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Vascular malformation
Vascular malformation
Other names	Vascular giantism or Lymphangioma
Specialty	Cardiovascular
Treatment	In low-flow lesions, sclerotherapy can be extremely effective, either alone, in small lesions, or combined with surgical resection or embolization, in larger lesions.

A vascular malformation is a type of vascular anomaly.^[2] They may cause aesthetic problems as they have a growth cycle, and can continue to grow throughout life.

Vascular malformations of the brain include those involving capillaries, and those involving the veins and arteries. Capillary malformations in the brain are known as cerebral cavernous malformations or capillary cavernous malformations. Those involving the mix of vessels are known as cerebral arteriovenous malformations (AVMs or cAVMs). The arteriovenous type is the most common in the brain.^[3]

Types

The International Society for the Study of Vascular Anomalies (ISSVA) classification has 5 types of Vascular Malformation.

Types of Vascular Malformations
Simple	Combined	Of Major Named Vessels	Associated with Other Anomalies
Capillary malformations	Defined as two or more vascular malformations found in one lesion.	Abnormalities in the origin/course/number of major blood vessels that have anatomical names	Syndromes in which vascular malformations are complicated by symptoms other than vascular anomalies
Lymphatic malformations
Venous malformations
Arteriovenous malformations*
Arteriovenous fistula*

* denotes high-flow malformation

Vascular malformations can also be divided into low-flow and high-flow types.^[2] Low-flow malformations involve a single type of blood or lymph vessel, and are known as simple vascular malformations; high-flow malformations involve an artery. There are also malformations that are of mixed-flow involving more than one type of vessel, such as an arteriovenous malformation.^[2] Low-flow vascular malformations include capillary malformations, venous malformations, and lymphatic malformations.^[4]

Simple Types

Capillary malformation

Capillary malformations involve the capillaries, and are the most common type. They used to refer only to port-wine stains but now include others.^[2] Capillary malformations are limited to the superficial layers of the skin but they can thicken, become nodular, and sometimes become disfiguring.^[5] It has been proposed that the category of capillary malformations, also called vascular stains, be classified into seven major clinical types including nevus flammeus nuchae also known as nevus simplex, commonly known as stork bite or salmon patch.^[6]

A capillary malformation is also a feature of the disorder macrocephaly-capillary malformation.^[7] An example of capillary malformation is cerebral cavernous malformations. This disease is linked to the central nervous system (brain, eye, spinal cord). They are abnormal clusters of closely packed, thin-walled blood vessels that usually form caverns. The lesions contain slow-moving or clotted blood. Lesions in the brain and spinal cord are particularly fragile and likely to bleed.^[8]

Lymphatic malformation

Lymphatic malformations are congenital, developing from badly-formed lymphatic vessels in early embryonic development.^[9] Abnormal development of the lymph vessels results in their failure to connect and drain into the venous system.^[9]

These lymph vessels can become blocked due to the collection of lymph which forms a cyst as a mass, and are known as lymphatic malformationss. They can be macrocystic, microcystic, or a combination of the two.^[9] Macrocystic have cysts greater than 2 cubic centimetres (0.12 cu in), and microcystic lymphatic malformation have cysts that are smaller than 2 cubic centimetres (0.12 cu in).^[10]

A severe venous malformation is known as a lymphaticovenous malformation that also involves the lymph vessels.^[11]

Venous malformations

Venous malformations are the type of vascular malformation that involves the veins. They can often extend deeper from their surface appearance, reaching underlying muscle or bone.^[12] In the neck they may extend into the lining of the mouth cavity or into the salivary glands.^[11] They are the most common of the vascular malformations.^[13] A severe venous malformation can involve the lymph vessels as a lymphaticovenous malformation.^[11]

Arteriovenous malformation

Arteriovenous malformations occur between an artery and a vein.

In the brain a cerebral arteriovenous malformation causes arterial blood to be directly shunted into the veins as there is an absence of a capillary bed. This carries a high risk of an intracranial hemorrhage.^[14]

Arteriovenous fistula

Combined Types

Combined types are defined as two or more vascular malformations found in one lesion. Examples of combined types include lymphatic-venous malformation (LVM) or capillary-venous-arteriovenous malformation (VAVM).

