CLINICAL STUDY

Algorithmic Approach to the Management of Hemangiomas

Ali M. Soltani, MD,* and John F. Reinisch, MD
Abstract: Hemangiomas are a common benign vascular tumor that can occur in all parts of the body. These lesions can be a distressing sight for both patient and parent. This unique vascular tumor has characteristic phases of growth. Historically, these tumors have been treated nonoperatively, but with variable results. Often, the residual-resolved tumor produces contour defects, unpredictable scarring, and pigmentation problems. The authors devised an algorithmic diagram for treating hemangiomas based on a 30-year experience with treating these unique tumors. This step-by-step method delineates the thinking method that should be used when presented with a difcult hemangioma. This algorithmic method takes into account a multifactorial approach to management. This includes anatomic location, growth velocity, treatment response, expected outcome, and psychosocial considerations. Key Words: Hemangioma, plastic surgery, management (J Craniofac Surg 2011;22: 585Y588) appearance. The hemangioma does not disappear but is instead replaced by a brofatty residuum, consisting of adipose tissue and scar tissue underneath a thin, wrinkled-appearing top skin layer. If the child had signicant ulcerations during the initial stages of the hemangioma, the scarring will be much worse and more noticeable after involution. The typical treatment scenario of hemangiomas involves conservative management and watchful waiting. The nonoperative treatment of these hemangiomas that is advocated most in the literature can cause additional anxiety to parents. It has been shown that 63% of parents with children with hemangiomas are concerned that their children will be teased at school.3 In addition, Weinstein et al demonstrated that many children with hemangiomas had negative reactions from strangers, social stigmatization, sadness, stress, and low self-esteem.4 It is this psychosocial disturbance that can be traumatizing to both child and parents, with lasting effects on condence and performance in school and later in life. In those hemangiomas that are large, rapidly growing, or ulcerating, intralesional or oral steroids have had measured success and are a standard treatment method.5,6 Further, treatments such as interferon >-2a have been used for refractory lesions but with common adverse effects such as fever, depression, and ulike symptoms reported.7,8 Interferons can also have signicantly rarer but devastating neurologic and hematologic sequelae such as spastic diplegia or febrile neutropenia.9,10 Laser treatment has been advocated by the dermatological community as a cure-all for vascular lesions. Although effective for supercial lesions as port-wine stains, data on cutaneous hemangiomas are controversial because of low penetrance and nonselectivity of tissue destruction. Recently, signicant adverse effects of laser-treated hemangiomas have been shown to occur immediately or appear many years later. Severe scarring, freckling, ulceration, and even hemorrhage have been reported in the literature.11,12 Surgical treatment is well established as a safe and effective treatment in the management of hemangiomas but is often viewed as a last resort option. Case series published by Reinisch et al13 and McCarthy et 14al have supported the early surgical approach to hemangiomas in certain anatomic areas. Exciting, newer systemic treatments with A-blockers are a promising option in the management of hemangiomas because of a mild adverse effect prole.15 This publication delineates an algorithmic approach to managing hemangiomas, using a multidisciplinary treatment focus that emphasizes the psychosocial effect of the lesion.

nfantile hemangiomas are the most common benign tumor of infancy, occurring in up to 12% of infants.1 These vascular lesions are distressing to parents, who most frequently visit their pediatrician or family practitioner for initial management and often the plastic surgeon is consulted for large, complex, or distressing hemangiomas. Typically, hemangiomas are not present at birth but rather arise during the rst year of life and undergo phases of growth and dissipation. The proliferating phase generally occurs in the rst 3 to 6 months of life. In this phase, the tumor has an exponential increase in endothelial cells, which is clinically distinguished by a bright red and swollen mass that is engorged with blood and has turgor on palpation.2 Next, the involuting phase occurs at around a year and a half of life, characterized by a softening of the tumor, fading of color, and decreased capillary factors. Finally, the hemangioma reaches it involuted phase, with a signicantly different

From the *Division of Plastic Surgery, Department of Surgery, Keck School of Medicine, University of Southern California; and Division of Plastic Surgery, Department of Surgery, Cedars-Sinai Medical Center, Los Angeles, California. Received July 30, 2010. Accepted for publication September 25, 2010. Address correspondence and reprint requests to Ali Soltani, MD, 1510 San Pablo St, Suite 415, Los Angeles, CA 90033; E-mail: asoltani@surgery.usc.edu Presented at the California Society of Plastic Surgeons meeting, May 29, 2010. The authors report no conicts of interest. Copyright * 2011 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0b013e31820873ac

MATERIALS AND METHODS

The authors devised an algorithmic management protocol capturing the evolving strategy used by the senior author in his 30-year career treating more than 1200 hemangioma patients with successful outcomes at the Childrens Hospital Los Angeles and Cedars-Sinai Medical Center.

