Diagnosis and Management of Achalasia: Updates of the Last Two Years

Amir Mari, Fadi Abu Baker, Rinaldo Pellicano, Tawfik Khoury, Amir Mari, Fadi Abu Baker, Rinaldo Pellicano, Tawfik Khoury

Abstract

Achalasia is a rare neurodegenerative disorder causing dysphagia and is characterized by abnormal esophageal motor function as well as the loss of lower esophageal sphincter (LES) relaxation. The assessment and management of achalasia has significantly progressed in recent years due to the advances in high-resolution manometry (HRM) technology along with the improvements and innovations of therapeutic endoscopy procedures. The recent evolution of HRM technology with the inclusion of an adjunctive test, fluoroscopy, and EndoFLIP has enabled more precise diagnoses of achalasia to be made and the subgrouping into therapeutically meaningful subtypes. Current management possibilities include endoscopic treatments such as Botulinum toxin injected to the LES and pneumatic balloon dilation. Surgical treatment includes laparoscopic Heller myotomy and esophagectomy. Furthermore, in recent years, per oral endoscopic myotomy (POEM) has established itself as a principal endoscopic therapeutic alternative to the traditional laparoscopic Heller myotomy. The latest randomized trials report that POEM, pneumatic balloon dilatation, and laparoscopic Heller's myotomy have comparable effectiveness and complications rates. The aim of the current review is to provide a practical clinical approach to dysphagia and to shed light on the most recent improvements in diagnostics and treatment of achalasia over the last two years.

Keywords: achalasia; diagnosis; dysphagia; high resolution manometry (HRM); management; per oral endoscopic myotomy (POEM).

Conflict of interest statement

The authors declare no conflict of interest regarding this manuscript.

Figures

Figure 1
Figure 1
A five milliliter water swallow starts with the opening of the upper esophageal sphincter (UES). One normal esophageal peristalsis and normal lower esophageal sphincter (LES) relaxation is shown. The LES relaxation is measured over a 10 s period, as shown in the box, by measuring the median integrated relaxation pressure (IRP) (supplied from the gastroenterology department at EMMS Nazareth hospital).
Figure 2
Figure 2
The three achalasia subtypes determined by the Chicago classification (supplied from the gastroenterology department at EMMS Nazareth hospital).

