New lasers and improved laser delivery systems have allowed
for the safe and effective treatment of port-wine stains in
patients of all ages. The satisfactory results obtained by
laser treatment have increased the number of patients seeking
consultation regarding their birthmarks. It is imperative
that physicians recognize the various medical syndromes and
problems associated with port-wine stains. A review of 415
patients with facial port-wine stains has revealed hypertrophy
and/or nodularity in 65% of patients by the fifth decade of
life, which increases significantly the morbidity of these
lesions. It is believed that laser treatment will minimize
the medical and psychologic complications that result from
the natural evolution of port-wine stains. J Dermatol Surg
Oncol 1991; 17:76-79. Recent developments in the treatment
of port-wine stains have allowed physicians to re-evaluate
the opportunity and necessity for treatment of these lesions.
Port-wine stains occur in 0.03% of the general population
and are noted to occur most commonly on the face over the
areas of the first and second trigeminal nerves. In the past
several years, the development of the flashlamp-pumped pulsed-dye
laser, continuous-wave tunable-dye yellow-light laser, copper
vapor laser and the use of other continuous-wave lasers in
conjunction with special scanning devices, have all led to
improved results in the treatment of these lesions. One of
these lasers (the flashlamp-pumped pulsed-dye laser) has even
allowed for the treatment of infants and young children. Thus,
physicians and patients afflicted with this congenital anomaly
are faced with the decision of whether or not to seek treatment
with any of these lasers, where previously treatment may have
been unsatisfactory or contraindicated. The superior results
now offered by lasers in the treatment of port-wine stains
have also allowed for the influx of patients with this congenital
malformation to seek medical attention. Thus it is important
that those physicians consulting these patients recognize
the associated medical problems that may be found with port-wine
stains. One important example is the Sturge-Weber syndrome,
which is commonly found among those patients with port-wine
stains involving the distribution of the first branch of the
trigeminal nerve. The Sturge-Weber syndrome has a high incidence
of congenital glaucoma of the ipsilateral eye, calcification
and vascular anomalies of the brain, associated seizure disorders,
and in some cases, mental redardation. Ophthalmologic and
neurologic evaluation in these patients are recommended early
in life. Other congenital syndromes associated with port-wine
stains include the Klippel-Trenaunay-Weber syndrom with the
associated hypertrophy of skeletal tissue, as well as the
Cobb syndrome, which may be a sign of underlying arteriovenous
malformation of the spinal cord. It is also not uncommon to
see a port-wine stain overlying an arteriovenous malformation,
arterial malformation, or venous malformation, therefore requiring
the physician to look beyond the skin for any underlying problem.
Port-win stains begin as macular lesions and become progressively
ectatic over time. This progressive ectasia can result in
significant darkening of the lesion as well as the development
of nodularity and occasional tumors. A review of 415 patients
presenting at the laser section of the Skin and Cancer Unit
of New York University Medical Center has revealed that approximately
two-thirds of all patients with port-wine stains develop nodularity
or hypertrophy in the fifth decade of life. These changes
are gradual in their development, with the mean age of hypertrophy
noted to be 37 years. The progression of the port-wine stain
from its macular stage to the hypertrophic stage may take
place at any point in the development of an individual's lifetime;
however, the most dramatic changes are noted as the patient
has become older. Associated with this nodularity and hypertrophy
is the risk of spontaneous bleeding and hemorrhaging upon
injury to the affected area. Although the exact risks have
not been established in the literature, they are clearly associated
with the degree of hypertrophy and the location of the lesion.
Men with port-wine stains on the face in the bearded area
run significant risks of excessive bleeding upon shaving,
as would a woman with a port-wine stain in the hair-bearing
area where epilation is desired. The development of hypertrophy
or nodularity in a periorbital area may inhibit the visual
field and, similarly, these changes around the nose or mouth
may also interfere with breathing or eating. It has become
clear that treatment of a port-wine stain in its macular stage
will prevent the development of the hypertrophic component
of the lesion. Postoperative biopsies upon laser treatment
of a port-wine stain reveal that the existing blood vessels
are smaller and fewer in number compared with pretreatment
biopsies. Thus, the opportunity for the progression of these
lesions to a more ectatic state is less likely to occur. Not
uncommonly, the development of pyogenic granulomas in patients
with port-wine stains can occur and these pyogenic granulomas
have the potential to also bleed spontaneously. In our experience,
the incidence of pyogenic granulomas is significantly lower
in those patients who have been treated with laser therapy.
The psychological problems associated with a congenital birthmark
of this type may have short and long-term implications for
the patient. Detailed studies have demonstrated lower self-esteem
and problems with social interaction for both the patient
and family alike. Successful treatment of a port-wine stain
with any modality helps minimize psychological problems that
occur as a result of the presence of the port-wine stain.
Although the treatment of these lesions has imporved dramatically
over the past several years with the development of newer
techniques and lasers, many patients are denied the opportunity
for treatment of these lesions by the unfortuante classification
of this treatment as a cosmetic procedure. It has been demonstrated
clearly that these safe treatments can minimize the chance
of nodularity, spontaneous bleeding, and the development of
pyogenic granulomas. The psychologic benefits of removing
port-wine stains in these patients are of considerable value
to their mental health and may, in fact, obviate the need
for psychological counseling at a later date. It is incumbent
upon those physicians who evaluate patients with port-wine
stains and insurance carriers who evaluate their claims to
realize the medical benefits in the treatment of these lesions.
Failure to recognize the treatment of port-wine stains as
a medical necessity may, in fact, be harmful to the medical
and psychologic well-being of the patient.