This condition develops when only veins are malformed and is most frequent among vascular malformations. Since the veins themselves seem normally developed, this malformation shows no pulsation. However, it is identifiable with soft lumps of vessels that can be easily pushed in when pressed. . This condition can occur in any part of the body including the head, the neck, the thorax, the extremities, the abdomen, and organs. When the lesion is located deep in the abdomen, the back, or the thorax, it is hardly visible.
Symptoms
Patients visit a doctor, usually reporting lumps, pain, fatigue, or aesthetic concerns. The lesion looks like a bluish and uneven lump and becomes larger as the patients grow. The size of bulge depends on dilation of veins under the skin and its growth into the skin. When it is under the subcutaneous fat, the skin color seems normal and the lesion is invisible outside. The severity varies from a tiny lesion similar to varicose veins to severe ones covering the entire face, arm, or leg. While it may be usually asymptomatic, the patients may experience pain after walking a lot or working out.
Treatment
When patients do not experience any symptom and have no difficulties in their daily life and malfunctions of the extremities, treatment is only optional. Patients may wear compression stockings to prevent blood pooling in the lesion from causing thrombus or consumptive cytopenia. If patients have pain, bleeding, discomfort while moving, or aesthetic concerns, treatment is required. It is particularly necessary when the lesion expands rapidly and develops severe pain due to hormonal changes during pregnancy or puberty, or after childbirth. If patients experience difficulties in walking due to the difference in leg lengths or joint conditions, they will also be treated by the Physical and Rehabilitation Medicine and Orthopedic Surgery Departments.
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Surgery
If the lesion is small and only in one spot, it is completely removable with surgery. However, if the lesion is widespread or deep, surgery might cause severe bleeding and a complete removal is impossible. Most of venous malformations can hardly be eliminated with surgery and might recur or grow bigger.
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Sclerotherapy
While viewing an X-ray image, doctors insert a tiny needle into the lesion and inject a sclerosing agent to burn malformed vessels. Typically used agents include alcohol (ethanol), ethanolamine oleate, sodium tetradecyl sulfate, and polidocanol. Alcohol is widely used when the lesion is deep or broad, and it results in severe pain, thus requiring general anesthesia. Other agents, however, do not cause severe pain and do not need anesthesia, so they can be treated on an outpatient basis. After treatment, about 75% of the lesions disappear without relapses. It is impossible to remove all the lesions at once or to cure the malformations completely. The lesions should be treated step-by-step. Therefore, sclerotherapy is focused on the most severe part.
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Combination of surgery and sclerotherapy
If the lesion is completely removable, but severe bleeding is expected due to the size of the lesion, sclerotherapy is first applied to block vessels around and the surgery follows.
Prognosis
If the patients have no or only mile complications, sclerotherapy can reduce the size of the lesion by more than 75% and alleviate or stop pain.
Complications
In many cases, it makes bulges in the skin. 5 to 10% of the patients experience skin complications like skin damage, discoloration, or necrosis. If malformed vessels wrap around nerve fascicles, agent injections might cause numbness and motor abnormalities, which requires physical and rehabilitation treatment.
The chance of unpredictable anaphylactic reactions is very low between 1/2,000 and 1/1.000. However, when it happens, patients might suffer cardiac arrest or shock.