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Jackhammer Esophagus: Clinical, Functional and Therapeutic Data. a Retrospective Multicenter Study in 272 Patients
Sa1661 ASSESSMENT FOR SYSTEMIC SCLEROSIS AND CONNECTIVE TISSUE DISEASES IN PATIENTS WITH INEFFECTIVE ESOPHAGEAL MOTILITY, ABSENT CONTRACTILITY, OR HYPOTENSIVE LOWER ESOPHAGEAL SPHINCTERAND A TERTIARY REFERRAL CENTRE'S EXPERIENCE Dorothy Y. Li, Michael Curley, Milli Gupta, Michelle Buresi, Yasmin Nasser, Lynn Wilsack, Joel David, Christopher N. Andrews
Sa1659 AN INCREASED PREVALENCE OF NEURODEGENERATIVE/ DEMYELINATING PROCESSES IN PATIENTS WITH ESOPHAGEAL ACHALASIA AND A PROSPECTIVE STUDY Hana Svecova, Martin Jerie, Eva Meluzinova, Dana Kautznerova, Julius Spicak, Jana Krajciova, Zuzana Vackova, Jan Martinek
BACKGROUND: Systemic sclerosis (SSc) is a connective tissue disease (CTD) with features of autoimmunity, vasculopathy and fibrosis. Gastrointestinal tract (GIT) involvement is seen in 50-90% of SSc patients and occurs early in the disease course. Esophageal involvement is common, and typical symptoms include dysphagia or heartburn. Classic findings on esophageal manometry are weak or absent esophageal peristalsis with a low resting lower esophageal sphincter (LES) pressure. AIMS: It is unclear how patients with these manometry findings are assessed and what proportion of these patients are diagnosed with SSc or another CTD after evaluation. This study addresses these uncertainties by describing the current standard of practice in a tertiary referral centre with expertise in gastrointestinal motility disorders. METHODS: The patient electronic database at the Calgary Gut Motility Centre was reviewed for patients consented between January 2013 and October 2016. Patients with ineffective esophageal motililty (IEM), aperistalsis, or hypotensive LES on high-resolution manometry (Chicago Classification) had their electronic medical charts reviewed to determine how they were evaluated and what proportion were diagnosed with CTD. RESULTS: A total of 353 patients had these findings on manometry and their charts were reviewed. 164 (46.5%) had IEM+hypotensive LES, 47 (13.3%) had aperistalsis+hypotensive LES, 27 (7.6%) had IEM alone, 34 (9.6%) had aperistalsis alone, and 81 (22.9%) had hypotensive LES alone. 259 (66.5%) of manometry reports included CTD on the differential diagnosis, with 6 (1.7%) specifically suggesting workup for CTD. CTD was most often listed on the differential diagnosis on manometry reports with aperistalsis+hypotensive LES (83%), hypotensive LES alone (77.4%), and IEM+hypotensive LES (73.5%). 54 (15.3%) of patients had autoantibody testing after manometry, and of these, 17 (31.5%) had a positive result. 14 (4%) patients had a Rheumatology referral after their manometry. 5 patients had a new diagnosis of CTD, 2 of which were SSc. CONCLUSIONS: These findings indicate that a new diagnosis of SSc or another CTD is uncommon among patients found to have IEM, aperistalsis, or hypotensive LES on esophageal manometry. However, this is likely reflective of current deficiencies in the diagnostic evaluation of patients with these manometry findings. Although CTD is often listed on the differential diagnosis on such manometry reports, only a small portion of these patients undergo further evaluation with antibody testing or Rheumatology assessment. Education of Gastroenterologists and primary care physicians may help increase awareness of the classic manometry findings associated with CTD and thereby increase the frequency of evaluation for these disorders in this patient population.
