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Getting to know Vascular Anomaly Lesions (3): Vascular Malformations

What are vascular malformations?

Vascular malformation is a localized lesion caused by the abnormal development of blood vessels. The abnormal tissue, which generally appears since birth, varies depending on the types of vessels that are involved. Several types of blood vessels in the body are arteries, veins, capillaries, and lymphatics, which can be on their own or combined. This anomaly is divided based on the flow of the blood vessels, which are slow-flow and high-flow; can appear localized in all parts of the body surface and become visible at the age of adolescents or adults. Lesions can grow, shrink, and damage the surrounding tissue. Unlike hemangioma, this lesion does not heal spontaneously, but rather continuously grows.

How often does it occur?

Approximately, vascular malformations occur in 1-4% of population; lymphatic malformations occur in 1 out of 2000-4000 lives; capillary malformations in 0.3% of children; and venous malformations affect 1 out of 10.000 lives.

What causes it?

Causes have not been clearly identified; however genetic factors may play a role in several types. Moreover, lymphatic malformations can be precipitated by trauma or infection.

How to detect this condition?

Diagnosis is made based on clinical history dan physical examination. Supporting modalities such as Doppler’s Ultrasound, MRI, and angiography are important.

What are the characteristics of vascular malformations?

Vascular malformations may cause several conditions based on the blood vessels and areas involved.

  • Venous malformations: Usually, this appears as a bulging, bluish or grayish lesion. Initially, symptoms appear following trauma or physiological changes, such as pregnancy or puberty. Symptoms may involve pain, blood clotting abnormalities, and organ damage (bones, muscles, or joints).

  • Lymphatic malformations: Can be differentiated into macrocystic (one or more cysts sized > 2cm3), microcystic (one or more cysts sized < 2cm3), or combination of the two. These lesions often get infected, which causes swelling, thus more prominent appearance, and pain. Elastic macrocystic lesions contain fluid, cannot be decompressed, and have color similar to the skin. Similarly, elastic microcycstic lesion also cannot be decompressed, thus causing blisters on the skin and mucous membranes.

  • Arteriovenous malformations (AVM): Lesions appear bluish since birth, warm to touch, with pulsating or vibrating sensations due to the high-flow venous vessels. Puberty and trauma may be the triggering factors. This condition can damage the surrounding vessels besides being life-threatening due to massive bleeding.

  • Capillary malformations: Differentiated based on its location, which includes mid-capillary malformations (“stork bites” on the neck or “angel kisses” on the forehead, which can heal spontaneously) and port wine stains. Both present as red- or purple-colored lesions that appear since birth, with irregular border, painless, and do not bleed.

How to treat it?

Treatments depend on the blood vessels involved; whether this condition appears on its own or causes other symptoms, as well as the patient’s general condition. Treatment is focused to relieve the patients’ symptoms – ranging from attention for aesthetic appearance to life-saving efforts in critical conditions. Regular observation is needed to assess the development of the lesions. Embolization and sclerotherapy using injected drugs or other specific materials is used to stop or clot the blood vessel. Laser is used to fade skin color as well as capillary lesions. Furthermore, surgeries and reconstructions can be performed simultaneously.

Written by Teddy Prasetyono, Ph.D and Amila Tikyayala, M.D.

Division of Plastic Surgery, Department of Surgery

Dr. Cipto Mangunkusumo Hospital / Faculty of Medicine, Universitas Indonesia

Translated by Illona Andromeda, M.D.


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