The physical examination usually confirms the diagnosis, particularly if the hemangioma is located in the dermis layer of the skin. Superficial hemangiomas display a classic strawberry red pigmentation. However, hemangiomas in the subcutaneous tissue appear bluish and have been mislabeled “cavernous” with the presumption that they contain dilated venules, but microscopic examination reveals proliferating capillaries identical to the dermal type. It is simply the deeper location that renders a bluish hue. In contrast, true venous vascular malformations tend to be softer and more compressible and may significantly expand when the head is held in a dependent position. In the older child, a venous malformation may also contain hard nodules (phleboliths), or they may show rapid painful enlargement and prolonged tenderness if rupture of the fragile ectatic vessels results in a transient hematoma.

Hemangiomas are often mixed lesions that have both a dermal and a subcutaneous component, with potential submuscular extension into the orbit. One will commonly see a central island of protruding bright red tissue atop a mound of bluish-tinted skin. It is the superficial component that is more likely to result in ulceration and permanent dermal scarring. Overdistention of the skin envelope may also result in atrophic skin redundancy, and incomplete resolution of the mass may result in residual fibrofatty tissue that will benefit from later judicious debulking. Purely subcutaneous and intramuscular hemangiomas may also expand the overlying skin, but there is greater potential for normal skin tone and texture following involution if the integrity of the dermis has not been violated. Twenty percent of infants will have more than one hemangioma, which may grow and regress at different rates and may differ in maximum size and degree of involution.

Hemangiomas in the periorbital region may involve either the upper or lower eyelid, the brow, the forehead, the paranasal regions and nasal dorsum, the cheek, or the temple. The orbit itself may be involved. Massive hemangiomas may involve all of these areas simultaneously.

Particular attention should be given to determining whether the hemangioma already obstructs vision or if continued growth will result in potential obstruction of the visual field. Extensive involvement of the head may also be associated with subglottic involvement, and any evidence of stridor or respiratory compromise warrants immediate otorhinolaryngology evaluation. Infants with large plaque-like facial hemangiomas and evidence of macrocephaly, enlarging head circumference, or unusual ophthalmologic findings (choroidal hemangioma, cryptophthalmos, microphthalmus, exophthalmos, and cataract) may have associated Dandy–Walker malformations (hypoplasia or absence of the cerebellar vermis and posterior fossa cysts) (Fig. 7.3). These patients may also display apnea, vomiting, posturing, developmental delay, and hemiparesis. The diagnosis can be confirmed with a computed tomography (CT) or a magnetic resonance imaging (MRI) scan [4].

The majority of hemangiomas will not require radiologic studies for confirmation, but the extent of periorbital hemangiomas is best determined with MRI and magnetic resonance angiography (MRA). Gadolinium-enhanced MRI scanning demonstrates progressive and total filling of intraorbital vascular lesions. Distortion of the globe and orbital walls from mass effect can be appreciated. This will also help to distinguish a hemangioma from a venous or lymphatic malformation or a high-flow arteriovenous malformation. While contrast-enhanced CT scans will also show the extent of hemangiomas, the ability of MR scanning to demonstrate flow characteristics and the lack of radiation exposure makes it preferable to CT scanning in the vast majority of patients.

Arteriography is not necessary, although it has been described in the management of problematic hepatic hemangiomas associated with high-output cardiac failure, where embolization is a potential treatment option. Plane films are generally not useful. Hemangiomas rarely show bony distortion, although there may be some orbital enlargement from the expanding mass. In contrast, vascular malformations may show skeletal overgrowth or, in high-flow situations, bone destruction. The presence of a phlebolith would favor a venous or venolymphatic malformation instead of a hemangioma as the proper diagnosis. Ultrasound has been utilized to monitor hepatic hemangiomas and to scan the body for other vascular anomalies.

In the rare event that a biopsy is performed, typical infantile hemangiomas will demonstrate high immunoreactivity for glucose transporter protein 1 [5]. Tissue that is GLUT1 positive is considered diagnostic of classic hemangiomas, whereas the less common rapidly involuting congenital hemangioma (RICH) and non-involuting congenital hemangioma (NICH) are GLUT1 negative.

The stimulus for angiogenesis usually persists until 6–12 months of age, but may extend to 18 months. As the hemangioma reaches the slow involutional phase, a plateau in growth is noted, the thrombosis and sclerosis of the tiny capillary vessels results in patchy gray pigmentation, and tissue turgor begins to diminish.

The average hemangioma takes about 5 years to fully involute, although some may take several years more. It is estimated that about 70% of hemangiomas will regress satisfactorily enough so as to need no intervention [6]. However, that still leaves at least 30% of patients who will have significant residual deformity (Fig. 7.4).

Additionally, there are certain regions of the body where the risk of unacceptable scarring and residual deformity requiring reconstructive surgery is more likely, particularly when the lips, cheeks, and periorbital regions are involved. Additionally, since obstruction of the visual axis by an enlarging hemangioma for as little as 1 week during the first year of life can result in deprivation amblyopia, periorbital hemangiomas must be regarded as a potential ophthalmologic emergency (Fig. 7.5). There is a linear corelation between the duration of complete eye obstruction and the loss of visual acuity. Refractive errors can result from the mass effect of an adjacent hemangioma on the globe or cornea (anisometropic amblyopia), and hemangiomas involving the extraocular muscles can result in strabismus due to distortion and scarring of the muscles despite later involution. Retrobulbar hemangiomas can also compress the optic nerve.

