Vascular Malformations are benign vascular lesions that include a wide variety of anomalies that are different from hemangioma.
Vascular Malformations are always present at birth, but may not be visible until days, weeks or even years after birth.VM grows over the lifetime of the patient and will never spontaneously regress.These lesions occur as commonly in males as in females and do not occur any more or less frequently between races.VM may be first diagnosed as hemangioma. While they are very different, they sometimes resemble a hemangioma in appearance.
Vascular Malformations are abnormally developed vessels. These abnormalities may occure in the small vessles or capillaries, the veins, the arteries or the lymphatic vessels. The diagnosis for the vascular malformation will depend on the vessel affected.
Types of Vascular Malformations
Midline venular malformations: commonly called "stork bites and angel kisses". This type of lesion is present at birth often in the back of the neck, on the forehead, eyelid or even the lip. It appears as a pale red blotch. Most of the time the area will lighten and become less noticeable
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Capillary Malformations : (Port Wine Stains) are always present at birth and can range from pale pink to dark purple in color. PWS occurs in about 0.3% of the population with equal incidence in males and females. They are present at birth and develop over time. In the past these lesions were called "capillary hemangiomas." The exact cause of PWS is not known although some physicians believe that the progression of a PWS is related to a problem in the nerve control over dilation and relaxation of the blood vessles. PWS treatment involes treatment with Pulse Dyle lasers. The results vary and may be temporary. As the PWS progresses maintence may be necessary through life. It is believed that early laser in childhoodmay prevent a darkening of the PWS or a "cobbling" and thickening of the skin which occurs in adult years. Patients diagnosed with PWS should be evaluated by a dermatologist in early childhood and followed through life.
Sturge Weber Syndrome is a syndrome seen in patients presenting with a large PWS of the face that does not cross the midline; an eye is involved as well as the covering of the brain. Patients with this pattern of PWS should be evaluated for Sturge Weber Syndrome. Early diagnosis is important for optimal treatment outcomes.
For more informaition on SWS contact our friends at the
Sturge Weber Foundation: http://www.sturge-weber.org/ |
Pyogenic Granuloma or Lobulated Capillary Hemangioma:
Pyogenic Granuloma are small reddish bumps on the skin that easily bleed due to a hgh concentration of blood vessels. The exact cause is unknown.Typical Presentation: Small almost always a singular solitary raspberry like structure ranging in size from 1mm – 2cm in size. May first appear as a small cherry red zone and grows rapidly with a frequency of bleeding often profusely.
Occurrence: Usually appears in children 6 months – 2 years of age although not contained to this age group. There is some indication that it is an acquired lesion starting from a scratch, cut or burn. Yet this is not always the case.
Histology: The epidermis is usually thin. The growth is composed of numerous newly formed capillaries suggesting a subcutaneous hemangioma. The capillaries are lined with endothelial cells in a single layer. The may also be an edematous fibroblastic proliferation in the stroma that surrounds the tumor itself.
Diagnosis: Diagnosis is usually easy, as the presentation is not consistent with hemangioma of infancy. They may be mistakenly diagnosed as a Kaposi's Sarcoma, melanoma or other tumor. However histological evaluation is distinctive enough to rule out other diagnosis. Most common misdiagnosis is hemangioma.
Treatment: Includes surgical excision of the entire lesion, including the dermal curette and base. Anesthesia may be necessary. There is a small chance of reoccurrence, which can be followed up with repeat excision and laser ablation.
Venous Malformations
An abnormality of the larger, deep vessels and often appears as a hemangioma. Venous Malformations do not go through the prolific growth phase that a hemangioma goes through and does not involute. A venous malformation can be deep or superficial, localized or diffused. Superficial malformations have more color and can be blue and red. Often the vessels are visualized. Deep lesions have little or no color and will cause a bulge in the skin. Venous Malformations are commonly found in the cheek, tongue, lip and jaw.
