Algorithmic Approach to the Management of.47

8/13/2019 Algorithmic Approach to the Management of.47

1/4

Algorithmic Approach to the Managementof 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 dis-

tressing sight for both patient and parent. This unique vascular tu-

mor 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 heman-

giomas 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 difficult hemangioma. This

algorithmic method takes into account a multifactorial approach to

management. This includes anatomic location, growth velocity, treat-

ment response, expected outcome, and psychosocial considerations.

Key Words: Hemangioma, plastic surgery, management

(J Craniofac Surg2011;22: 585Y588)

Infantile hemangiomas are the most common benign tumor of in-fancy, occurring in up to 12% of infants.1 These vascular lesionsare distressing to parents, who most frequently visit their pediatri-

cian or family practitioner for initial management and often theplastic surgeon is consulted for large, complex, or distressinghemangiomas.

Typically, hemangiomas are not present at birth but ratherarise during the first year of life and undergo phases of growth anddissipation. The proliferating phase generally occurs in the first 3to 6 months of life. In this phase, the tumor has an exponentialincrease in endothelial cells, which is clinically distinguished by abright red and swollen mass that is engorged with blood and hasturgor on palpation.2 Next, the involuting phase occurs at arounda year and a half of life, characterized by a softening of the tumor,fading of color, and decreased capillary factors. Finally, the hem-angioma reaches it involuted phase, with a significantly different

appearance. The hemangioma does not disappear but is insteadreplaced by a fibrofatty residuum, consisting of adipose tissue andscar tissue underneath a thin, wrinkled-appearing top skin layer. Ifthe child had significant ulcerations during the initial stages ofthe hemangioma, the scarring will be much worse and more no-ticeable after involution.

The typical treatment scenario of hemangiomas involvesconservative management and watchful waiting. The nonoperativetreatment of these hemangiomas that is advocated most in theliterature can cause additional anxiety to parents. It has been shown

that 63% of parents with children with hemangiomas are con-cerned that their children will be teased at school.3 In addition,Weinstein et al demonstrated that many children with hemangiomashad negative reactions from strangers, social stigmatization, sadness,stress, and low self-esteem.4 It is this psychosocial disturbancethat can be traumatizing to both child and parents, with lastingeffects on confidence and performance in school and later in life.

In those hemangiomas that are large, rapidly growing, orulcerating, intralesional or oral steroids have had measured successand are a standard treatment method.5,6 Further, treatments suchas interferon >-2a have been used for refractory lesions but withcommon adverse effects such as fever, depression, and flulikesymptoms reported.7,8 Interferons can also have significantly rarerbut devastating neurologic and hematologic sequelae such as spasticdiplegia or febrile neutropenia.9,10 Laser treatment has been advo-

cated by the dermatological community as a cure-all for vascularlesions. Although effective for superficial lesions as port-winestains, data on cutaneous hemangiomas are controversial because oflow penetrance and nonselectivity of tissue destruction. Recently,significant adverse effects of laser-treated hemangiomas have beenshown to occur immediately or appear many years later. Severescarring, freckling, ulceration, and even hemorrhage have beenreported in the literature.11,12 Surgical treatment is well establishedas a safe and effective treatment in the management of hemangio-mas but is often viewed as a last resort option. Case series publishedby Reinisch et al13 and McCarthy et 14al have supported the earlysurgical approach to hemangiomas in certain anatomic areas. Ex-citing, newer systemic treatments with A-blockers are a promisingoption in the management of hemangiomas because of a mildadverse effect profile.15 This publication delineates an algorith-

mic approach to managing hemangiomas, using a multidisciplinarytreatment focus that emphasizes the psychosocial effect of the lesion.

MATERIALS AND METHODS

The authors devised an algorithmic management protocolcapturing the evolving strategy used by the senior author in his30-year career treating more than 1200 hemangioma patients withsuccessful outcomes at the Childrens Hospital Los Angeles andCedars-Sinai Medical Center.

RESULTS AND DISCUSSION

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

CLINICALSTUDY

The Journal of Craniofacial Surgery & Volume 22, Number 2, March 2011 585

From the *Division of Plastic Surgery, Department of Surgery, Keck Schoolof Medicine, University of Southern California; andDivision of PlasticSurgery, Department of Surgery, Cedars-Sinai Medical Center, LosAngeles, 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: [email protected]

Presented at the California Society of Plastic Surgeons meeting,May 29, 2010.

The authors report no conflicts of interest.Copyright* 2011 by Mutaz B. Habal, MDISSN: 1049-2275DOI: 10.1097/SCS.0b013e31820873ac

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


2/4

anomalies and managed using a multifactorial approach, includinganatomic location, response to medication, growth velocity, andpsychosocial issues. We have developed an algorithmic approach tomanaging this difficult pediatric problem during a 30-year expe-rience. This algorithm can be used by many different specialties andtakes into account the many subtleties that should be addressedwhen treating patients with hemangiomas.

When referred a patient with possible hemangioma, the first

step is visual confirmation that the lesion is indeed a hemangioma(Fig. 1). Many practitioners still make the mistake of diagnosingother vascular lesions such as vascular malformations or granu-lomas as hemangiomas. In general, they are not present at birth andhave a rounded and lobular appearance that displaces rather thaninvades the adjacent skin. In the proliferating phase, the color isgenerally beefy red or pink. The tumor has a spongy texture thatwill inflate after pressure is applied. An involuting tumor may havea mottled skin appearance and a more fatty texture. Lastly, an in-voluted hemangioma has a papery, wrinkled skin overlying a fatty,rubbery residuum. Although some authors have described ultra-sound, computed tomographic scanning, or magnetic resonanceimaging for confirmation of diagnosis, in most instances, it is notnecessary to confirm the clinical diagnosis. Further, biopsy or otherlaboratory testing is not recommended for confirmatory diagnosis

of these lesions. Typically, a hemangioma is diagnosed solely onclinical judgment, and additional tests that add cost and possibleradiation exposure are not worthwhile.

