The Naevus Flammeus is not a Hemangioma.

Hemangioma

Hemangioma

The naevus flammeus is a benign, congenital vascular malformation of small (“capillary”) vessels of the skin.

In about 5% of occurrences, it is a partial symptom of syndromes and is then associated with malformations of larger vessels or other tissues such as the bones or fatty tissue.

A naevus flammeus (“port-wine stain”) belongs to the benign, congenital vascular malformations and is present at the time of birth – contrary to hemangiomas, which only occur after the birth. At the time of the birth, it usually appears as a light to dark red spot of varying size in the area of the head, the arms, or the legs, in rarer cases in the area of the upper body. With increasing age, a port-wine stain only grows proportionally to the person’s growth, but clearly increases in color intensity (from light red to dark blue), whereas hemangiomas may very well grow in size.

In most cases, the skin thickens in the area of the naevus flammeus, especially when the lip region is affected (soft tissue hyperplasia). Occasionally, individual dark-blue to blackish lumps (tuberous transformation, eruptive angiomas) develop as well, which burst and may bleed severely. The appearance of the naevus flammeus, particularly in the facial area, influences the sense of self-worth and the coexistence with the personal milieu such as in kindergarten and later in school, professional training, the job, and of course in interpersonal relationships. Therefore, a treatment should already be sought at the age of infants or toddlers.

Besides direct changes, one must not forget the great psychological stress of the patient due to the appearance of the naevus flammeus, especially in the area of the face. The disfiguration by the port-wine stain often leads to severe disturbances in the development of the patient and his or her sense of self-worth. In early childhood already, patients with a port-wine stain in the facial area experience more frequent social exclusion, clearly influencing the development in school and professional training as well as the personal development, especially with regard to partnerships or marriages. Therefore, an early treatment (which, according to the explanations, ought not to be designated a “cosmetic” treatment) should be realized comprehensively by the cost carriers in order to prevent complications and exacerbations of the symptoms. However, it should be noted that there are also people who consider such a mark as being part of them, who do not feel discriminated by their environment, and who do not want to have it removed.

It is important that a naevus flammeus can be sign for the presence of other malformations of vessels, for example in the area of the cerebral membranes. Therefore, it is always recommended to contact a specialized center near one’s place of residence if a naevus flammeus exists.

Up to now, the development and the genetics of vascular malformations and thus of the naevus flammeus are unclear. In any case, it is not passed on genetically and, as most malformations, is to be viewed as a “whim of nature”.

In no case is a naevus flammeus triggered or influenced by the lifestyle of the mother such as, for example, by tight-fitting clothes, drinking of red wine, etc. (as is frequently claimed), or by events during the pregnancy such as bumps, scares, grief, etc., or during the birth such as medication, a suction cup, the forceps, etc. It seems that a genetic correlation can only be confirmed for particular, very rare syndromes, in which the naevus flammeus, together with other symptoms, is part of the phenotype.

The naevus flammeus already exists at the time of the birth, usually in its complete extent. It “grows” proportionally with the size increase of the child. The vessels do not penetrate in depth, i.e. into the fatty or muscle tissue. Also, the extent of the area of the naevus flammeus does not increase during the course of the patient’s life, i.e. a naevus flammeus in the area of the index finger, for example, does not expand onto the hand or the arm, but remains in the area of the index finger. What can change, though, is the color intensity. A light red spot during infancy may turn dark red by the time of puberty and then become dark red to bluish red later and form dark red to blackish lumps (tuberous transformation, eruptive angiomas), which may burst spontaneously or after an injury, bleeding repeatedly and severely. In certain areas such as, for example, the middle part of the face and the upper lip in particular, a clear doughy thickening of the skin (soft tissue hypertrophy) with detectable, dilated vessels in the area of the skin and the fatty tissue develops.

Due to the appearance and the course of the malformations in the first months of life, the diagnosis can be easily made by experienced physicians. Samples from the affected skin and thorough examinations are unnecessary since only an increase of the number of vessels in the skin can be proven in early childhood. It is only with increasing age that enlarged diameters of the vessels (ectatic vascular lumina) in the skin and an increasing number of red blood cells can be recognized in the vessels.

If a naevus flammeus occurs in the area of the face and follows a facial nerve (trigeminus I - III) in its extent, regular examinations of the eye pressure by an eye doctor for the exclusion of a glaucoma and other examinations such as the measurement of the brain waves (EEG) by a neurologist should be performed as a so-called Sturge-Weber Syndrome may be present.

