Management of Congenital Vascular Lesions of the Head and Neck
Management of Congenital Vascular Lesions of the Head and Neck
There is a clear need to improve the clinical management of vascular lesions of the head and neck. This paper by Waner and Suen corrects some of the common misconceptions and emphasizes that each vascular lesion must be recognized, understood, and treated on an individual basis.
As the authors point out, Mulliken and Glowacki have contributed the most recent and useful attempt at classifying blood vessel tumors. They limit the term "hemangioma" to those congenital lesions that have a marked tendency to involute spontaneously. Unfortunately, if we are to improve care, what is most needed is an improvement in the clinical recognition of these lesions rather than in the names by which they are designated. The late Dr. Louis T. Byars suggested that, from a practical standpoint, it might be easier to allow people to continue to call all vascular lesions "hemangiomas" and instead concentrate efforts on clarifying the clinical differences among the multiple varieties.
Many Lesions Still Left Untreated
Currently, the biggest remaining problem is that many patients with vascular lesions are left untreated because their physicians are not aware of the exact prognosis or the methods of management most suitable for that individual patient. As the authors point out, some 30% of involuting (juvenile) hemangiomas are, indeed, present at birth. Some of these lesions never involute completely, and others take many years to undergo partial involution.
To further complicate the situation, quite a substantial number of vascular "malformations" enlarge markedly and cause increased morbidity after birth. Although this may not be due strictly to cellular proliferation, from a clinical standpoint, the family and pediatrician often see this increase in size as "progression." Further confusion occurs when involuting "cutaneous" hemangiomas are compared with port-wine stains that involve postcapillary venules. Both of these lesions are cutaneous and intradermal, although quite different in clinical prognosis.
I do take exception to the authors' statement that clinical characteristics do not give any clue as to the expected rates of involution of cutaneous hemangiomas. We have learned through the years that the site of the hemangioma may be quite helpful. For example, angiomas involving the midline of the nasal tip, the deep tissues of the eyelids, or the deep parotid gland are notoriously slow to involute and usually require surgical intervention at an early age. By the same token, hemangiomas with a spherical unilocular form located just beneath the skin involute very slowly and respond only rarely to steroid therapy.
Use of Steroids A Major Therapeutic Advance
I am pleased with the emphasis the authors place on steroids (ie, prednisone) in initiating involution of these vascular lesions. I believe this to be the major advance in therapy for these patients over the past 40 years. As one of the original authors advocating this treatment, however, I would point out that the dose recommended by Waner and Suen may be too conservative. At a dose of 2 to 4 mg/kg, a 10-lb healthy baby would receive only a 12-mg daily dose. They point out that this dose achieves only a 60% response rate. We have found that a dose of 40 mg for a 10-lb baby given every other day produces better than a 90% response rate.
The alternate-day regimen reduces side effects, and, in fact, we have never seen a serious documented long-term complication from this level of treatment. Separate studies in China using similar large doses have confirmed the apparent safety of these programs. The family should be warned that the child receiving alternate-day steroids may develop temporary edema and cushingoid appearance of the face, but that these effects disappear quickly as the steroid dose is tapered. I also agree with the authors that intralesional injections offer no additional benefit, are painful, and sometimes result in skin ulceration.
Unfortunately, interferon-alpha 2a (Roferon-A) is expensive and has yet not been shown to be more effective than steroid treatment, at least in instances of non-life-threatening hemangiomas.
If cardiac failure is, in fact, encountered, compression dressings to reduce platelet trapping may be helpful, and the possibility of an arteriovenous malformation must be suspected. In such cases, selected embolization can be enormously helpful.
Roles of Laser Therapy and Surgery
Although laser therapy can be quite helpful for very superficial hemangiomas, we prefer to defer laser treatment of overlying skin until the deep portion of a compound lesion has been surgically excised. In many instances, that will be followed by spontaneous lightening in the color of the overlying skin, making laser treatment unnecessary. I would hope that the authors are not suggesting that the result shown in Figure 8B is an acceptable final result. Hopefully, that child will be referred to a plastic surgeon to eliminate the residual lip deformity.
Hemangiomas (of any type) and vascular malformations should all be classified as phakomatoses. These conditions are clearly of neurogenic origin and are "related" to other conditions, such as neurofibromatosis.
There is ample evidence that the future pattern of an arteriovenous malformation is present at birth, and thus, is congenital. This may be true, although only a small part of that anatomic pattern is clinically evident. The clinical removal of the nidus of an arteriovenous malformation does not appear to suppress the later recruitment of adjacent capillary beds that occupy the tissue within the congenital pattern of that particular lesion. Thus, removal of apparently adequate margins of normal tissue in a small child may not prevent the recurrence of an arteriovenous malformation. These boundaries are understand- ably more predictable in older patients.
The use of MRI-guided placement of quartz fibers coupled to a neodymium/yttrium-aluminum-garnet (Nd:YAG) laser is a treatment approach well worth exploring. In addition, "intravascular glues" that "set up" within the lesion show some promise.
Although port-wine stains of "lighter" color may be effectively managed by laser therapy, those that are darker or elevated often are best treated by skin grafting or pedicle flap rotations. Physicians managing children with these lesions would be wise to consult a plastic surgeon when these children are at an early age in the hopes that the port-wine stain occupies an appropriate aesthetic unit for a one-stage removal and graft or flap repair.
Understandably, pediatricians, or even plastic surgeons and dermatologists, do not see enough vascular lesions in clinical practice to form clear pictures of the prognosis and alternative treatment results that are possible. Certainly, this paper by Waner and Suen will clear away some of the cobwebs.