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Neurenteric cyst

MedGen UID:
14343
•Concept ID:
C0027806
•
Congenital Abnormality
Synonyms: Cyst, Neurenteric; Cyst, Neuroenteric; Cysts, Neurenteric; Cysts, Neuroenteric; Neurenteric Cyst; Neurenteric Cysts; Neuroenteric Cyst; Neuroenteric Cysts
SNOMED CT: Neurenteric cyst (789120001); Neurenteric cyst (41353005)
 
HPO: HP:0030725
Monarch Initiative: MONDO:0017087
Orphanet: ORPHA268865

Definition

The neurenteric cyst is a rare lesion composed of heterotopic endodermal tissue. During the third week of human embryogenesis, the neurenteric canal unites the yolk sac and the amniotic cavity as it traverses the primitive notochordal plate. Persistence of the normally transient neurenteric canal prevents appropriate separation of endoderm and notochord. This results in a variable degree of communication between neural and enteric epithelium. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeurenteric cyst
Follow this link to review classifications for Neurenteric cyst in Orphanet.

Professional guidelines

PubMed

Neural tube defects: a review of global prevalence, causes, and primary prevention.
Kancherla V
Childs Nerv Syst 2023 Jul;39(7):1703-1710. Epub 2023 Mar 8 doi: 10.1007/s00381-023-05910-7. PMID: 36882610
Update on prenatal diagnosis and fetal surgery for myelomeningocele.
Meller C, Covini D, Aiello H, Izbizky G, Portillo Medina S, Otaño L
Arch Argent Pediatr 2021 Jun;119(3):e215-e228. doi: 10.5546/aap.2021.eng.e215. PMID: 34033426
Spina Bifida Management.
Phillips LA, Burton JM, Evans SH
Curr Probl Pediatr Adolesc Health Care 2017 Jul;47(7):173-177. Epub 2017 Jul 19 doi: 10.1016/j.cppeds.2017.06.007. PMID: 28734746

Recent clinical studies

Etiology

Neurenteric cysts, incidence and surgical treatment.
Kozak J, Bizik I, Surkala J, Steno J, Steno A
Bratisl Lek Listy 2019;120(9):680-685. doi: 10.4149/BLL_2019_114. PMID: 31475554
Neurenteric Cyst or Neuroendodermal Cyst? Immunohistochemical Study and Pathogenesis.
Chen CT, Lai HY, Jung SM, Lee CY, Wu CT, Lee ST
World Neurosurg 2016 Dec;96:85-90. Epub 2016 Aug 29 doi: 10.1016/j.wneu.2016.08.089. PMID: 27586176
Supratentorial Neurenteric Cysts: Case Series and Review of Pathology, Imaging, and Clinical Management.
Chakraborty S, Priamo F, Loven T, Li J, Insinga S, Schulder M
World Neurosurg 2016 Jan;85:143-52. Epub 2015 Sep 1 doi: 10.1016/j.wneu.2015.08.057. PMID: 26341448
Spinal Neurenteric Cyst in Association with Klippel-Feil Syndrome: Case Report and Literature Review.
Can A, Dos Santos Rubio EJ, Jasperse B, Verdijk RM, Harhangi BS
World Neurosurg 2015 Aug;84(2):592.e9-14. Epub 2015 Mar 17 doi: 10.1016/j.wneu.2015.03.015. PMID: 25790871
Neurenteric cyst presenting with stridor and dysphagia: case report and literature review.
Visvanathan V, Crowther J, Taylor W
Scott Med J 2015 Feb;60(1):e4-7. Epub 2014 Dec 3 doi: 10.1177/0036933014563239. PMID: 25471489

Diagnosis

Giant Craniospinal Intramedullary Neurenteric Cyst in Infant-Case Report and Review of Literature.
Diyora B, Bhende B, Kukreja S
World Neurosurg 2018 Oct;118:126-131. Epub 2018 Aug 4 doi: 10.1016/j.wneu.2018.07.027. PMID: 30010074
Neurenteric Cyst or Neuroendodermal Cyst? Immunohistochemical Study and Pathogenesis.
Chen CT, Lai HY, Jung SM, Lee CY, Wu CT, Lee ST
World Neurosurg 2016 Dec;96:85-90. Epub 2016 Aug 29 doi: 10.1016/j.wneu.2016.08.089. PMID: 27586176
Supratentorial Neurenteric Cysts: Case Series and Review of Pathology, Imaging, and Clinical Management.
Chakraborty S, Priamo F, Loven T, Li J, Insinga S, Schulder M
World Neurosurg 2016 Jan;85:143-52. Epub 2015 Sep 1 doi: 10.1016/j.wneu.2015.08.057. PMID: 26341448
Spinal Neurenteric Cyst in Association with Klippel-Feil Syndrome: Case Report and Literature Review.
Can A, Dos Santos Rubio EJ, Jasperse B, Verdijk RM, Harhangi BS
World Neurosurg 2015 Aug;84(2):592.e9-14. Epub 2015 Mar 17 doi: 10.1016/j.wneu.2015.03.015. PMID: 25790871
Neurenteric cyst presenting with stridor and dysphagia: case report and literature review.
Visvanathan V, Crowther J, Taylor W
Scott Med J 2015 Feb;60(1):e4-7. Epub 2014 Dec 3 doi: 10.1177/0036933014563239. PMID: 25471489

