Vascular malformation

Last updated
Vascular malformation
Other namesVascular giantism or Lymphangioma
Specialty Cardiovascular
TreatmentIn 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.

Contents

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

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 Port-wine stain on leg.jpg
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 Lymphangioma of the left side of the tongue Wellcome L0062760.jpg
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

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

Related Research Articles

<span class="mw-page-title-main">Arteriovenous malformation</span> Vascular anomaly

An arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system. Usually congenital, this vascular anomaly is widely known because of its occurrence in the central nervous system, 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.

<span class="mw-page-title-main">Cerebral arteriovenous malformation</span> Medical condition

A cerebral arteriovenous malformation is an abnormal connection between the arteries and veins in the brain—specifically, an arteriovenous malformation in the cerebrum.

<span class="mw-page-title-main">Vein</span> Blood vessels that carry blood towards the heart

Veins are blood vessels in the circulatory system of humans and most other animals that carry blood toward the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are those of the pulmonary and fetal circulations which carry oxygenated blood to the heart. In the systemic circulation arteries carry oxygenated blood away from the heart, and veins return deoxygenated blood to the heart, in the deep veins.

<span class="mw-page-title-main">Lymphatic system</span> Organ system in vertebrates

The lymphatic system, or lymphoid system, is an organ system in vertebrates that is part of the immune system, and complementary to the circulatory system. It consists of a large network of lymphatic vessels, lymph nodes, lymphoid organs, lymphoid tissues and lymph. Lymph is a clear fluid carried by the lymphatic vessels back to the heart for re-circulation.

<span class="mw-page-title-main">Lymphatic vessel</span> Tubular vessels that are involved in the transport of lymph and lymphocytes

The lymphatic vessels are thin-walled vessels (tubes), structured like blood vessels, that carry lymph. As part of the lymphatic system, lymph vessels are complementary to the cardiovascular system. Lymph vessels are lined by endothelial cells, and have a thin layer of smooth muscle, and adventitia that binds the lymph vessels to the surrounding tissue. Lymph vessels are devoted to the propulsion of the lymph from the lymph capillaries, which are mainly concerned with the absorption of interstitial fluid from the tissues. Lymph capillaries are slightly bigger than their counterpart capillaries of the vascular system. Lymph vessels that carry lymph to a lymph node are called afferent lymph vessels, and those that carry it from a lymph node are called efferent lymph vessels, from where the lymph may travel to another lymph node, may be returned to a vein, or may travel to a larger lymph duct. Lymph ducts drain the lymph into one of the subclavian veins and thus return it to general circulation.

<span class="mw-page-title-main">Sclerotherapy</span> Shrinking of the varicose blood vessels by the injection of medicine

Sclerotherapy is a procedure used to treat blood vessel malformations and also malformations of the lymphatic system. A medicine is injected into the vessels, which makes them shrink. It is used for children and young adults with vascular or lymphatic malformations. In adults, sclerotherapy is often used to treat spider veins, smaller varicose veins, hemorrhoids, and hydroceles.

<span class="mw-page-title-main">Arteriovenous fistula</span> Medical condition

An arteriovenous fistula is an abnormal connection or passageway between an artery and a vein. It may be congenital, surgically created for hemodialysis treatments, or acquired due to pathologic process, such as trauma or erosion of an arterial aneurysm.

<span class="mw-page-title-main">Lymphangioma</span> Malformations of the lymphatic system characterized by lesions that are thin-walled cysts

Lymphangiomas are malformations of the lymphatic system characterized by lesions that are thin-walled cysts; these cysts can be macroscopic, as in a cystic hygroma, or microscopic. The lymphatic system is the network of vessels responsible for returning to the venous system excess fluid from tissues as well as the lymph nodes that filter this fluid for signs of pathogens. These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and involve the head and neck. These malformations are either congenital or acquired. Congenital lymphangiomas are often associated with chromosomal abnormalities such as Turner syndrome, although they can also exist in isolation. Lymphangiomas are commonly diagnosed before birth using fetal ultrasonography. Acquired lymphangiomas may result from trauma, inflammation, or lymphatic obstruction.

<span class="mw-page-title-main">Dural arteriovenous fistula</span> Medical condition

A dural arteriovenous fistula (DAVF) or malformation is an abnormal direct connection (fistula) between a meningeal artery and a meningeal vein or dural venous sinus.

<span class="mw-page-title-main">Cystic hygroma</span> Human disease

A cystic hygroma is an abnormal growth that usually appears on a baby's neck or head. It consists of one or more cysts and tends to grow larger over time. The disorder usually develops while the fetus is still in the uterus, but can also appear after birth.

<span class="mw-page-title-main">Vascular tumor</span> Medical condition

A vascular tumor is a tumor of vascular origin; a soft tissue growth that can be either benign or malignant, formed from blood vessels or lymph vessels. Examples of vascular tumors include hemangiomas, lymphangiomas, hemangioendotheliomas, Kaposi's sarcomas, angiosarcomas, and hemangioblastomas. An angioma refers to any type of benign vascular tumor.

