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Hemangiomas are the most common tumor of infancy and childhood, affecting approximately 10% of infants by the age of 1 year. The diagnosis can nearly always be made by the patient’s history and findings on physical examination. Prior to the classification system outlined by Mulliken and Glowacki, the natural course of hemangiomas was poorly understood and treatment was inconsistent, ranging from benign neglect to deforming surgical intervention. However, with an improved understanding of the natural course of hemangiomas, as well as advances in anesthesia, laser technology, medical therapy, and surgical methods, an aesthetic approach to facial hemangiomas was developed by the senior author (E.F.W.) and is reviewed in this article.
Arch Facial Plast Surg. 2005;7:301-306

Hemangiomas are the most common tumor of infancy and childhood, affecting approximately 10% of infants by the age of 1 year.¹ Facial hemangiomas tend to be solitary lesions and occur spontaneously, with a female predilection of nearly 4 to 1.² In the rare patient presenting with 4 or more cutaneous hemangiomas, consideration should be given to the possibility of coexisting visceral hemangiomas.³

The diagnosis can nearly always be made by the patient’s history and physical examination findings. Hemangiomas usually are seen within the first few weeks of life as small red papules or blue subcutaneous lesions that grow with variable intensity. Superficial hemangiomas will appear as bright red macular or papular lesions with well-defined borders (Figure 1A). Deep hemangiomas usually appear as bluish, subcutaneous masses (Figure 1B). At times, hemangiomas may contain features of both superficial and deep hemangiomas and are therefore called compound hemangiomas (Figure 1C).

The natural course of hemangiomas is characterized by 2 distinct clinical stages: proliferation and involution. Proliferation occurs during the first 12 months of life and, occasionally, as late as 18 months.

The growth pattern varies greatly in both timing and severity from one lesion to the next; however, a bimodal growth pattern
is frequently observed with an initial growth phase during the first few months of life and a subsequent growth phase at
4 to 6 months of age. In the histologic diagnosis, proliferating hemangiomas are characterized by plump, proliferating endothelial cells with barely perceptible vascular channels.

Proliferation is invariably followed by involution, which, by definition, follows the completion of proliferation. The onset of involution is characterized clinically by a decrease in the growth of the lesion with subsequent cessation of growth.

The cutaneous component will change from a bright red to a dark maroon; eventually, patches of ashen gray will predominate. The histologic examination findings will show that the plump endothelial cells give way to a gradual flattening of endothelial cells with progressive deposition of fibrous tissue and ectatic vessels.

HISTORICAL PERSPECTIVE

Areview of the medical literature reveals debate regarding the natural course of hemangiomas and a lack of consensus as to the appropriate therapy. Several authors in the past advocated aggressive management of facial hemangiomas, particularly if, according to Kiehn et al, ^4(p340) "growth is infiltrating into the adjacent areas and perhaps threatening invasion of the orbit or nasal cavity, with possible danger to vision or breathing." Aggressive treatment included not only surgical excision but also hot cautery, carbon dioxide snow, surface radium, radioactive implants, external beam radiation, interstitial y-radiation, and injection of sclerosing agents.⁵ However, the available treatment options of the mid–20th century had serious complications, such as subsequent malignant transformation associated with radiation therapy and poor cosmetic results with scars secondary to surgery and other ablative procedures.⁶

Figure 1. Whereas superficial hemangiomas appear as bright red macular or papular lesions (A), deep hemangiomas appear as bluish, subcutaneous masses (B); compound hemangiomas contain features of both superficial and deep hemangiomas (C).

While some authors advocated aggressive management of hemangiomas, subsequent articles published in the mid–20th century argued that nearly all hemangiomas eventually involuted with no residual deformity.^7-9 Therefore, a strong opinion developed that the appropriate treatment for hemangiomas was no treatment; this became known as benign neglect.¹⁰

The lack of consistency in the literature stemmed, in large part, from confusing vascular malformations with hemangiomas. In many classic articles, the term capillary hemangioma was used to describe what is now known as a port-wine malformation, and the terms strawberry naevi and cavernous hemangioma were used to describe what are now recognized as true hemangiomas. The classification system described by Mulliken and Glowacki¹¹ systematically delineated the difference between hemangiomas and vascular malformations. Furthermore, Waner and Suen¹² suggested that the description of true hemangiomas as capillary or cavernous created confusion; thus, the authors recommended a simple classification system that categorized lesions in the papillary dermis as superficial hemangiomas and lesions in the reticular dermis or subcutaneous tissue as deep hemangiomas. This system allowed for consistent definition in the literature and the ability to more accurately follow the natural course of hemangiomas.

