Seminars in Pediatric Surgery (2006) 15, 124-132

Cervicofacial vascular anomalies. I. Hemangiomas and

other benign vascular tumors
Denise M. Adams, MD,a,c Anne W. Lucky, MDb

a
From the Division of Hematology/Oncology;
b
Division of Pediatric Dermatology; and
c
Hemangioma and Vascular Malformation Center, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio.

INDEX WORDS Before the early 1980s, the lack of an accepted system of nomenclature for vascular anomalies created
Head and neck confusion and frustration for clinicians and patients alike. Scientific advances over the past two decades
hemangiomas; have, however, led to international consensus among experts on a system of disease classification that
Vascular comprises vascular tumors and vascular malformations. The most common vascular tumor is heman-
malformations; gioma, which is frequently seen in the head and neck area. Some hemangiomas are best left untreated,
Benign vascular whereas others may cause significant complications and disfigurement, thus requiring intervention.
tumors; Early recognition and treatment of complications is imperative. This is best accomplished in an
Infantile interdisciplinary setting, where collaborative treatment strategies are planned on an individual basis.
hemangiomas; Steroids are the first line of medical treatment. Chemotherapy with vincristine is an effective treatment
Congenital for complicated lesions, although it requires further evaluation through prospective clinical trials.
hemangiomas; Because of known neurotoxicity, interferon is used as a last resort, especially in children younger than
Segmental 1 year of age. Laser therapy and surgical excision are also important therapeutic modalities. This article
hemangiomas; presents an overview of hemangiomas and other vascular tumors, highlighting cervicofacial lesions and
PHACES syndrome describing critical and underappreciated clinical presentations.
© 2006 Elsevier Inc. All rights reserved.

Historically, the care of patients with vascular anomalies
has been severely hampered by confusion and lack of com-
munication in the medical community at large. The absence
of a universally accepted system of nomenclature frequently
led to inadequate and improper management. Scientific ad-
vances over the past two decades have, however, led to
international consensus among experts on a binary system
of disease classification that comprises vascular tumors and
vascular malformations (Table 1). Proper disease classifi-
cation has in turn led to gradual improvement in the diag-
nosis and treatment of affected patients. In recent years, a
number of vascular anomalies centers have developed
worldwide. These centers bring together specialists in sur-
gery, radiology, dermatology, hematology– oncology, pa-
Address reprint requests and correspondence: Denise Adams, MD,
Division of Hematology/Oncology, Cincinnati Children’s Hospital Medi-
cal Center, 3333 Burnet Ave., Cincinnati, OH 45229.
E-mail: Denise.Adams@cchmc.org.
thology, and basic sciences who share their expertise and
develop integrated management strategies. This article pre-
sents an overview of the diagnosis and treatment of hem-
angiomas and other vascular tumors, highlighting cervico-
facial lesions and describing critical and under-appreciated
clinical presentations.
Classification of vascular anomalies
In 1982, Mulliken and Glowacki1 proposed a classifica-
tion system of vascular lesions that distinguished vascu-
lar tumors from vascular malformations based on clinical
appearance, histopathological features, and biologic be-
havior. Hemangiomas were identified as vascular tumors
that undergo an active growth phase characterized by
endothelial proliferation and hypercellularity followed by
a gradual involutional phase that occurs over several

1055-8586/$ -see front matter © 2006 Elsevier Inc. All rights reserved.
doi:10.1053/j.sempedsurg.2006.02.010
Adams and Lucky Cervicofacial Vascular Anomalies 125

