General Information
Vascular Anomalies |
The National Organization of Vascular Anomalies is a patient support, advocacy and educational agency for individuals affected by vascular tumors and vascular malformation. Traditionally these tumors and malformations have been called birthmarks. Medicine and science today dictate that they are so much more. |
Vascular Anomalies: More Than a Birthmark
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Vascular Tumors: Vascular tumors arise from abnormal circumscribed growth of blood vessel cells. There are three identified categories of vascular tumors, Benign Tumors, Locally Aggressive or Borderline Tumors and Malignant Tumors. Within each category are specific types of vascular tumors.
Benign Vascular Tumors- Include Infantile Hemangioma, Congenital Hemangioma, Tufted Angioima, Pyogenic Granuloma and other benign vascular tumors. Locally Aggressive or Borderline Vascular Tumors-Include Kaposiform Hemangioenothelioma (KHE), Kaposi Sarcoma, Retiform Hemangioendothelioma, Papillary intralymphatic angioendothelioma (PILA), Dabska tumor and others. Malignant Vascular Tumors – Include Angiosarcoma, Epithelioid hemangioendothelioma and othersThe most common benign vascular tumor is the Infantile Hemangioma. It is the most common benign tumor of infancy and children. Most develop in the first few weeks of life. They grow for about a year and then will spontaneously regress in a process called involution. IH occur in 4-10% of all children and are more common in females then in males. (MORE on Infantile Hemangioma) Some hemangioma are fully formed at birth these are called Congential Hemangioma. This type of hemangioma differs from IH. (More on Congenital Hemangioma) Some vascular tumors are associated with thrombocytopenia, and or anemia, these include KHE and Tufted Angioma (More on KHE and Tufted Angioma) There is a known association of Infantile Hemangioma with a spectrum of other abnormalities known as PHACE. (More on PHACE) |
Vascular Malformations are abnormally developed vessels that can occur in any of the blood vessels or lymphatic vessels. There are four identified categories of vascular anomalies.
Simple– Inclucdes Capillary Malformations (Port Wine Stain), Lymphatic Malformations, Venous Malformations, Ateriovenous Malformations and Arteriovenous fistula.
Combined– Includes CVM-CLM, LVM-CLVM, CAVM, CLAVM and others. Named after Major Vessels– Affect lymphatics, veins, arteries, Anomalies of origin, course, number, length, diameter, valves communication and persistence (of embryonal vessel) Associated with other Anomalies– Klippel Trenaunay Syndrome, Parkes Weber Syndrome, Servelle-Martorell Syndrome, Sturge Weber Sndrome, Maffucci Syndrome, Cloves Syndrome, Proteus,
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Vascular Anomalies are associated with “syndromes”. In medical terminology a syndrome is an association of clinically recognizable signs, symptoms and features that often occur together but with significant variation. A syndrome in itself is not a disease but rather an association of observable features that allows clinicians to form treatment and direct research. Some Syndromes related to Vascular Anomalies include: Sturge Weber, Kilppel Trenaunay, PHACE, CLOVES.Vascular Anomalies are often confused and misdiagnosed. This leads to frustration for the parents and the families of those affected.
Classification of Vascular Anomalies was first published by Dr. John Mulliken in 1981, it has recently been revised at the 2014 meeting of ISSVA in Melbourne Australia. The information above is from this newly revised classification.
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NOVA provides this information as a resource. It is not intended to engage in the practice of medicine or to replace the physician. NOVA does not claim to have medical knowlege. NOVA does not endorse any particular physician, treating facility or treatment protocol. In all cases NOVA, the NOVA Board of Directors and associates recommends that you seek the opinion of a physician experienced in the diagnosis and treatment of hemangioma and vascular malformations. |
Rev 04/2014 |