The Medical Necessity of Evaluation and Treatment of Port-Wine Stains

Last update: 9/4/1999



by Roy G. Geronemus, MD
   Robin Ashinoff, MD

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-dy 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, wehre 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;s 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.


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