Introduction
Port-wine stains (now termed capillary malformations) are congenital
vascular malformations characterised by ectatic vessels within the
cutaneous superficial vascular plexus.1 They affect 0.3% of children
at birth, with an equal sex distribution.2 Most port-wine stains
are found on the head and neck, with 85% occurring in a unilateral,
dermatomal distribution.3 Their natural history is to progress from
a pink, macular lesion at birth to a dark red (or even purple),
nodular, proportionately larger lesion in adulthood.1,4
Cosmetic disfigurement can cause considerable psychological morbidity,
and be socially disadvantageous.5,6 Hypertrophic, nodular lesions
may bleed, either spontaneously or after trauma, encroach on essential
facial structures or even develop into pyogenic granulomas.7 All
port-wine stains should be treated, preferably early in life,
to prevent or reduce the potential physical and psychological
complications.
Before the development of laser technology, treatment for port-wine
stains was often unsatisfactory.8 Earlier lasers, including ruby,
carbon dioxide and argon lasers, improved the lesions in most
patients, but, as they were non-selective in their effects on
tissue, the frequency of side effects, such as scarring and pigmentary
changes, was unacceptably high.9 The flashlamp-pumped pulsed dye
laser (PDL) was the first laser to be based on selective photothermolysis;10
it produces vascular- specific damage without affecting surrounding
dermal structures or the epidermis.11-13 Studies have confirmed
its effectiveness in the treatment of port-wine stains in adults,
children and infants, with an extraordinarily low incidence of
side effects.14-19 The PDL is now regarded as the first-line treatment
for port-wine stains whenever possible.8,9,20-22
In Australia, the PDL has been in use since the late 1980s for
the treatment of cutaneous vascular lesions, especially port-wine
stains. However, no Australian study of its effects has been published.
Therefore, we undertook a retrospective clinical review of all
patients with port-wine stains treated with PDL at Royal Perth
Hospital between August 1989 and December 1992.
Methods
The study was a retrospective review of patient medical records.
If response to treatment was not recorded, attempts were made
to review the patient between January and December 1993.
Patients
All patients (adult and paediatric) with a port-wine stain treated
with PDL at Royal Perth Hospital between August 1989 and December
1992 were eligible. Patients attended outpatient clinics, where
the site and size of the port-wine stain and demographic data
were recorded and the treatment procedure and its risks and benefits
were explained. Patients were photographed before treatment by
a professional photographer in a studio dedicated to medical photography,
with efforts to use the same magnification, lighting and exposure.
Laser and technique
A flashlamp-pumped pulsed dye laser (Candela SPTL-1, Candela Corp,
Wayland, Mass, United States) was used. It emitted yellow light
at a wavelength of 585 nm, with a pulse duration of 450 µs and
a 3 s pause between pulses. The laser beam was transmitted down
a 1 mm fibre by a planoconvex lens and focused as a 5 mm spot
beam. Energy densities were measured by an energy meter (Ophir,
Jerusalem, Israel), calibrated to 10% accuracy. Both the physicians
and the patients eyes were protected from laser light during treatment.
Some patients had a small initial test patch treated, depending
on patient anxiety and time of presentation (before 1991, most
had a patch test). Otherwise, the entire lesion was treated at
once, unless it involved a large surface area (> 100 cm2).
Treatments were repeated at intervals of 23 months.
The energy density used varied with the age of the patient and
colour, nodularity and location of the lesion and was adjusted
according to the degree of purpura produced and the patients response
to the previous treatment. Pulses were overlapped by a maximum
of 10% across the affected area.
The anaesthetic varied according to the site and area to be treated
and the level of patient cooperation. EMLA cream (eutectic mixture
of 2.5% lignocaine and 2.5% prilocaine cream, Astra Pharmaceuticals,
North Ryde, NSW) was used for topical anaesthesia, applied under
occlusion for 60120 minutes before treatment. Local anaesthesia
involved an injection of 1% lignocaine, either locally or as a
regional nerve block. General anaesthesia was given to children
who had extensive lesions or were uncooperative with topical or
local anaesthesia.
The treated area developed purpura within a few minutes, usually
persisting for 710 days. No immediate postoperative care was necessary,
except for an occasional ice pack to reduce oedema in those with
large treatment areas. Postoperative instructions were to protect
the area from trauma, avoid excessive exposure to sunlight and
use a topical antiseptic cream for any scaling or crusting.
Treatment evaluation
Each port-wine stain was evaluated, either before the next treatment
or 34 months after the final treatment. Lesional lightening was
assessed as the percentage reduction in colour compared with the
pretreatment photo (fading < 25%, poor; 25% to 50%, fair; >
50% to 75%, good; and > 75%, excellent) (Figures 1-4).
Adverse effects, such as scarring and textural or pigmentary changes,
were also noted. All patients were individually assessed by one
or both investigators. The endpoint of treatment was assessed
clinically.
