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Arteriovenous malformation

From Wikipedia, the free encyclopedia
Abnormal connection between arteries and veins, bypassing the capillaries
This article is about the anomaly generally. For its occurrence in the brain, seeCerebral arteriovenous malformation.

Medical condition
Arteriovenous malformation
Other namesAVM
Micrograph of an arteriovenous malformation in the brain.HPS stain.
SpecialtyNeurosurgery
Diagnostic methodCT, MRI, MRA

Anarteriovenous malformation (AVM) is an abnormal connection betweenarteries andveins, bypassing thecapillary system. Usuallycongenital, thisvascular anomaly is widely known because of its occurrence in thecentral nervous system (usually as acerebral AVM), but can appear anywhere in the body. The symptoms of AVMs can range from none at all to intense pain or bleeding, and they can lead to other serious medical problems.[1]

Signs and symptoms

[edit]

Symptoms of AVMs vary according to their location. Mostneurological AVMs produce few to no symptoms. Often the malformation is discovered as part of anautopsy or during treatment of an unrelated disorder (an "incidental finding"); in rare cases, its expansion or a micro-bleed from an AVM in the brain can causeepilepsy,neurological deficit, or pain.[2]

The most general symptoms of acerebral AVM includeheadaches and epilepticseizures, with more specific symptoms that normally depend on its location and the individual, including:[1]

Cerebral AVMs may present themselves in a number of different ways:[citation needed]

  • Bleeding (45% of cases)
  • "parkinsonism" 4 symptoms inParkinson's disease.
  • Acute onset of severe headache. May be described as the worst headache of the patient's life. Depending on the location of bleeding, may be associated with new fixed neurologic deficit. In unruptured brain AVMs, the risk of spontaneous bleeding may be as low as 1% per year. After a first rupture, the annual bleeding risk may increase to more than 5%.[3]
  • Seizure or brain seizure (46%). Depending on the place of the AVM, it can contribute toloss of vision.
  • Headache (34%)
  • Progressive neurologic deficit (21%)
    • May be caused by mass effect or venous dilations. Presence and nature of the deficit depends on location of the lesion and the draining veins.[4]
  • Pediatric patients

Pulmonary arteriovenous malformations

[edit]

Pulmonary arteriovenous malformations are abnormal communications between the veins and arteries of thepulmonary circulation, leading to a right-to-left blood shunt.[5][6] They have no symptoms in up to 29% of all cases,[7] however they can give rise to serious complications includinghemorrhage, and infection.[5] They are most commonly associated withhereditary hemorrhagic telangiectasia.[6]

Genetics

[edit]

AVMs are usuallycongenital and are part of theRASopathy family of developmental syndromes.The understanding of the anomaly'sgenetic transmission patterns are incomplete, but there are known geneticmutations (for instance in theepithelial line, tumor suppressorPTEN gene) which can lead to an increased occurrence throughout the body.[citation needed]

The anomaly can occur due to autosomal dominant diseases, such ashereditary hemorrhagic telangiectasia.[8]

Pathophysiology

[edit]

In thecirculatory system, arteries carry blood away from the heart to thelungs and the rest of the body, where the blood normally passes throughcapillaries—where oxygen is released and waste products likecarbon dioxide (CO2) absorbed—before veins return blood to the heart.[9] An AVM interferes with this process by forming a direct connection of the arteries and veins, bypassing thecapillary bed.[10] AVMs can cause intense pain and lead to serious medical problems. Although AVMs are often associated with the brain and spinal cord, they can develop in other parts of the body.[10]

As an AVM lacks the dampening effect of capillaries on the blood flow, the AVM can get progressively larger over time as the amount of blood flowing through it increases, forcing the heart to work harder to keep up with the extra blood flow. It also causes the surrounding area to be deprived of the functions of the capillaries. The resulting tangle ofblood vessels, often called anidus (Latin for 'nest'), has no capillaries. It can be extremely fragile and prone to bleeding because of the abnormally direct connections between high-pressure arteries and low-pressure veins.[11] One indicator is a pulsing 'whoosh' sound caused by rapid blood flow through arteries and veins, which has been given the termbruit (French for 'noise'). If the AVM is severe, this may produce an audible symptom which can interfere with hearing and sleep as well as cause psychological distress.[1]

Diagnosis

[edit]
An arterial venous malformation of the left kidney and a simple cyst of the right kidney
An arterial venous malformation of the left kidney leading toaneurysmal dilatation of the leftrenal vein andinferior vena cava

