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2. Kahrilas PJ, Bredenoord AJ, Fox M, Gyawali CP, Roman S, Smout AJ, Pandolno JE, International High Resolution Manometry Working Group. The Chicago classication of esophageal motility disorders, v3.0. Neurogastroenterol Motil. 2015;27(2):160–74. https://doi.org/10.1111/
nmo.12477. Epub 2014 Dec 3.
3. Jia Y, Arenas J, Hejazi RA, Elhana S, Saadi M, McCallum RW.Frequency of jackhammer esophagus as the extreme phenotypes of esophageal hypercontractility based on the new Chicago classication. J Clin Gastroenterol. 2016;50(8):615–8. https://doi.org/10.1097/
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4. Kamal A, Shakya S, Lopez R, Thota PN.Gender, medication use and other factors associated with esophageal motility disorders in non­obstructive dysphagia. Gastroenterol Rep (Oxf). 2018;6(3):177–83.
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5. Kristo I, Schwameis K, Maschke S, Kainz A, Rieder E, Paireder M, Jomrich G, Schoppmann SF. Phenotypes of jackhammer esophagus in patients with typical symptoms of gastroesophageal reux disease responsive to proton pump inhibitors. Sci Rep. 2018;8(1):9949. https://
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6. Clément M, etal. Jackhammer esophagus: from manometric diagnosis to clinical presentation. Can J Gastroenterol Hepatol. 2019;2019:5036160. PMID: 30941328.
7. Al-Qaisi MT, Siddiki HA, Crowell MD, Burdick GE, Fleischer DE, Ramirez FC, Vela MF.The clinical signicance of hypercontractile peri­stalsis: comparison of high-resolution manometric features, demograph­ics, symptom presentation, and response to therapy in patients with jackhammer esophagus versus nutcracker esophagus. Dis Esophagus. 2017;30(12):1–7. https://doi.org/10.1093/dote/dox085.
8. Sato H, Takeuchi M, Takahashi K.Eosinophilic inltration of the muscu­laris propria in a patient with jackhammer esophagus treated with per­Oral endoscopic myotomy. Clin Gastroenterol Hepatol. 2015;13(4):e33–4.
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11. Abdallah J, Fass R. Progression of jackhammer esophagus to type II achalasia. J Neurogastroenterol Motil. 2016;22(1):153–6. https://doi.
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13. Amieva-Balmori M, Cano-Contreras AD, Remes-Troche JM. “Jackhammer esophagus” and eosinophilic esophagitis. Rev Gastroenterol Mex. 2015;80(3):217–9. https://doi.org/10.1016/j.rgmx.2015.03.006. Epub 2015 Aug 12.
14. Snyder DL, Crowell MD, Horsley-Silva J, Ravi K, Lacy BE, Vela MF.Opioid-induced esophageal dysfunction: differential effects of type and dose. Am J Gastroenterol. 2019;114(9):1464–9. https://doi.
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15. Herregods TVK, Smout AJPM, Ooi JLS, Sifrim D, Bredenoord AJ. Jackhammer esophagus: observations on a European Cohort. Neurogastroenterol Motil. 2017;29(4) https://doi.org/10.1111/
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17. Csucska M, Masuda T, Bremner RM, Mittal SK.Clinical symptom pre­sentation of hypercontractile peristalsis in the era of high-resolution manometry: a single-center experience. Dig Dis. 2020;38:355–63. https://
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18. Sato H, Hasegawa G, Takeuchi M, Takahashi K, Sato Y, Hashimoto S, Mizuno K, Kobayashi M.Education and imaging. Gastroenterology: a unique endoscopic technique for full-layer histology of jackhammer esophagus. J Gastroenterol Hepatol. 2015;30(8):1226. https://doi.
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19. García-Lledó J, Clemente-Sánchez A, Merino-Rodríguez B, Nogales­Rincón Ó, González-Asanza C, Menchén-Fernández-Pacheco P. Hypercontractile “jackhammer esophagus”. Rev Esp Enferm Dig. 2015;107(4):234. PMID: 25824925.
