Congenital vascular lesions

Congenital vascular lesions are not uncommon, and may worry a mother, so you should know how to advise her. There is little you can do for most of them except, to watch a child carefully, and refer him when necessary.

A capillary haemangioma, or ''strawberry naevus' (D, in Fig. 28-11) is a skin lesion, and is commonest on the face. It is bright red, and a little raised, sometimes quite markedly. It may occur on the mucous membranes and may bleed severely. A baby is usually normal at birth; the lesions appear one to four weeks later, and enlarge for a few months. Rapid growth is unusual after six months, but slow growth is common up to a year. If you leave them alone, 95% will resolve spontaneously. The first sign that this is happening is the appearance of a lighter, flatter area. Resolution may be precipitated by trauma, but this usually causes only minor scarring. Advise his mother that his lesion will probably disappear slowly. Warn her not to allow traditional healers to scarify the lesion, which may cause bleeding, infection, and worse scarring. If resolution is slow, or she presses you, refer him.

A port-wine stain (B, in Fig. 28-11) consists of cavernous channels, and usually occurs on the face or neck, but is not uncommon on the trunk. It is usually present at birth and does not progress, but it may be quite extensive. The texture of his skin is normal, and is not usually thickened; occasionally there is some hypertrophy and irregularity.

If a lesion is particularly ugly, as in a light skin, cosmetic creams may help. Otherwise, there is no treatment. Reassure his mother that his lesion will not enlarge.

Lesions on the face, in the area of distribution of the ophthalmic and maxillary branches of the fifth nerve, may be associated with vascular abnormalities of the cerebral cortex (Sturge[nd]Weber syndrome), and present with seizures. Glaucoma of the ipsilateral eye sometimes occurs, and is treatable (24.6). Refer him to a neurosurgeon.

A capillary cavernous haemangioma consists of abnormal capillaries, arteries, and veins, and is partly compressible. It is usually present at birth, and commonly occurs on a child's face, axillae, or neck, where it may extend into his mediastinum. It may occasionally resolve spontaneously over several years (unusual), or it may enlarge rapidly.

Watch him carefully, and at each visit use a measuring tape to record the exact size of his lesion in two dimensions at right angles. If it does enlarge, refer him[md]surgery is likely to be difficult, and bleeding severe.

A lymphangioma is a congenital capillary cavernous lesion, which is usually present at birth, in the same sites, commonly the neck, as a haemangioma. Some are well circumscribed, but others merge with the surrounding tissues. Some become slowly smaller, others enlarge, sometimes quite quickly, and cause pressure effects in the chest. Most eventually need surgery, which can be difficult and may not be complete.

Watch him and measure his lesion regularly. Refer him if it enlarges, especially if it enlarges rapidly.

A cystic hygroma (E, and F, in Fig. 28-11) is a fluctuant, lobulated, translucent mass of abnormal lymphatic vessels, which presents as a soft, diffuse swelling in the posterior triangle of the neck, or occasionally the axilla. It appears early, and may be present at birth. The mass is not attached to the skin, and does not move on the deep tissues. A cystic hygroma usually causes no serious problems, and usually enlarges slowly. Occasionally, it advances rapidly and causes pressure symptoms at the root of the neck, or, rarely, it may regress spontaneously. The main differential diagnoses are: (1) A lymphangioma. (2) A branchial cyst; this is unilocular and low in the neck, along the anterior border of the sternomastoid; aspiration yields a thicker opalescent fluid, instead of the thin, clear, watery fluid from a hygroma. (3) A capillary cavernous haemangioma.

Surgery can be difficult, because a hygroma may penetrate deeply, or have lymphangiomatous extensions. Watch him carefully, and unless it is clearly regressing, refer him. If it is very large, it should be operated on earlier. If it is not so large, removal will be easier when he is more than a year old.