The plastic surgeon who deals with hemangiomas should have a close working relationship with a multidisciplinary team of specialists familiar with all vascular anomalies. In addition to an interventional radiologist, dermatologist, and nursing coordinator, other team members may include a hematologist–oncologist, an orthopedic surgeon, and, when the periorbital region is involved, an oculoplastic surgeon and/or ophthalmologist.

While the plastic surgeon may choose to primarily manage the patient, any infant with a hemangioma that already does or may eventually obstruct even part of the visual field should be immediately evaluated by an ophthalmologist or oculoplastic surgeon. Thomson et al. [7] reviewed 51 patients with eyelid hemangiomas. Of these, 17 patients who had normal vision had only partial eyelid obstruction (smaller or more localized hemangiomas) that involuted at an earlier age (2.8 years) compared with patients with ultimately compromised vision. These favorable hemangiomas occupied only one-third of the lid margin, did not extend beyond the region of the eyelid, and/or involved only the lower lid.

Seven patients with isolated anisometropic (astigmatic) amblyopia had larger and bulkier lesions that took longer to resolve (4 years). Fifteen patients with deprivation amblyopia had large hemangiomas extending beyond the eyelid region that completely obstructed the eye for variable periods and, on average, took 4.5 years to resolve. They recommended measures to splint the affected eye open while patching the dominant eye. Lesions that were refractory to conservative measures were candidates for surgical excision if frequent ophthalmologic examinations showed developing amblyopia.

At our institution, the diagnosis of amblyopia secondary to an enlarging hemangioma in an infant is suggested by an abnormal Teller preferential looking test. A consultation between the pediatric ophthalmologist or oculoplastic surgeon and the plastic surgeon determines the need for a patching schedule for the contralateral side, and strong consideration is given for the immediate institution of suppressive antiangiogenic therapy.

Prior to 2008, intralesional or systemic steroids were the treatment of choice for problematic hemangiomas [8–11]. Oral steroid therapy was highly effective in up to 90% of patients, and regression was often observed within a week of steroid institution. However, the common side effects of steroids which included irritability, growth suppression, gastrointestinal upset, and hypertension prompted ophthalmologists to try intralesional injection with a combination of a rapid-acting steroid such a betamethasone and a long-acting steroid such as triamcinolone.

Intralesional steroid injection was promoted for the treatment of periorbital hemangiomas as early as 1978 by Mazzola [12], who treated 11 patients, with rapid benefit noted in 7 patients after 1 or 2 injections. Kushner was a strong proponent of intralesional steroid therapy in the ophthalmologic literature [13], citing the ease of administration, speed of action, paucity of side effects, and repeatability of injections. Using both a rapid-acting (betamethasone) and a long-acting (triamcinolone) steroid combination, visible change (blanching) was noted in as early as 1–2 days and dramatic results by 1 week. Doses ranged from 40 to 80 mg triamcinolone and 6 to 12 mg betamethasone per injection, delivered through 27-gauge needles. Most patients received two injections a month apart. In a later report, Kushner [14] limited his injections to 40 mg triamcinolone and 6 mg betamethasone because of potential complications of higher doses in small infants.

The safety of intralesional therapy for periorbital hemangiomas has been questioned [15, 16], with concerns noted regarding the use of general anesthesia during injection, the development of cholestrin plaques at the site of injection, and potential hematoma development and localized necrosis and scarring of the skin where concentrations of steroid might be heavy. Fat atrophy can also occur, with subsequent soft tissue contour abnormalities. Kushner himself noted the theoretical possibility of retinal artery occlusion with injections into retrobulbar or nasal mucosal hemangiomas. Furthermore, steroid injection into a vascular mass probably does not totally avoid systemic dosing, and the beneficial effect may not be due entirely to local effects.

Bilyk et al. [17] provide a comprehensive overview of treatment options for periorbital hemangiomas of infancy, again listing the risks and benefits of intralesional versus systemic steroid therapy. Other treatment options of historical interest include cryotherapy, which can cause skin scarring and hypopigmentation; surgical ligation of the feeding arteries, which has equivocal results on involution; radiation, which can cause damage to the lens, retina, periorbital bone, and overlying skin; sclerosing agents, which can cause significant destruction and scarring of local tissues; and embolization, which risks infarction of critical local tissues. While embolization has been reported, retinal artery occlusion and blindness is a significant risk. Embolization appears to have a more appropriate role in the treatment of high-output cardiac failure associated with visceral hemangiomas or as an adjunct to excision of high-flow arteriovenous malformations.

Interferon [18–20] is also of mostly historical interest, having received a great deal of enthusiasm in the 1990s as an alternative to steroid therapy, but its many significant side effects including neurologic sequelae restricted its use to the most serious life- and vision-threatening situations where steroids were ineffective. With the advent of beta blocker therapy in 2008, both steroids and interferon have taken a back seat in the treatment of problematic hemangiomas.

