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.[1]

A vascular malformation is a blood vessel or lymph vessel abnormality. Vascular malformations are one of the classifications of vascular anomalies, the other grouping is vascular tumors.[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]

## Contents

* 1 Types
* 1.1 Capillary malformation
* 1.1.1 Vascular anomalies
* 1.2 Lymphatic malformation
* 1.3 Arteriovenous malformation
* 2 See also
* 3 References

## Types

A simple division of the vascular malformations is made 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]

### Capillary malformation

Port-wine stain on leg

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]

#### Vascular anomalies

A vascular anomaly can be either a vascular tumor or a birthmark, or a vascular malformation. [2] In a tumor such as infantile hemangioma the mass is soft, and easily compressed, and their coloring is due to the dilated anomalous involved veins.[8] They are most commonly found in the head and neck. 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.[9] In the neck they may extend into the lining of the mouth cavity or into the salivary glands.[8] They are the most common of the vascular malformations.[10] A severe venous malformation can involve the lymph vessels as a lymphaticovenous malformation.[8]

### Lymphatic malformation

Drawing of a microcystic lymphangioma on the left side of a boy's tongue

Lymphatic malformations are congenital, developing from badly-formed lymphatic vessels in early embryonic development.[11] Abnormal development of the lymph vessels results in their failure to connect and drain into the venous system.[11]

These lymph vessels can become blocked due to the collection of lymph which forms a cyst as a mass, and are known as lymphangiomas. They can be macrocystic, microcystic, or a combination of the two.[11] Macrocystic have cysts greater than 2 cubic centimetres (0.12 cu in), and microcystic lymphangiomas have cysts that are smaller than 2 cubic centimetres (0.12 cu in).[12] A macrocystic lymphangioma is also known as a cystic hygroma. Cystic hygromas most often occur in the neck where they are known as nuchal hygromas.[13]

A severe venous malformation is known as a lymphaticovenous malformation that also involves the lymph vessels.[8]

### Arteriovenous malformation

Main article: Medicine:Arteriovenous malformation

3D image of an arteriovenous malformation shown in purple on the ring finger of a hand

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]

## See also

* Arteriovenous fistula
* Lymphohemangioma
* Telangiectasia
* Vascular disease

## References

1. ↑ 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.
2. ↑ 2.0 2.1 2.2 2.3 2.4 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. PMID 28955111.
3. ↑ Leblanc, GG; Golanov, E; Awad, IA; Young, WL; Biology of Vascular Malformations of the Brain NINDS Workshop, Collaborators. (December 2009). "Biology of vascular malformations of the brain.". Stroke 40 (12): e694-702. doi:10.1161/STROKEAHA.109.563692. PMID 19834013.
4. ↑ 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.
5. ↑ 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.
6. ↑ 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.
7. ↑ 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.
8. ↑ 8.0 8.1 8.2 8.3 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.
9. ↑ 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.
10. ↑ 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.
11. ↑ 11.0 11.1 11.2 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.
12. ↑ "Microcystic lymphatic malformation | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". https://rarediseases.info.nih.gov/diseases/13020/microcystic-lymphatic-malformation.
13. ↑ D'Souza, Donna. "Cystic hygroma | Radiology Reference Article | Radiopaedia.org". https://radiopaedia.org/articles/cystic-hygroma-1?lang=gb.
14. ↑ Mouchtouris, N; Jabbour, PM; Starke, RM; Hasan, DM; Zanaty, M; Theofanis, T; Ding, D; Tjoumakaris, SI et al. (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. PMID 25407267.

Classification|

* MeSH: D054079

|

* v
* t
* e

Cardiovascular disease (vessels) (I70–I99, 440–456)

Arteries, arterioles
and capillaries|

| Inflammation|

* Arteritis
* Aortitis
* Buerger's disease

|

Peripheral artery disease|

| Arteriosclerosis|

* Atherosclerosis
* Foam cell
* Fatty streak
* Atheroma
* Intermittent claudication
* Critical limb ischemia

* Monckeberg's arteriosclerosis
* Arteriolosclerosis
* Hyaline
* Hyperplastic
* Cholesterol
* LDL
* Oxycholesterol
* Trans fat

|

Stenosis|

* Carotid artery stenosis
* Renal artery stenosis

Other|

* Aortoiliac occlusive disease
* Degos disease
* Erythromelalgia
* Fibromuscular dysplasia
* Raynaud's phenomenon

Aneurysm / dissection /
pseudoaneurysm|

* torso: Aortic aneurysm
* Abdominal aortic aneurysm
* Thoracic aortic aneurysm
* Aneurysm of sinus of Valsalva
* Aortic dissection
* Aortic rupture
* Coronary artery aneurysm
* head / neck
* Intracranial aneurysm
* Intracranial berry aneurysm
* Carotid artery dissection
* Vertebral artery dissection
* Familial aortic dissection

Vascular malformation|

* Arteriovenous fistula
* Arteriovenous malformation
* Telangiectasia
* Hereditary hemorrhagic telangiectasia

Vascular nevus|

* Cherry hemangioma
* Halo nevus
* Spider angioma

Veins|

| Inflammation|

* Phlebitis

|

Venous thrombosis /
Thrombophlebitis|

* primarily lower limb
* Deep vein thrombosis
* abdomen
* Hepatic veno-occlusive disease
* Budd–Chiari syndrome
* May–Thurner syndrome
* Portal vein thrombosis
* Renal vein thrombosis
* upper limb / torso
* Mondor's disease
* Paget–Schroetter disease
* head
* Cerebral venous sinus thrombosis
* Post-thrombotic syndrome

Varicose veins|

* Gastric varices
* Portacaval anastomosis
* Caput medusae
* Esophageal varices
* Hemorrhoid
* Varicocele

Other|

* Chronic venous insufficiency
* Chronic cerebrospinal venous insufficiency
* Superior vena cava syndrome
* Inferior vena cava syndrome
* Venous ulcer

Arteries or veins|

* Angiopathy
* Macroangiopathy
* Microangiopathy
* Embolism
* Pulmonary embolism
* Cholesterol embolism
* Paradoxical embolism
* Thrombosis
* Vasculitis

Blood pressure|

| Hypertension|

* Hypertensive heart disease
* Hypertensive emergency
* Hypertensive nephropathy
* Essential hypertension
* Secondary hypertension
* Renovascular hypertension
* Benign hypertension
* Pulmonary hypertension
* Systolic hypertension
* White coat hypertension

|

Hypotension|

* Orthostatic hypotension

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