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Q: What is port wine stain ?

A:  Port wine stain is a birth mark beneath the surface of the skin. It affects 1 in 3000 babies. It appears reddish to purplish, and can occur at any parts of the body. It is seen at birth and persists throughout the lifetime.

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Why is my baby born with port wine stain?

Port wine stain happens due to the formation of abnormal small blood vessels (capillaries) in the skin which occurs during fetus development. This is not a hereditary disease and it is not caused by anything related to the mother during pregnancy. No parents should feel guilty or responsible for these port wine stains.

Does port wine stain changes as my baby grows up?

Yes. When they are babies and young children, port wine stain is flat and pink in colour. As they age, it changes to purplish colour, gets thicker (picture B) and can form small lumps on the surface (picture A). Thus port wine stain should be treated promptly to avoid the natural progression.

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Is my child’s port wine stain associated with other problems or diseases?

Most port wine stains have no associated problems or diseases.

Children with port wine stain near or around the eyes may be associated with eye disease named “glaucoma”. Thus they should be referred to the eye specialist for assessment. Early detection and treatment is vital as it is potentially serious if missed.

Port wine stain over the head and/or forehead may rarely be associated with deeper blood vessels malformation of the brain known as Sturge Weber syndrome. MRI brain is required to obtain diagnosis.  These children may experience seizures that may be difficult to control with medication. They should be referred to paediatric neurologist for further management.

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Extensive port wine stains over the limbs are associated with “Klippel Trenaunay Syndrome”.  This involves abnormalities of venous and/or lymphatics vessels deeper in the limbs and causes enlargement in that limb(s). These children should be monitored and followed up by a paediatric dermatologist.

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What are the psychological effects?

It can cause great misery in children with port wine stain, particularly when it affects the face. This is usually more prominent in the school going age group as they may be teased and bullied by other children in school. This could result in low self-esteem, depression and school avoidance. They may need to be referred to a child psychologist or child psychiatrist for counselling and further therapy.

What are the available treatment modalities?

Pulsed dye laser is the most effective therapy for treatment of port wine stain.

How does pulsed dye laser therapy works?                                                  

Lasers produce powerful beam of light which targets specific tissues within the body. The yellow beam of the pulsed dye laser is absorbed by the haemoglobin the red blood cells within the port wine stain, resulting in heat damage to the targeted blood vessels. The precision of the beam and short exposure time (less than a second) means that the surrounding tissues are hardly affected while treating the blood vessels in the port wine stain. Thus the risk of scarring is negligible.

Which patients benefit from this form of treatment?

Majority of port wine stains responds well to treatment with pulsed dye laser. However the response may vary from the colour becoming lighter and less obvious to total clearance in some patients. Occasionally, there is no response. Experience to date has shown that pulsed dye laser is safe and effective in treating port wine stains, with some evidence that younger children (3 months to 6 years) tend to respond better than adults. This may be related to the pink colour, relatively smaller size of the birthmark, the depth within the skin and the smaller blood vessels.

What does treatment entails?

Laser therapy is done as a “day care” procedure. Most children require sedation, although older children may undergo the procedure without sedation. The number of treatment sessions needed depends on the size of port wine stain. Preference is given to birthmark on the face.

What happens after treatment?

Immediately after treatment, the laser areas appear like small bruises and the skin can be mildly swollen. This will subside within 1-2 weeks and the treated areas will fade in colour over the next two months. Thus treatment is scheduled about 2-3 months apart. The amount of improvement varies and several treatments are usually needed.

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Pictures showing port wine stain before and after pulsed dye laser treatment.

( Written by Dr Aileen Wee )

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