20. Kim JH, Kim SE, Cho YK, Lim C-H, In Park M, Hwang JW, Jang J-S, Minkyung O, Motility Study Club of Korean Society of Neurogastroenterology and Motility. Factors determining the inter­observer variability and diagnostic accuracy of high-resolution manome­try for esophageal motility disorders. J Neurogastroenterol Motil. 2018;24(1):58–69. https://doi.org/10.5056/jnm17064.
21. Mauro A, Quader F, Tolone S, Savarino E, De Bortoli N, Marianna Franchina C, Gyawali P, Penagini R.Provocative testing in patients with jackhammer esophagus: evidence for altered neural control. Am J Physiol Gastrointest Liver Physiol. 2019;316(3):G397–403. https://doi.
org/10.1152/ajpgi.00342.2018. Epub 2018 Dec 13.
22. Goel R, Anggiansah A, Wong T, Wilkinson M.A jackhammer in the gul­let: high amplitude oesophageal contractions as a cause of atypical chest pain. BMJ Case Rep. 2015;2015:bcr2015210631. https://doi.org/10.1136/
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23. Carlson DA, Gluskin AB, Mogni B, Koo J, Sood R, Lin Z, Pandolno JE.Esophageal diverticula are associated with propagating peristalsis: a study utilizing high-resolution manometry. Neurogastroenterol Motil. 2016;28(3):392–8. https://doi.org/10.1111/nmo.12739. Epub 2015 Dec
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24. Mallet A-L, Ropert A, Bouguen G, Siproudhis L, Boutroux D, Bretagne J-F, Brochard C.Prevalence and characteristics of acid gastro- oesophageal reux disease in jackhammer oesophagus. Dig Liver Dis. 2016;48(10):1136–41. https://doi.org/10.1016/j.dld.2016.06.030. Epub 2016 Jul 9.
25. Hong YS, Min YW, Rhee P-L.Two distinct types of hypercontractile esophagus: classic and spastic jackhammer. Gut Liver. 2016;10(5):859–
63. https://doi.org/10.5009/gnl15388.
26. Roman S, Kahrilas PJ.Management of spastic disorders of the esopha­gus. Gastroenterol Clin North Am. 2013;42(1):27–43. https://doi.
org/10.1016/j.gtc.2012.11.002. Epub 2013 Jan 4. PMID: 23452629;
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27. Swamy N.Esophageal spasm: clinical and manometric response to nitro­glycerine and long acting nitrites. Gastroenterology. 1977;72(1):23–7. PMID: 401445.
28. Hosaka H, Kawami N, Manabe N, Kuribayashi S, Sato H, Funaki Y, Ayaki M, Hara K, Ueda C, Matsumura T, Fujiwara Y, Wada M, Kishino M, Yano F, Masaoka T, Ishimura N, Akiyama J, Ochiai Y, Uraoka T, Iwakiri K.Clinical presentation and therapeutic outcome of patients with jackhammer esophagus-a multicenter cohort study in Japan. Esophagus. 2022;19(3):393–400. https://doi.org/10.1007/s10388- 022- 00916- 7. Epub 2022 Mar 6. PMID: 35249162; PMCID: PMC9166863.
29. Babaei A, Shad S, Massey BT.Esophageal hypercontractility is abolished by cholinergic blockade. Neurogastroenterol Motil. 2021;33(8):e14017.
https://doi.org/10.1111/nmo.14017. Epub 2020 Nov 13. PMID:
33185322.
30. Funaki Y, Ogasawara N, Kawamura Y, Yoshimine T, Tamura Y, Izawa S, Tsuyuki T, Ebi M, Sasaki M, Kasugai K.Markedly effective steroid treat­ment of three patients with allergy-related jackhammer esophagus. Intern Med. 2020;59(5):633–9. https://doi.org/10.2169/internalmedicine.3865-
19. Epub 2019 Nov 8.
31. Sato H, Takeuchi M, Takahashi K, Sato Y, Hashimoto S, Mizuno K, Suzuki K, Kobayashi M, Honma T, Inoue H, Terai S.Nutcracker and jackhammer esophagus treatment: a three-case survey, including two novel cases of eosinophilic inltration into the muscularis propria. Endoscopy. 2015;47(9):855–7. https://doi.org/10.1055/s- 0034- 1391985. Epub 2015 May 11.