Once the diagnosis is secure, the next critical step is deter-mining 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 obscuredby clothing and hair. These areas are treated significantly differentfrom visible areas of the body. If the lesion is quite noticeable andvisible to the naked eye with clothes, it may cause great distress tothe child and be a source of embarrassment. Many children areteased and mocked when they reach school-age or even earlier.Thus, these hemangiomas should be treated more aggressively thanlesions not visible at a conversational distance (Figs. 2 and 3).

Most hemangiomas in nonvisible areas can be safely ob-served as the child develops. Most of these hemangiomas willeventually involute and leave behind the familiar characteristicsof hemangiomas: thin, wrinkled skin and a fibrofatty residuum. Inthe case of abnormal or excessive scarring, contour deformities, ordistortion, surgical therapy can be indicated for the involuted lesion.There are exceptions to observing these hidden hemangiomas, asseen in the algorithm. In Figure 4, an infant with a severe recalci-trant bleeding hemangioma that was resistant to conservative man-agement is shown. Eventually, the patient required surgical excisionwith good results.

Hemangiomas that are proliferating rapidly or increasing insize may be treated with intralesional steroids initially. If refrac-tory 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 fordifficult-to-treat refractory hemangiomas.15 They have minimal ad-verse effects in relation to other medical treatments, such as sys-temic steroids. A-Blocker therapy represents a possible paradigmshift in the management of hemangiomas, with propranolol and ace-butolol being evaluated in many trials worldwide.16,17 The indica-tions and uses ofA-blockers for hemangiomas are still nascent buthave been incorporated into our algorithm using the most available

literature and opinions. Further,A-blocker therapy is becoming morecompelling than systemic steroid therapy. In our opinion, we do notrecommend the use of interferon > or chemotherapeutic agentsbecause of their adverse effect profiles.9,10 There are many othermedical and surgical options that have fewer risks than these medi-cations to justify their use.

FIGURE 1. Initial evaluation of hemangioma referral.

FIGURE 2. Hemangioma algorithm for nonvisible lesions.

Soltani and Reinisch The Journal of Craniofacial Surgery & Volume 22, Number 2, March 2011

586 * 2011 Mutaz B. Habal, MD



3/4

Ulcerating hemangiomas present a distressing sight to theparents of many children with hemangiomas. These lesions arehighly vascular but typically do not produce severe enough bleed-ing to cause systemic compromise. Local wound care can heal theseulcerated areas including lidocaine gel, hydrocolloid dressings, orother wound dressings. Improved hygiene of the hemangioma canreduce the ulceration and allow the open areas to heal. However,refractory ulceration occasionally makes surgical excision the opti-mal choice to provide the fastest recovery for patient and parents.

Hemangiomas present on visible areas, most often in thehead and neck region, should be treated aggressively. Any surgicalexcision should be avoided during the proliferative phase of thetumor. If the lesion is large and periorbital, the patient should beevaluated by an ophthalmologist. Periorbital hemangiomas needto be treated expediently to avoid amblyopia in the developingchild. This may include intralesional steroids, systemic steroids, orA-blockers. If these fail, the child may need surgical excision ex-peditiously to avoid developmental problems in the eye. Experi-

mental treatment with topical A-blockers has been reported forthese periorbital hemangiomas and represents an exciting option

for hemangiomas in general.

18

Here is an example of a successfully treated patient with ahemangioma of the forehead with excellent aesthetic results (Fig. 5).This 2-year-old boy presented with a large unsightly hemangiomaof the forehead, which was being managed nonoperatively. Thehemangioma was excised, and long-term follow-up with patientand family revealed great satisfaction with the outcome and time-liness of treatment.

As the hemangioma involutes and ceases to proliferate, thelesion can be resected with little chance for regrowth. Surgical ex-cision should be tailored to each patient and each particular ana-tomic location. In many instances, total resection is not warranted,but debulking of the tumor to achieve good symmetry and contouris the best option. The operative goal is to envision how the finalinvoluted hemangioma might appear in the child after the colorfades. Totally involuted hemangiomas can often be problematic tothe growing child and adolescent. If severe scarring, contour de-formities, undesirable pigmentation, or skin texture is presentin the involuted hemangioma, we would advise surgical excision.In Figure 6, a young girl is shown, who presented late with a com-pletely involuted hemangioma. The resulting lesion was mottledwith hypopigmentation and hyperpigmentation with contour irregu-larities. The patient desired surgical treatment of this involuted lesionwith satisfactory long-term results.

FIGURE 4. A 4-month-old infant boy with a large ulceratinghemangioma over the upper back (left). Appearance 1 yearlater after excision and closure (right).

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

FIGURE 3. Hemangioma algorithm for visible lesions.

The Journal of Craniofacial Surgery & Volume 22, Number 2, March 2011 Algorithm in Managing Hemangiomas

* 2011 Mutaz B. Habal, MD 587



4/4

Documents

Algorithmic Approach to the Management of.47