It is important to differentiate the naevus flammeus from a hemangioma (“strawberry mark”), which may also appear as an extensive, reddish spot in the first weeks of life, but which quickly changes within the first 4-6 weeks. The hemangioma turns darker, expands at the edges (contrary to the port-wine stain), and becomes more elevated or knotty. Infants with an unclear reddish spot should be examined by an experienced doctor on a weekly basis in order to begin an early treatment in case of a hemangioma and to avoid unnecessary complications.

Nowadays, the treatment is performed exclusively with a flashlamp pumped dye laser, whose laser light bursts the vessels in a shock-like manner. The treatment can and must be repeated several times (up to 20 sessions). When performed with expertise, complications are very rare. Therefore, the first treatments may already be performed in infancy, i.e. beginning in the sixth month with the application of short-acting anesthesia. Other lasers (Nd:YAG lasers, KTP lasers, argon lasers, etc.) are only used for treatment in case of a special indication. Alternatively, the area of the naevus flammeus may be covered with camouflage or make-up after professional instruction by a cosmetician or a make-up artist. However, this does not prevent the further development of the naevus flammeus (soft tissue hypertrophy, tuberous transformation, bleeding)!

The first laser that was used successfully for the treatment of the port-wine stain was the argon laser. Until the 1980s, it was the “gold standard” in the laser treatment of this malformation and then replaced by the flashlamp pumped dye laser. While the argon laser welds the vessels through the application of heat, the FPDL causes a tearing or bursting of small vessels due to its short pulse lengths of 200 to 1,500 µs (1µs = 10-6 s).

The destroyed vessels are then removed by phagocytes (white blood cells) in subsequent weeks, followed by another treatment after 4-6 weeks at the earliest. The bursting of the vessels causes the release of red blood cells into the surrounding tissue. This leads to a bluish mark in the area treated (purpura), analogous, for example, to the blue spot after a blow to the eye. This blue discoloration regresses very quickly and without any problems within a few days.

This treatment has very few complications. Particularly the tendency for the formation of scars, which had been estimated at between 5-10% when argon lasers are used, is about 1,000 times smaller when the FPDL is used. Moreover, light red parts of the naevus flammeus, which could not be treated with the argon laser, can be successfully brightened with the FPDL. Thus, treatments with the aid of short-acting anesthesia are already possible in infancy. The treatment is slightly painful (burning, pinching), so that a anesthetizing cream (EMLA cream), pre-cooled air, cooling sprays, or ice are used effectively for the reduction of pain.

Other laser systems such as, for example, the copper vapor laser, KTP laser, and Nd:YAG laser are solely used for special indications such as, for example, knotty changes (tuberous transformations, eruptive angiomas), but should not be used for the treatment of the uncomplicated naevus flammeus due to the significantly higher complication rate.

Prior to the era of the laser therapy, many different treatment methods were tried. Besides excising the mark and subsequently covering the wound with skin grafts, X-ray irradiation, compounds with radioactive substances, and cold treatments (liquid nitrogen, carbon dioxide spray) were used in therapies. These treatment methods are only mentioned as historic therapies and are no longer important for the treatment of the naevus flammeus.

Any form of treatment of a congenital vascular malformation, whether through surgery or laser surgery, rare leads to a healing. Therefore, the objective of the treatment of a naevus flammeus is a lowering of the symptoms, i.e. the reddening, the swelling of the soft tissue, as well as of the bleeding, etc., while minimizing the complications of the treatment. Nowadays and in the future, this objective can be realized by experienced doctors through the use of different laser processes so that the prognosis of a naevus flammeus can overall be rated as good. Malignant changes in a naevus flammeus were only found after the application of X-rays and radioactive substances. To date, after about 40 years of laser treatments, there is no indication for similar malignant changes triggered by lasers.

The treatment with a dye laser, which may already begin in infancy, leads to a fast and long lasting brightening of the naevus flammeus at an early age. Nevertheless, individual treatments during childhood and the teenage years are necessary at a later stage in order to treat recurring vascular dilatations in the area of the naevus flammeus and to maintain the brightened condition. At that time, though, the child may develop normally, i.e. without any bullying and social exclusion. Furthermore, the risk of developing complications is clearly reduced.

There is still a controversial debate about the treatment of the naevus flammeus in infancy. Of course, a naevus flammeus may be treated with different laser techniques at any age. The crucial difference to a later treatment compared to an early one in infancy or early childhood consists in the unaffected development of the child in its social environment and the low tendency of its recurrence.

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