resolution manometry (HRM) differentiate achalasia subtypes.

BACKGROUND: The upper esophageal sphincter (UES) reflexively responds to bolus presence within the esophageal lumen, therefore UES metrics can vary in achalasia. METHODS: Within consecutive patients undergoing esophageal high-resolution manometry (HRM), 302 patients (58.2±1.0 year, 57% F) with esophageal outflow obstruction were identified, and compared to 16 asymptomatic controls (27.7±0.7 year, 56% F). Esophageal outflow obstruction was segregated into achalasia subtypes 1, 2, and 3, and esophagogastric junction outflow obstruction (EGJOO with intact peristalsis) using Chicago Classification v3.0. UES and lower esophageal sphincter (LES) metrics were compared between esophageal outflow obstruction and normal controls using univariate and multivariate analysis. Linear regression excluded multicollinearity of pressure metrics that demonstrated significant differences across individual subtype comparisons. KEY RESULTS: LES integrated relaxation pressure (IRP) had utility in differentiating achalasia from controls (P<.0001), but no utility in segregating between subtypes (P=.27). In comparison to controls, patients collectively demonstrated univariate differences in UES mean basal pressure, relaxation time to nadir, recovery time, and residual pressure (UES-RP) (P≤.049). UES-RP was highest in type 2 achalasia (P<.0001 compared to other subtypes and controls). In multivariate analysis, only UES-RP retained significance in comparison between each of the subgroups (P≤.02 for each comparison). Intrabolus pressure was highest in type 3 achalasia; this demonstrated significant differences across some but not all subtype comparisons. CONCLUSIONS AND INFERENCES: Nadir UES-RP can differentiate achalasia subtypes within the esophageal outflow obstruction spectrum, with highest values in type 2 achalasia. This metric likely represents a surrogate marker for esophageal pressurization.