Background: In recent years, there has been increasing recognition of the presence of gastrointestinal (GI) dysfunction in the setting of neurologic diseases. There are no studies examining a relationship between achalasia and neurodegenerative/ demyelinating diseases of central nervous system. Although both achalasia and neurodegenerative/demyelinating processes are not considered hereditary, a number of genetic variations have been shown to increase the risk of both conditions. For example, HLA-DQB1-insertion (chromosome 6) may be strongly associated with both achalasia and multiple sclerosis suggesting that autoimmune processes are involved in their etiopathogenesis. Several other findings (e.g. inflammatory infiltrates, Lewy's bodies, geospatial gradient etc.) are also described in patients with achalasia as well as in neurodegenerative/demyelinating diseases. Aim: The aim of our prospective study was to examine a prevalence of neurodegenerative/demyelinating diseases in a cohort of consecutive patients with confirmed esophageal achalasia. Achalasia was diagnosed by high-resolution manometry, endoscopy and esophagogram. Methods: One hundred and forty consecutive patients with esophageal achalasia have been questioned so far by using a detailed questionnaire about the occurrence of neurological diseases and symptoms in their personal or family history. Those with a suspicion of a neurological disease were referred for MR imaging of the brain and for detailed neurological examination. Results: A total of 51 out of 140 patients (36.4 %) exhibited of neurological symptoms most often visual disturbances in 33.3% (n=17), paresthesia of limbs in 23.5% (n=12) and hypotension in 19.6% (n=10). Among patients with a presence of neurological symptoms, 5 patients (3.6 %) have definitely been diagnosed with a neurodegeneration/demyelinating disease (multiple sclerosis - 2 patients, Leber's optic neuropathy - 1 patient, Parkinson's disease - 1 patient and Allgrove syndrome - 1 patient). Furthemore, 7 patients with a positive questionnaire have been diagnosed with other neurological diseases (tetany n=2, carpal tunnel syndrome n=3, epilepsy n=2). A total of 14 patients (27.4%) among those with the presence of neurological symptoms (vs. 0 from 89 patients without neurological symptoms) have had a positive family history of a neurodegenerative or a demyelinating disease. Conclusion: Our results imply an increased prevalence of neurodegenerative/demyelinating diseases in patients with achalasia (3.6% vs. approx. 1.4% in the Czech controls) and also a high prevalence of these disorders in families of patients with esophageal achalasia and simultaneous neurologic symptomatology. These results warrant further confirmation in a large population-based study.
Sa1662 IN INEFFECTIVE ESOPHAGEAL MOTILITY, FAILED SWALLOWS ARE MORE FUNCTIONALLY RELEVANT THAN WEAK SWALLOWS Anand S. Jain, Jason Baker, Joan Chen
Sa1660 JACKHAMMER ESOPHAGUS: CLINICAL, FUNCTIONAL AND THERAPEUTIC DATA. A RETROSPECTIVE MULTICENTER STUDY IN 272 PATIENTS Sara Philonenko, Guillaume Gourcerol, Frank Zerbib, Alain Ropert, Stanislas Bruley des Varannes, Maximilien Barret, Veronique Vitton, Michel Bouchoucha, Chloe Melchior, Yaeesh Badat, Francois Mion, Charlene Brochard, Yann Touchefeu, Ariane Chryssostalis, Caroline Gorbatchef, Alban Benezech, Robert Benamouzig, Sabine Roman, Benoit Coffin
Background: Esophageal pressure topography (EPT) diagnosis of Ineffective Esophageal Motility (IEM) can be nonspecific with unclear clinical significance. Aims: To determine whether peristaltic vigor or lower esophageal sphincter (LES) integrity is associated with functional outcomes of bolus clearance and acid reflux in IEM. Methods: Consecutive Highresolution impedance manometry (HRIM) studies of adult patients diagnosed with IEM performed at our institution were retrospectively reviewed. Bolus clearance was assessed via colored impedance contour method and conventional impedance line tracings over supine wet swallows to determine Functional Clearance (FC) and Complete Bolus Transit (CBT), respectively. Available reflux data in IEM subjects were also reviewed. Concomitant EPT parameters and bolus clearance and acid reflux variables were explored. Results: Eightyeight patients with IEM were included. Clinical data are presented in Table 1. Overall agreement between FC and CBT was excellent (k=0.83). CBT frequency across a total of 880 supine swallows was 0.71. Clearance was assessed in swallows of varying peristaltic vigor as defined by the Distal contractile integral (DCI). Bolus clearance according to DCI subgroups is as shown in Figure 1. In swallows with DCI ≤100 mmHg-s-cm, bolus clearance was lower compared to all other swallows (p=0.0001). DCI 250-449 and >450 mmHg-scm groups resulted in higher clearance (p=0.003 and 0.0001, respectively). Using ROC curve analysis, DCI was associated with CBT (AUC .77), with a DCI ≥100 mmHg-s-cm having an accuracy of 76% and a positive predictive value of 83% in correlation with CBT (p=0.0001). Failed swallows, but not failed + weak swallows, correlated with CBT <80% (AUCs .76 and .53 respectively). 