Vascular anomalies

Many different and confusing terms have been used to describe vascular lesions. Mulliken and Glowacki’s classifica­tion based on endothelial characteristics, helps in diagnosis, planning, management and predicting the course of these lesions. This classification has three major categories:

haernangiomas: show endothelial hyperplasia;

malformations: have normal endothelial turnover;

ectasias: have normal endothelial turnover, but also have vascular dilatation.

Haemangioma (Fig. 13.25)

These are the most common turnouts of infancy and have a typical history. The baby is normal at birth and at the age of 1—3 weeks is noted to have a red mark. This increases rapidly for some weeks or even up to 3 months, until the typical ‘strawberry’ or raspberry-like swelling is present. Clinically, the sign of emptying may be demonstrable. The lesion is composed of immature vasoformative tissue. The subcutaneous tissue as well as the skin is often involved, and in severe cases the muscles may be affected. From the age of 3 months to 1 year the haemangioma grows with the child, and then it ceases to grow Eventually the colour fades and flattening occurs so that at the age of 9 years 90 per cent demonstrate complete involution. Bleeding is rarely a prob­lem and responds to local pressure. Ulceration occurs in about 5 per cent of the cases, particularly in those areas that are commonly abraded, e.g. lips and perineum. Wound care will prevent infection and promote re-epithelialisation. Pulsed dye laser therapy has also been used to promote the involution of the superficial vessels. Visual obstruction from periorbital haemangiomas (Fig. 13.26) can result in amblyopia or anismetropia if total visual obstruction occurs for more than a week in the first year. Nasopharyngeal obstruction may cause obstruction in infants who are obligate nose breathers. Auditory canal obstruction is seen occa­sionally with lesions affecting the parotid gland. Intervention should be considered in bilateral conditions which last beyond 1 year of age.

Kasabach—Merritt syndrome occurs owing to platelet trap­ping within the lesions. Congestive cardiac failure with multiple cutaneous haemangiomas or large visceral haeman­giomas results from dilated vessels and arteriovenous shunt­ing. Skeletal distortion and cosmetic deformities in the form of telangectasias, hypopigmentation and contour abnor­malities have been observed. The final result (Fig. 13.27) is better if natural involution is allowed to occur rather than if an attempt is made to hasten regression by any operative or physical methods. Many interventions are possible and should be considered only in special circumstances. Intra­lesional and oral steroids have been demonstrated to produce involution of these lesions. Surgical debulking and laser photocoagulations — both superficially and intralesionally —have also been used. Radiation therapy carries a high risk of disturbance of growth and is dangerous.

  Vascular malformations

These are structural and morphological anomalies due to faulty embryological morphogenesis. The lesions are present at birth, grow commensurate with the child and do nor regress. They can lead to underlying soft tissue or bony hypertrophy, nodular development and discoloration as a consequence of blood vessel ectasia with age. The natural history of these lesions is determined by their haemodynamic and Iymphodynamic characteristics.

  High-flow lesions include arterial malformations and arteriovenous malformations (arterial plexiform angiomas, cirsoid aneurysm).

Low-flow lesions include lymphatic (LM) venous (VM) and capillary (CM — port-wine stain). Frequently these lesions combine arterial, venous and lymphatic elements.

Port-wine stains

Port-wine stains (Fig. 13.28) are intradermal capillary mal­formations that change very little throughout life, although the colour may alter a little and they may become nodular in some areas. Treatment is for reason of appearance. Treatment of choice for these lesions is the use of the pulsed tunable dye laser.

Glomus tumours

 Glomus tumours, frequently in the subungal regions, repre­sent arteriovenous anastomoses (Suquet—Hoyer canals). They are frequently removed because of the pain and to obtain a diagnosis.

Ectasias

These represent acquired lesions such as telangectasias (spider naevus). Lesions on the lower extremities are venular (thread veins); however, facial telangiectasia is mostly due to arteriolar vasodilatation. Lower extremity lesions respond to either microsclerotherapy or laser photocoagulation. Those on the face can be treated by photothermolysis using a pulsed dye laser.

Miscellaneous vascular lesions

Pyogenic granuloma

Pyogenic granulomas (Fig. 13.29) are common acquired, reactive, proliferative vascular lesions of the skin and mucous membranes, arising after minor trauma. The lesion looks like a haemangioma, but has a typical natural history. Usually single, it consists of a red, soft or moderately firm, more or less pedunculated nodule that grows rapidly to a size varying from 0.5 to 2.0 cm in diameter. Most commonly it is seen on the face, the fingers and toes. Treatment is excision.

Macular stains

Macular stains (salmon patch, also called ‘stork bites’; not to be confused with the ‘patch’ in syphilis) are present at birth over the forehead in the midline, and over the occipit. They disappear by the age of 1 year and so may represent a physiological process rather than a pathological lesion.