References

    1. Pandolfino J.E., Gawron A.J. Achalasia: A systematic review. JAMA. 2015;313:1841–1852. doi: 10.1001/jama.2015.2996.
    1. Williams V.A., Peters J.H. Achalasia of the esophagus: A surgical disease. J. Am. Coll. Surg. 2009;208:151–162. doi: 10.1016/j.jamcollsurg.2008.08.027.
    1. Khashab M.A., Vela M.F., Thosani N., Agrawal D., Buxbaum J.L., Abbas Fehmi S.M., Fishman D.S., Gurudu S.R., Jamil L.H., Jue T.L., et al. ASGE guideline on the management of achalasia. Gastrointest. Endosc. 2020;91:213–227. doi: 10.1016/j.gie.2019.04.231.
    1. Vaezi M.F., Pandolfino J.E., Yadlapati R.H., Greer K.B., Kavitt R.T. ACG Clinical Guidelines: Diagnosis and Management of Achalasia. Am. J. Gastroenterol. 2020;115:1393–1411. doi: 10.14309/ajg.0000000000000731.
    1. Eckardt V.F., Hoischen T., Bernhard G. Life expectancy, complications, and causes of death in patients with achalasia: Results of a 33-year follow-up investigation. Eur. J. Gastroenterol. Hepatol. 2008;20:956–960. doi: 10.1097/MEG.0b013e3282fbf5e5.
    1. Zaninotto G., Bennett C., Boeckxstaens G., Costantini M., Ferguson M.K., Pandolfino J.E., Patti M.G., Ribeiro U., Jr., Richter J., Swanstrom L., et al. The 2018 ISDE achalasia guidelines. Dis. Esophagus. 2018;31 doi: 10.1093/dote/doy071.
    1. Cassella R.R., Ellis F.H., Jr., Brown A.L., Jr. Fine-Structure Changes in Achalasia of Esophagus. Ii. Esophageal Smooth Muscle. Am. J. Pathol. 1965;46:467–475.
    1. Ota M., Narumiya K., Kudo K., Yagawa Y., Maeda S., Osugi H., Yamamoto M. Incidence of Esophageal Carcinomas After Surgery for Achalasia: Usefulness of Long-Term and Periodic Follow-up. Am. J. Case Rep. 2016;17:845–849. doi: 10.12659/AJCR.899800.
    1. Torres-Aguilera M., Remes Troche J.M. Achalasia and esophageal cancer: Risks and links. Clin. Exp. Gastroenterol. 2018;11:309–316. doi: 10.2147/CEG.S141642.
    1. Francis D.L., Katzka D.A. Achalasia: Update on the disease and its treatment. Gastroenterology. 2010;139:369–374. doi: 10.1053/j.gastro.2010.06.024.
    1. Gockel I., Becker J., Wouters M.M., Niebisch S., Gockel H.R., Hess T., Ramonet D., Zimmermann J., Vigo A.G., Trynka G., et al. Common variants in the HLA-DQ region confer susceptibility to idiopathic achalasia. Nat. Genet. 2014;46:901–904. doi: 10.1038/ng.3029.
    1. Raymond L., Lach B., Shamji F.M. Inflammatory aetiology of primary oesophageal achalasia: An immunohistochemical and ultrastructural study of Auerbach's plexus. Histopathology. 1999;35:445–453. doi: 10.1046/j.1365-2559.1999.035005445.x.
    1. Jin H., Wang B., Zhang L.L., Zhao W. Activated Eosinophils are Present in Esophageal Muscle in Patients with Achalasia of the Esophagus. Med. Sci. Monit. 2018;24:2377–2383. doi: 10.12659/MSM.909727.
    1. Liu Z.Q., Chen W.F., Wang Y., Xu X.Y., Zeng Y.G., Lee Dillon D., Cheng J., Xu M.D., Zhong Y.S., Zhang Y.Q., et al. Mast cell infiltration associated with loss of interstitial cells of Cajal and neuronal degeneration in achalasia. Neurogastroenterol. Motil. 2019;31:e13565. doi: 10.1111/nmo.13565.
    1. Clayton S., Cauble E., Kumar A., Patil N., Ledford D., Kolliputi N., Lopes-Virella M.F., Castell D., Richter J. Plasma levels of TNF-alpha, IL-6, IFN-gamma, IL-12, IL-17, IL-22, and IL-23 in achalasia, eosinophilic esophagitis (EoE), and gastroesophageal reflux disease (GERD) BMC Gastroenterol. 2019;19:28. doi: 10.1186/s12876-019-0937-9.