RESULTS AND DISCUSSION

Although it is agreed that not all hemangiomas need surgical intervention, each should be evaluated by a specialist in vascular

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Copyright 2011 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

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FIGURE 1. Initial evaluation of hemangioma referral. anomalies and managed using a multifactorial approach, including anatomic location, response to medication, growth velocity, and psychosocial issues. We have developed an algorithmic approach to managing this difcult pediatric problem during a 30-year experience. This algorithm can be used by many different specialties and takes into account the many subtleties that should be addressed when treating patients with hemangiomas. When referred a patient with possible hemangioma, the rst step is visual conrmation that the lesion is indeed a hemangioma (Fig. 1). Many practitioners still make the mistake of diagnosing other vascular lesions such as vascular malformations or granulomas as hemangiomas. In general, they are not present at birth and have a rounded and lobular appearance that displaces rather than invades the adjacent skin. In the proliferating phase, the color is generally beefy red or pink. The tumor has a spongy texture that will inate after pressure is applied. An involuting tumor may have a mottled skin appearance and a more fatty texture. Lastly, an involuted hemangioma has a papery, wrinkled skin overlying a fatty, rubbery residuum. Although some authors have described ultrasound, computed tomographic scanning, or magnetic resonance imaging for conrmation of diagnosis, in most instances, it is not necessary to conrm the clinical diagnosis. Further, biopsy or other laboratory testing is not recommended for conrmatory diagnosis of these lesions. Typically, a hemangioma is diagnosed solely on clinical judgment, and additional tests that add cost and possible radiation exposure are not worthwhile. Once the diagnosis is secure, the next critical step is determining whether the lesion is visible with the child wearing clothes. This may vary by culture, ethnicity, and even season, but in general, the scalp, trunk, abdomen, upper legs, and upper arms are obscured by clothing and hair. These areas are treated signicantly different from visible areas of the body. If the lesion is quite noticeable and visible to the naked eye with clothes, it may cause great distress to the child and be a source of embarrassment. Many children are teased and mocked when they reach school-age or even earlier. Thus, these hemangiomas should be treated more aggressively than lesions not visible at a conversational distance (Figs. 2 and 3). Most hemangiomas in nonvisible areas can be safely observed as the child develops. Most of these hemangiomas will eventually involute and leave behind the familiar characteristics of hemangiomas: thin, wrinkled skin and a brofatty residuum. In the case of abnormal or excessive scarring, contour deformities, or distortion, surgical therapy can be indicated for the involuted lesion. There are exceptions to observing these hidden hemangiomas, as seen in the algorithm. In Figure 4, an infant with a severe recalcitrant bleeding hemangioma that was resistant to conservative management is shown. Eventually, the patient required surgical excision with good results. Hemangiomas that are proliferating rapidly or increasing in size may be treated with intralesional steroids initially. If refractory to steroid injections, systemic therapy would be indicated. This may be accomplished with systemic steroids or A-blockers. Recent published reports have used the A-blocker propranolol for difcult-to-treat refractory hemangiomas.15 They have minimal adverse effects in relation to other medical treatments, such as systemic steroids. A-Blocker therapy represents a possible paradigm shift in the management of hemangiomas, with propranolol and acebutolol being evaluated in many trials worldwide.16,17 The indications and uses of A-blockers for hemangiomas are still nascent but have been incorporated into our algorithm using the most available literature and opinions. Further, A-blocker therapy is becoming more compelling than systemic steroid therapy. In our opinion, we do not recommend the use of interferon > or chemotherapeutic agents because of their adverse effect proles.9,10 There are many other medical and surgical options that have fewer risks than these medications to justify their use.

FIGURE 2. Hemangioma algorithm for nonvisible lesions. * 2011 Mutaz B. Habal, MD

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Algorithm in Managing Hemangiomas

FIGURE 4. A 4-month-old infant boy with a large ulcerating hemangioma over the upper back (left). Appearance 1 year later after excision and closure (right). mental treatment with topical A-blockers has been reported for these periorbital hemangiomas and represents an exciting option for hemangiomas in general.18 Here is an example of a successfully treated patient with a hemangioma of the forehead with excellent aesthetic results (Fig. 5). This 2-year-old boy presented with a large unsightly hemangioma of the forehead, which was being managed nonoperatively. The hemangioma was excised, and long-term follow-up with patient and family revealed great satisfaction with the outcome and timeliness of treatment. As the hemangioma involutes and ceases to proliferate, the lesion can be resected with little chance for regrowth. Surgical excision should be tailored to each patient and each particular anatomic location. In many instances, total resection is not warranted, but debulking of the tumor to achieve good symmetry and contour is the best option. The operative goal is to envision how the nal involuted hemangioma might appear in the child after the color fades. Totally involuted hemangiomas can often be problematic to the growing child and adolescent. If severe scarring, contour deformities, undesirable pigmentation, or skin texture is present in the involuted hemangioma, we would advise surgical excision. In Figure 6, a young girl is shown, who presented late with a completely involuted hemangioma. The resulting lesion was mottled with hypopigmentation and hyperpigmentation with contour irregularities. The patient desired surgical treatment of this involuted lesion with satisfactory long-term results.