AVMs are diagnosed primarily by the followingimaging methods:[12]

  • Computed tomography (CT) scan is a noninvasive X-ray to view the anatomical structures within the brain to detect blood in or around the brain. A newer technology calledCT angiography involves the injection ofcontrast into the blood stream to view the arteries of the brain. This type of test provides the best pictures of blood vessels through angiography and soft tissues through CT.
  • Magnetic resonance imaging (MRI) scan is a noninvasive test, which uses a magnetic field and radio-frequency waves to give a detailed view of the soft tissues of the brain.
  • Magnetic resonance angiography (MRA) – scans created using magnetic resonance imaging to specifically image the blood vessels and structures of the brain. A magnetic resonance angiogram can be an invasive procedure, involving the introduction of contrast dyes (e.g.,gadolinium MRcontrast agents) into the vasculature (circulatory system) of a patient using acatheter inserted into an artery and passed through the blood vessels to the brain. Once the catheter is in place, the contrast dye is injected into the bloodstream and the MR images are taken. Additionally or alternatively, flow-dependent or other contrast-free magnetic resonance imaging techniques can be used to determine the location and other properties of the vasculature.

AVMs can occur in various parts of the body:

AVMs may occur in isolation or as a part of another disease (for example,Sturge-Weber syndrome orhereditary hemorrhagic telangiectasia).[22]

AVMs have been shown to be associated withaortic stenosis.[23]

Bleeding from an AVM can be relatively mild or devastating. It can cause severe and less often fatalstrokes.[1]

Treatment

[edit]

Treatment for AVMs in the brain can be symptomatic, and patients should be followed by a neurologist for any seizures, headaches, or focal neurologic deficits. AVM-specific treatment may also involveendovascular embolization, neurosurgery or radiosurgery.[1]Embolization, that is, cutting off the blood supply to the AVM with coils, particles, acrylates, or polymers introduced by a radiographically guided catheter, may be used in addition to neurosurgery or radiosurgery, but is rarely successful in isolation except in smaller AVMs.[24] Agamma knife may also be used.[25]

If a cerebral AVM is detected before a stroke occurs, usually the arteries feeding blood into the nidus can be closed off to avert the danger.[26] Interventional therapy may be relatively risky in the short term.[27]

Treatment of lung AVMs is typically performed with endovascular embolization alone, which is considered the standard of care.[15]

Epidemiology

[edit]

The estimated detection rate of AVM in the US general population is 1.4/100,000 per year.[28] This is approximately one-fifth to one-seventh the incidence ofintracranial aneurysms. An estimated 300,000 Americans have AVMs, of whom 12% (approximately 36,000) will exhibit symptoms of greatly varying severity.[1]

History

[edit]

Hubert von Luschka (1820–1875) andRudolf Virchow (1821–1902) first described arteriovenous malformations in the mid-1800s.Herbert Olivecrona (1891–1980) performed the first surgical excision of an intracranial AVM in 1932.

Society and culture

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Notable cases

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  • ActorRicardo Montalbán was born with spinal AVM.[29] During the filming of the 1951 filmAcross the Wide Missouri, Montalbán was thrown from his horse, knocked unconscious, and trampled by another horse which aggravated his AVM and resulted in a painful back injury that never healed. The pain increased as he aged, and in 1993, Montalbán underwent9+12 hours of spinal surgery which left him paralyzed below the waist and using a wheelchair.[30]
  • Composer and lyricistWilliam Finn was diagnosed with AVM and underwent gamma knife surgery in September 1992, soon after he won the 1992Tony Award for best musical, awarded toFalsettos.[31] Finn wrote the 1998 Off-Broadway musicalA New Brain about the experience.
  • Phoenix Suns NBA basketball point guardAJ Price nearly died from AVM in 2004 while a student at theUniversity of Connecticut.
  • On December 13, 2006, US SenatorTim Johnson of South Dakota was diagnosed with AVM and treated atGeorge Washington University Hospital.[32]
  • Actor/comedianT. J. Miller was diagnosed with AVM in 2010; Miller had a seizure and was unable to sleep for a period. He successfully underwent surgery that had a mortality rate of 10%.[33]
  • On August 3, 2011,Mike Patterson of thePhiladelphia Eagles collapsed on the field and had a seizure during a practice, leading to him being diagnosed with AVM.[34]
  • Former Florida Gators and Oakland Raiders linebackerNeiron Ball was diagnosed with AVM in 2011 while playing forFlorida, but recovered and was cleared to play. On September 16, 2018, Ball was placed in a medically induced coma due to complications of the disease, which lasted until his death on September 10, 2019.[35]
  • Indonesian actressEgidia Savitri [id] died from complications of AVM on November 29, 2013.[36]
  • Jazz guitaristPat Martino experienced an AVM and subsequently developedamnesia andmanic depression. He eventually re-learned to play the guitar by listening to his own recordings from before theaneurysm.[37]
  • YouTube vloggerNikki Lilly (Nikki Christou), winner of the2016 season ofJunior Bake Off was born with AVM, which has resulted in some facial disfigurement.[38]
  • Country music singerDrake White was diagnosed with AVM in January 2019, and is undergoing treatment.
  • Singer-songwriterDino Valenti (actual name Chester William Powers, Jr.) of the psychedelic rock bandQuicksilver Messenger underwent brain surgery for AVM in the late 1980s.
  • ModelLucy Markovic died in April 2025 at age 27 shortly after undergoing surgery to treat AVM.[39]

