Samuel M. Lam, M.D* and Edwin F. Williams, III, M.D.**
*Lam Facial Plastic Surgery Center,
Plano, Texas
**Clinical Associate Professor, Division of Otolaryngology, Department
of Surgery,
Albany Medical College,
Albany, New York
Chief of Division,
Facial Plastic & Reconstructive Surgery,
Albany Medical College,
Albany, New York
Abstract
Port-wine stains represent the most common type of
vascular malformation. With the advent of pulse-dye laser therapy,
treatment of these lesions has become achievable with minimal prospect
of adverse scarring. Although many scientific articles have heralded
the favorable outcomes in patients who have undergone pulse-dye
laser treatments, few papers address the practical considerations
(preoperative, intraoperative, and postoperative) that must be followed
in order to ensure success. This paper presents the ten-year experience
of the senior author (EFW) in the management of port-wine malformations
and the clinical insights that have been gleaned from that extensive
experience. Introductory remarks are made as to the nature of port-wine
stains, related syndromes, and the evolution of treatment; and practical
management guidelines are then discussed.
Introduction
Port-wine stains (PWS), or more
correctly, capillary vascular malformations, represent the most
common type of vascular malformation. They occur in 3 of 1000 births,
arise predominantly in the head and neck region (80%), and exhibit
an equal sex distribution.1 PWS manifests initially
as a flat lesion with a red to pink hue which may lighten during
the first year but then tends to darken throughout life turning
a deeper shade of red or blue and may even become thicker or more
nodular as the individual matures.2 Two related syndromes
have been linked with PWS: Sturge-Weber (SWS) and Klippel-Trenaunay
(KTS).
Sturge-Weber, or encephalotrigeminal angiomatosis, refers to a
PWS that is distributed in the first trigeminal division (V1),
with or without V2 or V3 involvement, and
with central nervous system (CNS) abnormalities. CNS defects include
cerebral atrophy, leptomeningeal angiomas, and cortical calcifications
that may lead to seizures, mental retardation and hemiparesis.
Magnetic resonance imaging (MRI) should be conducted to screen the
high-risk infant. In addition, ocular examinations should be undertaken
to determine whether glaucoma is present with the syndrome. Klippel-Trenaunay
syndrome, or angio-osteohypertrophy, is characterized by a PWS that
usually involves a unilateral, lower extremity marked by hypertrophy,
varicose veins, lymphedema and phleboliths. 3
Prior to the introduction of laser therapy, the only
method of treatment for PWS was simply cosmetic camouflage. Initially,
the argon laser showed promise in the treatment of vascular diseases,
but the incidence of scarring and the advent of the pulse-dye laser
have largely relegated the argon to historical interest. Now with
the pulse-dye laser that selectively targets the vascular chromophore
in PWS, patients have found a new hope in minimizing their deformity
with little morbidity. Numerous studies have documented the proven
reliability of the pulse-dye laser in treating PWS.4,5
Some studies have investigated the role of a 532-nm KTP laser in
the treatment of resistant PWS lesions or of mature PWS lesions
that manifest nodularity and found clinical efficacy6,
but scarring has been reported with this laser type.6,7
Intense pulsed light has also proven to be highly effective and
safe for the treatment of PWS according to one study.8
However, the pulse-dye laser remains the proven gold standard for
treatment of PWS.
Recent studies have emphasized the psychological aspect
as much as the efficacy of treatment.9-12 Most studies
have demonstrated the ostracizing effects of PWS and the benefit
of intervention to the psychological welfare of the afflicted individual.
One small study contended that the patients in the series (n=9)
revealed no subjective benefit to treatment despite objective physical
improvement.10 As the child matures, he or she not
only confronts the psychosocial trauma of bearing the unsightly
mark but also may develop a lesion that is more difficult to treat
as age and hormonal factors darken and thicken the PWS. Proper
counseling and early treatment should be initiated to avoid these
adverse outcomes.
Preoperative Considerations
The first step in preoperative assessment is a thorough
history and physical examination. The detailed history should address
pertinent birth history, change in color or texture of the lesion,
prior workup for relevant syndromes (Sturge-Weber or Klippel-Trenaunay),
psychological effects of bearing the disfigurement, previous treatments,
and standard inquiries into the past medical, surgical, social,
and family history. More specifically, the patient should be asked
if any alteration in color or texture of the lesion occurred with
puberty, pregnancy (or other hormonal factors), prior trauma, or
simply due to gradual maturation of the lesion.