30% or more failed swallows was associated with CBT <80% (sensitivity 85%, p=0.0001); but the Chicago Classification v3.0 definition of IEM (failed + weak swallows ≥50%) was not. Peristaltic reserve (DCI of Multiple Rapid Swallow/ median DCI of wet swallows) was not associated with CBT. PH studies were available in 47 patients after excluding studies in 6 patients with prior fundoplication. Median DCI <100 mmHg-s-cm had an accuracy of 70.2% and a positive predictive value of 100% (p= 0.002) in correlation with elevated AET in combined on/off proton-pump inhibitor analysis. Low basal LES pressure or Integrated Relaxation Pressure (IRP) were not associated with abnormal AET. Conclusions: Failed peristalsis, as defined by DCI <100 mmHg-s-cm, is associated with impaired bolus clearance and abnormal AET in IEM. This observation should be taken into consideration in designing a prospective outcome study before modifications to the current diagnostic criteria for IEM can be made.
Introduction: Jackhammer esophagus (JE) is an esophageal motor disorder identified with the development of high resolution manometry (HRM). It is defined by at least 2 esophageal contractions with a distal contractile integral (DCI) > 8000 mmHg.s.cm (Chicago classification v3.0). Clinical and therapeutic data of JE patients have been so far poorly evaluated. Patients and methods: Among HRM performed in 9 academic French centers between 01/01/2010 and 08/31/2016, patients with at least 2 swallows with DCI > 8000mmHg.s.cm were included. All HRM studies were retrospectively analyzed by one single investigator. Patients' charts were reviewed to collect clinical, and therapeutic data. Results: Among the 16264 HRM performed during this period, 227 patients had JE (1.7%). Sex ratio M/F was 0.7, mean age 60.7 ± 25.6 years (SD), and median duration of symptoms before diagnosis was 20 ±60 months. Dysphagia was the most frequent symptom (74%), followed by GERD symptoms (38%), chest pain (34%) and ENT symptoms (19%). 13 patients (5%) were asymptomatic (systematic evaluation before bariatric surgery). Upper GI endoscopy was available in 204 patients: esophagitis was present in 7%; 71 patients had esophageal biopsies and eosinophilic esophagitis was diagnosed in 4 patients (6%). 107 patients had other morphological evaluations (CT scan, barium esophagogram, ultrasound endoscopy); 51 of them (48%) had some abnormalities that were considered as non-clinically relevant in 26%. 34 patients underwent esophageal pH-monitoring: pathological acid reflux was diagnosed in 9 patients (26%). Regarding HRM data, mean esophago-gastric junction (EGJ) resting pressure was 19.2 ± 15.8 mmHg and mean integrated relaxation pressure was 7.7 ± 7.2 mm Hg. 4.5 ± 2.7 swallows per patient had a DCI > 8000. Mean DCI of 10 swallows performed during the manometry protocol was 8942 ± 4549 mmHg.s.cm. Therapeutic data were available in 133 patients (49%): 38 did not receive any treatment; first line treatment was medical in 73 (PPI, calcium antagonist, nitrates) with a poor efficacy (50% no efficacy, 35.5 % moderate efficacy, 14.5 % success). 2nd and 3rd line treatments were mainly endoscopic: botox injection in 29 patients (success: 37.5 %; moderate efficacy: 37.3 %; no efficacy: 29.2%) and endoscopic dilation (success: 30 %; moderate efficacy: 40%; no efficacy: 30%). In patients with initial response, relapse rate was 35.3 % after Botox and 57.1 % after dilation. 2 patients were treated successfully with POEM (4 and 2.5 months follow up). Conclusion: This large cohort confirms that JE is a rare motility disorders. It might be associated with eosinophilic esophagitis or GERD. Diagnostic work-up is heterogeneous and should be standardized. Medical treatment and endoscopic therapies currently used have a low efficacy. POEM must be evaluated in a larger group of patients.
