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Esophageal myenteric plexus degeneration

MedGen UID:
1841707
•Concept ID:
C5826446
•
Disease or Syndrome
HPO: HP:0034520

Definition

Deterioration of the cells of the myenteric plexus (also known as the Auerbach plexus) located between the layers of the muscular propria of the esophagus. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEsophageal myenteric plexus degeneration

Professional guidelines

PubMed

ASGE guideline on the management of achalasia.
Khashab MA, Vela MF, Thosani N, Agrawal D, Buxbaum JL, Abbas Fehmi SM, Fishman DS, Gurudu SR, Jamil LH, Jue TL, Kannadath BS, Law JK, Lee JK, Naveed M, Qumseya BJ, Sawhney MS, Yang J, Wani S
Gastrointest Endosc 2020 Feb;91(2):213-227.e6. Epub 2019 Dec 13 doi: 10.1016/j.gie.2019.04.231. PMID: 31839408
A controversy that has been tough to swallow: is the treatment of achalasia now digested?
Roll GR, Rabl C, Ciovica R, Peeva S, Campos GM
J Gastrointest Surg 2010 Feb;14 Suppl 1(Suppl 1):S33-45. Epub 2009 Sep 17 doi: 10.1007/s11605-009-1013-5. PMID: 19760373Free PMC Article

Recent clinical studies

Etiology

Achalasia alters physiological networks depending on sex.
Furuzawa-Carballeda J, Barajas-Martínez A, Olguín-Rodríguez PV, Ibarra-Coronado E, Fossion R, Coss-Adame E, Valdovinos MA, Torres-Villalobos G, Rivera AL
Sci Rep 2024 Jan 24;14(1):2072. doi: 10.1038/s41598-024-52273-3. PMID: 38267468Free PMC Article
Whole-exome sequencing reveals common and rare variants in immunologic and neurological genes implicated in achalasia.
Li Q, Chen W, Wang C, Liu Z, Gu Y, Xu X, Xu J, Jiang T, Xu M, Wang Y, Chen C, Zhong Y, Zhang Y, Yao L, Jin G, Hu Z, Zhou P
Am J Hum Genet 2021 Aug 5;108(8):1478-1487. Epub 2021 Jun 30 doi: 10.1016/j.ajhg.2021.06.004. PMID: 34197731Free PMC Article
Pathogenesis of achalasia cardia.
Ghoshal UC, Daschakraborty SB, Singh R
World J Gastroenterol 2012 Jun 28;18(24):3050-7. doi: 10.3748/wjg.v18.i24.3050. PMID: 22791940Free PMC Article
Achalasia in the elderly patient: a comparative study.
Schechter RB, Lemme EM, Novais P, Biccas B
Arq Gastroenterol 2011 Jan-Mar;48(1):19-23. doi: 10.1590/s0004-28032011000100005. PMID: 21537537
Etiology and pathogenesis of achalasia: the current understanding.
Park W, Vaezi MF
Am J Gastroenterol 2005 Jun;100(6):1404-14. doi: 10.1111/j.1572-0241.2005.41775.x. PMID: 15929777

Diagnosis

Systematic Review of Treatment of Patients with Achalasia: Heller Myotomy, Pneumatic Dilation, and Peroral Endoscopic Myotomy.
Ciomperlik H, Dhanani NH, Mohr C, Hannon C, Olavarria OA, Holihan JL, Liang MK
J Am Coll Surg 2023 Mar 1;236(3):523-532. Epub 2022 Nov 16 doi: 10.1097/XCS.0000000000000484. PMID: 36382896
ASGE guideline on the management of achalasia.
Khashab MA, Vela MF, Thosani N, Agrawal D, Buxbaum JL, Abbas Fehmi SM, Fishman DS, Gurudu SR, Jamil LH, Jue TL, Kannadath BS, Law JK, Lee JK, Naveed M, Qumseya BJ, Sawhney MS, Yang J, Wani S
Gastrointest Endosc 2020 Feb;91(2):213-227.e6. Epub 2019 Dec 13 doi: 10.1016/j.gie.2019.04.231. PMID: 31839408
Achalasia in the elderly patient: a comparative study.
Schechter RB, Lemme EM, Novais P, Biccas B
Arq Gastroenterol 2011 Jan-Mar;48(1):19-23. doi: 10.1590/s0004-28032011000100005. PMID: 21537537
A controversy that has been tough to swallow: is the treatment of achalasia now digested?
Roll GR, Rabl C, Ciovica R, Peeva S, Campos GM
J Gastrointest Surg 2010 Feb;14 Suppl 1(Suppl 1):S33-45. Epub 2009 Sep 17 doi: 10.1007/s11605-009-1013-5. PMID: 19760373Free PMC Article
Radiologic and histologic differentiation of neuromuscular disorders of the gastrointestinal tract: visceral myopathies, visceral neuropathies, and progressive systemic sclerosis.
Rohrmann CA Jr, Ricci MT, Krishnamurthy S, Schuffler MD
AJR Am J Roentgenol 1984 Nov;143(5):933-41. doi: 10.2214/ajr.143.5.933. PMID: 6333163