These lesions are soft to the touch; the color disappears and can be compressed if pressure is applied. Sometimes parents report that if the child cries or is lying down, the lesion gets larger and darker.
Growth or Venous Malformations occur over the life of the patients. Certain things may cause a venous malformation to grow. Serious illness, trauma, infection and hormone changes of puberty and pregnancy are associated with growth of the lesions.
Cutis Marmorata Telangiectatica Congenita is a congenital disorder known as CMTC. This disorder is uncommon and consists of symptoms including a persistent marbled look to the skin along with chronic dilation of blood vessels, varicosities of veins, ulceration of the skin and atrophy of the skin. CMTC is seen to occur in association with Sturge Weber Syndrome and Klippel Trenaunay Syndrome. Approximately 50% of patients have one or more other congenital abnormalities. There is no known racial predilection; the disorder affects slightly more females then males. CMTC is generally present at birth. The cause appears to be multifactorial, including genetic, viral, geography and due to fetal development. No treatment is needed unless there are associated anomalies that require medical intervention. Often the skin lesions improve by 2 years of age.
Klipple Trenanay Syndrome is a syndrome seen in patients presenting with a capillary or venule malformation on an extremity in the first weeks after birth. As the infanct grows large venous varicosities develop. Limb overgrowth and excess fatty tissue devleopment occurs. KT is associated with several significant complications including thrombophlebitis, cellulits and pain.
Lymphatic Malformations formerly called cystic -hygroma, hemangiolymphangioma, or lymphangiomas. The lymphatic system is a collection and transfer system for fluid in the body's tissue. Abnormally developed lymphatic channels result in excess fluid accumulation in both the tissue and the vessels themselves. When the vessels involved are close to the surface of the skin there will be a prominent enlargement of the area. If the face is involved the face will swell and become disfigured. If it occurs in the mouth or tongue it can interfere with eating and breathing. (lesions in the tongue are often describe as appearing as small papules or frog eggs) They may enlarge following an upper respiratory infection. These lesions may occur anywhere but are most common in the head and neck. Lymphatic Malformations increase or grow with the patient. They are difficult to treat.
Proteus Syndrome is a very rare hereditary disorder characterized by multiple lesions of the lymph vessls, overgrowth of one side of the body, (hemihypertrophy), an abnormally enlargedhead, parital giagantism of the fee and multiple brown nevi (cafe-au-lait birthmarks) on the skin.
Arteriovenous Malformations (AVM)
This type of malormation is present at birth but are usually not noticed until later in life. Abnormal blood flow is associated with these lesions. As the lesion ages, the vessels enlarge and thicken to compensate for the increased blood supply. There are two major types: Low Grade AVM and High Grade AVM. A Low Flow AVM will grow slowly with the child and H High Flow AVM will expand rapidly, growing faster than the child until the lesion may eventually become life threatening. Because blood flow can be affected and shunted away from normal flow patterns treatment is usually necessary. An AV malformation feels like a firm mass and does not compress easily. Common sites are the lips and other head and neck areas.
Cavernous Angioma refers to abnormally developed blood vessels of the brain, spinal cord and rarely other ares of the body. Sometimes these anomalies are referred to as, cavernous angioma, cerebral cavernouse malformations (CCM), cavernoma and cavernous hemangioma. Cavernous angioma can cause severe symptoms including neurological deficits, weakness in the limbs, vision and balance problems, headaches and seizures. Cavernous Angiomas typically become symptomatic when the blood vessels involved begin to bleed. For more information on Cavernous Angioma contact our friends at :www.angiomaalliance.org
Glomuvenous Malformations (glomangioma) GMV have traditionally been known as venous malforamtions wiht glomus cells. The are cutaneous venous lesions characterized by the presence of smooth muscle like glomus cells in the media surrounding distended vascular lumens. These anomalies are similar to venous malformations with a few exceptions. GVM's have a cobblestone appearance and have a consistency that is harder then venous malformations. They are also painful to palpate.
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