See also

[edit]

References

[edit]
  1. ^abcdefArteriovenous Malformation Information Page atNINDS
  2. ^"Arteriovenous Malformations".Johns Hopkins Medicine. RetrievedOctober 26, 2022.
  3. ^Stapf, C.; Mast, H.; Sciacca, R. R.; Choi, J. H.; Khaw, A. V.;Connolly, E. S.; Pile-Spellman, J.; Mohr, J. P. (2006). "Predictors of hemorrhage in patients with untreated brain arteriovenous malformation".Neurology.66 (9):1350–5.doi:10.1212/01.wnl.0000210524.68507.87.PMID 16682666.S2CID 22004276.
  4. ^Choi, J.H.; Mast, H.; Hartmann, A.; Marshall, R.S.; Pile-Spellman, J.; Mohr, J.P.; Stapf, C. (2009)."Clinical and morphological determinants of focal neurological deficits in patients with unruptured brain arteriovenous malformation".Journal of the Neurological Sciences.287 (1–2):126–30.doi:10.1016/j.jns.2009.08.011.PMC 2783734.PMID 19729171.
  5. ^abReichert, M; Kerber, S; Alkoudmani, I; Bodner, J (April 2016). "Management of a solitary pulmonary arteriovenous malformation by video-assisted thoracoscopic surgery and anatomic lingula resection: video and review".Surgical Endoscopy.30 (4):1667–9.doi:10.1007/s00464-015-4337-0.PMID 26156615.S2CID 22394114.
  6. ^abTellapuri, S; Park, HS; Kalva, SP (August 2019). "Pulmonary arteriovenous malformations".The International Journal of Cardiovascular Imaging.35 (8):1421–1428.doi:10.1007/s10554-018-1479-x.PMID 30386957.S2CID 53144651.
  7. ^Goodenberger DM (2008). "Chapter 84 Pulmonary arteriovenous malformations".Fishman's Pulmonary Diseases and Disorders (4th ed.). McGraw-Hill. p. 1470.ISBN 978-0-07-145739-2.
  8. ^"Hereditary hemorrhagic telangiectasia: MedlinePlus Genetics".medlineplus.gov. RetrievedJuly 23, 2022.
  9. ^"Circulatory System: Anatomy and Function".Cleveland Clinic. September 21, 2021. RetrievedMarch 8, 2023.
  10. ^abSchimmel, Katharina; Ali, Md Khadem; Tan, Serena Y.; Teng, Joyce; Do, Huy M.; Steinberg, Gary K.; Stevenson, David A.; Spiekerkoetter, Edda (August 21, 2021)."Arteriovenous Malformations—Current Understanding of the Pathogenesis with Implications for Treatment".International Journal of Molecular Sciences.22 (16): 9037.doi:10.3390/ijms22169037.ISSN 1422-0067.PMC 8396465.PMID 34445743.
  11. ^Mouchtouris, Nikolaos; Jabbour, Pascal M.; Starke, Robert M.; Hasan, David M.; Zanaty, Mario; Theofanis, Thana; Ding, Dale; Tjoumakaris, Stavropoula I.; Dumont, Aaron S.; Ghobrial, George M.; Kung, David (February 2015)."Biology of cerebral arteriovenous malformations with a focus on inflammation".Journal of Cerebral Blood Flow and Metabolism.35 (2):167–175.doi:10.1038/jcbfm.2014.179.PMC 4426734.PMID 25407267.
  12. ^Mokin, Maxim (February 2014)."Novel Multimodality Imaging Techniques for Diagnosis and Evaluation of Arteriovenous Malformations".Neurologic Clinics.32 (1):225–236.doi:10.1016/j.ncl.2013.07.006.PMID 24287392. RetrievedFebruary 11, 2023.
  13. ^Agrawal, Aditya; Whitehouse, Richard; Johnson, Robert W.; Augustine, Titus (2006)."Giant splenic artery aneurysm associated with arteriovenous malformation".Journal of Vascular Surgery.44 (6):1345–9.doi:10.1016/j.jvs.2006.06.049.PMID 17145440.