If the lesion involves the V1 distribution
with or without V2 or V3 involvement, we recommend
an appropriate evaluation for possible Sturge-Weber syndrome. However,
an isolated V1 manifestation that does not encroach on
either eyelid is rarely indicative of SWS, and we do not compel
patients to seek further workup. In fact, the extent of eyelid
involvement (i.e., only upper versus both upper- and lower-eyelid
extension) may reflect the likelihood that glaucoma may be present
(personal observation & communication with other specialists).
As part of the workup for SWS, an MRI scan should be performed after
the first 6 months of life, as the abnormal cerebral vascular findings
usually are not radiographically apparent prior to this time. Any
abnormal findings would be conclusive of SWS and would warrant medical
prophylaxis for seizures. We refer these individuals to their pediatrician
for further counseling and management of this condition. As mentioned,
part of SWS also involves the potential for the development of glaucoma
in the ipsilateral eye. At the time of diagnosis of the PWS, we
refer appropriate candidates to an ophthalmologist for evaluation
and further care. Furthermore, patients may only have ocular and
cutaneous manifestations and warrant treatment for both conditions
but not constitute SWS for lack of cerebral findings. After two
years of age, if the child with V1 PWS has not exhibited
any central manifestations of SWS, namely seizures, hemiparesis,
etc., then the likelihood for any syndromic abnormality is rare.
However, risk of glaucoma is still present and should be assessed.
All of these ocular and cerebral examinations should be carefully
documented in the patient’s medical record.
Unlike Sturge-Weber syndrome, Klippel-Trenaunay syndrome
does not carry commensurately grave morbidity. If a child with
PWS of the trunk or limbs, particularly involving the lower extremities,
presents with pain in the affected limb or limb disparity (of length
or circumference vis-à-vis the contralateral extremity) then the
diagnosis of KTS may be entertained. KTS extends beyond the superficial
cutaneous dimensions of typical PWS to involve the deeper tissues;
and this extension may be readily apparent on MRI scanning. If
the underlying soft-tissue appears to be involved, then pressure
application with support stockings may alleviate some discomfort
and retard the progress of vascular expansion. Also, deep extension
of this type of PWS is not readily amenable to pulse-dye therapy
and may be more properly addressed with surgical excision with or
without preoperative tissue expansion, if conservative measures,
e.g., pulse-dye laser and pressure application, fail.
A physical examination should address the color, texture
(presence or absence of nodularity), and extent of the lesion in
addition to the formal inspection of the entire body per routine.
Photographic documentation is critical to assess clinical improvement,
document findings, secure insurance approval, and promote scientific
communication. If the child is mature enough to cooperate, we attempt
to obtain a full set of standardized photographs (frontal, oblique,
lateral, and close-up frontal) prior to any procedure. If the lesion
appears in the head and neck region, we consider this appropriate
for submission to the insurance carrier for compensation. However,
if the PWS is situated elsewhere, particularly the trunk or lower
extremities, we believe that such a location would be more properly
deemed cosmetic in nature (unless an underlying syndrome such as
KTS may be giving rise to limb disparity or concomitant pain).
Also, PWS that affects the trunk or extremity may be more refractory
to treatment and has a proclivity to recur. For these reasons,
we discourage treatment of these types of lesions; and, therefore,
we also feel an ethical responsibility not to submit uncomplicated
trunk or limb PWS lesions to the insurance carrier for compensation.
Part of the preoperative consultation requires education
of the patient about the natural course of this disease and the need
for repeated treatments to effect an improvement. 
The
patient should fully understand that the PWS is a progressive disorder
and may become darker and more nodular in nature if left untreated.
Also it should be clearly stated that the PWS cannot be entirely eradicated
but only lightened and perhaps flattened by pulse-dye laser therapy.
Despite treatments, the lesion may continue to progress but usually
at a considerably reduced rate. Studies have shown that 50% of PWS
tend to return over time to their initial pretreatment appearance, and
this unfortunate tendency should be honestly communicated to the patient.
13 On the other hand, some scholars contend that early and aggressive
laser therapy may fully arrest, and reverse, the evolution of PWS.