32. Tanaka S, Toyonaga T, Kawara F, Watanabe D, Hoshi N, Abe H, Ariyoshi R, Ohara Y, Ishida T, Takao T, Morita Y, Umegaki E.A case of jackham­mer esophagus caused by eosinophilic esophagitis in which per-oral
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0868- y. Epub 2018 May 22.
33. Zerbib F, Roman S.Current therapeutic options for esophageal motor dis­orders as dened by the Chicago classication. J Clin Gastroenterol. 2015;49(6):451–60. https://doi.org/10.1097/MCG.0000000000000317.
34. Kawami N, Hoshino S, Hoshikawa Y, Takenouchi N, Hanada Y, Tanabe T, Koeda M, Monma E, Goto O, Kaise M, Iwakiri K.Differences in clinical characteristics between conservative-treatment-response group and refractory (surgical-treatment) group in patients with jackhammer esoph­agus. Esophagus. 2021;18(1):138–43. https://doi.org/10.1007/s10388-
020- 00748- 3. Epub 2020 May 26. PMID: 32458101.
35. Sakai Y, Tsuyuguchi T, Ishihara T, Kato K, Tsuboi M, Ooka Y, Katsuura K, Ohara T, Takayama S, Kimura M, Kasanuki J, Ai M, Yokosuka O.Conrmation of the antispasmodic effect of shakuyaku-kanzo-to (TJ-
68), a Chinese herbal medicine, on the duodenal wall by direct spraying during endoscopic retrograde cholangiopancreatography. J Nat Med. 2009;63(2):200–3. https://doi.org/10.1007/s11418- 008- 0304- 6. Epub 2008 Dec 3. PMID: 19050990.
36. Feng J, Ali RW, Hao J-y, Kong G-x, Yang L-h, Huang X-j. Peroral endo­scopic myotomy for esophageal motility disorders. Esophagus. 2020;17(1):11–8. https://doi.org/10.1007/s10388- 019- 00693- w.
37. Khan M, A, Kumbhari V, Ngamruengphong S, Ismail A, Chen Y-I, Chavez YH, Bukhari M, Nollan R, Ismail MK, Onimaru M, Balassone V, Sharata A, Swanstrom L, Inoue H, Repici A, Khashab MA.Is POEM the answer for management of spastic esophageal disorders? A systematic review and meta-analysis. Dig Dis Sci. 2017;62(1):35–44. https://doi.
org/10.1007/s10620- 016- 4373- 1. Epub 2016 Nov 17.
38. Bechara R, Ikeda H, Inoue H.Peroral endoscopic myotomy for jackham­mer esophagus: to cut or not to cut the lower esophageal sphincter. Endosc Int Open. 2016;4(5):E585–8. https://doi.
org/10.1055/s- 0042- 105204. Epub 2016 Apr 8. PMID: 27274539.
39. Khashab MA, Familiari P, Draganov PV, Aridi HD, Cho JY, Ujiki M, Tinto RR, Louis H, Desai PN, Velanovich V, Albéniz E, Haji A, Marks J, Costamagna G, Devière J, Perbtani Y, Hedberg M, Estremera F, Martin Del Campo LA, Yang D, Bukhari M, Brewer O, Sanaei O, Fayad L, Agarwal A, Kumbhari V, Chen Y-I.Peroral endoscopic myotomy is effec­tive and safe in non-achalasia esophageal motility disorders: an interna­tional multicenter study. Endosc Int Open. 2018;6(8):E1031–6. https://
doi.org/10.1055/a- 0625- 6288. Epub 2018 Aug 10.
