Medical Therapy
Medical therapy includes monitoring the lesion with appropriate imaging if an associated syndrome is suspected. Regular ophthalmologic review is also necessary when there is periocular involvement. [11]
Uncommonly, capillary malformations bleed after minor trauma, and the bleeding can be difficult to stop. Compression of the area and immediate medical assistance may be necessary. Topical timolol can be used to help stop bleeding.
Attempts can be made to conceal the lesion using specially designed water-resistant makeup. The use of superficial tattooing has also been described.
Compression garments and physiotherapy may be required for patients with associated limb edema.
Surgical Therapy
Laser therapy
The current treatment of choice for capillary vascular malformations is the flashlamp-pumped pulsed-dye laser, although only 15-20% of lesions clear completely. [15, 16] The 585 nm wavelength is most commonly used for a duration of 450 μsec, which is long enough to heat the vessels but not the surrounding tissue. [17] The anatomy of the malformation, however, ultimately guides the physician's choice of laser parameters.
Photothermolysis, using ultrashort pulses of yellow light, targets superficial vessels, improving the appearance of the lesion by lightening the color. Immediately following therapy, skin can develop edema and purpura. The safety of pulsed-dye laser therapy can be enhanced by cooling the skin during treatment. Skin cooling also allows the use of higher wavelengths and fluences. Cooling strategies include the use of convective air cooling, ice cubes, cold gels, cryogen spray cooling, and aluminum rollers. [18, 19, 20, 21, 22, 23]
The therapeutic response is better in the head and neck region compared with the extremities, with superior outcomes seen for lesions in the periorbital region, neck, and temple. Small and/or lighter lesions and patients with skin types I-III also respond better. [10]
The outcome for treatment with pulsed-dye laser is better when administered at a younger age, as response is improved and fewer treatments are thus required. [10] This therapy is not as effective in patients with skin types IV and V and is associated with more complications such as hypertrophic scarring. Skin lesions can recur, but not to the extent seen prior to the initiation of treatment. Recurrence rates are reported to be 11-50%.
Topical imiquimod has reportedly been used following laser therapy to aid involution of the lesion. [24] However, this is not yet considered an accepted treatment, although there is ongoing research into this and the use of angiogenesis inhibitors.
Other, less commonly used lasers are KTP (potassium-titanyl-phosphate), Nd:YAG (neodymium-doped yttrium aluminium garnet), and IPL (intense pulsed light), but these are associated with greater side effects. [25]
Surgical treatment
Patients with extensive port wine stains who have not responded adequately to laser therapy or who have been left with hypertrophic lesions, scarring, or hyperpigmentation may require surgical excision and reconstruction with the most appropriate technique. Soft tissue hypertrophy occurs most commonly in the upper lip, and bone hypertrophy occurs most often in the maxillary bone (and can also be treated with surgery). [8]
Cerrati et al described a systematic approach to the surgical treatment of head and neck port wine stains. The main aim of excision is to restore facial symmetry and contour. The authors described techniques of primary resection performed in staged procedures, advising that the bulk of the lesion be resected in the first procedure, while the second procedure be focused more on accurate aesthetic analysis. [7]
Preoperative and postoperative laser therapy can be used to minimize the amount of tissue that needs to be removed. Soft tissue hypertrophy of underlying tissue can cause difficulty in predicting how the overlying skin flap will heal, as it will not be of the same uniformity and depth throughout the lesion; this is another reason that a staged approach is preferred. [7]
Flaps
If the deficit after resection is going to be too large to close primarily using the skin flap, then local flaps, rotational flaps, free flaps, or full-thickness skin grafts may be used. However these are often more noticeable than local skin faded with laser, and with time, skin grafts can also become infiltrated by the port wine stain.
Local flaps may be used with or without tissue expansion. Expansion flaps can be induced to cover the skin deficit left by excision, while causing minimal donor site morbidity and providing a better match for texture and color than full-thickness skin grafts. [26] Tissue expanders are placed in the skin adjacent to the lesion, with the base of the expander being as close as possible to the size of defect. The expanders are then filled every 2-3 weeks, with this being continued until an area of tissue twice the size of the defect has been produced.
Use of free flaps in successful deficit reconstruction has been described. In a series of five patients, thoracodorsal artery perforator free flaps were used for reconstruction, with all flaps surviving without the recurrence of the port wine stain within them. [27] This option may be used for patients when local flaps are not appropriate.
Orthognathic procedures
When mandibular prognathism or occlusal canting from hemimaxillary vertical overgrowth occurs, orthognathic procedures are indicated. Maxillary overgrowth can be surgically reduced by contouring.
Follow-up
Provide monthly follow-up care to neonates with birthmarks. Hemangiomas begin proliferating within the first month, while capillary malformations enlarge commensurately with the child’s growth. Invasion of important anatomic structures, cosmetic deformity, pain, and swelling may prompt surgical treatment. Monitor patients for recurrence after lesions are resected.
Future and Controversies
Classification of vascular malformations remains controversial. Findings by Breugem et al suggest that the pathologic abnormalities of capillary malformations appear to be located in postcapillary venules rather than in the capillaries themselves. [28] Thus, port wine stains may need to be redefined from their original classification under capillary malformations.
Future advancements in the treatment of capillary malformations include improved selective laser ablation and gene therapy. However, gene therapy remains experimental, with target cells still being evaluated. The use of angiogenesis inhibition is also being researched.
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A 12-year-old boy presenting with bluish discoloration of the right thenar eminence and index finger. The lesion has been present since birth and changes in size when he raises his arms or exercises strenuously. Upon physical examination, the mass is soft and rubbery. No palpable thrill or audible bruit is present.
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An image from an MRI study of the right hand of the patient in the image above, detailing the vascularity of the lesion.
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Intraoperative view of the lesion in the first and second images shown above. Note the irregular mass of vessels that are adherent to the digital neurovascular bundles, tendons, and lumbrical muscle belly.
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The excised specimen from the patient in the previous images.
Tables
Grade 1
|
Sparse, pale, nonconfluent discoloration |
Grade 2 |
Denser pink, nonconfluent discoloration |
Grade 3
|
Red, discrete, ectatic vessels |
Grade 4
|
Confluent patch |
Grade 5
|
Nodular lesion |