S-323
AGA Abstracts
AGA Abstracts
area, by manual definition of the borders, provides a more precise estimate of the volume of retained barium.
Sa1659 AN INCREASED PREVALENCE OF NEURODEGENERATIVE/ DEMYELINATING PROCESSES IN PATIENTS WITH ESOPHAGEAL ACHALASIA AND A PROSPECTIVE STUDY Hana Svecova, Martin Jerie, Eva Meluzinova, Dana Kautznerova, Julius Spicak, Jana Krajciova, Zuzana Vackova, Jan Martinek
BACKGROUND: Systemic sclerosis (SSc) is a connective tissue disease (CTD) with features of autoimmunity, vasculopathy and fibrosis. Gastrointestinal tract (GIT) involvement is seen in 50-90% of SSc patients and occurs early in the disease course. Esophageal involvement is common, and typical symptoms include dysphagia or heartburn. Classic findings on esophageal manometry are weak or absent esophageal peristalsis with a low resting lower esophageal sphincter (LES) pressure. AIMS: It is unclear how patients with these manometry findings are assessed and what proportion of these patients are diagnosed with SSc or another CTD after evaluation. This study addresses these uncertainties by describing the current standard of practice in a tertiary referral centre with expertise in gastrointestinal motility disorders. METHODS: The patient electronic database at the Calgary Gut Motility Centre was reviewed for patients consented between January 2013 and October 2016. Patients with ineffective esophageal motililty (IEM), aperistalsis, or hypotensive LES on high-resolution manometry (Chicago Classification) had their electronic medical charts reviewed to determine how they were evaluated and what proportion were diagnosed with CTD. RESULTS: A total of 353 patients had these findings on manometry and their charts were reviewed. 164 (46.5%) had IEM+hypotensive LES, 47 (13.3%) had aperistalsis+hypotensive LES, 27 (7.6%) had IEM alone, 34 (9.6%) had aperistalsis alone, and 81 (22.9%) had hypotensive LES alone. 259 (66.5%) of manometry reports included CTD on the differential diagnosis, with 6 (1.7%) specifically suggesting workup for CTD. CTD was most often listed on the differential diagnosis on manometry reports with aperistalsis+hypotensive LES (83%), hypotensive LES alone (77.4%), and IEM+hypotensive LES (73.5%). 54 (15.3%) of patients had autoantibody testing after manometry, and of these, 17 (31.5%) had a positive result. 14 (4%) patients had a Rheumatology referral after their manometry. 5 patients had a new diagnosis of CTD, 2 of which were SSc. CONCLUSIONS: These findings indicate that a new diagnosis of SSc or another CTD is uncommon among patients found to have IEM, aperistalsis, or hypotensive LES on esophageal manometry. However, this is likely reflective of current deficiencies in the diagnostic evaluation of patients with these manometry findings. Although CTD is often listed on the differential diagnosis on such manometry reports, only a small portion of these patients undergo further evaluation with antibody testing or Rheumatology assessment. Education of Gastroenterologists and primary care physicians may help increase awareness of the classic manometry findings associated with CTD and thereby increase the frequency of evaluation for these disorders in this patient population.
