Port-wine stains (Naevi flammei)

1. Port-wine stains (Naevi flammei) are congenital malformations of blood vessels of the upper layers of the skin. Currently, it is suspected that in those areas blood vessels are dilated as muscles of these blood vessels have not formed properly. Thus, the skin appears red in these areas. About 80 % of port-wine stains are localized in the face, and even though they are not dangerous or malignant, they may be associated with other malformations of deeper blood vessels. Therefore, in case of naevi flammei in the occular area, examination of the eyes and especially pressure measurements are necessary. Also, port-wine stains of the face (forehead-area) can be associated with more complex syndroms (e.g. Sturge-Weber-Syndrome) and may be associated with epileptic seizures. In those cases, additional pediatric and/or neurologic assessment is necessary. Port-wine stains do not regress over the years but have a tendency to get darker and even form small nodules with age. These nodules are benign dilatations of the blood vessels.
   A special form of port-wine stain in the area of the middle face is the so-called "storch bite". These visible erythemas in the central part of the face, especially forehead, nose and upper lip, tend to regress within the first few years of life, so that a treatment is not necessary.
2. Naevi flammei have to be distinguished from so-called haemangiomas which are benign vascular growths that occur after birth and often show fast progression both in growth of size and depth. Only in the very beginning, in their macular growth phase, may they be mistaken for naevi flammeii. As soon as a plaque or a small nodule appears, the diagnosis of haemangioma should be established so potential treatment can be initiated to prevent further enlargement.  
   
3. The 'gold standard' of therapy for naevi flammei nowadays is the pulsed dye-laser therapy. This treatment was established in 1982 in Germany and is available as standard therapy all over Germany since 1994. Treatment comprises application of this laser with a yellow wavelength to the entire area of the port-wine stain. The high-energetic light is absorbed by erythrocytes (red blood cells) which heat up and transmit the heat to the blood vessel wall which subsequently ruptures. Therefore, immediately after therapy with the pulsed dye-laser the skin appears bruised. Bruising, however, completely regresses in time. Treatment with the pulsed dye-laser is extremely safe and except for bruising, almost free of unwanted side effects. Typically, 8 - 10 treatments, at least two months apart, need to be carried out to achieve sufficient blanching/regression of the port-wine stain. Port-wine stains of the face tend to respond better than those on the extremities (arms or legs). 
   
4. Current practice: Should a child be diagnosed with a port-wine stain at birth, it is prudent to first wait to assess whether this region remains a port-wine stain or whether it represents an initial haemangioma. Should it prove to be a port-wine stain,certain casesmay require additional pediatric exams to exclude more complex syndromes with malformations or associated diseases. In our department, the treatment is initiated at the age of 1 year and the first treatment is carried out under general anaesthesia with the child being supervised in our day-care-clinic for the day. We choose to carry out therapy under general anaesthesia, as treatment for infants is especially painful. We start treatment at 1 year of age since some port-wine stains may lighten within the first year. After the first treatment, repetitive laser-therapy is carried out at intervals of at least 2 - 3 months, sometimes even longer, until satisfactory ligthening of the port-wine stain is achieved. By the time the child reaches its third year (an age at which children typically will go to kindergarten), the port-wine stain should be hardly visible to prevent psychosocial stigmatisation of the child. Even though this may not be possible in all cases, satisfactory treatment results of the pulsed dye-laser-therapy can be achieved in about 70 % of the cases.