Finn et al¹³ evaluated the clinical application of the new classification system. In summary, 60% of all lesions resolved with excellent results and 40% did not. Fifty percent of hemangiomas involuted early (before 6 years of age), and of those, 40% resulted in a substantial aesthetic deformity. Of the 50% of hemangiomas that involuted late (after 6 years of age), 80% were aesthetically unacceptable, with residual scars, redundant skin, or telangectasia. Furthermore, the psychological impact on a child with a facial hemangioma or its resultant scar cannot be underestimated. Children begin to develop selfawareness
at 18 to 24 months of age.¹⁴ Williams et al¹⁵ demonstrate a negative psychological impact on children with facial emangiomas when compared with a cohort group. Recent advances in anesthesia, laser technologies, medical treatment, and surgical methods, however, allow effective intervention to treat not only those lesions that would otherwise leave a grossly unacceptable result in years to come but also permit safe treatment to prevent children from suffering the psychological impact of having a facial hemangioma. Review of the contemporary medical literature regarding facial
hemangiomas in children demonstrates that 25% to 40% of hemangiomas will result in unacceptable cosmesis that may be improved with medical and/or surgical intervention.

MANAGEMENT OF HEMANGIOMAS

In an effort to determine which patients would benefit from intervention, Williams et al¹⁶ presented a retrospective review of 168 patients with hemangiomas and developed an algorithm for management (Figure 2).

PROLIFERATING HEMANGIOMAS

The decision to treat a proliferating hemangioma is based on the rate of proliferation, presence of ulceration or pending ulceration, and aesthetic result. Because nearly 60% of hemangiomas will involute with no cosmetic deformity, perhaps the most important aspect of treating the proliferating hemangioma is determining when to observe. Unlike the previous practice of benign neglect, observation is an active treatment and includes monitor-ing the rate of proliferation with evaluation every 3 months; if the hemangioma is in a cosmetically sensitive area and/or demonstrates interval change, then evaluation occurs every 2 to 4 weeks. If the hemangioma is proliferating rapidly, undergoes ulceration, or causes functional problems (visual impairment or nasal airway obstruction), pulsed-dye laser therapy is indicated. We deliver pulsed-dye laser treatments at 4- to 6-week intervals until the ulceration heals or proliferation is aborted and involution occurs. Currently, a wavelength of 595 nm is used to allow deeper penetration into the thickened aspects of the hemangioma. In our experience, the starting fluence with a 5-mm handpiece is usually approximately 9 J/cm², and this is increased until the desired degree of purpura is attained. Previous studies¹⁷ have demonstrated the efficacy of pulsed-dye laser treatment for proliferating hemangiomas with ulceration. The proposed theory explaining the use of the pulsed-dye laser for proliferating hemangiomas with ulceration is that the ulcer develops secondary to a growth rate of the hemangioma, which exceeds the growth rate of the epidermis.

The pulsed-dye laser treatment essentially decreases the growth rate of the hemangioma, thus allowing epithelialization.

Figure 2. Algorithm developed by the senior author (E.F.W.)¹⁶ for management of hemangiomas, depending on whether the hemangioma is in the proliferative phase (A) or involutional phase (B).

Although the pulsed-dye laser is useful for superficial hemangiomas, it is ineffective for deep hemangiomas. Intralesional laser therapy with the potassium-titanylphosphate or Nd:YAG laser has been suggested for this use, but scarring and damage to underlying structures has been a concern.¹⁸ The primary treatment modality for deep and compound hemangiomas that are either rapidly proliferating in a cosmetically sensitive area or a functional threat is systemic or intralesional steroids. The mechanism of action is not well delineated; however, treatment with corticosteroids may influence vasoconstriction and/or angiogenesis of the hemangioma.

Figure 3. Incision line is shortened with the use of an M-plasty. Z-plasty is used for scar camouflage,

INVOLUTING HEMANGIOMAS

Involution begins by 12 months but may occur as late as 18 months of age. As the lesion enters involution, it becomes necessary to observe the process for 8 to 12 months. As the hemangioma is monitored during early involution, it becomes apparent from serial photography and parent questioning whether the lesion demonstrates regression or remains stable. Hemangiomas that regress by the age of 2 years are classified as “early involuters,” and those hemangiomas that do not regress but rather remain stable are classified as “late involuters.” Hemangiomas that demonstrate early involution are monitored approximately every 6 months until 4 to 4½ years of age. If substantial deformity is evident, surgical therapy is offered for atrophic skin or fibrofatty residuum, whereas pulsed-dye laser therapy is offered for telangectasia. Surgical excision or laser treatment is offered for children with hemangiomas that are considered late involuters at approximately 2 years of age. In light of the aforementioned psychological impact that hemangiomas have on children, we elect to intervene at 2 years of age, before a child has a fully developed body image and before initiation of schooling.