<span class="mw-page-title-main">Vascular disease</span> Medical condition

Vascular disease is a class of diseases of the vessels of the circulatory system in the body, including blood vessels – the arteries and veins, and the lymphatic vessels. Vascular disease is a subgroup of cardiovascular disease. Disorders in this vast network of blood and lymph vessels can cause a range of health problems that can sometimes become severe, and fatal. Coronary heart disease for example, is the leading cause of death for men and women in the United States.

<span class="mw-page-title-main">Klippel–Trénaunay syndrome</span> Medical condition

Klippel–Trénaunay syndrome, formerly Klippel–Trénaunay–Weber syndrome and sometimes angioosteohypertrophy syndrome and hemangiectatic hypertrophy, is a rare congenital medical condition in which blood vessels and/or lymph vessels fail to form properly. The three main features are nevus flammeus, venous and lymphatic malformations, and soft-tissue hypertrophy of the affected limb. It is similar to, though distinctly separate from, the less common Parkes Weber syndrome.

<span class="mw-page-title-main">Bonnet–Dechaume–Blanc syndrome</span> Medical condition

Bonnet–Dechaume–Blanc syndrome, also known as Wyburn-Mason syndrome, is a rare congenital disorder characterized by arteriovenous malformations of the brain, retina or facial nevi. The syndrome has a number of possible symptoms and can, more rarely, affect the skin, bones, kidneys, muscles, and gastrointestinal tract. When the syndrome affects the brain, people can experience severe headaches, seizures, acute stroke, meningism, and progressive neurological deficits due to acute or chronic ischaemia caused by arteriovenous shunting.

A vascular anomaly is any of a range of lesions from a simple birthmark to a large tumor that may be disfiguring. They are caused by a disorder of the vascular system. A vascular anomaly is a localized defect in blood or lymph vessels. These defects are characterized by an increased number of vessels, and vessels that are both enlarged and sinuous. Some vascular anomalies are congenital, others appear within weeks to years after birth, and others are acquired by trauma or during pregnancy. Inherited vascular anomalies are also described and often present with a number of lesions that increase with age. Vascular anomalies can also be a part of a syndrome.

<span class="mw-page-title-main">Parkes Weber syndrome</span> Medical condition

Parkes Weber syndrome (PWS) is a congenital disorder of the vascular system. It is an extremely rare condition, and its exact prevalence is unknown. It is named after British dermatologist Frederick Parkes Weber, who first described the syndrome in 1907.

<span class="mw-page-title-main">Vein of Galen aneurysmal malformations</span> Medical condition

Vein of Galen aneurysmal malformations(VGAMs) and Vein of Galen aneurysmal dilations (VGADs) are the most frequent arteriovenous malformations in infants and fetuses. A VGAM consists of a tangled mass of dilated vessels supplied by an enlarged artery. The malformation increases greatly in size with age, although the mechanism of the increase is unknown. Dilation of the great cerebral vein of Galen is a secondary result of the force of arterial blood either directly from an artery via an arteriovenous fistula or by way of a tributary vein that receives the blood directly from an artery. There is usually a venous anomaly downstream from the draining vein that, together with the high blood flow into the great cerebral vein of Galen causes its dilation. The right sided cardiac chambers and pulmonary arteries also develop mild to severe dilation.

Lymphohemangioma is a disease characterized by swelling of the lymph nodes and blood vessels. It is variously described as a "mixture of clear fluid and blood-filled cysts", a mass of abnormal swollen veins and lymph nodes, or a tumorous growth of lymph and blood vessels. Oftentimes, it is described as a misnomer for combined lymphatic and capillary malformation. The lymphangiomas are known to be malformations of the lymph tissue, 75% of which is found near the head and neck. However, it can affect any region of the body. This disease is most commonly observed in children and male patients. In fact, Lymphangiomas account for 4% of all vascular tumors and 25% of the benign vascular tumors in children. Additionally, 50% of Lymphangiomas are noted at birth and become more evident by age 5.

<span class="mw-page-title-main">CLOVES syndrome</span> Medical condition

CLOVES syndrome is a rare overgrowth syndrome with complex vascular anomalies. CLOVES syndrome affects people with various symptoms, ranging from mild fatty soft-tissue tumors to vascular malformations encompassing the spine or internal organs.

Diffuse capillary malformation with overgrowth (DCMO) is a subset of capillary malformations (CM) associated with hypertrophy, i.e. increased size of body structures. CM can be considered an umbrella term for various vascular anomalies caused by increased diameter or number of capillary blood vessels. It is commonly referred to as "port-wine stain", and is thought to affect approximately 0.5% of the population. Typically capillaries in the papillary dermis are involved, and this gives rise to pink or violaceous colored lesions. The majority of DCMO lesions are diffuse, reticulated pale-colored stains.

References

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