Port-wine Stain

Synonyms: PWS, capillary haemangioma, naevus flammeus, nevus flammeus, strawberry patch and strawberry birth-mark.

Most involve the head and the neck. On the face, 45% are restricted to just one of the three regions of the trigeminal nerve, with 55% crossing to more than one area. Those that do are often bilateral.

Natural history

There seems to be some conflict of opinion.
eMedicine states that:
'It remains present for life and has no tendency toward involution'.

NICE guidance on intralesional photocoagulation of subcutaneous congenital vascular disorders states:
'Often these abnormalities require no treatment, as they may spontaneously resolve or cause only mild cosmetic problems.'

Dermnet New Zealand takes more of the American line with:
'Some port wine stains may fade over time but most remain unchanged or may even deepen in colour.'

• In early life, the lesions are flat and usually clearly demarcated. The colour does not correlate with depth or diameter of capillaries. Blanching on pressure is variable. They are usually unilateral with a clear demarcation at the midline.
• The lesions may change from pink in infancy to red in early adulthood to deep purple during middle age.
• Nodular vascular lesions may develop, usually in adulthood.
• Pyogenic granulomas with bleeding may develop, even in childhood.
• Later in life, the vasculature dilates and the lesions may evolve into a raised, thickened plaque with a cobblestone contour. This may occur in up to 65% of facial lesions during adulthood.
• Lobulated capillary haemangiomas (pyogenic granulomas) may form, especially with intraoral lesions.

The diagnosis of a port wine stain or capillary haemangioma is usually clear but there may be concern that it is not an isolated lesion but part of a syndrome.

Glaucoma

There is an association between port wine stains involving the eyelids and glaucoma. Glaucoma may occur in about 10% of facial port wine stains but the figure rises to between a quarter and a half when both the ophthalmic and maxillary divisions are involved.

Sturge-Weber syndrome

PWS in association with ocular intracranial angiomas causes blindness, focal epilepsy, hemiplegia or mental retardation - Sturge-Weber syndrome.¹ The lesion is clearly unilateral and in the trigeminal area, usually in the ophthalmic division. There are ipsilateral capillary malformation on the leptomeninges and cerebral cortex. The condition may be bilateral.

Klippel-Trenaunay-Weber syndrome

This is also known as angio-osteo-hypertrophy syndrome.² It is association of a port-wine stain, varicose veins, and hypertrophy of a limb. The lower limbs are involved in 95% of patients, and it is unilateral in 85%. Most babies are asymptomatic at birth, but have problems later in childhood. Complications include:

• Varicose veins with venous thrombosis and pulmonary embolism
• Bleeding from varices, the rectum, or the bladder
• Skin ulceration
• Increased sweating overlying the lesion
• Leg circumference or length discrepancy with resultant scoliosis
• Oedema and recurrent infections

Parkes-Weber syndrome

This consists of arterio-venous malformation in addition to those of the Klippel-Trenaunay syndrome. AV fistulae are usually diffuse and ablation is difficult. Most children present in childhood with an enlarged, warm extremity. The prognosis is worse than with Klippel-Trenaunay syndrome.

Cobb syndrome

This includes cutaneomeningospinal angiomatosis. A lesion in the skin overlying the spine is associated with vascular malformations in the underlying spinal meninges. There may be pressure on the spinal cord or nerves, bone erosion, and subarachnoid haemorrhage.

Wyburn-Mason syndrome

This is also called unilateral retinocephalic syndrome and also Bonnet-Dechaume-Blanc syndrome. There are facial port wine stains with unilateral AV malformation of the retina and the intracranial optic nerves. Lesions may occur anywhere on the face and there may be facial hypertrophy or occasional involvement of the optic chiasm, the hypothalamus, the midbrain and the basal ganglia, with associated mental retardation or neurological features.

If Sturge Weber syndrome is suspected, MRI of the brain is required. In very early life, results may give false reassurance.³Photoacoustic imaging can be used to give information relating to the depth of the lesion and its vasculature.⁴

Refer as young as possible, usually around 1 year old, to a centre which has the laser and anaesthetic facilities. Some centres may treat earlier.⁵ The British Association of Dermatologists recommends early treatment:

• The tunable pulse dye laser (PDL) emits light at 585nm which is preferentially absorbed by haemoglobin.
• The length of the pulse of light (450 microseconds) is just long enough to heat small vessels but not the surrounding tissue.
• This results in destruction of the blood vessels without any scarring. The results are best in babies or infants.

Current research is looking at the added efficacy of PDL with topical imiquimod.⁶
Sometimes a PDL laser is used in conjunction with a KTP (potassium titanyl phosphate) laser.

• Treatment is painful and in small children requires a general anaesthetic, but small infants have smaller areas to be treated (before they have grown). Local anaesthetic is helpful.⁷
• The treatment is tedious, time consuming and expensive, and may not be universally available without charge on the NHS.
• Several treatments may be required, spaced at 2-3 monthly intervals; and postoperatively patients are usually advised to avoid the sun, avoid injury and use an emollient.

Cosmetic camouflage can be useful in hiding the area. It may be prescribed on an FP10.

Hyperpigmentation is usual in treated areas but this is transient. Permanent hypopigmentation may occur in treated areas. Pyogenic granuloma in treated areas has been described.⁸

Failure to treat can result in considerable psychological morbidity.⁹

The lesion is permanent during life and in some may become a major cosmetic disability. Laser treatment can produce good results.