Medical Care
Affected areas can be tattooed with a skin-colored pigment; however, this is not routinely performed.
Use of a cosmetic cover-up is an alternative. Opaque makeup, sold commercially without a prescription under brand names like Dermablend and Covermark, disguises capillary malformations but does not treat them.
At least one report describes partial clearing of a capillary malformation on the arm of a 59-year-old woman after the use of topical imiquimod under plastic wrap occlusion 5 times per week for 4 weeks. The authors attribute the response to antiangiogenic properties of imiquimod through the induction of tissue inhibitor of matrix metalloproteinase-1, an inhibitor of vascular tumor growth. [38] A prospective, controlled trial demonstrated that imiquimod application 3 times weekly for 8 weeks after pulsed-dye laser (PDL) therapy resulted in measurable objective improvement in lightening of the stain than PDL therapy followed by placebo. [39]
Surgical Care
Flashlamp-pumped pulsed-dye laser (PDL) surgery is the treatment of choice for capillary malformations. [40, 41, 42] It uses selective photothermolysis with ultrashort pulses of monochromatic yellow light (585-600 nm), which are preferentially absorbed by oxyhemoglobin in the abnormally dilated superficial dermal blood vessels. Flashlamp-pumped PDL causes selective destruction of these superficial target blood vessels, inducing intravascular coagulation and rupture of some smaller vessels, which later become absorbed and replaced by collagen. The ultrashort laser pulses are shorter than the thermal relaxation time of the vessels, thereby limiting adjacent dermal and epidermal heating and preventing subsequent damage. Kono et al published a thorough review of PDL therapy for capillary malformations, [43] which examines many of the idiosyncrasies of this treatment modality.
Despite few, high quality, randomized-controlled trials comparing the efficacy amongst various laser or light sources, PDL has proven to be effective for clinically relevant clearance of capillary malformations. A Cochrane review demonstrated overall greater than 25% reduction in redness after 1-3 treatments in over half of all subjects treated in these studies. [40]
Skin treated with a PDL immediately develops edema and purpura, the latter of which generally last 1-2 weeks. PDL surgery is repeated between 2 weeks and 3 months. Preliminary findings suggest that a 2-week interval between treatments is as safe and effective as a 3-month interval. The shorter interval serves to increase the treatments in early infancy, potentially avoiding general anesthesia later in childhood. [44] Flashlamp-pumped PDL is relatively safe; major risks include pigmentation alterations (hyperpigmentation, which is always transient, and potentially permanent hypopigmentation), crusting, and, rarely, scarring. Immediate adverse events associated with PDL therapy are pain (a hot snapping sensation that can increase with repeated pulses) and combustion (especially of hair-bearing areas and in the presence of increased ambient oxygen). PDL therapy can be performed on patients with skin types V and VI, but with a potentially higher risk of pigmentary complications.
Decreasing the time between treatments increases the number of treatments that can be delivered prior to age 6 months, thereby reducing the number of subsequent treatments needed under general anesthesia. The safety and efficacy of more frequent PDL treatments of capillary malformations in infancy was investigated in pilot, prospective, patient-controlled study of 10 patients. The entire capillary malformation was treated initially using a 595-nm (Vbeam) PDL, and then half the capillary malformation was randomly selected for 2-week interval PDL treatment and half to 3-month intervals for two additional PDL treatments. An independent, blinded dermatologist evaluated photographs of the capillary malformations taken 3 months after treatment. Nine infants finished the study. More improvement on the 2-week interval treated side was seen in 3 infants (33%), and more improvement was seen on the 3-month interval treated side in four infants (44%). No difference in the two sides was noted in two infants (22%). Treatments were well tolerated, with no complications reported. The study results suggest that 2-week interval PDL treatments of capillary malformations in infants younger than 6 months is effective and well tolerated, without adverse effects. The preliminary data suggested possible superior efficacy with the 3-month interval treatment; however, larger studies are warranted for stronger evidence. More frequent treatment of capillary malformations without general anesthesia should be investigated further to decrease the risk of repeated exposure to general anesthesia in young children.