    1. Spechler S.J., Konda V., Souza R. Can Eosinophilic Esophagitis Cause Achalasia and Other Esophageal Motility Disorders? Am. J. Gastroenterol. 2018;113:1594–1599. doi: 10.1038/s41395-018-0240-3.
    1. Skaper S.D., Facci L., Zusso M., Giusti P. Neuroinflammation, Mast Cells, and Glia: Dangerous Liaisons. Neuroscientist. 2017;23:478–498. doi: 10.1177/1073858416687249.
    1. Nakajima N., Sato H., Takahashi K., Hasegawa G., Mizuno K., Hashimoto S., Sato Y., Terai S. Muscle layer histopathology and manometry pattern of primary esophageal motility disorders including achalasia. Neurogastroenterol. Motil. 2017;29 doi: 10.1111/nmo.12968.
    1. Goldblum J.R., Rice T.W., Richter J.E. Histopathologic features in esophagomyotomy specimens from patients with achalasia. Gastroenterology. 1996;111:648–654. doi: 10.1053/gast.1996.v111.pm8780569.
    1. Zarate N., Wang X.Y., Tougas G., Anvari M., Birch D., Mearin F., Malagelada J.R., Huizinga J.D. Intramuscular interstitial cells of Cajal associated with mast cells survive nitrergic nerves in achalasia. Neurogastroenterol. Motil. 2006;18:556–568. doi: 10.1111/j.1365-2982.2006.00788.x.
    1. Villanacci V., Annese V., Cuttitta A., Fisogni S., Scaramuzzi G., De Santo E., Corazzi N., Bassotti G. An immunohistochemical study of the myenteric plexus in idiopathic achalasia. J. Clin. Gastroenterol. 2010;44:407–410. doi: 10.1097/MCG.0b013e3181bc9ebf.
    1. Clark S.B., Rice T.W., Tubbs R.R., Richter J.E., Goldblum J.R. The nature of the myenteric infiltrate in achalasia: An immunohistochemical analysis. Am. J. Surg. Pathol. 2000;24:1153–1158. doi: 10.1097/00000478-200008000-00014.
    1. Furuzawa-Carballeda J., Aguilar-Leon D., Gamboa-Dominguez A., Valdovinos M.A., Nunez-Alvarez C., Martin-del-Campo L.A., Enriquez A.B., Coss-Adame E., Svarch A.E., Flores-Najera A., et al. Achalasia--An Autoimmune Inflammatory Disease: A Cross-Sectional Study. J. Immunol. Res. 2015;2015:729217. doi: 10.1155/2015/729217.
    1. Torres-Landa S., Furuzawa-Carballeda J., Coss-Adame E., Valdovinos M.A., Alejandro-Medrano E., Ramos-Avalos B., Martinez-Benitez B., Torres-Villalobos G. Barrett’s Oesophagus in an Achalasia Patient: Immunological Analysis and Comparison with a Group of Achalasia Patients. Case Rep. Gastrointest. Med. 2016;2016:5681590. doi: 10.1155/2016/5681590.
    1. Kilic A., Owens S.R., Pennathur A., Luketich J.D., Landreneau R.J., Schuchert M.J. An increased proportion of inflammatory cells express tumor necrosis factor alpha in idiopathic achalasia of the esophagus. Dis. Esophagus. 2009;22:382–385. doi: 10.1111/j.1442-2050.2008.00922.x.
    1. Wu X.Y., Liu Z.Q., Wang Y., Chen W.F., Gao P.T., Li Q.L., Zhou P.H. The etiology of achalasia: An immune-dominant disease. J. Dig. Dis. 2021;22:126–135. doi: 10.1111/1751-2980.12973.
    1. Ganem D., Kistler A., DeRisi J. Achalasia and viral infection: New insights from veterinary medicine. Sci. Transl. Med. 2010;2:33ps24. doi: 10.1126/scitranslmed.3000986.
    1. Pressman A., Behar J. Etiology and Pathogenesis of Idiopathic Achalasia. J. Clin. Gastroenterol. 2017;51:195–202. doi: 10.1097/MCG.0000000000000780.