Background: In recent years, there has been increasing recognition of the presence of gastrointestinal (GI) dysfunction in the setting of neurologic diseases. There are no studies examining a relationship between achalasia and neurodegenerative/ demyelinating diseases of central nervous system. Although both achalasia and neurodegenerative/demyelinating processes are not considered hereditary, a number of genetic variations have been shown to increase the risk of both conditions. For example, HLA-DQB1-insertion (chromosome 6) may be strongly associated with both achalasia and multiple sclerosis suggesting that autoimmune processes are involved in their etiopathogenesis. Several other findings (e.g. inflammatory infiltrates, Lewy's bodies, geospatial gradient etc.) are also described in patients with achalasia as well as in neurodegenerative/demyelinating diseases. Aim: The aim of our prospective study was to examine a prevalence of neurodegenerative/demyelinating diseases in a cohort of consecutive patients with confirmed esophageal achalasia. Achalasia was diagnosed by high-resolution manometry, endoscopy and esophagogram. Methods: One hundred and forty consecutive patients with esophageal achalasia have been questioned so far by using a detailed questionnaire about the occurrence of neurological diseases and symptoms in their personal or family history. Those with a suspicion of a neurological disease were referred for MR imaging of the brain and for detailed neurological examination. Results: A total of 51 out of 140 patients (36.4 %) exhibited of neurological symptoms most often visual disturbances in 33.3% (n=17), paresthesia of limbs in 23.5% (n=12) and hypotension in 19.6% (n=10). Among patients with a presence of neurological symptoms, 5 patients (3.6 %) have definitely been diagnosed with a neurodegeneration/demyelinating disease (multiple sclerosis - 2 patients, Leber's optic neuropathy - 1 patient, Parkinson's disease - 1 patient and Allgrove syndrome - 1 patient). Furthemore, 7 patients with a positive questionnaire have been diagnosed with other neurological diseases (tetany n=2, carpal tunnel syndrome n=3, epilepsy n=2). A total of 14 patients (27.4%) among those with the presence of neurological symptoms (vs. 0 from 89 patients without neurological symptoms) have had a positive family history of a neurodegenerative or a demyelinating disease. Conclusion: Our results imply an increased prevalence of neurodegenerative/demyelinating diseases in patients with achalasia (3.6% vs. approx. 1.4% in the Czech controls) and also a high prevalence of these disorders in families of patients with esophageal achalasia and simultaneous neurologic symptomatology. These results warrant further confirmation in a large population-based study.
Sa1662 IN INEFFECTIVE ESOPHAGEAL MOTILITY, FAILED SWALLOWS ARE MORE FUNCTIONALLY RELEVANT THAN WEAK SWALLOWS Anand S. Jain, Jason Baker, Joan Chen
Sa1660 JACKHAMMER ESOPHAGUS: CLINICAL, FUNCTIONAL AND THERAPEUTIC DATA. A RETROSPECTIVE MULTICENTER STUDY IN 272 PATIENTS Sara Philonenko, Guillaume Gourcerol, Frank Zerbib, Alain Ropert, Stanislas Bruley des Varannes, Maximilien Barret, Veronique Vitton, Michel Bouchoucha, Chloe Melchior, Yaeesh Badat, Francois Mion, Charlene Brochard, Yann Touchefeu, Ariane Chryssostalis, Caroline Gorbatchef, Alban Benezech, Robert Benamouzig, Sabine Roman, Benoit Coffin
Background: Esophageal pressure topography (EPT) diagnosis of Ineffective Esophageal Motility (IEM) can be nonspecific with unclear clinical significance. Aims: To determine whether peristaltic vigor or lower esophageal sphincter (LES) integrity is associated with functional outcomes of bolus clearance and acid reflux in IEM. Methods: Consecutive Highresolution impedance manometry (HRIM) studies of adult patients diagnosed with IEM performed at our institution were retrospectively reviewed. Bolus clearance was assessed via colored impedance contour method and conventional impedance line tracings over supine wet swallows to determine Functional Clearance (FC) and Complete Bolus Transit (CBT), respectively. Available reflux data in IEM subjects were also reviewed. Concomitant EPT parameters and bolus clearance and acid reflux variables were explored. Results: Eightyeight patients with IEM were included. Clinical data are presented in Table 1. Overall agreement between FC and CBT was excellent (k=0.83). CBT frequency across a total of 880 supine swallows was 0.71. Clearance was assessed in swallows of varying peristaltic vigor as defined by the Distal contractile integral (DCI). Bolus clearance according to DCI subgroups is as shown in Figure 1. In swallows with DCI ≤100 mmHg-s-cm, bolus clearance was lower compared to all other swallows (p=0.0001). DCI 250-449 and >450 mmHg-scm groups resulted in higher clearance (p=0.003 and 0.0001, respectively). Using ROC curve analysis, DCI was associated with CBT (AUC .77), with a DCI ≥100 mmHg-s-cm having an accuracy of 76% and a positive predictive value of 83% in correlation with CBT (p=0.0001). Failed swallows, but not failed + weak swallows, correlated with CBT <80% (AUCs .76 and .53 respectively). 30% or more failed swallows was associated with CBT <80% (sensitivity 85%, p=0.0001); but the Chicago Classification v3.0 definition of IEM (failed + weak swallows ≥50%) was not. Peristaltic reserve (DCI of Multiple Rapid Swallow/ median DCI of wet swallows) was not associated with CBT. PH studies were available in 47 patients after excluding studies in 6 patients with prior fundoplication. Median DCI <100 mmHg-s-cm had an accuracy of 70.2% and a positive predictive value of 100% (p= 0.002) in correlation with elevated AET in combined on/off proton-pump inhibitor analysis. Low basal LES pressure or Integrated Relaxation Pressure (IRP) were not associated with abnormal AET. Conclusions: Failed peristalsis, as defined by DCI <100 mmHg-s-cm, is associated with impaired bolus clearance and abnormal AET in IEM. This observation should be taken into consideration in designing a prospective outcome study before modifications to the current diagnostic criteria for IEM can be made.
