Hemangioma Update Pearls From 30 Years of Treatment
Barry M. Zide, DMD, MD, and Steven M. Levine, MD
Abstract: This manuscript is a compilation of clinical lessons learned from30 years of treating hemangiomas. We review relevant clinical anatomy, the role of lasers and medications, timing of surgery, and operative judgment, as it pertains to the treatment of hemangiomas.
Key Words: hemangioma, vascular malformation
This is a “biased” (informal) review, though some current concepts from the literature have been referred. Readers of clinical review articles are looking for recipes for treatment, and so we are providing time-tested truths. Our goal for this manuscript was to avoid the pitfalls of a standard review article that attempts to pay equal attention to all level 4 and 5 evidences, despite the fact that such evidences are never created equally. We focus on teaching the points garnered from the senior author’s practice (B.M.Z.). We review relevant anatomy when appropriate, the role of lasers and medications, timing of surgery, and operative judgment, as it pertains to the management of hemangiomas.
Vascular birthmarks are classified as either hemangiomas or vascular malformations. Hemangiomas are the most prevalent benign tumor of infancy that affect up to 12% of full-term white infants. Hemangiomas are usually first noticed shortly after the birth and they present as small, faint, irregular, red or blue cutaneous marks. However, they quickly undergo a proliferative phase that is variable in length and intensity, but usually lasts for 3 to 9 months. Eventually, in most cases, endothelial cell hyperplasia plateaus, which leads to a period of spontaneous regression. This involuting period usually occurs after 7 to 8 months through 7 to 9 years. To date, there exists no evidence-based algorithm to stratify these tumors into lesions likely to involute and those likely to remain status quo. In fact, some lesions (eg, noninvoluting congenital hemangiomas) will hardly undergo any involution, and more than 50% of lesions will leave stigma evidenced by telangiectasias, scarring from ulceration, hypoelastic patches, facial discoloration, pitting, or fibrofatty replacement of tissue.
Of interest, hemangiomas of the liver, vertebrae, spine, and facial bones can be detected by radiographic studies in adults without being symptomatic. Some of these can be left alone, depending on their size, whereas others seem to grow. The senior author has seen them in the forehead, maxilla, and cheek skin presenting as slow-growing masses that were not present at birth or shortly after. Perhaps, they are true intraosseous hemangiomas or intrahepatic hemangiomas, for example, but we have had numerous problems identifying pathologists who can accurately differentiate hemangiomas from vascular malformations. We have sent hemangiomas, arteriovenous malformations, capillary malformations, and port-wine stains to pathology for which their histological diagnosis was “hemangioma.” Following are the 7 essential points that should be conveyed to parents on initial visit (especially if the visit is for seeking secondopinion).
1. Hemangiomas lack dermis
Normal skin has a dermal layer that contains adnexal structures usually responsible for reepithelialization (eg, hair follicles, sweat glands). Because these dermal structures are largely replaced in the hemangiomatous tissue, it does not heal like normal skin if dermabraded or sutured. If a hemangioma is excised, one often finds that the central portion (where there was little or no dermis, or maybe residual hemangioma) will heal with a wider scar than the remainder of the lesion. It is imperative to alert parents in advance to the likely need for some revision, as well as the decreased amount of hair that will grow in that portion of the scar, if its is on the scalp. Further, any attempt at dermabrasion of the scar left after involution cannot be expected to reepithelialize normally. In the same way, resurfacing (mechanically or chemically) of the skin that had been recently treated with an isotretinoin-based product due to suppression of skin appendage activity should not be performed, as one cannot expect normal healing from hemangiomatous skin.
2. Involute does not mean disappearance
Hemangiomas are often subcategorized into the following 2 types:involuting and noninvoluting. Although it is convenient to memorize that 30% of hemangiomas will involute by age 3, 50% by age 5, 70% by age 7, and 90% by age 9, these numbers are only approximations. Certainly, published rates of involution vary from report to report, but the likelihood of the hemangioma to undergo significant involution is guesswork, whereas observing a specific lesion over time can provide great insight. Of note, for example, in patients with multiple hemangiomas, each one will involute at their own rate. A more important question is whether the tumor will involute, is/will the lesion involute enough to not cause an image problem, or cause parents shame or discomfort with family or out in public. Most of the reports publish numbers suggesting that approximately 50% of hemangiomas will lead to permanent disfigurement.This means that parents need to understand that involuting does not mean disappearance of hemangiomas. For example, the senior author has never observed a bulky hemangioma on the forehead, greater than 1 cm in diameter, involute without leaving some stigma (discolored skin, atrophic skin, fibrofatty tissue).