  14. ^Chowdhury, Ujjwal K.; Kothari, Shyam S.; Bishnoi, Arvind K.; Gupta, Ruchika; Mittal, Chander M.; Reddy, Srikrishna (2009). "Successful Lobectomy for Pulmonary Arteriovenous Malformation Causing Recurrent Massive Haemoptysis".Heart, Lung and Circulation.18 (2):135–9.doi:10.1016/j.hlc.2007.11.142.PMID 18294908.
  15. ^abCusumano, Lucas R.; Duckwiler, Gary R.; Roberts, Dustin G.; McWilliams, Justin P. (August 30, 2019). "Treatment of Recurrent Pulmonary Arteriovenous Malformations: Comparison of Proximal Versus Distal Embolization Technique".CardioVascular and Interventional Radiology.43 (1):29–36.doi:10.1007/s00270-019-02328-0.ISSN 1432-086X.PMID 31471718.S2CID 201675132.
  16. ^Barley, Fay L.; Kessel, David; Nicholson, Tony; Robertson, Iain (2006). "Selective Embolization of Large Symptomatic Iatrogenic Renal Transplant Arteriovenous Fistula".CardioVascular and Interventional Radiology.29 (6):1084–7.doi:10.1007/s00270-005-0265-z.PMID 16794894.S2CID 9335750.
  17. ^Kishi, K; Shirai, S; Sonomura, T; Sato, M (2005). "Selective conformal radiotherapy for arteriovenous malformation involving the spinal cord".The British Journal of Radiology.78 (927):252–4.doi:10.1259/bjr/50653404.PMID 15730991.
  18. ^Bauer, Tilman; Britton, Peter; Lomas, David; Wight, Derek G.D.; Friend, Peter J.; Alexander, Graeme J.M. (1995). "Liver transplantation for hepatic arteriovenous malformation in hereditary haemorrhagic telangiectasia".Journal of Hepatology.22 (5):586–90.doi:10.1016/0168-8278(95)80455-2.PMID 7650340.
  19. ^Rivera, Peter P.; Kole, Max K.; Pelz, David M.; Gulka, Irene B.; McKenzie, F. Neil; Lownie, Stephen P. (2006). "Congenital Intercostal Arteriovenous Malformation".American Journal of Roentgenology.187 (5): W503–6.doi:10.2214/AJR.05.0367.PMID 17056881.
  20. ^Shields, Jerry A.; Streicher, Theodor F. E.; Spirkova, Jane H. J.; Stubna, Michal; Shields, Carol L. (2006). "Arteriovenous Malformation of the Iris in 14 Cases".Archives of Ophthalmology.124 (3):370–5.doi:10.1001/archopht.124.3.370.PMID 16534057.
  21. ^Sountoulides, Petros; Bantis, Athanasios; Asouhidou, Irene; Aggelonidou, Hellen (2007)."Arteriovenous malformation of the spermatic cord as the cause of acute scrotal pain: a case report".Journal of Medical Case Reports.1 110.doi:10.1186/1752-1947-1-110.PMC 2194703.PMID 17939869.
  22. ^"Arteriovenous Malformations (AVMs)".National Institute of Neurological Disorders and Stroke,NIH. February 22, 2024. RetrievedMarch 30, 2024.
  23. ^Batur, Pelin; Stewart, William J.; Isaacson, J. Harry (2003). "Increased Prevalence of Aortic Stenosis in Patients With Arteriovenous Malformations of the Gastrointestinal Tract in Heyde Syndrome".Archives of Internal Medicine.163 (15):1821–4.doi:10.1001/archinte.163.15.1821.PMID 12912718.
  24. ^Jafar, Jafar J.; Davis, Adam J.; Berenstein, Alejandro; Choi, In Sup; Kupersmith, Mark J. (January 1, 1993). "The effect of embolization with N-butyl cyanoacrylate prior to surgical resection of cerebral arteriovenous malformations".Journal of Neurosurgery.78 (1):60–69.doi:10.3171/jns.1993.78.1.0060.ISSN 0022-3085.PMID 8416244.
  25. ^"Conditions We Treat". Macquarie University Hospital. Archived fromthe original on June 13, 2013.
  26. ^"Arteriovenous Malformations".Neurosurgery. RetrievedJune 26, 2023.