Table 1 Distinguishing features of vascular tumors and Hemangiomas of infancy

vascular malformations
Hemangiomas of infancy are the most common tumors of
Hemangiomas of Infancy Vascular Malformations infancy, occurring in about 1 in 100 live births. These
Benign tumor Congenital abnormality tumors are composed of a mixture of cell types, including
30% visible at birth, seen as Present at birth, but may not endothelial cells (CD31⫹), pericytes (SMA⫹), dendritic
red macule; 70% become be evident until months or cells (factor XIIIa⫹), and mast cells. Unlike vascular mal-
apparent during first few even years later formations, they demonstrate immunopositivity for biologic
weeks of life markers GLUT1, Fc␥RII, merosin, and Lewis Y antigen.2-4
Females more commonly No gender predilection Hemangiomas of infancy occur more commonly in white
affected (gender ratio of newborns and have a higher incidence in females and pre-
3:1)
Rapid postnatal growth Slow steady growth, with no
mature infants.5-7 They frequently (60%) occur around the
followed by slow involution; may expand neck; however, 25% occur on the trunk and 15% occur on
involution secondary to sepsis, trauma, the extremities. Although most tumors present singularly,
or hormonal changes up to 20% of affected infants have multiple (⬎5) cutaneous
Endothelial cell hyperplasia Normal endothelial cell lesions. This increases the risk of having visceral tumors
turnover involving the liver, spleen, lung, brain, and intestines, which
Increased mast cells Normal mast cell count can lead to serious consequences, such as hepatomegaly,
Multilaminated basement Normal thin basement
congestive heart failure, and anemia.8,9 About one-third of
membrane membrane
Primary thrombophilia, No Primary stasis (venous); hemangiomas are seen at birth, appearing as erythematous
coagulation abnormalities localized consumptive macules, some with a pale halo or a telangiectatic patch;
coagulopathy however, most lesions are observed during the first several
Radiographic findings: well- Radiographic findings: weeks of life. Based on anatomic depth, hemangiomas are
circumscribed, intense diffuse, no parenchyma categorized as superficial, deep, or combined. Superficial
lobular-parenchymal Low-flow: phleboliths, lesions tend to be soft, red, and raised, or rarely, telangiec-
staining with equatorial ectatic channels tatic. Deep lesions may show a spectrum of appearance and
vessels High-flow: enlarged, consistency, ranging from soft and supple to raised and
tortuous arteries with
more firm warm masses with a bluish color. Combined
arteriovenous shunting
Infrequent “mass effect” on Low-flow: distortion, lesions appear as red dermal tumors with epidermal and
adjacent bone; hypertrophy, or hypoplasia dermal components, along with a subcutaneous mass that is
hypertrophy rare High-flow: bone destruction, either blue or flesh-colored.
distortion, or hypertrophy Hemangiomas of infancy follow a predetermined course
80%-90% respond No response to of proliferation and involution. The proliferative phase be-
dramatically to corticosteroids or gins in the first few weeks of life and continues for 4 to 10
corticosteroid treatment antiangiogenic agents months. During this phase, lesions rapidly expand and the
in 2 to 3 weeks superficial component of the hemangioma becomes more
Immunopositive for biologic Immunonegative for these
markers GLUT1, Fc␥RII, biologic markers
erythematous or violaceous. Deep hemangiomas may pro-
merosin, and Lewis Y liferate until 2 years of age. The growth rate of lesions then
antigen. stabilizes and the hemangioma grows in proportion to the
child. This quiescent period is followed by an involuting
phase, during which hemangiomas become gray in color;
this phase can last for up to 5 to 6 years. Maximum invo-
lution occurs in approximately 50% of children by age 5
years. In contrast, vascular malformations were described years and in 90% of children by age 9 years.5-7 In most
as congenital malformations of the vasculature derived patients, tumor regression results in the restoration of nor-
from capillaries, veins, lymphatic vessels, arteries, or a mal skin, although 20% to 40% exhibit residual changes of
combination of these. In 1996, this classification system the skin such as laxity, discoloration, telangiectasia, fibro-
was modified with consensus from the International So- fatty masses, or scarring.
ciety for the Study of Vascular Anomalies. The modifi-
cation expanded the category of vascular tumors to in-
clude kaposiform hemangioendothelioma (KHE), tufted
angiomas, pyogenic granulomas, and hemangiopericyto-
Congenital hemangiomas
mas. These lesions, which had formerly been classified as
hemangiomas, were shown to have distinct clinical and Congenital hemangiomas are thought to be either a variant
histopathologic characteristics. This modified binary sys- of hemangiomas of infancy or a related but distinct his-
tem is now the international classification standard used topathologic entity. Unlike hemangiomas of infancy, these
by all experts in the field of vascular anomalies. lesions are fully developed at birth and do not undergo
126
Figure 1 Large, segmental facial hemangioma in a child with PHACES syndrome (A). The “beard” distribution is often associated with
airway hemangiomas, here requiring tracheostomy (B). (Color version of figure is available online.)