3. Lasers are not magic
Parents, especially first-time mothers, will virtually always want to get rid of the lesions immediately. Many parents present to the surgeon after either consulting with another practitioner or reading an article on the Internet, which touts lasers as some sort of panacea. And remarkably, insurance companies are willing to pay large amount of money for the lasers when the patient actually needs an excision. Pulsed-dye lasers that penetrate an average of 1.2-mm depth are often used to treat hemangiomas that are 10 times thicker than the depth of penetration. For example, a pulsed-dye laser will only scar the surface of a Cyrano nose; however, if it is first treated by laser, the nose tends to loose skin after undermining. Undermining skin that has been treated by laser will usually lead to some edge necrosis. The parents should be encouraged to reserve laser treatment as the final modality for any remaining telangiectasias, especially on the lip and nose. However, the senior author can often achieve results similar to laser treatment by using a Colorado tipped electrocautery (set to 5), or an electrolysis needle, or even a 25- or 27-gauge needle attached to the blade of a standard electrocautery tip with a piece of tubing.
4. Address medical management
Address medication, a good pediatrician will help the patients with appropriate management and monitoring. Some parents are distressed by the thought of steroids because of its side effects that includes Cushingoid facies (71%), temporary growth failure (35%), mental status changes (29%), and gastrointestinal distress (21%). Despite this, the surgeons need to assure parents that this treatment can work, especially if they believe that the lesion is likely to ulcerate (ie, lip lesions frequently ulcerate).
Parents should be made aware that this systemic steroid treatment is one of the few therapies for hemangiomas that have been time-tested.More recently, Bleomycin (using multiple injections at timely intervals) and now propranolol for short-term use (3–9 months) have been highly effective and should replace steroids as first-line treatment.
5. Confirm the diagnosis
The diagnosis of venous vascular malformation that grows pari passu with the child is missed constantly. To make the diagnosis, the surgeon should turn the child upside down to see whether the lesion fills. If there is any doubt, administer a small amount of local anesthesia first before looking for dark blood with a 25- or 27-gauge needle and consider venous malformation as the diagnosis. Most importantly, a possibility that it is not even a hemangioma exists and should be considered. On the tailbone region, for example, it may be a harbinger of a tethered cord, and a magnetic resonance imaging may be warranted. If it seems to be too firm, a needle biopsy should be considered. If it was there at the time of birth, it is likely to be something else. Therefore, the surgeons should have an open mind. They should make the diagnosis first, and then make decisions.
6. A caution about lip lesions
Lip lesions are very common, and the parents (especially of girls) need to be aware of certain things. First, inform the parents, no matter how well closure of the lesion after reduction was performed, that there is a great chance the child will bite out or “binky out” the sutures. In addition, tell them it is normal, and will not bleed badly. In fact, tell them it is this opening that will expedite scarring and involution. They should be prepared to use bland food to avoid irritation to the open area. Many of the lip lesions will need secondary revisions, especially for telangiectasias. The hemangiomatous skin will always be flaky or dry (remember, no adnexae) and may need gloss or lip balm forever. Further, lip lesions treated early with laser may lose pigment and require repeated tattoos.
7. For medical necessity, only excise what is necessary
Removal of total hemangioma may not be crucial and may be detrimental. A lesion protruding below the eye and partially inside the orbit will enlarge the orbital volume. If the obvious frontal part is removed with some cautery to the intraorbital fat, the rest will involute and the lower rim will come up afterward because much of the weight has decreased. Total removal may leave the child with orbital dystopia.
The child should not be operated before the age of 1 year except in certain cases (eg, loose skin, scalp, painful ulcerations leading to failure to thrive, visual problems, astigmatism), which will be discussed. Otherwise, surgeon is operating for the parents- one should operate for the child. Even with laser, although some may disagree, we do not recommend operation before the age of 15 months. The treatment plan should aim to be completed by age of 3 1⁄2 years when body image starts developing as well as the taunts of other children. These goals should be specified to the parents. Instances when surgical intervention prior to 1 year of age is sometimes recommended are reviewed below.