Therapy

Systematic Review of Treatment of Patients with Achalasia: Heller Myotomy, Pneumatic Dilation, and Peroral Endoscopic Myotomy.
Ciomperlik H, Dhanani NH, Mohr C, Hannon C, Olavarria OA, Holihan JL, Liang MK
J Am Coll Surg 2023 Mar 1;236(3):523-532. Epub 2022 Nov 16 doi: 10.1097/XCS.0000000000000484. PMID: 36382896
ASGE guideline on the management of achalasia.
Khashab MA, Vela MF, Thosani N, Agrawal D, Buxbaum JL, Abbas Fehmi SM, Fishman DS, Gurudu SR, Jamil LH, Jue TL, Kannadath BS, Law JK, Lee JK, Naveed M, Qumseya BJ, Sawhney MS, Yang J, Wani S
Gastrointest Endosc 2020 Feb;91(2):213-227.e6. Epub 2019 Dec 13 doi: 10.1016/j.gie.2019.04.231. PMID: 31839408
Chronic intestinal pseudo-obstruction due to buserelin-induced formation of anti-GnRH antibodies.
Ohlsson B, Veress B, Janciauskiene S, Montgomery A, Haglund M, Wallmark A
Gastroenterology 2007 Jan;132(1):45-51. doi: 10.1053/j.gastro.2006.10.036. PMID: 17241858
Achalasia. A morphologic study of 42 resected specimens.
Goldblum JR, Whyte RI, Orringer MB, Appelman HD
Am J Surg Pathol 1994 Apr;18(4):327-37. PMID: 8141427

Prognosis

The Number of Interstitial Cells of Cajal Differs Among Different Subtypes of Achalasia and is Related to Patients' Prognosis.
Chen S, Zhang M, Liang M, Tan N, Cui Y, Wang J, Xin X, Ye Z, Zhuang Q, Xiao Y
Clin Transl Gastroenterol 2021 Aug 24;12(8):e00388. doi: 10.14309/ctg.0000000000000388. PMID: 34428185Free PMC Article
Extremely Late Recovery of Unusual Cervical Ankylosing Spondylitis-Related Dysphagia: Anesthesiologic, Surgical, and Pathophysiological Considerations, and Review of the Literature.
Stifano V, Leone A, Revelli L, Tosi F, Visocchi M
World Neurosurg 2020 Jul;139:97-100. Epub 2020 Apr 16 doi: 10.1016/j.wneu.2020.04.031. PMID: 32305610
Pathogenesis of achalasia cardia.
Ghoshal UC, Daschakraborty SB, Singh R
World J Gastroenterol 2012 Jun 28;18(24):3050-7. doi: 10.3748/wjg.v18.i24.3050. PMID: 22791940Free PMC Article
Megaesophagus and hereditary nervous system degeneration.
Longstreth GF, Walker FD
J Clin Gastroenterol 1994 Sep;19(2):125-7. doi: 10.1097/00004836-199409000-00010. PMID: 7963358

Clinical prediction guides

Achalasia alters physiological networks depending on sex.
Furuzawa-Carballeda J, Barajas-Martínez A, Olguín-Rodríguez PV, Ibarra-Coronado E, Fossion R, Coss-Adame E, Valdovinos MA, Torres-Villalobos G, Rivera AL
Sci Rep 2024 Jan 24;14(1):2072. doi: 10.1038/s41598-024-52273-3. PMID: 38267468Free PMC Article
The ageing gastrointestinal tract.
Soenen S, Rayner CK, Jones KL, Horowitz M
Curr Opin Clin Nutr Metab Care 2016 Jan;19(1):12-8. doi: 10.1097/MCO.0000000000000238. PMID: 26560524
Intramuscular interstitial cells of Cajal associated with mast cells survive nitrergic nerves in achalasia.
Zarate N, Wang XY, Tougas G, Anvari M, Birch D, Mearin F, Malagelada JR, Huizinga JD
Neurogastroenterol Motil 2006 Jul;18(7):556-68. doi: 10.1111/j.1365-2982.2006.00788.x. PMID: 16771771
Apoptosis and autoimmunity as proposed pathogenetic links between Helicobacter pylori infection and idiopathic achalasia.
Kountouras J, Zavos C, Chatzopoulos D
Med Hypotheses 2004;63(4):624-9. doi: 10.1016/j.mehy.2004.04.003. PMID: 15325006
Chronic sensory and autonomic neuropathy.
Okajima T, Yamamura S, Hamada K, Kawasaki S, Ideta T, Ueno H, Tokuomi H
Neurology 1983 Aug;33(8):1061-4. doi: 10.1212/wnl.33.8.1061. PMID: 6683803

Recent systematic reviews

Systematic Review of Treatment of Patients with Achalasia: Heller Myotomy, Pneumatic Dilation, and Peroral Endoscopic Myotomy.
Ciomperlik H, Dhanani NH, Mohr C, Hannon C, Olavarria OA, Holihan JL, Liang MK
J Am Coll Surg 2023 Mar 1;236(3):523-532. Epub 2022 Nov 16 doi: 10.1097/XCS.0000000000000484. PMID: 36382896
ASGE guideline on the management of achalasia.
Khashab MA, Vela MF, Thosani N, Agrawal D, Buxbaum JL, Abbas Fehmi SM, Fishman DS, Gurudu SR, Jamil LH, Jue TL, Kannadath BS, Law JK, Lee JK, Naveed M, Qumseya BJ, Sawhney MS, Yang J, Wani S
Gastrointest Endosc 2020 Feb;91(2):213-227.e6. Epub 2019 Dec 13 doi: 10.1016/j.gie.2019.04.231. PMID: 31839408

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