We
advocate the first treatment session to begin no earlier than 6 months
of age to ensure that the infant has the fortitude to undergo anesthesia
safely (Figure 3). In addition, we tend to favor earlier laser
therapy, as mature lesions may be more advanced or recalcitrant to laser
intervention. However, we tailor our treatment to patients’ desire
and motivation. Further, we inform patients that the degree of response
after the first laser-treatment session may prognosticate the likelihood
of the lesion to respond equally well, or poorly, after future sessions.
Usually, we outline a conservative plan for 3 to 5 treatment sessions
separated by a minimum of 6-week intervals as needed to achieve a desired
endpoint. If that objective is not met after the projected treatment
timeframe, then further sessions are designed accordingly.
Intraoperative Considerations
Prior to an in-depth discussion concerning technical aspects of
intraoperative care, we should clearly state our philosophy that
supports judicious and appropriate treatment of PWS. We disagree
with the need to perform “test spots” before treatment of the PWS,
as the safety of the pulse-dye laser, if used correctly, has been
well established. Further, segmental treatment of the PWS only
prolongs the unnecessary misery of the patient and his or her family,
who already must return on numerous occasions even when the entire
lesion is treated at once. Usually, segmental laser therapy is
performed to minimize patient discomfort. However, as we rely on
sedated or general anesthesia to perform all of our PWS treatment
sessions, we have not encountered any reports of patient discomfort.
In our experience, younger children who have undergone laser therapy
without any modicum of anesthesia are severely psychologically crippled
from the experience and present to us less trusting and more fearful
of any further intervention. Even older individuals are more favorably
inclined to undergo further sessions, as they do not harbor any
anxiety about potential pain or discomfort. Ethically, we also
contend that treatment of the entire lesion at each session is less
costly to the insurance carrier, to whom we should be responsible.
We will now discuss the myriad details to which one should adhere to
optimize a successful outcome. The patient’s photographic record should
be brought into the operative suite to confirm the extent of the actual
lesion. After anesthesia is administered, when the patient lies supine,
or when the child cries, the adjacent, normal tissue becomes engorged,
and the delineation between PWS and normal skin is diminished. Further,
a surgical marking pen should be used to outline the lesion before induction
of anesthesia 
to
ensure that the pulse-dye laser adequately treats the entire lesion
and nothing else (Figure 1). The particular safety goggles worn
for pulse-dye laser administration renders the PWS less visually distinct,
and the marking pen facilitates ease of determining the perimeter of
the lesion during the procedure. All staff must also don appropriate
protective goggles prior to commencement of laser therapy. In addition,
the patient should wear corneal shields to protect against inadvertent
retinal injury (Figure 1). If the child’s frame is too small
to accommodate corneal shields, he or she should have the eyes held
shut by the staff and/or surgeon during the procedure. We have found
that the fluences required to effect a favorable change are sufficiently
high to cause some patient discomfort. Therefore, we advocate use of
monitored anesthesia care in which the patient is sedated but spontaneously
respiring. In the younger child (less than 2 years of age), general
mask anesthesia is routinely required to ensure cooperation and to avoid
the aforementioned unfavorable traumatic stress induced by lack of anesthesia
and sedation. The anesthetic mask is temporarily removed as the laser
passes over the area covered by the mask. The affected eyebrow is coated
with surgical lubricating gel in order to avoid damage to the hair follicles
but which still permits treatment of the underlying skin.
After the initial laser spot is administered, the surgeon pauses
to inspect the tissue for appropriate response, i.e., the tissue
should appear a dark purple but not assume a grayish hue, the latter
of which portends potential over-treatment that may engender cicatricial
formation. We then adjust the fluence accordingly to achieve the
desired purpuric endpoint. However, in older individuals who have
resistant PWS, we do attempt to attain a grayish endpoint in order
to ensure a lightening of color. We tend to use higher fluences
(8 to 12 J/cm2) in older (> 2 years old) patients
particularly along the perimeter of the lesion (vide infra),
whereas we rely on lower fluences (5 to 7 J/cm2) in infants
who have more sensitive skin and may be more prone to scarring.
