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Spinal arteriovenous malformation

MedGen UID:
83876
Concept ID:
C0348023
Congenital Abnormality
Synonym: AVM - Spinal arteriovenous malformation
SNOMED CT: Spinal arteriovenous malformation (261482004); AVM - Spinal arteriovenous malformation (261482004)
 
HPO: HP:0002390

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSpinal arteriovenous malformation

Conditions with this feature

Telangiectasia, hereditary hemorrhagic, type 2
MedGen UID:
324960
Concept ID:
C1838163
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Hereditary hemorrhagic telangiectasia type 4
MedGen UID:
341824
Concept ID:
C1857688
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.
Telangiectasia, hereditary hemorrhagic, type 1
MedGen UID:
1643786
Concept ID:
C4551861
Disease or Syndrome
Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of multiple arteriovenous malformations (AVMs) that lack intervening capillaries and result in direct connections between arteries and veins. The most common clinical manifestation is spontaneous and recurrent nosebleeds (epistaxis) beginning on average at age 12 years. Telangiectases (small AVMs) are characteristically found on the lips, tongue, buccal and gastrointestinal (GI) mucosa, face, and fingers. The appearance of telangiectases is generally later than epistaxis but may be during childhood. Large AVMs occur most often in the lungs, liver, or brain; complications from bleeding or shunting may be sudden and catastrophic. A minority of individuals with HHT have GI bleeding, which is rarely seen before age 50 years.

Professional guidelines

PubMed

Mamaril-Davis J, Aguilar-Salinas P, Avila MJ, Dumont T, Avery MB
World Neurosurg 2023 May;173:e250-e297. Epub 2023 Feb 12 doi: 10.1016/j.wneu.2023.02.040. PMID: 36787855
Ehresman J, Catapano JS, Baranoski JF, Jadhav AP, Ducruet AF, Albuquerque FC
Neurosurg Clin N Am 2022 Apr;33(2):193-206. doi: 10.1016/j.nec.2021.11.005. PMID: 35346451
Brinjikji W, Lanzino G
Handb Clin Neurol 2017;143:161-174. doi: 10.1016/B978-0-444-63640-9.00016-3. PMID: 28552139

Recent clinical studies

Etiology

Watabe D, Tominaga M, Matsuda H, Takamori K, Amano H
Acta Derm Venereol 2019 Jun 1;99(7):681-682. doi: 10.2340/00015555-3177. PMID: 30896775
Day AL, Turkmani AH, Chen PR
Handb Clin Neurol 2017;143:189-198. doi: 10.1016/B978-0-444-63640-9.00018-7. PMID: 28552141
Flores BC, Klinger DR, White JA, Batjer HH
Neurosurg Rev 2017 Jan;40(1):15-28. Epub 2016 Apr 13 doi: 10.1007/s10143-016-0713-z. PMID: 27075861
Andersen O
Curr Opin Neurol 2000 Jun;13(3):311-6. doi: 10.1097/00019052-200006000-00014. PMID: 10871257
Regan EQ, Spencer D, Levy JM, Russell EJ
J Spinal Disord 1991 Jun;4(2):238-41. doi: 10.1097/00002517-199106000-00016. PMID: 1839613

Diagnosis

Day AL, Turkmani AH, Chen PR
Handb Clin Neurol 2017;143:189-198. doi: 10.1016/B978-0-444-63640-9.00018-7. PMID: 28552141
Takai K
Neurol Med Chir (Tokyo) 2017 Jul 15;57(7):356-365. Epub 2017 May 18 doi: 10.2176/nmc.ra.2016-0316. PMID: 28515372Free PMC Article
Drislane FW, Matheson JK, Dubuisson D
Arch Neurol 2003 Jan;60(1):125. doi: 10.1001/archneur.60.1.125. PMID: 12533099
Andersen O
Curr Opin Neurol 2000 Jun;13(3):311-6. doi: 10.1097/00019052-200006000-00014. PMID: 10871257
Rutten MJ, Merx JL, Kesselring FO, Grotenhuis JA
JBR-BTR 2000 Feb;83(1):23. PMID: 10769509

