U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Abnormal enteric nervous system morphology

MedGen UID:
927602
•Concept ID:
C4293693
•
Anatomical Abnormality
Synonym: Abnormality of enteric nervous system morphology
 
HPO: HP:0025028

Definition

A structural anomaly of nerves of the enteric nervous system. [from HPO]

Professional guidelines

PubMed

Clinical aspects of neurointestinal disease: Pathophysiology, diagnosis, and treatment.
Goldstein AM, Thapar N, Karunaratne TB, De Giorgio R
Dev Biol 2016 Sep 15;417(2):217-28. Epub 2016 Apr 6 doi: 10.1016/j.ydbio.2016.03.032. PMID: 27059882
Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention.
Demehri FR, Halaweish IF, Coran AG, Teitelbaum DH
Pediatr Surg Int 2013 Sep;29(9):873-81. doi: 10.1007/s00383-013-3353-1. PMID: 23913261
New perspectives in the diagnosis and management of enteric neuropathies.
Knowles CH, Lindberg G, Panza E, De Giorgio R
Nat Rev Gastroenterol Hepatol 2013 Apr;10(4):206-18. Epub 2013 Feb 12 doi: 10.1038/nrgastro.2013.18. PMID: 23399525

Recent clinical studies

Etiology

PTEN Immunohistochemistry.
Terra SA, de Arruda Lourenção PLT, Rodrigues MAM
Arch Pathol Lab Med 2022 May 1;147(5):577-583. doi: 10.5858/arpa.2021-0424-OA. PMID: 35943858
SOX10: 20 years of phenotypic plurality and current understanding of its developmental function.
Pingault V, Zerad L, Bertani-Torres W, Bondurand N
J Med Genet 2022 Feb;59(2):105-114. Epub 2021 Oct 19 doi: 10.1136/jmedgenet-2021-108105. PMID: 34667088Free PMC Article
Effects of Serotonin and Slow-Release 5-Hydroxytryptophan on Gastrointestinal Motility in a Mouse Model of Depression.
Israelyan N, Del Colle A, Li Z, Park Y, Xing A, Jacobsen JPR, Luna RA, Jensen DD, Madra M, Saurman V, Rahim R, Latorre R, Law K, Carson W, Bunnett NW, Caron MG, Margolis KG
Gastroenterology 2019 Aug;157(2):507-521.e4. Epub 2019 May 7 doi: 10.1053/j.gastro.2019.04.022. PMID: 31071306Free PMC Article
Parkinson's disease from the gut.
Liddle RA
Brain Res 2018 Aug 15;1693(Pt B):201-206. Epub 2018 Jan 31 doi: 10.1016/j.brainres.2018.01.010. PMID: 29360467Free PMC Article
Hirschsprung's disease.
Kenny SE, Tam PK, Garcia-Barcelo M
Semin Pediatr Surg 2010 Aug;19(3):194-200. doi: 10.1053/j.sempedsurg.2010.03.004. PMID: 20610192

Diagnosis

PTEN Immunohistochemistry.
Terra SA, de Arruda Lourenção PLT, Rodrigues MAM
Arch Pathol Lab Med 2022 May 1;147(5):577-583. doi: 10.5858/arpa.2021-0424-OA. PMID: 35943858
Gastroparesis.
Camilleri M, Sanders KM
Gastroenterology 2022 Jan;162(1):68-87.e1. Epub 2021 Oct 27 doi: 10.1053/j.gastro.2021.10.028. PMID: 34717924Free PMC Article
SOX10: 20 years of phenotypic plurality and current understanding of its developmental function.
Pingault V, Zerad L, Bertani-Torres W, Bondurand N
J Med Genet 2022 Feb;59(2):105-114. Epub 2021 Oct 19 doi: 10.1136/jmedgenet-2021-108105. PMID: 34667088Free PMC Article
Gastrointestinal motility disorders in neurologic disease.
Camilleri M
J Clin Invest 2021 Feb 15;131(4) doi: 10.1172/JCI143771. PMID: 33586685Free PMC Article
The developmental etiology and pathogenesis of Hirschsprung disease.
Butler Tjaden NE, Trainor PA
Transl Res 2013 Jul;162(1):1-15. Epub 2013 Mar 22 doi: 10.1016/j.trsl.2013.03.001. PMID: 23528997Free PMC Article