Some authorities advocate treatment beginning as early as 7-14 days of life, yet the age at which laser treatment achieves maximal response is unknown. Some studies indicate a better response if treatment is commenced before the patient is aged 1 year, while others reveal no difference. From a psychosocial perspective, treatment commencing in infancy is most beneficial.
The response rate depends on several variables. The first is anatomical location, with rates in descending order of location from best to worst as follows: neck, torso, face, and hand and arm; and with rates in descending order of location from best to worst for facial capillary malformations as follows: forehead, lateral part of the face, temple, and central part of the face. The second variable is size; smaller lesions appear to have higher response rates. These variables are influenced by the histologic features of capillary malformations. In 2004, videodermoscopy was used to demonstrate that capillary malformations with smaller vessels deep in the dermis were the most difficult to eradicate with PDL. [45]
Investigations are ongoing to explore methods to improve the outcome of laser surgery, including adjunctive use of exogenous heat, [46] the topical application of the angiogenesis inhibitors rapamycin [47] or imiquimod [48] postoperatively, or intravenous porphyrin derivatives (photodynamic therapy) [49] prior to laser surgery. While the early observations are promising, these interventions are strictly experimental at this time.
See the images below.
Capillary malformation on the left preauricular aspect of the cheek, the ear, and the neck in a neonate (same patient as in Media Files 3-4).
Same patient as in Media Files 2 and 4 immediately after test spots with the pulsed-dye laser at 585 nm. Note the purpuric macules where the laser impacted in a linear distribution on the preauricular aspect of the cheek.
Same patient as in Media Files 2-3 after 4 treatments with the pulsed-dye laser. Treatments were given at 2-month intervals in an outpatient setting using topical anesthetic.
Redarkening of capillary malformations can occur in patients treated by laser. [50] The exact percentage of patients in which this occurs is unknown, with estimates from 11-50%. Currently, no characteristics help predict which lesions will redarken. No capillary malformations darken to the degree they were prior to embarking on laser treatment. Fifty-nine percent of patients are satisfied with the overall treatment result. [51]
Earlier laser therapies that caused an unacceptably high rate of scarring are not recommended; these therapies include carbon dioxide laser, copper vapor laser, and argon laser. Other modalities, such as intense pulsed light devices, [52] alexandrite laser, [53] and the long-pulse [54] or frequency-doubled Nd:YAG laser, [55] are reported to be effective in certain instances, but they are generally considered to have higher complication rates than the PDL.
Previous nonlaser treatments determined unreliable or deleterious include surgical excision, radium implants, cryosurgery, electrocautery, sclerotherapy, and grenz ray radiotherapy.
Innovative methods such as laser speckle imaging [56] and photoacoustic imaging [57] to more accurately assess the vessel density, depth, and diameter and immediate efficacy of laser surgery are ongoing and represent the next frontiers toward improved treatment outcomes.
Consultations
Consult a pediatric dermatologist, a general dermatologist, or a specialist at a laser center or a vascular lesion center for evaluation and early intervention if indicated.
Consult an ophthalmologist for tonometry to exclude glaucoma in infants with CN V1 and CN V2 or eyelid involvement. An ophthalmologist can also evaluate for evidence of ocular capillary malformation in patients with Sturge-Weber syndrome.
Consult a radiologist or refer the patient to a vascular lesion center if underlying complicating vascular, neural, or skeletal malformations are suspected. If a leg-length discrepancy is apparent in a patient with a lower extremity capillary malformation, consult a pediatric orthopedic surgeon.
Consult a neurologist if Sturge-Weber syndrome is suspected.
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Histopathologic features of a capillary malformation (nevus flammeus) showing telangiectatic vessels lined by mature-appearing endothelial cells.
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Capillary malformation on the left preauricular aspect of the cheek, the ear, and the neck in a neonate (same patient as in Media Files 3-4).
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Same patient as in Media Files 2 and 4 immediately after test spots with the pulsed-dye laser at 585 nm. Note the purpuric macules where the laser impacted in a linear distribution on the preauricular aspect of the cheek.
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Same patient as in Media Files 2-3 after 4 treatments with the pulsed-dye laser. Treatments were given at 2-month intervals in an outpatient setting using topical anesthetic.