    1. Facco M., Brun P., Baesso I., Costantini M., Rizzetto C., Berto A., Baldan N., Palu G., Semenzato G., Castagliuolo I., et al. T cells in the myenteric plexus of achalasia patients show a skewed TCR repertoire and react to HSV-1 antigens. Am. J. Gastroenterol. 2008;103:1598–1609. doi: 10.1111/j.1572-0241.2008.01956.x.
    1. Kahrilas P.J., Boeckxstaens G. The spectrum of achalasia: Lessons from studies of pathophysiology and high-resolution manometry. Gastroenterology. 2013;145:954–965. doi: 10.1053/j.gastro.2013.08.038.
    1. Jones D.B., Mayberry J.F., Rhodes J., Munro J. Preliminary report of an association between measles virus and achalasia. J. Clin. Pathol. 1983;36:655–657. doi: 10.1136/jcp.36.6.655.
    1. Wang A.J., Tu L.X., Yu C., Zheng X.L., Hong J.B., Lu N.H. Achalasia secondary to cardial tuberculosis caused by AIDS. J. Dig. Dis. 2015;16:752–753. doi: 10.1111/1751-2980.12287.
    1. Mari A., Patel K., Mahamid M., Khoury T., Pesce M. Achalasia: Insights into Diagnostic and Therapeutic Advances for an Ancient Disease. Rambam Maimonides Med. J. 2019;10 doi: 10.5041/RMMJ.10361.
    1. Woodfield C.A., Levine M.S., Rubesin S.E., Langlotz C.P., Laufer I. Diagnosis of primary versus secondary achalasia: Reassessment of clinical and radiographic criteria. AJR Am. J. Roentgenol. 2000;175:727–731. doi: 10.2214/ajr.175.3.1750727.
    1. Mari A., Abu Baker F., Mahamid M., Khoury T., Sbeit W., Pellicano R. Eosinophilic esophagitis: Pitfalls and controversies in diagnosis and management. Minerva Med. 2020;111:9–17. doi: 10.23736/S0026-4806.19.06322-5.
    1. Mari A., Sweis R. Assessment and management of dysphagia and achalasia. Clin. Med. 2021;21:119–123. doi: 10.7861/clinmed.2021-0069.
    1. Yadlapati R., Kahrilas P.J., Fox M.R., Bredenoord A.J., Prakash Gyawali C., Roman S., Babaei A., Mittal R.K., Rommel N., Savarino E., et al. Esophageal motility disorders on high-resolution manometry: Chicago classification version 4.0((c)) Neurogastroenterol. Motil. 2021;33:e14058. doi: 10.1111/nmo.14058.
    1. Kahrilas P.J., Bredenoord A.J., Fox M., Gyawali C.P., Roman S., Smout A.J., Pandolfino J.E., International High Resolution Manometry Working G. The Chicago Classification of esophageal motility disorders, v3.0. Neurogastroenterol. Motil. 2015;27:160–174. doi: 10.1111/nmo.12477.
    1. Sanagapalli S., McGuire J., Leong R.W., Patel K., Raeburn A., Abdul-Razakq H., Plumb A., Banks M., Haidry R., Lovat L., et al. The Clinical Relevance of Manometric Esophagogastric Junction Outflow Obstruction Can Be Determined Using Rapid Drink Challenge and Solid Swallows. Am. J. Gastroenterol. 2021;116:280–288. doi: 10.14309/ajg.0000000000000988.
    1. Ang D., Misselwitz B., Hollenstein M., Knowles K., Wright J., Tucker E., Sweis R., Fox M. Diagnostic yield of high-resolution manometry with a solid test meal for clinically relevant, symptomatic oesophageal motility disorders: Serial diagnostic study. Lancet Gastroenterol. Hepatol. 2017;2:654–661. doi: 10.1016/S2468-1253(17)30148-6.
    1. Wang Y.T., Tai L.F., Yazaki E., Jafari J., Sweis R., Tucker E., Knowles K., Wright J., Ahmad S., Kasi M., et al. Investigation of Dysphagia After Antireflux Surgery by High-resolution Manometry: Impact of Multiple Water Swallows and a Solid Test Meal on Diagnosis, Management, and Clinical Outcome. Clin. Gastroenterol. Hepatol. 2015;13:1575–1583. doi: 10.1016/j.cgh.2015.04.181.