Introduction: Jackhammer esophagus (JE) is an esophageal motor disorder identified with the development of high resolution manometry (HRM). It is defined by at least 2 esophageal contractions with a distal contractile integral (DCI) > 8000 mmHg.s.cm (Chicago classification v3.0). Clinical and therapeutic data of JE patients have been so far poorly evaluated. Patients and methods: Among HRM performed in 9 academic French centers between 01/01/2010 and 08/31/2016, patients with at least 2 swallows with DCI > 8000mmHg.s.cm were included. All HRM studies were retrospectively analyzed by one single investigator. Patients' charts were reviewed to collect clinical, and therapeutic data. Results: Among the 16264 HRM performed during this period, 227 patients had JE (1.7%). Sex ratio M/F was 0.7, mean age 60.7 ± 25.6 years (SD), and median duration of symptoms before diagnosis was 20 ±60 months. Dysphagia was the most frequent symptom (74%), followed by GERD symptoms (38%), chest pain (34%) and ENT symptoms (19%). 13 patients (5%) were asymptomatic (systematic evaluation before bariatric surgery). Upper GI endoscopy was available in 204 patients: esophagitis was present in 7%; 71 patients had esophageal biopsies and eosinophilic esophagitis was diagnosed in 4 patients (6%). 107 patients had other morphological evaluations (CT scan, barium esophagogram, ultrasound endoscopy); 51 of them (48%) had some abnormalities that were considered as non-clinically relevant in 26%. 34 patients underwent esophageal pH-monitoring: pathological acid reflux was diagnosed in 9 patients (26%). Regarding HRM data, mean esophago-gastric junction (EGJ) resting pressure was 19.2 ± 15.8 mmHg and mean integrated relaxation pressure was 7.7 ± 7.2 mm Hg. 4.5 ± 2.7 swallows per patient had a DCI > 8000. Mean DCI of 10 swallows performed during the manometry protocol was 8942 ± 4549 mmHg.s.cm. Therapeutic data were available in 133 patients (49%): 38 did not receive any treatment; first line treatment was medical in 73 (PPI, calcium antagonist, nitrates) with a poor efficacy (50% no efficacy, 35.5 % moderate efficacy, 14.5 % success). 2nd and 3rd line treatments were mainly endoscopic: botox injection in 29 patients (success: 37.5 %; moderate efficacy: 37.3 %; no efficacy: 29.2%) and endoscopic dilation (success: 30 %; moderate efficacy: 40%; no efficacy: 30%). In patients with initial response, relapse rate was 35.3 % after Botox and 57.1 % after dilation. 2 patients were treated successfully with POEM (4 and 2.5 months follow up). Conclusion: This large cohort confirms that JE is a rare motility disorders. It might be associated with eosinophilic esophagitis or GERD. Diagnostic work-up is heterogeneous and should be standardized. Medical treatment and endoscopic therapies currently used have a low efficacy. POEM must be evaluated in a larger group of patients.
S-323
AGA Abstracts
AGA Abstracts
area, by manual definition of the borders, provides a more precise estimate of the volume of retained barium.