Leauté-Labréze et al. reported 11 patients treated with propranolol who showed significant hemangioma regression [21]. The mechanism of action may be related to vasoconstriction, suppression of vascular endothelial growth factor and basic fibroblast growth factor, and stimulation of capillary endothelial cell apoptosis. At the Children’s Hospital of Philadelphia, propranolol has replaced steroids as first-line therapy for problematic hemangiomas, including those involving the periorbital area and airway. Initially, because of the potential cardiovascular, pulmonary, and hypoglycemic effects of beta blocker therapy, the propranolol protocol required hospital admission for blood pressure, heart rate, and glucose monitoring, while children were gradually administered therapeutic levels. Both inpatient and outpatient propranolol administration were directed by our dermatology colleagues. Currently, the dermatology protocol follows the FDA guideline that children of 45 weeks gestation or older are candidates for outpatient propranolol therapy if they have a normal EKG. Blood pressure and heart rate are monitored for 1–2 h during an initial partial dose administration and again the following week when the full dose is administered. Propranolol appears to have fewer adverse side effects and may even be more effective than steroids in suppressing hemangiomas and causing them to involute more rapidly (Figs. 7.6 and 7.7).

A review of 100 patients with periocular hemangiomas treated with propranolol revealed it was the first-line treatment in the majority, with improvement in 96% of patients and minimal side effects. The most common dose was 2 mg/kg/day [22].

Topical beta blocker in the form of timolol maleate gel, 0.25% applied twice daily, may also be effective and is easily applied by parents [23].

Historically, a variety of vascular lesion lasers have been used for the treatment of undesirable cutaneous blood vessels [24–29]. All operate on the principle that blood (oxyhemoglobin) absorbs certain wavelengths of light preferentially, thereby allowing selective or semiselective destruction of blood vessels by heating the blood inside. These have included argon (wavelength 488,515 nm; blue-green light), KTP (wavelength 532 nm, green light), pulsed yellow dye (current wavelength 595 nm, yellow light), tunable yellow dye (wavelength 577–630 nm, usually tuned to 585 nm), and copper vapor (wavelength 578 nm, yellow light) lasers. The pulsed yellow dye laser delivers a high-energy pulse of laser light that causes rapid expansion and rupture of the blood vessels, resulting in purpura. All of the other lasers cause vascular coagulation and blanching. None of the lasers can penetrate more than 1–2 mm into the skin, and therefore treatment is limited to thin cutaneous lesions. Neodymium:YAG lasers (wavelength 1064) will penetrate 5–6 mm, but the light is not specifically absorbed by blood. It will cause nonspecific deep thermal coagulation, and therefore has potential in treating highly vascular lesions, but at the risk of significant thermal damage to surrounding tissue.

The pulsed yellow dye laser has become well accepted as the laser of choice for pediatric port-wine stains, because of its low risk of scarring, favorable results, and rapidity and ease of delivery [30]. It may be very effective in aborting small flat hemangiomas, but in many cases, rebound growth may warrant repeated laser treatments until the hemangioma undergoes permanent regression. In such cases, interim oral propranolol or topical application of timolol may help to suppress rebound growth.

In general, thick hemangiomas do not appear to respond well to laser because of the limited depth of penetration. They may lose their bright red surface pigmentation without decreasing in size and may still require surgical debulking in the case of an obstructing hemangioma.

The pulsed dye laser is best used for early flat hemangiomas or for those that have regressed but have left persistent vascular pigmentation in the dermis (Fig. 7.8).

Small hemangiomas can be excised or debulked using standard surgical techniques. If the surgeon has the luxury of being able to stay away from the hemangioma, the dissection is through normal tissue planes, and bleeding is minimal. Usually there are 1–2 feeding vessels, and vascular control and hemostasis are straightforward. Ideally, incisions are placed inconspicuously or along natural expression lines, but this is not always possible depending on the size and location of the hemangioma (Fig. 7.9).

Certain problematic periorbital hemangiomas, however, are not amenable to complete excision, and debulking becomes necessary to prevent or relieve visual obstruction. In such cases, incisions must be made within the hypervascular tissue, and hemostasis may be difficult. Electric cautery, particularly the bipolar cautery, is extremely useful to shrink and seal off engorged capillary vessels, and the bipolar forceps can even be used to dissect through the hemangioma by pinching the tissue with the tips of the forceps. Standard cautery tends to be less hemostatic, but may be useful for making initial incisions. Vaporization with a CO₂ laser can also prove useful, but the laser does not coagulate as well as other lasers that target oxyhemoglobin. After removing the necessary bulk of the hemangioma, residual raw edges must be meticulously cauterized or oversewn to avoid a hematoma. Suture closure with a tapered needle is preferable to a cutting needle, to avoid further bleeding.

Regressed hemangiomas that have left redundant skin and fibrofatty tissue as well as persistent vascular pigmentation may be treated by a combination of excision and pulsed yellow dye laser photocoagulation (Fig. 7.10).