40. Khashab MA, Messallam AA, Onimaru M, Teitelbaum EN, Ujiki MB, Gitelis ME, Modayil RJ, Hungness ES, Stavropoulos SN, El Zein MH, Shiwaku H, Kunda R, Repici A, Minami H, Chiu PW, Ponsky J, Kumbhari V, Saxena P, Maydeo AP, Inoue H.International multicenter experience with peroral endoscopic myotomy for the treatment of spastic esophageal
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41. Ramos RI, Guidi M, Kakisu MH, Rocca AM, Sakai P.Peroral endoscopic myotomy in pediatric jackhammer esophagus. Rev Esp Enferm Dig. 2018;110(7):464–5. https://doi.org/10.17235/reed.2018.5090/2017. PMID: 29667413.
42. Estremera-Arévalo F, Albéniz E, Rullán M, Areste I, Iglesias R, Vila JJ.Efcacy of peroral endoscopic myotomy compared with other inva­sive treatment options for the different esophageal motor disorders. Rev Esp Enferm Dig. 2017;109(8):578–86. https://doi.org/10.17235/
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06819- 6. Epub 2019 May 9.
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Motility Disorders:
21
Diagnosis andManagement
MeredithA.Harrison, RonakModi, RodrigoDuarte-Chavez, andAndrewM.Brown
Introduction
Disorders of esophageal motility are dened by using high resolu­tion manometry (HRM) with color pressure topography plots and categorized according to version 3.0 of the Chicago Classication of esophageal motility disorders, last updated in 2014 [1]. Using a tiered approach, the Chicago Classication system denes the primary esophageal motility disorders. In addition to those major and minor disorders, there are other systemic and localized pathologies which secondarily lead to disorders of motility. As a result of these secondary esophageal motility disorders being rel-
M. A. Harrison · A. M. Brown (*) Department of Surgery, St. Luke’s University Hospital, Bethlehem, PA, USA e-mail: Meredith.harrison@sluhn.org; andrew.brown2@sluhn.org
R. Modi · R. Duarte-Chavez Department of Gastroenterology, St. Luke’s University Hospital, Bethlehem, PA, USA e-mail: Ronak.Modi@sluhn.org; Rodrigo.Chavez@sluhn.org
© Society of American Gastrointestinal and Endoscopic Surgeons (SAGES) 2023 A. D. Patel et al. (eds.), The SAGES Manual of Physiologic Evaluation of Foregut Diseases,
https://doi.org/10.1007/978-3-031-39199-6_21
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M. A. Harrison et al.
atively rare, diagnoses can often be delayed, and management can be challenging. The knowledgeable foregut surgeon should be prepared for the complexities of these conditions and able to treat these patients in conjunction with a multidisciplinary team.
Case #1
Patient Information
A 62-year-old female presents to the outpatient ofce with atypical chest pain and dysphagia to solids and liquids associated with uncontrollable heartburn despite medical therapy. Physical exam reveals telangiectasia, muscle atrophy with sclerodactyly, and Raynaud phenomenon. On laboratory workup, anti­centromere antibodies were positive.
Manometry
Figure 21.1 shows the HRM for this patient. By Chicago clas­sication, this patient has ineffective esophageal motility with scleroderma pattern. The mean distal contractile integral (DCI) for this patient was 50 mmHg/s/cm, with a median integrated relaxation pressure (IRP) of 1mmHg.
Fig. 21.1 High Resolution Manometry in Scleroderma. Mean DCI: 50mmHg/s/cm, Median IRP: 1mmHg
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293
Background
Systemic Sclerosis (SSc) or Scleroderma is a connective tissue disorder with 8:2 female to male predominance and documented histological esophageal involvement in up to 90% of patients [2].
While the percentage of histologic involvement of the esopha­gus in patients with SSc is quite high, the incidence of esophageal symptoms ranges from 40 to 80%. Patients with esophageal dys­function have two broad categories of symptoms: heartburn and regurgitation secondary to gastroesophageal reux disease (GERD), or dysphagia and chest pain secondary to esophageal dysmotility [2].
Workup can include pH testing with or without impedance and upper endoscopy, but manometry is imperative. Classic sclero­derma esophagus is characterized by absent or ineffective peri­stalsis with a hypotensive lower esophageal sphincter (LES). A hypotensive LES is dened as less than 10mmHg. Upwards of 50% of symptomatic patients will present with this manometric pattern. Additionally, chest CT may reveal a dilated esophagus in >70% of patients who present with the classic scleroderma esoph­agus pattern on esophageal manometry [3].
