Arteriovenous malformation
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| Arteriovenous malformation Classification and external resources |
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| ICD-10 | Q27.3, Q28.0, Q28.2 |
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| ICD-9 | 747.6, 747.81 |
| DiseasesDB | 15235 |
| MedlinePlus | 000779 |
| eMedicine | topic list |
| MeSH | D001165 |
Arteriovenous malformation or AVM is a congenital disorder of the connections between veins and arteries in the vascular system. The genetic transmission patterns of AVM (if any) are unknown, and AVM is not generally thought to be an inherited disorder--unless in the context of a specific hereditary syndrome.
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Signs and symptoms
Symptoms of AVM vary according to the location of the malformation. Roughly 88% of those with an AVM are asymptomatic; often the malformation is discovered as part of an autopsy or during treatment for an unrelated disorder. The most general symptoms include headache and seizures, with more specific symptoms occurring that normally depend on the location of the malformation and the individual. Other symptoms can include paralysis, muscle weakness, poor coordination, inability to plan and carry out tasks, dizziness, alogia or paralysis, loss of coordination, difficulties carrying out tasks that require planning, vision problems, apraxia, abnormal sensations (numbness, tingling, or spontaneous pain), problems with memory, mental confusion, dementia or hallucinations.[1]
Pathophysiology
Arteries and veins are part of the human cardiovascular system. Normally, the arteries in the vascular system carry oxygen-rich blood at a relatively high pressure. Structurally, arteries divide and sub-divide repeatedly, eventually forming a sponge-like capillary bed. Blood moves through the capillaries, giving up oxygen and taking up waste products from the surrounding cells. Capillaries successively join together, one upon the other, to form the veins that carry blood away at a relatively low pressure. The heart acts to pump blood from the high pressure arteries to the low pressure veins.
If the capillary bed is thought of as a sponge, then an AVM is the rough equivalent of jamming a tangle of flexible soda straws from artery to vein through that sponge. On arteriogram films AVM formation often resemble a tangle of spaghetti noodles. This tangle of blood vessels forms a relatively direct connection between high pressure arteries and low pressure veins.
The result is a collection of blood vessels with abnormal connections and no capillaries. This collection, often called a nidus, can be extremely fragile and prone to bleeding.
Diagnosis
AVMs can occur in various parts of the body
- brain, causing a cerebral arteriovenous malformation
- spleen[2]
- lung[3]
- kidney[4]
- spinal cord[5]
- liver[6]
- intercostal space[7]
- iris[8]
- spermatic cord[9]
AVMs may occur in isolation or as a part of another disease (e.g. Von Hippel-Lindau disease or hereditary hemorrhagic telangiectasia).
This bleeding can be devastating, particularly in the brain. It can cause severe and often fatal strokes. If detected before a stroke occurs, usually the arteries feeding blood into the nidus can be closed off, ensuring the safety of the patient.
Treatment
Treatment can be symptomatic, or it can involve surgery or radiation therapy.[1]
Epidemiology
An estimated 300,000 Americans have AVMs, of which 12% (approximately 36,000) will exhibit symptoms that differ greatly in severity.[1]
Society and culture
- On December 13, 2006, Senator Tim Johnson was diagnosed with AVM and treated at George Washington University Hospital.[10]
Cases in fiction
- The plot of William Finn's 1998 Off-Broadway musical, A New Brain, revolves around the main character's battle with AVM.
- The character Nate Fisher on the HBO television show Six Feet Under suffers from AVM.
- Patients with AVM appeared in two episodes of the series House, DNR and Top Secret.
Notes
- ^ a b c Arteriovenous Malformation Information Page at NINDS
- ^ Agrawal A, Whitehouse R, Johnson RW, Augustine T (December 2006). "Giant splenic artery aneurysm associated with arteriovenous malformation". J. Vasc. Surg. 44 (6): 1345–9. doi:. PMID 17145440.
- ^ Chowdhury UK, Kothari SS, Bishnoi AK, Gupta R, Mittal CM, Reddy S (February 2008). "Successful Lobectomy for Pulmonary Arteriovenous Malformation Causing Recurrent Massive Haemoptysis". Heart Lung Circ. doi:. PMID 18294908.
- ^ Barley FL, Kessel D, Nicholson T, Robertson I (2006). "Selective embolization of large symptomatic iatrogenic renal transplant arteriovenous fistula". Cardiovasc Intervent Radiol 29 (6): 1084–7. doi:. PMID 16794894.
- ^ Kishi K, Shirai S, Sonomura T, Sato M (March 2005). "Selective conformal radiotherapy for arteriovenous malformation involving the spinal cord". Br J Radiol 78 (927): 252–4. PMID 15730991.
- ^ Bauer T, Britton P, Lomas D, Wight DG, Friend PJ, Alexander GJ (May 1995). "Liver transplantation for hepatic arteriovenous malformation in hereditary haemorrhagic telangiectasia". J. Hepatol. 22 (5): 586–90. PMID 7650340.
- ^ Rivera PP, Kole MK, Pelz DM, Gulka IB, McKenzie FN, Lownie SP (November 2006). "Congenital intercostal arteriovenous malformation". AJR Am J Roentgenol 187 (5): W503–6. doi:. PMID 17056881.
- ^ Shields JA, Streicher TF, Spirkova JH, Stubna M, Shields CL (March 2006). "Arteriovenous malformation of the iris in 14 cases". Arch. Ophthalmol. 124 (3): 370–5. doi:. PMID 16534057.
- ^ Sountoulides P, Bantis A, Asouhidou I, Aggelonidou H (2007). "Arteriovenous malformation of the spermatic cord as the cause of acute scrotal pain: a case report". J Med Case Reports 1: 110. doi:. PMID 17939869.
- ^ "Sen. Johnson recovering after brain surgery", AP, December 14, 2006.
External links
- Information from the AVM Support UK
- Information from AVM Support
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