    1. Araujo I.K., Roman S., Napoleon M., Mion F. Diagnostic yield of adding solid food swallows during high-resolution manometry in esophageal motility disorders. Neurogastroenterol. Motil. 2021;33:e14060. doi: 10.1111/nmo.14060.
    1. Hollenstein M., Thwaites P., Butikofer S., Heinrich H., Sauter M., Ulmer I., Pohl D., Ang D., Eberli D., Schwizer W., et al. Pharyngeal swallowing and oesophageal motility during a solid meal test: A prospective study in healthy volunteers and patients with major motility disorders. Lancet Gastroenterol. Hepatol. 2017;2:644–653. doi: 10.1016/S2468-1253(17)30151-6.
    1. Ang D., Hollenstein M., Misselwitz B., Knowles K., Wright J., Tucker E., Sweis R., Fox M. Rapid Drink Challenge in high-resolution manometry: An adjunctive test for detection of esophageal motility disorders. Neurogastroenterol. Motil. 2017;29 doi: 10.1111/nmo.12902.
    1. Babaei A., Szabo A., Yorio S.D., Massey B.T. Pressure exposure and catheter impingement affect the recorded pressure in the Manoscan 360 system. Neurogastroenterol. Motil. 2018 doi: 10.1111/nmo.13329.
    1. Lynch K.L., Yang Y.X., Metz D.C., Falk G.W. Clinical presentation and disease course of patients with esophagogastric junction outflow obstruction. Dis. Esophagus. 2017;30:1–6. doi: 10.1093/dote/dox004.
    1. DeLay K., Austin G.L., Menard-Katcher P. Anatomic abnormalities are common potential explanations of manometric esophagogastric junction outflow obstruction. Neurogastroenterol. Motil. 2016;28:1166–1171. doi: 10.1111/nmo.12814.
    1. Biasutto D., Mion F., Garros A., Roman S. Rapid drink challenge test during esophageal high resolution manometry in patients with esophago-gastric junction outflow obstruction. Neurogastroenterol. Motil. 2018;30:e13293. doi: 10.1111/nmo.13293.
    1. Triggs J.R., Carlson D.A., Beveridge C., Jain A., Tye M.Y., Kahrilas P.J., Pandolfino J.E. Upright Integrated Relaxation Pressure Facilitates Characterization of Esophagogastric Junction Outflow Obstruction. Clin. Gastroenterol. Hepatol. 2019;17:2218–2226.e2212. doi: 10.1016/j.cgh.2019.01.024.
    1. Rohof W.O., Lei A., Boeckxstaens G.E. Esophageal stasis on a timed barium esophagogram predicts recurrent symptoms in patients with long-standing achalasia. Am. J. Gastroenterol. 2013;108:49–55. doi: 10.1038/ajg.2012.318.
    1. Sanagapalli S., Plumb A., Maynard J., Leong R.W., Sweis R. The timed barium swallow and its relationship to symptoms in achalasia: Analysis of surface area and emptying rate. Neurogastroenterol. Motil. 2020;32:e13928. doi: 10.1111/nmo.13928.
    1. Carlson D.A., Kahrilas P.J., Lin Z., Hirano I., Gonsalves N., Listernick Z., Ritter K., Tye M., Ponds F.A., Wong I., et al. Evaluation of Esophageal Motility Utilizing the Functional Lumen Imaging Probe. Am. J. Gastroenterol. 2016;111:1726–1735. doi: 10.1038/ajg.2016.454.
    1. O’Neill O.M., Johnston B.T., Coleman H.G. Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomes. World J. Gastroenterol. 2013;19:5806–5812. doi: 10.3748/wjg.v19.i35.5806.
    1. Eckardt V.F., Gockel I., Bernhard G. Pneumatic dilation for achalasia: Late results of a prospective follow up investigation. Gut. 2004;53:629–633. doi: 10.1136/gut.2003.029298.
    1. Patel D.A., Sharda R., Hovis K.L., Nichols E.E., Sathe N., Penson D.F., Feurer I.D., McPheeters M.L., Vaezi M.F., Francis D.O. Patient-reported outcome measures in dysphagia: A systematic review of instrument development and validation. Dis. Esophagus. 2017;30:1–23. doi: 10.1093/dote/dow028.