Management is aimed at symptom improvement, either man­aging GERD symptoms or dysmotility symptoms. GERD man­agement is centered on proton pump inhibitor (PPI) therapy, but although there is short term benet, the long-term efcacy is not sustained, and there is no prevention of progression of esophageal dysfunction [4, 5]. Common practice includes a 2–4× increase in the daily dose of PPI to gain better symptom control [6]. Surgical management for GERD symptoms of SSc should avoid anti-reux procedures such as Nissen fundoplication, as 38–71% of patients can develop postoperative dysphagia despite improvement of reux symptoms [79]. Some authors advocate Roux-en-y gastric bypass to prevent reux, which has been shown to have better post-op GER-related quality of life and less dysphagia as com­pared to Nissen [10]. However, bypass in a scleroderma patient should be pursued with caution given the propensity of small intestinal dysmotility in SSc patients. An esophagectomy can be considered for end-stage scleroderma of the esophagus.
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Esophageal dysmotility symptoms can be improved with vari­ous prokinetic drugs such as metoclopramide, erythromycin, and cisapride [1113]. Ciaspride has been shown to increase LES pressure and the amplitude of distal esophageal body peristalsis in SSc patients [11, 14]. Domperidone, a peripheral dopamine antagonist, is commonly used, but the literature questions the effect of symptom and manometric improvement of this drug’s administration [15]. An emerging therapy, buspirone a 5- hydroxytryptamine 1A (5-HT1A) agonist, has been shown to increase LES resting pressure and improve the severity of heart­burn and regurgitation in SSc patients in small studies [16].
Case #2
Patient Information
A 39-year-old male presents to the ofce with complaints of signicant heartburn and regurgitation. His medical history is sig­nicant for chronic GERD on medical therapy and obesity, with a social history signicant for smoking.
Manometry
Figure 21.2 shows the HRM for this patient. The manometry pinpoints a specic reux event.
Fig. 21.2 High Resolution Manometry in GERD. Arrow indicates a reux event during study.
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Background
Gastroesophageal reux disease (GERD), while highly preva­lent in Western countries, displays heterogeneity in terms of clini­cal features, pathophysiologic mechanisms, and response to acid suppression [17]. The pathogenesis is multifactorial, but includes: transient lower esophageal sphincter (LES) relaxations, hypoten­sion of the LES, impairment of the gastroesophageal junction (i.e., hiatal hernia), ineffective esophageal acid and bolus clear­ance, delayed gastric emptying, and impaired mucosal defensive factors. The major mechanisms for esophageal dysmotility in GERD are due to impairments at the gastroesophageal junction (transient LES relaxation, hypotensive LES, or hiatal hernia) and ineffective esophageal motility [1719]. Furthermore, persistent injury to the esophageal mucosa is associated with dysmotility.
Transient LES relaxations are 10–60s periods of LES relax­ation that are not preceded by a swallow and are deemed sponta­neous [20]. These episodes make up the majority of the periods of reux in patients with moderate GERD.As the severity of GERD increases, a hypotensive LES becomes the prevalent pathophysiological mechanism. Dened as an LES basal pressure less than 10mmHg, a hypotensive LES is found with increasing prevalence as the severity of GERD increases, such as in patients with Barrett’s esophagus, erosive esophagitis, and nonerosive reux disease [21, 22].
Ineffective esophageal motility, dened as hypocontractility in greater than 30% of swallows, is the most prevalent esophageal motor disorder in GERD [23, 24]. Furthermore, worsening peri­staltic dysfunction is increasingly prevalent with more severe GERD [21, 23, 25, 26]. When contractility is preserved, major peristaltic breaks can be observed resulting in fragmented peri­stalsis [27].
Diagnosis of esophageal dysmotility in the setting of GERD is done with high resolution manometry, often combined with impedance, which can help identify abnormal bolus transport and clearance during swallows and investigate the relationships between bolus transit and LES relaxation [28]. Treatment with a proton pump inhibitor will decrease acid exposure, but does not improve esophageal dysmotility. Additionally, weight loss will