Therapy

Risk Factors in a Pediatric Population for Postoperative Intracranial Infection Following Endoscopic Endonasal Skull Base Surgery and the Role of Antibiotic Prophylaxis.
Palmieri D, Champagne PO, Valappil B, McDowell MM, Gardner PA, Snyderman CH
Am J Rhinol Allergy 2023 Jan;37(1):13-18. Epub 2022 Sep 1 doi: 10.1177/19458924221123113. PMID: 36050937
Supratentorial Neurenteric Cysts: Systematic Literature Review and Case Report.
Stopa BM, Cuoco JA, Stump MS, Rogers CM
World Neurosurg 2022 Aug;164:8-24. Epub 2022 Apr 20 doi: 10.1016/j.wneu.2022.04.057. PMID: 35460907
Management of an unusual, recurrent neurenteric cyst in an infant: case report and review of the literature.
Esfahani DR, Burokas L, Brown HG, Hahn YS, Nikas D
Childs Nerv Syst 2017 Sep;33(9):1603-1607. Epub 2017 Jun 22 doi: 10.1007/s00381-017-3487-x. PMID: 28643040
Neurenteric cyst: an unusual cause of third nerve palsy.
Wait SD, Choi LS, Teo C
Childs Nerv Syst 2011 Apr;27(4):639-41. Epub 2010 Nov 25 doi: 10.1007/s00381-010-1332-6. PMID: 21107583
Surgical approaches: postoperative care and complications "posterolateral-far lateral transcondylar approach to the ventral foramen magnum and upper cervical spinal canal".
Menezes AH
Childs Nerv Syst 2008 Oct;24(10):1203-7. Epub 2008 Mar 26 doi: 10.1007/s00381-008-0597-5. PMID: 18365213

Prognosis

Pediatric intracranial neurenteric cyst of the oculomotor nerve: a case-based review.
Oppenhuizen B, Ragheb J, Leuchter JD, Clarke JE, Pelaez L, Wang S
Childs Nerv Syst 2021 Dec;37(12):3681-3693. Epub 2021 Aug 17 doi: 10.1007/s00381-021-05308-3. PMID: 34401938
Neurenteric cysts, incidence and surgical treatment.
Kozak J, Bizik I, Surkala J, Steno J, Steno A
Bratisl Lek Listy 2019;120(9):680-685. doi: 10.4149/BLL_2019_114. PMID: 31475554
Supratentorial Neurenteric Cysts: Case Series and Review of Pathology, Imaging, and Clinical Management.
Chakraborty S, Priamo F, Loven T, Li J, Insinga S, Schulder M
World Neurosurg 2016 Jan;85:143-52. Epub 2015 Sep 1 doi: 10.1016/j.wneu.2015.08.057. PMID: 26341448
Spinal Neurenteric Cyst in Association with Klippel-Feil Syndrome: Case Report and Literature Review.
Can A, Dos Santos Rubio EJ, Jasperse B, Verdijk RM, Harhangi BS
World Neurosurg 2015 Aug;84(2):592.e9-14. Epub 2015 Mar 17 doi: 10.1016/j.wneu.2015.03.015. PMID: 25790871
Neurenteric cyst presenting with stridor and dysphagia: case report and literature review.
Visvanathan V, Crowther J, Taylor W
Scott Med J 2015 Feb;60(1):e4-7. Epub 2014 Dec 3 doi: 10.1177/0036933014563239. PMID: 25471489

Clinical prediction guides

Spinal neurenteric cysts: experience with 9 cases via an anterior cervical approach.
Xin Z, Du Y, Zhang C, Zhang B, Qi M, Meng H, Jin T, Chan CK, Chen Z, Duan W
J Neurosurg Spine 2024 Sep 1;41(3):407-415. Epub 2024 Jun 7 doi: 10.3171/2024.3.SPINE231161. PMID: 38848604
Giant Craniospinal Intramedullary Neurenteric Cyst in Infant-Case Report and Review of Literature.
Diyora B, Bhende B, Kukreja S
World Neurosurg 2018 Oct;118:126-131. Epub 2018 Aug 4 doi: 10.1016/j.wneu.2018.07.027. PMID: 30010074
Clinical presentation and surgical outcomes of intramedullary neurenteric cysts.
Yang T, Wu L, Fang J, Yang C, Deng X, Xu Y
J Neurosurg Spine 2015 Jul;23(1):99-110. Epub 2015 Apr 24 doi: 10.3171/2014.11.SPINE14352. PMID: 25909275
Diagnostic and surgical implications of ventral vertebrobasilar displacement by posterior fossa neurenteric cysts.
Uschold T, Xu DS, Wilson DA, Abla AA, Nakaji P, Spetzler RF, Chang SW
World Neurosurg 2014 Sep-Oct;82(3-4):480-4. Epub 2013 Oct 16 doi: 10.1016/j.wneu.2013.10.037. PMID: 24141002
Spectrum of nonterminal myelocystoceles.
Rossi A, Piatelli G, Gandolfo C, Pavanello M, Hoffmann C, Van Goethem JW, Cama A, Tortori-Donati P
Neurosurgery 2006 Mar;58(3):509-15; discussion 509-15. doi: 10.1227/01.NEU.0000197122.92954.82. PMID: 16528191

Recent systematic reviews

Resection of intradural spinal lesions with concomitant instrumented fusion in children: a systematic review and representative cases.
Prather KY, Baier MP, Coulibaly NJ, Stephens ME, Chen S, Omini ME, Jea A
J Neurosurg Pediatr 2024 Jul 1;34(1):99-110. Epub 2024 Apr 5 doi: 10.3171/2024.1.PEDS23444. PMID: 38579345
Supratentorial Neurenteric Cysts: Systematic Literature Review and Case Report.
Stopa BM, Cuoco JA, Stump MS, Rogers CM
World Neurosurg 2022 Aug;164:8-24. Epub 2022 Apr 20 doi: 10.1016/j.wneu.2022.04.057. PMID: 35460907

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