Terminology

The International Society for the Study of Vascular Anomalies (ISSVA) classification is a basic and systematic classification of vascular anomalies with international acceptance. As such terms such as "Lymphangioma" and "Cystic Hygroma", which were used widely in the past, are outdated. Newer research may only reference ISSVA terminology and, as a consequence, sources of information can be missed by doctors and patients unaware of the ISSVA convention.

Terminology
ISSVA name	Outdated names
Lymphatic malformation	Lymphangioma, Cystic hygroma, Lymphangioma circumscriptum, Cavernous lymphangioma, lymphangiomatosis
Venous malformation	Caveronous Hemangioma
Capillary Malformation	Port-wine strain, Capillary hemangioma

References

^ Jackson, Ian T., et al. "Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment." Plastic and reconstructive surgery 91.7 (1993): 1216-1230.
^ ^a ^b ^c ^d Steiner, JE; Drolet, BA (September 2017). "Classification of Vascular Anomalies: An Update". Seminars in Interventional Radiology. 34 (3): 225–232. doi:10.1055/s-0037-1604295. PMC 5615389. PMID 28955111.
^ Leblanc, GG; Golanov, E; Awad, IA; Young, WL (December 2009). "Biology of vascular malformations of the brain". Stroke. 40 (12): e694-702. doi:10.1161/STROKEAHA.109.563692. PMC 2810509. PMID 19834013.
^ Sadick, M; Müller-Wille, R; Wildgruber, M; Wohlgemuth, WA (September 2018). "Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies". RöFo. 190 (9): 825–835. doi:10.1055/a-0620-8925. PMID 29874693.
^ Puttgen, KB; Pearl, M; Tekes, A; Mitchell, SE (October 2010). "Update on pediatric extracranial vascular anomalies of the head and neck". Child's Nervous System. 26 (10): 1417–33. doi:10.1007/s00381-010-1202-2. PMID 20697721. S2CID 12359248.
^ Rozas-Muñoz, E; Frieden, IJ; Roé, E; Puig, L; Baselga, E (November 2016). "Vascular Stains: Proposal for a Clinical Classification to Improve Diagnosis and Management". Pediatric Dermatology. 33 (6): 570–584. doi:10.1111/pde.12939. PMID 27456075. S2CID 22474320.
^ Gonzalez, ME; Burk, CJ; Barbouth, DS; Connelly, EA (May–June 2009). "Macrocephaly-capillary malformation: a report of three cases and review of the literature". Pediatric Dermatology. 26 (3): 342–6. doi:10.1111/j.1525-1470.2009.00924.x. PMID 19706101. S2CID 27334360.
^ "Cerebral Cavernous Malformations | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Retrieved 2024-09-12.
^ ^a ^b ^c Elluru, RG; Balakrishnan, K; Padua, HM (August 2014). "Lymphatic malformations: diagnosis and management". Seminars in Pediatric Surgery. 23 (4): 178–85. doi:10.1053/j.sempedsurg.2014.07.002. PMID 25241095.
^ "Microcystic lymphatic malformation | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov.
^ ^a ^b ^c Chim, H; Drolet, B; Duffy, K; Koshima, I; Gosain, AK (August 2010). "Vascular anomalies and lymphedema". Plastic and Reconstructive Surgery. 126 (2): 55e – 69e. doi:10.1097/PRS.0b013e3181df803d. PMID 20679788. S2CID 30865392.
^ Chen, RJ; Vrazas, JI; Penington, AJ (January 2021). "Surgical Management of Intramuscular Venous Malformations". Journal of Pediatric Orthopedics. 41 (1): e67 – e73. doi:10.1097/BPO.0000000000001667. PMID 32815867. S2CID 221199574.
^ Markovic, JN; Shortell, CK (October 2021). "Venous malformations". The Journal of Cardiovascular Surgery. 62 (5): 456–466. doi:10.23736/S0021-9509.21.11911-1. PMID 34105926.
^ Mouchtouris, N; Jabbour, PM; Starke, RM; Hasan, DM; Zanaty, M; Theofanis, T; Ding, D; Tjoumakaris, SI; Dumont, AS; Ghobrial, GM; Kung, D; Rosenwasser, RH; Chalouhi, N (February 2015). "Biology of cerebral arteriovenous malformations with a focus on inflammation". Journal of Cerebral Blood Flow and Metabolism. 35 (2): 167–75. doi:10.1038/jcbfm.2014.179. PMC 4426734. PMID 25407267.