Therapy

Effects of Serotonin and Slow-Release 5-Hydroxytryptophan on Gastrointestinal Motility in a Mouse Model of Depression.
Israelyan N, Del Colle A, Li Z, Park Y, Xing A, Jacobsen JPR, Luna RA, Jensen DD, Madra M, Saurman V, Rahim R, Latorre R, Law K, Carson W, Bunnett NW, Caron MG, Margolis KG
Gastroenterology 2019 Aug;157(2):507-521.e4. Epub 2019 May 7 doi: 10.1053/j.gastro.2019.04.022. PMID: 31071306Free PMC Article
Upper GI Disorders: Pathophysiology and Current Therapeutic Approaches.
Parkman HP
Handb Exp Pharmacol 2017;239:17-37. doi: 10.1007/164_2016_114. PMID: 28105529
Achalasia: new perspectives on an old disease.
Gyawali CP
Neurogastroenterol Motil 2016 Jan;28(1):4-11. doi: 10.1111/nmo.12750. PMID: 26690870
Disturbed development of the enteric nervous system after in utero exposure of selective serotonin re-uptake inhibitors and tricyclic antidepressants. Part 1: Literature review.
Nijenhuis CM, Horst PG, Berg LT, Wilffert B
Br J Clin Pharmacol 2012 Jan;73(1):16-26. doi: 10.1111/j.1365-2125.2011.04075.x. PMID: 21815911Free PMC Article
Role of opioid ligands in the irritable bowel syndrome.
Corazziari E
Can J Gastroenterol 1999 Mar;13 Suppl A:71A-75A. doi: 10.1155/1999/598659. PMID: 10202212

Prognosis

Gastroparesis.
Camilleri M, Sanders KM
Gastroenterology 2022 Jan;162(1):68-87.e1. Epub 2021 Oct 27 doi: 10.1053/j.gastro.2021.10.028. PMID: 34717924Free PMC Article
Neurological Phenotype of Mowat-Wilson Syndrome.
Cordelli DM, Di Pisa V, Fetta A, Garavelli L, Maltoni L, Soliani L, Ricci E
Genes (Basel) 2021 Jun 27;12(7) doi: 10.3390/genes12070982. PMID: 34199024Free PMC Article
Hirschsprung's disease.
Kenny SE, Tam PK, Garcia-Barcelo M
Semin Pediatr Surg 2010 Aug;19(3):194-200. doi: 10.1053/j.sempedsurg.2010.03.004. PMID: 20610192
Intestinal neuronal dysplasia.
Skába R, Frantlová M, Horák J
Eur J Gastroenterol Hepatol 2006 Jul;18(7):699-701. doi: 10.1097/01.meg.0000224476.51428.7b. PMID: 16772824
Variant Hirschsprung's disease.
Puri P, Rolle U
Semin Pediatr Surg 2004 Nov;13(4):293-9. doi: 10.1053/j.sempedsurg.2004.10.017. PMID: 15660323

Clinical prediction guides

PTEN Immunohistochemistry.
Terra SA, de Arruda Lourenção PLT, Rodrigues MAM
Arch Pathol Lab Med 2022 May 1;147(5):577-583. doi: 10.5858/arpa.2021-0424-OA. PMID: 35943858
Gastroparesis.
Camilleri M, Sanders KM
Gastroenterology 2022 Jan;162(1):68-87.e1. Epub 2021 Oct 27 doi: 10.1053/j.gastro.2021.10.028. PMID: 34717924Free PMC Article
SOX10: 20 years of phenotypic plurality and current understanding of its developmental function.
Pingault V, Zerad L, Bertani-Torres W, Bondurand N
J Med Genet 2022 Feb;59(2):105-114. Epub 2021 Oct 19 doi: 10.1136/jmedgenet-2021-108105. PMID: 34667088Free PMC Article
Glial Cell-Derived Neurotrophic Factor Induces Enteric Neurogenesis and Improves Colon Structure and Function in Mouse Models of Hirschsprung Disease.
Soret R, Schneider S, Bernas G, Christophers B, Souchkova O, Charrier B, Righini-Grunder F, Aspirot A, Landry M, Kembel SW, Faure C, Heuckeroth RO, Pilon N
Gastroenterology 2020 Nov;159(5):1824-1838.e17. Epub 2020 Jul 17 doi: 10.1053/j.gastro.2020.07.018. PMID: 32687927
Intestinal neuronal dysplasia.
Puri P
Semin Pediatr Surg 2003 Nov;12(4):259-64. doi: 10.1053/j.sempedsurg.2003.08.007. PMID: 14655165

Recent systematic reviews

Congenital anomalies of the kidney and urinary tract (CAKUT) associated with Hirschsprung's disease: a systematic review.
Hofmann AD, Duess JW, Puri P
Pediatr Surg Int 2014 Aug;30(8):757-61. Epub 2014 Jun 29 doi: 10.1007/s00383-014-3529-3. PMID: 24974188
Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect.
Dingemann J, Puri P
Pediatr Surg Int 2010 Nov;26(11):1111-5. doi: 10.1007/s00383-010-2693-3. PMID: 20721562
Skip segment Hirschsprung's disease: a systematic review.
O'Donnell AM, Puri P
Pediatr Surg Int 2010 Nov;26(11):1065-9. doi: 10.1007/s00383-010-2692-4. PMID: 20714729
Effects of proton pump inhibitors on gastric emptying: a systematic review.
Sanaka M, Yamamoto T, Kuyama Y
Dig Dis Sci 2010 Sep;55(9):2431-40. Epub 2009 Dec 10 doi: 10.1007/s10620-009-1076-x. PMID: 20012198

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...