FIGURE 3. Hemangioma algorithm for visible lesions.

Ulcerating hemangiomas present a distressing sight to the parents of many children with hemangiomas. These lesions are highly vascular but typically do not produce severe enough bleeding to cause systemic compromise. Local wound care can heal these ulcerated areas including lidocaine gel, hydrocolloid dressings, or other wound dressings. Improved hygiene of the hemangioma can reduce the ulceration and allow the open areas to heal. However, refractory ulceration occasionally makes surgical excision the optimal choice to provide the fastest recovery for patient and parents. Hemangiomas present on visible areas, most often in the head and neck region, should be treated aggressively. Any surgical excision should be avoided during the proliferative phase of the tumor. If the lesion is large and periorbital, the patient should be evaluated by an ophthalmologist. Periorbital hemangiomas need to be treated expediently to avoid amblyopia in the developing child. This may include intralesional steroids, systemic steroids, or A-blockers. If these fail, the child may need surgical excision expeditiously to avoid developmental problems in the eye. Experi* 2011 Mutaz B. Habal, MD

FIGURE 5. A 2-year-old boy with a highly conspicuous hemangioma over the right side of the forehead (left). Appearance 1 year later after excision and advancement ap closure (right).

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FIGURE 6. A 9-year-old girl with pigmentation and contour irregularities after complete involution of her facial hemangioma. Lastly, there have been reports of hemangiomas being treated with lasers in the literature. We typically do not advocate their use except for occasional residual telangiectasia or pigmentation that may occur around hemangiomas. Lasers do not penetrate deep enough to have value in treating hemangiomas and generally cause additional scarring when the lesion does eventually involute. The management of hemangiomas has evolved greatly in the past few decades, with new developments and techniques changing the confusing landscape. Standard treatments such as observation, intralesional steroids, and surgical excision still apply; however, newer modalities such as A-blocker therapy and laser treatments have complicated the eld. Sorting out all of these evolving techniques and treatments is important in tailoring it to each patient for optimal success, and we hope this algorithm assists in simplifying the decision process.

REFERENCES
1. Esterly NB. Cutaneous hemangiomas, vascular stains and malformations, and associated syndromes. Curr Probl Pediatr 1996;26:3Y39 2. Weinstein JM, Chamlin SL. Quality of life in vascular anomalies. Lymph Res Biol 2005;3:256Y259 3. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982;69:412Y420

4. Sandler S, Adams S, Taylor C. Paediatric vascular birthmarksVthe psychological impact and the role of the GP. Aust Fam Phys 2009;38:169Y171 5. Bennett ML, Fleischer AB, Chamlin SL, et al. Oral corticosteroid use is effective for cutaneous hemangiomas: an evidence-based evaluation. Arch Dermatol 2001;137:1208 6. Boon LM, MacDonald DM, Mulliken JB. Complications of systemic corticosteroid therapy for problematic hemangioma. Plast Reconstr Surg 1999;104:1616Y1623 7. Ezekowitz RAB, Mulliken JB, Folkman J. Interferon alfa-2a therapy for life-threatening hemangiomas of infancy. N Engl J Med 1992;326:1456Y1463 8. Kaselas C, Tsikopoulos G, Papouis G, et al. Intralesional administration of interferon A for the management of severe haemangiomas. Pediatr Surg Int 2007;23:215Y218 9. Dubois J, Hershon L, Carmant L, et al. Toxicity profile of interferon alfa-2b in children: a prospective evaluation. J Pediatr 1999;135:782Y785 10. Barlow CF, Priebe CJ, Mulliken JB. Spastic diplegia as a complication of interferon alfa-2a treatment of hemangiomas of infancy. J Pediatr 1998;132:527Y530 11. Bayliss SJ, Berk DR. Freckling 11 years after pulsed dye laser therapy for an infantile hemangioma: coincidence or a previously unrecognized complication? Clin Pediatr (Phila) 2008;47: 189Y190 12. Witman PM, Wagner AM, Scherer K, et al. Complications following pulsed dye laser treatment of superficial hemangiomas. Lasers Surg Med 2006;38:116Y123 13. Reinisch JF, Kim RY, Harshbarger RJ, et al. Surgical management of parotid hemangioma. Plast Reconstr Surg 2004;113:1940Y1948 14. McCarthy JG, Borud LJ, Schreiber JS. Hemangiomas of the nasal tip. Plast Reconstr Surg 2002;109:31Y40 15. Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al. Propranolol for severe hemangiomas of infancy. N Engl J Med 2008;358: 2649Y2651 16. Holmes WJ, Mishra A, Gorst C, et al. Propranolol as first-line treatment for infantile hemangiomas. Plast Reconstr Surg 2010;125:420Y421 17. Bigorre M, Van Kien AK, Valette H. A-Blocking agent for treatment of infantile hemangioma. Plast Reconstr Surg 2009;123:195eY196e 18. Guo S, Ni N. Topical treatment of capillary hemangioma of the eyelid using A-blocker solution. Arch Ophthalmol 2010;128:255Y256

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