Data analysis
Data were analysed with the Statistical Analysis Systems software
package.23 The chi-squared statistic was used to assess the difference
in response between age groups.
Results
There were 186 patients treated by PDL: 131 completed treatment
(55 either did not complete treatment or were having ongoing treatment).
Patients were either Caucasian or Asian and aged 8 months to 66
years (mean, 25.6 years). There were 59 males (32%) and 127 females
(68%).
Most of the treated lesions were present from birth (97%). Acquired
lesions appeared most commonly between the ages of six and 12.
Most lesions were on the face and neck (87%), with the rest distributed
unilaterally on the arms (4%), legs (5%), back (2%) and chest
(2%).
The size of treated lesions ranged from 1 cm2 to 280 cm2 (mean,
42 cm2). All responded to energy fluences between 5 and 10 joules/cm2(mean,
6.7 joules/cm2). Sixty-two per cent of the patients had a patch
test before treatment. Anaesthesia was used for 44% of patients
(general anaesthesia by 20%, topical by 19% and local or regional
block by 5%).
Responses of patients who completed treatment are shown in the
Box. A good-to-excellent response was achieved in 78% and a poor
response in only 9%. An average 3.4 treatments per lesion were
required to achieve a good-to-excellent response.
Adverse side effects occurred in 11% of patients who completed
treatment; all had some fading of the lesion. The most common
adverse effect was pigmentary change (6.1%), which was usually
transient and resolved in 23 months. Only 4.6% had significant
permanent adverse effects; two had scarring (in both the port-wine
stain was on the face and neck region).
More children than adults had a good or excellent response, but
the difference was not significant when compared with a 2 x 2
contingency table and chi-squared test (r = 0.60). Similarly,
fewer children than adults had a poor response.
Discussion
Our results compare favourably with those of other studies. A
good-to-excellent response (more than 50% fading) was obtained
in 78% of our patients (including both adults and children, with
lesions on sites including the trunk and lower limbs), with an
average 3.4 treatments required. Others have found more than 50%
fading in 73%-95% of patients after 2.4-2.8 treatments.14-17,24,25
Response to treatment varies between sites: the periorbital area,
temple, lateral aspect of the cheek, neck and chin have been observed
to be more responsive18,25 and the centrofacial area and lower
leg to be less responsive.18,19 We found that a poor response
was more common in adults than in children (although the difference
was not significant), possibly because port-wine stains become
progressively hypertrophied and nodular in adults.
We found a higher rate of adverse effects (11%) than in other
studies. The most common (usually transient) adverse effect was
pigmentary change (increase or decrease), possibly because of
excessive sunlight exposure after treatment. This transient change
may not have been recorded in other studies; when it was excluded
from our figures, the rate of adverse reactions was reduced to
less than 5%, which is comparable with that found in other studies.
Scarring was seen in two of our patients, with lesions on the
face and neck, where damage to dermal structures with fibrosis
occurs when excessive energy fluence is used. A low energy fluence
should be used initially when treating port-wine stains on the
neck and anterior chest.
The PDL is the first laser specifically designed for cutaneous
vascular malformations. It is based on the theory of selective
photothermolysis, which predicts selective destruction of blood
vessels without damage to the surrounding tissues.10 Laser light
emitted by the PDL is absorbed by oxyhaemoglobin in the dilated
vessels of the lesion, producing agglutination of erythrocytes,
thrombus formation and eventual destruction of the vessels.11
They are replaced by non-dilated superficial dermal blood vessels
with a normal appearance.12 A recent comparison of PDL and the
copper vapour laser showed that PDL produced significantly better
fading of port-wine stains.22
The characteristics and degree of pain associated with PDL treatment
have been well described.26 Initially, there is a sharp stinging
pain, very similar to the snap of a rubber band against the skin.
Accompanying this is a second distinct heat sensation that can
be at least as unpleasant as the initial sting. Pain rapidly subsides
but seems to build up if successive pulses are used for a moderately
sized lesion.
Our current practice is to give general anaesthesia to all children
from four weeks of age, until they are able to co-operate with
topical or local anaesthesia, usually at eight to 10 years. Young
children undergoing multiple painful treatments with inadequate
anaesthesia under restraint may develop phobic responses. Furthermore,
a struggling child may compromise the clinicians ability to perform
the procedure optimally.
In conclusion, this study supports the contention that all port-wine
stains should be treated with PDL, as it has a high therapeutic
index with a low incidence of adverse effects. Patients should
preferably be treated in infancy or childhood, under general anaesthesia,
to minimise the potential psychological morbidity of disfiguring
lesions. In addition, the response to treatment seems better in
children than in adults, although the difference was not significant,
possibly because of the relatively small sample size. Laser treatment
of port-wine stains should no longer be considered just cosmetic,
but a medical necessity for a problem that can cause psychological
and physical morbidity.
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(Received 11 Sep, accepted 23 Nov 1995)
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