  27. ^"A Randomized Trial of Unruptured Brain Arteriovenous Malformations (ARUBA)".National Institute of Neurological Disorders and Stroke. January 29, 2014. Archived fromthe original on July 4, 2016. RetrievedMarch 6, 2023.
  28. ^Stapf, C.; Mast, H.; Sciacca, R.R.; Berenstein, A.; Nelson, P.K.; Gobin, Y.P.; Pile-Spellman, J.; Mohr, J.P. (2003)."The New York Islands AVM Study: Design, Study Progress, and Initial Results".Stroke.34 (5): e29–33.doi:10.1161/01.STR.0000068784.36838.19.PMID 12690217.
  29. ^"Ricardo Montalban tribute" YouTube, acceptance speech video of Easter Seals Lifetime Achievement Award
  30. ^"Inside".mahalo.com. RetrievedMarch 17, 2018.
  31. ^Pall, Ellen (June 14, 1998)."The Long-Running Musical of William Finn's Life".The New York Times. RetrievedMarch 17, 2018.
  32. ^"Sen. Johnson recovering after brain surgery". AP. December 14, 2006.
  33. ^Holmes, Pete (October 27, 2011)."You Made It Weird with Pete Holmes".You Made It Weird #2: TJ Miller (Podcast). Nerdist Industries. Event occurs at 37:45. Archived fromthe original on February 12, 2013. RetrievedDecember 5, 2012.
  34. ^"Mike Patterson's Collapse Reportedly Related To Brain AVM".sbnation.com. August 4, 2011. RetrievedMarch 17, 2018.
  35. ^"Neiron Ball, former Raiders LB, in medically induced coma after aneurysm".ESPN. September 26, 2018.
  36. ^Suhendra, Ichsan."Pesinetron Egidia Savitri Telah Pergi".KOMPAS.com (in Indonesian). Kompas Cyber Media. RetrievedMay 15, 2019.
  37. ^Vida, Vendela.Confidence, or the Appearance of Confidence: The Best of the Believer Music Interviews. No ed. San Francisco, CA: Believer, a Tiny Division of McSweeney's Which Is Also Tiny, 2014. Print.
  38. ^"Junior Bake Off 2016 Winner announced".BBC Media Centre.BBC Online. November 25, 2016. RetrievedApril 9, 2017.
  39. ^Holland, Oscar (April 11, 2025)."Lucy Markovic, star of 'Australia's Next Top Model,' dies age 27".CNN. RetrievedApril 12, 2025.
Classification
External resources
Vascular tumors
Benign
Locally aggressive or borderline
Malignant
Vascular malformations
Simple
Capillary malformations
Lymphatic malformations (LM)
Incorrect name: Lymphangioma
Venous malformations
  • Common VM
  • Familial VM cutaneo-mucosal
  • Blue rubber bleb nevus syndrome
  • Glomuvenous malformation
  • Cerebral cavernous malformation
  • Familial intraosseous vascular malformation
  • Verrucous venous malformation
Arteriovenous malformations
  • Sporadic
  • In HHT
  • In CM-AVM
Arteriovenous fistula
  • Sporadic
  • In HHT
  • In CM-AVM
Combined
  • Lymphatic-venous malformation
  • Capillary-arteriovenous malformation
Associated with other anomalies
Great arteries/
other arteries
Aorta
Pulmonary artery
Subclavian artery
Umbilical artery
Great veins
Superior/inferior vena cava
Pulmonary vein
Arteriovenous malformation
Arteries,arterioles
andcapillaries
Inflammation
Arteriosclerosis
Peripheral artery disease
Aneurysm /dissection /
pseudoaneurysm
Vascular malformation
Vascular nevus
Veins
Inflammation
Venous thrombosis /
Thrombophlebitis
Varicose veins
Other
Arteries or veins
Blood pressure
Hypertension
Hypotension
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