We have found that the 5-mm spot-size handpiece allows us to achieve
higher fluences and more precise control when addressing the periphery
of the lesion. The 7-mm handpiece permits a wider arc of coverage
for the more central areas when lower fluences are preferred. However,
the 5-mm handpiece is often adequate when treating the central areas
of an infant with a smaller lesion when the fluence is adjusted
downward from that used on the periphery. These aforementioned
fluences and spot-size parameters are only meant to serve as guidelines
for the reader, and clinical judgment must be exercised. In addition,
we have determined that these fluences and spot sizes are optimal
for our laser device (ScleroPlus, Candela Corp., Wayland, MA) and
safe when used with the proprietary Dynamic Cooling Device (DCD)
spray, which ejects a pre-laser cryogen burst to protect the epidermis.
For subsequent treatment sessions, we usually adhere to the same
parameters if the patient has noticed a favorable change.
We target the perimeter of the lesion first with a direct, 
perpendicular
aim of the laser at a higher fluence in order to soften the transition
between normal and abnormal skin. We have found that this method effectively
obscures the PWS by attenuating the border of demarcation 14
(Figure 1,2). It is imperative that the edge of the laser beam
not overlap at all with normal adjacent skin because the unaffected
skin will lighten and form a halo around the lesion (particularly in
darker complected patients), which in turn only accentuates the border.
The central areas of the lesion are then treated with either the same
or lower fluence of the laser aimed tangentially to the skin surface
in order to avoid the uneven, lattice-like appearance of partially treated
areas (Figure 1,2). We also aim the laser beam obliquely over
the eyelid and perform at most one pass of the laser, as the eyelid
skin is very thin. At the end of the treatment, we apply a thin coating
of Bacitracin ointment to facilitate healing, which the patient or patient’s
family maintains twice daily for the first postoperative week.
Postoperative Considerations
We routinely see patients six weeks
after the treatment session, as any time earlier is too premature
to gauge whether the effected change has occurred. Further treatment
sessions depend on patient satisfaction with prior sessions and
motivation to pursue another cycle of therapy. Some patients desire
intermittent treatments to accommodate their work or school schedule
and at times express the need to abstain from treatments for awhile
until they are psychologically ready to continue with therapy.
Potential complications from pulse-dye laser therapy are rare and
include scarring, uneven pigmentary ablation, hypopigmentation of
surrounding normal skin, ocular injuries, transient alopecia, herpetic
outbreaks, and cutaneous infections. An area of scarred skin should
be avoided on subsequent treatment sessions and hopefully allowed
to heal over time. Uneven pigmentary changes can be addressed by
focusing the laser on the residual darker patches and feathering
the laser gently around these areas. Hypopigmentation may resolve
with time, but frank depigmentation may be more recalcitrant. Ocular
injuries are very rare, as the laser’s efficacy is significantly
reduced after a 0.5-mm distance and as diligent protective measures,
e.g., use of corneal shields, should be undertaken during every
procedure. Alopecia, even in the eyebrow area, is temporary, and
the patient should be reassured of this fact. We do not routinely
treat patients prophylactically with antiviral medication but would
initiate treatment if a herpetic outbreak should manifest itself.
Cutaneous infections, albeit unusual, may be adequately treated
with antibiotics aimed at staphylococcus aureus and/or pseudomonas
aeruginosa. Fortunately, complications are very infrequent
and usually readily amenable to treatment.
Conclusions
Pulse-dye laser therapy for PWS is a safe and effective treatment
that may lighten but not eliminate the stigma of the hyperpigmented
lesion. Due to the paucity of practical treatment information that
exists in the literature, we have endeavored to provide a succinct,
but comprehensive, management outline that addresses pertinent pre-,
intra-, and postoperative care for the PWS patient so as to maximize
benefit and minimize morbidity.
References
1. Miller AC, Pit-Ten Cate IM, Watson HS.
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3. Dohil MA, Baugh WP, Eichenfield LF. Vascular
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dye laser can improve results in port-wine stain therapy. Br
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Legends
Figure 1: Illustration highlighting the pertinent intraoperative
considerations that should be respected to ensure a favorable outcome.
Figure 2: Illustration demonstrating the tangential angle that
the laser beam should be directed to treat the central areas of
the PWS evenly and to avoid the lattice-like appearance that arises
from a direct, perpendicular aim of the laser (A), and the perpendicular
angle to the skin that the laser should be aimed at the periphery
of the lesion to diminish the abrupt transition between the PWS
lesion and normal adjacent skin with the use of a high-fluence setting
and the laser maximally focused on the skin (B).
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