Therapy

Mamaril-Davis J, Aguilar-Salinas P, Avila MJ, Dumont T, Avery MB
World Neurosurg 2023 May;173:e250-e297. Epub 2023 Feb 12 doi: 10.1016/j.wneu.2023.02.040. PMID: 36787855
Pagiola I, Caroff J, Mihalea C, Aghakhani N, Ikka L, Chalumeau V, Iacobucci M, Ozanne A, Gallas S, Carrete H, Marques MC, Nalli D, Caldas JG, Frudit ME, Moret J, Parker F, Spelle L
J Neurointerv Surg 2020 May;12(5):e4. Epub 2020 Mar 17 doi: 10.1136/neurintsurg-2019-015268.rep. PMID: 32184273
Vázquez EJ, Gefaell J, Fernandez L, López E, Areitio E, Elexpuru JA
Acta Neurochir (Wien) 2017 Jun;159(6):1107-1111. Epub 2017 Apr 4 doi: 10.1007/s00701-017-3150-z. PMID: 28378096
Ghobrial GM, Maulucci CM, Dalyai RT, Chalouhi N, Rosenwasser RH, Harrop JS
J Neurol Surg A Cent Eur Neurosurg 2015 Sep;76(5):392-8. Epub 2015 Jul 3 doi: 10.1055/s-0035-1551824. PMID: 26140419
Bentson J, Rand R, Calcaterra T, Lasjaunias P
Neuroradiology 1978;16:420-3. doi: 10.1007/BF00395321. PMID: 745726

Prognosis

Hiramatsu M, Ishibashi R, Suzuki E, Miyazaki Y, Murai S, Takai H, Takasugi Y, Yamaoka Y, Nishi K, Takahashi Y, Haruma J, Hishikawa T, Yasuhara T, Chin M, Matsubara S, Uno M, Tokunaga K, Sugiu K, Date I
J Neurosurg Spine 2022 Apr 1;36(4):670-677. Epub 2021 Oct 29 doi: 10.3171/2021.7.SPINE21233. PMID: 34715647
Watabe D, Tominaga M, Matsuda H, Takamori K, Amano H
Acta Derm Venereol 2019 Jun 1;99(7):681-682. doi: 10.2340/00015555-3177. PMID: 30896775
Dossani RH, Kalakoti P, Thakur JD, Nanda A
Neurosurgery 2017 Feb 1;80(2):324-330. doi: 10.1093/neuros/nyw031. PMID: 28173516
Flores BC, Klinger DR, White JA, Batjer HH
Neurosurg Rev 2017 Jan;40(1):15-28. Epub 2016 Apr 13 doi: 10.1007/s10143-016-0713-z. PMID: 27075861
Koch C
Curr Opin Neurol 2006 Feb;19(1):69-75. doi: 10.1097/01.wco.0000200547.22292.11. PMID: 16415680

Clinical prediction guides

Ronald AA, Yao B, Winkelman RD, Piraino D, Masaryk TJ, Krishnaney AA
World Neurosurg 2020 Dec;144:e306-e315. Epub 2020 Aug 25 doi: 10.1016/j.wneu.2020.08.126. PMID: 32858225
Pagiola I, Caroff J, Mihalea C, Aghakhani N, Ikka L, Chalumeau V, Iacobucci M, Ozanne A, Gallas S, Carrete H, Marques MC, Nalli D, Caldas JG, Frudit ME, Moret J, Parker F, Spelle L
J Neurointerv Surg 2020 May;12(5):e4. Epub 2020 Mar 17 doi: 10.1136/neurintsurg-2019-015268.rep. PMID: 32184273
Pagiola I, Caroff J, Mihalea C, Aghakhani N, Ikka L, Chalumeau V, Iacobucci M, Ozanne A, Gallas S, Carrete H, Marques MC, Nalli D, Caldas JG, Frudit ME, Moret J, Parker F, Spelle L
BMJ Case Rep 2020 Mar 3;13(3) doi: 10.1136/bcr-2019-015268. PMID: 32132099Free PMC Article
Nikova A, Ganchev D, Birbilis T
Pediatr Neurosurg 2018;53(5):291-298. Epub 2018 Jul 23 doi: 10.1159/000490420. PMID: 30036876
Flores BC, Klinger DR, White JA, Batjer HH
Neurosurg Rev 2017 Jan;40(1):15-28. Epub 2016 Apr 13 doi: 10.1007/s10143-016-0713-z. PMID: 27075861

Recent systematic reviews

Mamaril-Davis J, Aguilar-Salinas P, Avila MJ, Dumont T, Avery MB
World Neurosurg 2023 May;173:e250-e297. Epub 2023 Feb 12 doi: 10.1016/j.wneu.2023.02.040. PMID: 36787855
Goethe E, LoPresti MA, Kan P, Lam SK
Childs Nerv Syst 2020 Feb;36(2):325-332. Epub 2019 Aug 14 doi: 10.1007/s00381-019-04329-3. PMID: 31414169
Clark MT, Brooks EL, Chong W, Pappas C, Fahey M
Pediatr Neurol 2008 Dec;39(6):423-5. doi: 10.1016/j.pediatrneurol.2008.08.001. PMID: 19027589

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