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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

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
Patsalides A, Santillan A, Knopman J, Tsiouris AJ, Riina HA, Gobin YP
J Neurointerv Surg 2011 Mar;3(1):80-4. Epub 2010 Sep 29 doi: 10.1136/jnis.2010.003178. PMID: 21990796

Recent clinical studies

Etiology

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
Islak C, Kandemirli SG, Kizilkilic O, Kocer N, Tuzgen S, Hanci MM
World Neurosurg 2018 Feb;110:407-413. Epub 2017 Dec 6 doi: 10.1016/j.wneu.2017.11.162. PMID: 29223517
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

Diagnosis

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
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
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

Therapy

Suzuki T, Kagawa K, Sato K, Nomura R, Irie K, Ichi S
World Neurosurg 2023 Jul;175:e230-e237. Epub 2023 Mar 20 doi: 10.1016/j.wneu.2023.03.058. PMID: 36940806
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
Schoonover A, Nanda S, Singer J, Benner C
Am J Emerg Med 2022 Jun;56:396.e5-396.e8. Epub 2022 Apr 1 doi: 10.1016/j.ajem.2022.03.062. PMID: 35437201
Jablawi F, Schubert GA, Hans FJ, Mull M
World Neurosurg 2018 Jun;114:e698-e705. Epub 2018 Mar 14 doi: 10.1016/j.wneu.2018.03.061. PMID: 29550595
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

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
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
Li ZF, Li Q, Xu Y, Hong B, Huang QH, Liu JM
World Neurosurg 2017 Nov;107:1043.e7-1043.e13. Epub 2017 Jun 20 doi: 10.1016/j.wneu.2017.06.080. PMID: 28645597
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

Suzuki T, Kagawa K, Sato K, Nomura R, Irie K, Ichi S
World Neurosurg 2023 Jul;175:e230-e237. Epub 2023 Mar 20 doi: 10.1016/j.wneu.2023.03.058. PMID: 36940806
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
Nikova A, Ganchev D, Birbilis T
Pediatr Neurosurg 2018;53(5):291-298. Epub 2018 Jul 23 doi: 10.1159/000490420. PMID: 30036876
Li ZF, Li Q, Xu Y, Hong B, Huang QH, Liu JM
World Neurosurg 2017 Nov;107:1043.e7-1043.e13. Epub 2017 Jun 20 doi: 10.1016/j.wneu.2017.06.080. PMID: 28645597
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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