SURGICAL PRINCIPLES

When undertaking surgical excision of a hemangioma, physicians should consider several important surgical principles:

• Consider surgical intervention during involution, thus resulting in less blood loss. With careful identification and management of the feeding vessels with bipolar cautery, very little bleeding should be encountered; therefore, blood products are not needed.
• Incisions are placed in the junction of facial units, subunits, or relaxed skin tension lines to camouflage scars and thus improve cosmetic outcomes.
• The incision line can often be shortened with the use of an M-plasty at 1 or both extremes of the incision (Figure 3).
• Typically, there is an easily defined surgical plane deep to the hemangioma; deeper structures are rarely involved.
  Incorporate atrophic scar tissue and ulceration with skin excision.
• The tumor acts as a tissue expander; therefore, adequate tissue is nearly always available for advancement as needed to close the excision site (Figure 4).
• When the hemangioma has an extensive superficial component and the entire lesion cannot be excised without violating aesthetic lines or making an unduly large incision, 10% of the hemangioma is left behind and allowed to undergo involution. Pulsed-dye laser therapy can be used postoperatively to treat the remaining hemangioma.

SPECIAL CONSIDERATIONS

Periorbital Hemangiomas

Surgical management of proliferating hemangiomas of the periorbital region is usually reserved for those that do not respond to steroid or laser therapy and are a functional concern. In addition to their cosmetic significance, these cases carry a more ominous threat of amblyopia.¹⁹ If amblyopia is suspected or the hemangioma proves to be rapidly proliferating, steroid therapy with intralesional injections or surgery is indicated. Intralesional steroid injection has an 80% to 88% favorable response rate with fewer complications than systemic steroid therapy.²⁰

Surgical excision for periorbital hemangiomas has been recommended by authors²¹ both as primary treatment and for medical treatment failures. The surgical technique involves an incision in the eyelid crease or transconjunctival approach, followed by sharp dissection around the extent of the tumor and en bloc removal. However, when a portion of the tumor adheres to the surrounding orbital structures and is difficult to remove, a portion of the hemangioma is left behind. The remaining hemangioma is treated with intralesional steroid or pulsed-dye therapy.

Figure 4. At times, the hemangioma functions as a tissue expander, thus enabling the surgeon to perform excision and closure with minimum tension. A, preoperative; B, intraoperative; and C, postoperative views.

Figure 5. External rhinoplasty approach to deep hemangioma of the nose. A, preoperative; B, intraoperative; and C, postoperative views.

Figure 6. Hemangioma of the lip. Placement and reconstruction of the white roll is essential in lip reconstruction. A, preoperative; B, intraoperative; and C, postoperative views.

Nasal Hemangiomas

Hemangiomas of the nasal tip may cause deformity of the lower lateral cartilages and result in nasal obstruction. Furthermore, the psychological impact of nasal hemangiomas cannot be overstated. The incisions must be placed within the aesthetic subunit junctions of the nose, they must provide access for complete removal of the hemangioma, and they must allow for both horizontal and vertical trimming of the redundant nasal soft tissue envelope for repositioning; at times, an external rhinoplasty approach is indicated. Once the hemangioma is removed, the alar cartilages should be repositioned and sutured to place the domes in anatomical approximation. Following this, the soft tissue envelope is repositioned, trimmed, and sutured into position (Figure 5).

Lip Hemangiomas

Zide et al²² presented several observations from their experience in surgical excision of lip hemangiomas. Many of the surgical principles mentioned in this section are pertinent; however, there are some special considerations with the lip. As hemangiomas grow within the red or white lip, expansion of the lip occurs. Removal of the lesion requires that both the vertical and horizontal proportion of the red and white lip are adjusted to the normal anatomical relationship. Usually the hemangioma will not invade through the muscle; it is advisable to not resect orbicularis muscle. Placement and reconstruction of the white roll is essential in lip reconstruction because malposition of this border is obviously perceptible (Figure 6).

CONCLUSIONS

Hemangiomas are the most common neoplasm of infancy and early childhood. Because of an improved understanding
of the natural course of hemangiomas and a more accurate classification scheme, benign neglect is no longer in favor. Rather, observation is encouraged and, for most hemangiomas, no further intervention is necessary. However, if the hemangioma proves to be rapidly proliferating, ulcerative, in a cosmetically and/or functionally sensitive area, or is a late involuter, then further intervention with steroid therapy, pulsed-dye laser, or surgery may be warranted. Recent advances in anesthesia, laser technologies, medical treatment, and surgical methods allow effective intervention to treat not only those lesions that would otherwise leave a grossly unacceptable result in years to come but also permit safe treatment to prevent children from suffering the psychological impact of a facial hemangioma.

Accepted for Publication: May 18, 2005.
Correspondence: Edwin F. Williams III, MD, Williams Center for Facial Plastic Surgery, 1072 Troy Schenectady Rd, Latham, NY 12110 (edwilliams@nelasersurg.com).

REFERENCES

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