Large Scalp Lesions
The scalp has an “accordion-like” laxity inherent to infants up to age 3 1⁄2 to 4 months, but not after this. This is secondary to the absence of a thick fibrous galeal layer. As the infant grows, this flimsiness disappears considerably. Because of this relative tissue excess, large lesions of the scalp (especially temporally and occipitally) can be removed only before 4 months, not later.
Failure to Thrive
If the child’s large hemangiomas ulcerate, pain can interfere with some parents’ ability to hold the child. In this case, early removal of the lesion eliminates this pain. For small lesions (approximately 3 cm), the senior author has successfully used Lidoderm (Endo Pharmaceuticals, Newark, DE) patches (topically used for postherpetic neuralgia) to provide dramatic pain relief. These Lidoderm pads can be applied in small doses, just where lesion is ulcerated and can provide relief up to 6 hours at a time. They are especially useful in the perineal region where urine or feces over the ulcerated lesion increases discomfort. When multiple lesions cause constant crying or inability to pick up the child, excision may be indicated.
Orifice Obstruction, Astigmatism, and/or Visual
Field Obstruction Usually Dictate Early Removal To these classic examples, we add disturbing anatomy (eg, pushing down on an ear). Hemangiomas of the parietal scalp may impinge on the ear causing a secondary defect in the ear cartilage. The well-known pliability of the newborn ear may result in a permanent deformity if not addressed. After excision, if the ear has become deformed, there is a window of opportunity to reshape the ear by splint. Regarding parotid hemangiomas, we acknowledge that some may meet certain criteria for excision; however, we strongly object to early facial nerve dissections, as advocated by some. We might consider a debulking procedure, a procedure where the plane of dissection is kept superficial to the facial nerve, but the senior author has never practiced it. We favor waiting for shrinkage and removing skin excess in the future.
Sometimes a surgical treatment, sometimes medical management, sometimes both surgical treatment and medical management, and sometimes none of them are indicated. To this end, the plastic surgeon should represent only one portion of the multidisciplinary approach needed to adequately address vascular malformations, some of which are hemangiomas. At present, there are 3 nonsurgical therapies (steroids, bleomycin, and propranolol), which may help proliferating hemangiomas that require excision.
Systemic steroids may help reduce the frequency of ulceration and/or may decrease lesion size. They will also temporarily halt growth and weight gain, along with other aforementioned side effects. Historical response rates to systemic steroid treatment ranged from 30% to 90%. We have found at least partial benefits in 60% to 80% of our patients treated with systemic steroids. Our patients’ usual dose of systemic steroids is 2 to 3 mg/kg and is continued for at least 2 weeks, with a slow weaning process. It is generally recommended to use the lowest possible effective dose, though we do believe that the drug does have a direct dose response in terms of hemangioma reduction and intensity of side effect. The higher dose may require shorter length of treatment, but the side effects (like immediate stoppage of weight gain), although reversible, can be too difficult for parents to endure. Fortunately, this is now the second-line medical treatment in the senior author’s practice.
Intralesional injection of long-acting corticosteroids is well described. Published reports cite response rates of approximately 50% reduction in one-third of patients, less than 50% reduction in one-third of patient, and little response in one-third of patients. The inconsistent response rates with only 30% to 50% reductions in the lesion reduce its usefulness. Although some authors favor intralesional steroids for lesions involving the eyelid, we avoid these injections around the orbit due to the low, but possible risk of visual problems from steroid emboli. Intervals should be at least 6 to 8 weeks for this modality. Dermatologists who are reluctant to operate cite this treatment more in their literature than we plastic surgeons do.
This drug has great promise as well as great benefits for many cases. For infants, it requires multiple intralesional injections under general anesthesia (due to pain), but the dosages are low enough to be very safe. The literature on this is well documented in both our journal, in South African cases, and the dermatology literature.
