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What is a vascular anomaly?

A vascular anomaly results from a localized disturbance in the embryonic development of the blood vessel system. All types of blood vessels, as well as the lymphatic drainage system, can be involved. Two types of vascular anomalies can be differentiated based upon their natural behavior during life: the hemangiomas and the vascular malformations.

Hemangiomas

A hemangioma is mostly diagnosed immediately after birth, since these kinds of lesions are clearly visible at birth or shortly thereafter. Usually the lesions are superficial and mostly slightly elevated in height. They are irregularly colored with variations between red and pink. Their size is variable. Hemangiomas may be located at every part of the body. Interestingly, these lesions are seen more often in girls than in boys. Shortly after birth, these lesions show a period of extremely fast growth, sporadically resulting in ulceration or bleeding. The diagnosis can easily be made on the basis of these features. An additional examination needed to establish the depth of the hemangioma is seldom required. After a period of expansive growth, hemangiomas will shrink until the age of 5 or 6 year is reached. Finally, what is left is a scarred skin area, sometimes displaying a few remaining blood vessel irregularities.

Despite the fact that all these lesions regress spontaneously, treatment may indicated under the following circumstances:

1. Very large fast growing lesions can be treated by a pediatrician using mediation that inhibits growth (f.e. corticosteroids), attempting to prevent excessive ulceration resulting in extensive scarring after regression.
2. Small lesions may cause functional problems due to their location (f.e. the eye socket). These need to be treated surgically in order to prevent irreversible functional problems (f.e. a lazy eye).
3. Larger lesions will finally leave a large scarred skin zone, independent of their location. If these lesions can be removed surgically without major risks, they should be removed early. This will prevent that the child has to bear the burden of an abnormality, as the final scarred zone after regression would require a surgical correction anyway.

Whether a lesion is classified as belonging in one of the two of the last categories needs to be assessed by your plastic surgeon. An individual personal consultation is therefore inevitable.

Vascular malformations

Vascular malformations are mostly unnoticeable at birth; consequently they are usually diagnosed at later age. These malformations grow at the same speed as the body. Their incidence is about equal in boys and girls. In contrast to hemangiomas, these lesions can be embedded in deep tissue layers, such as muscle or bone. Due to the volume increase during growth, complaints may occur due to compression of other structures, such as nerves or the trachea. Hormonal variations, such as can be observed during pregnancy, may induce an increase in growth rate of these lesions.

Malformations are defined based upon the type of blood vessels involved. If the capillary system is involved, the lesion is called capillary malformation or port wine stain. The skin is completely involved but the deeper layers are seldom affected. In most cases treatment is possible with the aid of a pulsed dye laser.

A venous malformation marks an area with abnormal dilated venous vessels and impairing normal blood drainage; the skin overlying the lesion frequently displays bluish discolorations. Due to the dilatation, blood flow will be sludged, resulting in abnormal blood clotting and thrombus formation causing local pain. Pain may also be caused by the tissue sponge itself, since the tissue excess may compress adjacent nerves.

If the lymphatic drainage system is affected, a lymphatic tissue sponge is seen, which is called lymphatic malformation. The lesion is called lympho-venous malformation if the draining venous vessels are simultaneously affected. These kind of lesions mostly cause problems due to fluid accumulation and swelling.

The last type of malformation is an arterio-venous malformation; the main affected blood vessels are dilated arteries resulting in a major increase in blood flow rate through the tissue, causing visible or palpable abnormal pulsations. Due to the increased blood flow rate, drainage venous vessels may become secondarily dilated.

How is the diagnosis made?

Most of the vascular malformations can be distinguished from hemangiomas solely by its natural development and its physical appearance. Further investigations are indicated in case of complaints about pain, volume increase, bleeding, impaired function, or nerve problems. A MRI investigation is then needed to exactly locate the problem. In contrast to hemangiomas, malformations do not respond to conservative medical treatment.

Which treatment and when?

If complaints as described above are present, curative or palliative surgical treatment, dependent on the extent and location of the lesion might be offered. Sometimes also conservative measures, such as application of compressive stockings, may be useful to alleviate the complaints. Well-aligned lesions, especially located at the extremities, might be treated with curative resection. Infiltrative, badly aligned lesions can only be treated surgically if resection will not cause any major functional impairment. After incomplete resection, a lesion will always reoccur, mostly after several years. Lesions located in the head and neck region are mostly difficult to treat; sometimes sclerotherapy (injection of scarificative fluid into the lesion) may result in some improvement.

In conclusion, an individualized approach is required, due to the extreme diversity in extent, location, and nature of these lesions. Consequently, each patient needs to be seen and examined, before any statement concerning potential treatments of the anomaly can be made.