Biomedical Engineering Reference
In-Depth Information
may be more extensive) occurring in association with ipsilat-
eral central nervous system vascular malformation (leptomen-
ingeal angiomatosis) and vascular malformation of the choroid
of the eye associated with glaucoma. The risk for SWS is deter-
mined by the distribution of the facial PWS, a fact that has
been somewhat complicated by authors' defi nitions and ana-
tomic variations in the distribution of V1 and V2 of the upper
and lower eyelids (the so-called watershed areas). Overall, the
risk of SWS with V1 PWS involving the upper eyelid approxi-
mates 10%; that risk increases to about 25% when either
bilateral stains are present or when multiple dermatomes
(V1, V2, V3) are involved (67,68). Because SWS can cause sig-
nifi cant medical and ophthalmologic problems ranging from
potential visual loss and hypothalamic-pituitary dysfunction
(69) to seizures and mental retardation, the possibility of SWS
should be considered in any infant with a PWS that includes
the V1 distribution.
Other congenital syndromes include the Klippel-Trenaunay
and Parkes-Weber syndromes (PWS with associated varicose
vein and hypertrophy of skeletal tissue with or without AVMs,
respectively) and Cobb syndrome (PWS with underlying AVM
of the spinal cord). A PWS may be associated with an underly-
ing venous malformation or occasionally an AVM, so the exis-
tence of these associated lesions should not cause confusion in
diagnosis.
interactions with others that should be required reading for all
physicians.
Multiple studies have demonstrated an improvement in psy-
chological health after successful treatment of PWS (72-76).
We have noted a change in personal perceptions dramatically
in our treated patients. On a personal level, a 12-year-old girl
fi rst sought treatment in our practice for a PWS on the right
cheek. Initially, although of above-average intelligence, she was
introverted and interacted sparingly with her classmates. After
three treatment sessions, resulting in 75% clearance, she began
dating, joined the school band, and excelled academically.
These are the observations so gratifying to the physician and
medical staff involved in laser treatment.
Despite the psychological and medical complications of
PWS, insurance coverage in the United States for laser treat-
ment of PWS varies from state to state. A study by McClean
and Hanke (64) of insurance reimbursement in 18 states
found that determination for approval of treatment was made
on a case-by-case basis, with the majority requiring preautho-
rization. The percentage of requests approved for coverage
varied from 50% to 100% without apparent reason. Some
insurance carriers would only approve treatment if functional
impairment existed and some only if the patient was less than
1 year of age. Only Minnesota has a law requiring all health
insurance to cover the elimination or maximum feasible treat-
ment of PWS.
Various support groups are available for children with con-
genital vascular abnormalities and their families. The Sturge-
Weber Foundation (http://www.sturge-weber.org; Mt. Freedom,
New Jersey, USA) publishes an excellent booklet for children
that clearly explains the syndrome as well as multiple treatment
options. The Klippel-Trenaunay Support Group (Edina, USA)
publishes a useful quarterly newsletter and holds support group
and educational meetings for the public. The National Congen-
ital Port-Wine Stain Foundation (New York, USA) also provides
information and support to patients and families of children
with PWSs; www.birthmarks.com is an excellent website for
patient information. Patients and parents should be encouraged
to use these resources.
psychologic impact
In addition to producing physical deformity, a PWS carries a
defi nite risk for lasting detrimental effects on a child's psycho-
logic, social, interpersonal, and cognitive development. The
exact age when psychosocial development is affected is
speculative.
A common misperception regarding PWSs in adults is that
if one has reached adulthood without psychologic damage
from a cosmetic deformity, one does not require treatment.
Adult PWS are known to impact social relationships. A ques-
tionnaire given to 186 patients who sought treatment for
their PWS found that 29% thought the PWS was disadvanta-
geous in forming interpersonal relationships with members
of the opposite sex (70). A half rated their PWS as unattract-
ive, although only 33% thought that other people perceived
their PWS to be moderately to very unattractive. The true
incidence of psychologic problems from PWS may be higher
or lower because this study was obviously skewed to patients
who were actively seeking treatment. Nevertheless, the sur-
vey does show that a signifi cant number of adults with PWS
would benefi t psychologically from treatment.
Psychologic diffi culties in interactions with others occur as
often in adults as in children. The Soviet news agency, Tass,
for example, famously airbrushed out former president
Mikail Gorbachev's PWS from published photographs until
perestroika
as a means of hiding his “cosmetic handicap.” Simi-
larly, an experiment during the 1989 annual meeting of the
American Academy of Dermatology dramatically demon-
strates this point. A model had a PWS painted on her face and
then feigned an illness that led to unconsciousness on a public
bus. Not one passenger came to her aid. When the same model
feigned the same illness on a bus without the facial PWS,
all those present eagerly came to her aid. Pena Clementina
Masclarelli (71), a senior occupational therapist who also
has an extensive PWS, wrote a poignant chapter about her
childhood port-wine stains
Because of the long-term physical and psychosocial comor-
bidities associated with PWSs, many specialists advocate for
treatment of these lesions to begin as soon as possible after
birth. Support for intervention at the earliest possible age is
based on the fact that the lesions themselves are physically
smaller in size and are comprised of vessels that are smaller
in diameter and more superfi cial. Thus, early treatment may
improve the responsiveness, decrease the number of treat-
ments, and reduce the likelihood of permanent adverse
sequelae (77-79).
Many therapeutic methods have been attempted to treat PWS.
These include surgery (excision, grafts, fl aps, dermabrasion)
(80-82), radium implants, X-ray therapy (83), cryosurgery (84),
electrocautery, sclerotherapy (85), tattooing (86), and cosmetic
camoufl age (Fig. 2.20). Vascular-targeted photodynamic ther-
apy has also emerged as a potential option for patients with
darker skin types (Fitzpatrick V) or for those patients with nod-
ular lesions, with long-term studies in the Chinese population
demonstrating 50% clearance in about 30% of patients receiv-
ing, on average, 2.6-8.2 treatment sessions (87). Many of these