A drug with good tolerance and very few side effects has been discovered as a top tier weapon. In infants younger than 3 months, starting the medication requires hospital admission to watch for bradycardia, hypotension, and hypoglycemia, though just for 1 day. Preoperatively, photographs, ultrasound, routine blood work, and a cardiology visit (for echo and EKG) are required. The drug works well if started at 2 to 3 mg/kg/d, divided into 2 or 3 doses, and can be used for 3 to 9 months. Gradual discontinuation can take 3 to 4 weeks. Results can be grippingly rapid, and the literature certainly supports its use. Topical propranalol as a 1% cream or timolol 0.5% can be tried for some lesions around the eye to jump-start involution.
For periorbital lesions, the studies demonstrate greater efficacy. The bottom line in this case should read as follows: start with propranalol, consult a cardiologist to do your baseline work, admit to the hospital, and start with 2 to 3 mg/kg/d in 2 to 3 divided doses.
We are including insights for the surgical treatment of hemangiomas categorized by anatomic location on face.
Scalp lesions, when involuted will leave lipomatous bumps with less hair. Larger ones, if possible, should be treated before 3 1⁄2 to 4 months when the scalp is very loose, and primary closure may be simple. After that, cosmesis and regular flap decisions determines the next step. To minimize complications resulting from bleeding, all excisions in the scalp should be performed after the lesion and surrounding tissue have been infiltrated with “tumescent” solution including dilute epinephrine. Further, hemostatic polypropylene sutures (2–0 or 3–0 prolene) can be placed around the hemangioma to limit vascular inflow to the lesion. It should be kept in mind that the lesion never goes below the galea, whereas the plane of dissection around the lesion should be in the avascular subgaleal layers. Finally, one should resist the temptation to eliminate tiny dog-ears on scalp after excision. These will almost always resolve.
Forehead lesions with bulk will never just go away. Lesions in the upper forehead can almost always be horizontally excised without excess eyebrow movement due to the tissue expander-like effect of the hemangioma. Lower near brow lesions may require direct excision or flaps but the surgeon should not elevate the brow with this method. Secondary procedures to level the brow are common.
Nasal lesions can be excised in stages. We prefer the anatomic incisions of Burget and Menick with tip cartilage suturing over most published methods, but an open rhinoplasty method may be effective for some lesions, depending on the condition of the skin. The Burget and Menick approach focuses on the excision of all the very red areas, whereas open rhinoplasty methods may leave those areas with residual redness.
Laser treatment first, without trying other modalities, will lead to the loss of skin. Very blue lesions must be distinguished from venous malformations. Late treatment of nasal hemangiomas (after 8 years) may have distorted or destroyed underlying cartilage and the surgeon may need grafts to reinforce or repair the native nasal framework. A tissue glue is very helpful in all these nasal cases because delayed bleeding can occur. Closure is best with some deep sutures, glue, and arm restraints in infants and toddlers. Children who fall routinely can hit their noses and they should be carried or walked in the early postoperative period.
Lip lesions can be approached in many ways, and vermilion/mucosal reductions can work on many lesions (Fig. 7). The child will uniformly bite sutures out, opening the wounds, which will rarely change the final result. It may actually expedite involution, and parents must be warned and told what to expect.
Cheek lesions can be completely excised, partially excised, and then closed primarily or with a local flap. One should not be tempted to excise lesions only in the direction of the lesion; the dog-ear may end up to be disfiguring. For cheek lesions, even large ones, stay in the relaxed skin tension lines, no matter the size. All may require secondary work that will be small.
Eye lesions that cause astigmatism or visual block in any field must be treated early. Injections might be problematic. Total removal in the lower lid may not be crucial, but excessive weight causing ptosis (field loss) or astigmatism must be dealt with by surgically debulking. After this debulking, astigmatism may reverse to a great extent if accomplished early enough.
Parotid lesions usually resolve and occasionally, extra skin will need to be excised later. Facial nerve dissection for early removal is not advised.
For many patients with these lesions, the plastic surgeon can appear to be a hero. But they should not try to achieve this status too early (eg, with parotid lesions) or too late (like on the nose), which may affect patient’s body image. They should work with a good pediatrician who can monitor drug treatments, and always inform the parents; although hemangiomas may look blood filled, they can stop the bleeding if it occurs with pressure. They should inform the parents that revisions are common, but do not be afraid to shoot for the stars.