    1. Pasricha P.J., Ravich W.J., Hendrix T.R., Sostre S., Jones B., Kalloo A.N. Intrasphincteric botulinum toxin for the treatment of achalasia. N. Engl. J. Med. 1995;332:774–778. doi: 10.1056/NEJM199503233321203.
    1. Boeckxstaens G.E., Annese V., des Varannes S.B., Chaussade S., Costantini M., Cuttitta A., Elizalde J.I., Fumagalli U., Gaudric M., Rohof W.O., et al. Pneumatic dilation versus laparoscopic Heller’s myotomy for idiopathic achalasia. N. Engl. J. Med. 2011;364:1807–1816. doi: 10.1056/NEJMoa1010502.
    1. Vaezi M.F., Pandolfino J.E., Vela M.F. ACG clinical guideline: Diagnosis and management of achalasia. Am. J. Gastroenterol. 2013;108:1238–1249. doi: 10.1038/ajg.2013.196. quiz 1250.
    1. Kilic A., Schuchert M.J., Pennathur A., Gilbert S., Landreneau R.J., Luketich J.D. Long-term outcomes of laparoscopic Heller myotomy for achalasia. Surgery. 2009;146:826–831. doi: 10.1016/j.surg.2009.06.049.
    1. Lynch K.L., Pandolfino J.E., Howden C.W., Kahrilas P.J. Major complications of pneumatic dilation and Heller myotomy for achalasia: Single-center experience and systematic review of the literature. Am. J. Gastroenterol. 2012;107:1817–1825. doi: 10.1038/ajg.2012.332.
    1. Campos G.M., Vittinghoff E., Rabl C., Takata M., Gadenstatter M., Lin F., Ciovica R. Endoscopic and surgical treatments for achalasia: A systematic review and meta-analysis. Ann. Surg. 2009;249:45–57. doi: 10.1097/SLA.0b013e31818e43ab.
    1. Ujiki M.B., Yetasook A.K., Zapf M., Linn J.G., Carbray J.M., Denham W. Peroral endoscopic myotomy: A short-term comparison with the standard laparoscopic approach. Surgery. 2013;154:893–897. doi: 10.1016/j.surg.2013.04.042.
    1. Kumbhari V., Tieu A.H., Onimaru M., El Zein M.H., Teitelbaum E.N., Ujiki M.B., Gitelis M.E., Modayil R.J., Hungness E.S., Stavropoulos S.N., et al. Peroral endoscopic myotomy (POEM) vs laparoscopic Heller myotomy (LHM) for the treatment of Type III achalasia in 75 patients: A multicenter comparative study. Endosc. Int. Open. 2015;3:E195–E201. doi: 10.1055/s-0034-1391668.
    1. Kahaleh M., Tyberg A., Suresh S., Lambroza A., Casas F.R., Rey M., Nieto J., Martinez G.M., Zamarripa F., Arantes V., et al. The Learning Curve for Peroral Endoscopic Myotomy in Latin America: A Slide to the Right? Clin. Endosc. 2021 doi: 10.5946/ce.2020.290.
    1. Patel K.S., Calixte R., Modayil R.J., Friedel D., Brathwaite C.E., Stavropoulos S.N. The light at the end of the tunnel: A single-operator learning curve analysis for per oral endoscopic myotomy. Gastrointest. Endosc. 2015;81:1181–1187. doi: 10.1016/j.gie.2014.10.002.
    1. Rohof W.O., Salvador R., Annese V., Bruley des Varannes S., Chaussade S., Costantini M., Elizalde J.I., Gaudric M., Smout A.J., Tack J., et al. Outcomes of treatment for achalasia depend on manometric subtype. Gastroenterology. 2013;144:718–725. doi: 10.1053/j.gastro.2012.12.027.
    1. Oude Nijhuis R.A.B., Prins L.I., Mostafavi N., van Etten-Jamaludin F.S., Smout A., Bredenoord A.J. Factors Associated With Achalasia Treatment Outcomes: Systematic Review and Meta-Analysis. Clin. Gastroenterol. Hepatol. 2020;18:1442–1453. doi: 10.1016/j.cgh.2019.10.008. quiz e713–714.

Source: PubMed

3
Předplatit