additional postnatal growth; they have occasionally been
diagnosed in utero. Congenital lesions fall into two distinct
subgroups: rapidly involuting congenital hemangiomas
(RICHs) and noninvoluting congenital hemangiomas
(NICHs).10 These two tumor types share similar morphol-
ogy and generally occur as solitary lesions that are viola-
ceous in color at birth. RICHs rapidly regress over the first
year of life, whereas NICHs do not involute. Both lesions
are high-flow lesions that can be misdiagnosed as arterio-
venous malformations. Congenital hemangiomas can cause
congestive heart failure that has been reported in utero.
Unlike hemangiomas of infancy, congenital hemangiomas
are Glut-1 negative.
Head and neck hemangiomas
Segmental lesions
Hemangiomas that occur in developmental segments are
referred to as “segmental” lesions. Such hemangiomas have
Seminars in Pediatric Surgery, Vol 15, No 2, May 2006
a higher risk of being life or function threatening and are
often associated with other congenital anomalies.
Large cervicofacial segmental hemangiomas, particu-
larly those on the forehead, temple, upper cheek, and around
the eye, can be accompanied by a constellation of findings
termed PHACES association (Figure 1A and B).11,12 This
association is characterized by posterior fossa abnormali-
ties, hemangiomas that are typically plaque-like and seg-
mental in the cervicofacial area, arterial anomalies, cardiac
and aortic arch defects, eye anomalies, and sternal clefts or
supraumbilical raphes. A large variety of structural brain
abnormalities such as Dandy–Walker syndrome and hydro-
cephalus, as well as cerebrovascular arterial anomalies have
been described. Over 50% of these patients have neurologic
sequelae, such as seizures, stroke, developmental delay, and
migraines.13 Eye abnormalities include optic atrophy, con-
genital cataracts, and retinal vascular abnormalities. Aortic
arch anomalies may include coarctation, aneurysms, and
congenital valvular aortic stenosis. Other cardiac defects
include septal defects, pulmonary stenosis, and anomalous
pulmonary veins. Patients suspected of having a diagnosis
of PHACES should undergo an eye examination, screening
Adams and Lucky Cervicofacial Vascular Anomalies
echocardiogram, and either magnetic resonance imaging
(MRI) or magnetic resonance angiography (MRA). If a
central nervous system (CNS) abnormality is noted, patients
should undergo follow-up imaging every 3 months until 18
months of age. Either an MRI or MRA is required if there
is CNS compromise.
Risk of airway involvement
Sixty-five percent of patients with hemangiomas in the
cervicofacial region that cover a beard distribution includ-
ing the chin, jawline, and preauricular areas have associated
airway involvement.14 The majority of airway hemangio-
mas are localized in the glottic or subglottic region, al-
though they may be segmental or diffuse. Infants with
hemangiomas in the beard area should be monitored
closely, and treatment should be initiated if airway involve-
ment is suspected. Localized lesions are managed with laser
therapy, intralesional steroids, or surgical resection. Seg-
mental hemangiomas are treated with systemic agents such
as steroids or vincristine. Tracheostomy should be reserved
for patients who fail medical therapy. Parotid gland hem-
angiomas can be superficial and deep and can grow rapidly,
causing extrinsic compression of the upper pharyngeal air-
way. These lesions can proliferate for up to 12 to 16 months
and require systemic treatment if the airway is compro-
mised.
Periocular and orbital hemangiomas
Periocular hemangiomas can involve the upper lid, lower
lid, and or retrobulbar space; amblyopia is reported in 40%
to 60% of cases.15 Because of pressure from the hemangi-
oma on the globe, astigmatism often occurs. Strabismic
amblyopia occurs when a hemangioma affects the move-
ment of extraocular muscles, and deprivation amblyopia
occurs when there is partial or complete eyelid closure
(Figure 2A). When deprivation amblyopia occurs in the first
few months of life, it can cause profound visual impairment.
Periorbital hemangiomas that impair vision or create pres-
sure on the globe require immediate treatment. This may
include medical approaches such as topical high-potency
steroids, intralesional steroids, systemic steroids, or surgical
resection (Figure 2B). Ophthalmologic care, such as patch-
ing the contralateral eye and monitoring the need for cor-
rective lenses, is imperative.
Diagnosis
The diagnosis of hemangioma of infancy is based on clin-
ical presentation, history, and physical examination, with
lesions having varying degrees of compressibility to the
limits of their underlying fibrofatty architecture. If lesions
are complicated or if surgical intervention is required, ad-
ditional investigation may be helpful in determining the
127
extent of involvement. In such cases, a variety of noninva-
sive imaging modalities are extremely helpful in verifying
the diagnosis. Ultrasonography (US) with color flow Dopp-
ler is extremely useful in differentiated hemangiomas from
similarly appearing vascular malformations such as lym-
phatic malformations. On US, an infantile hemangioma
appears hypoechoic, well defined, and heterogenous in tex-
ture, with small cystic and sinusoidal spaces. In most pro-
liferating hemangiomas, a characteristic fast-flow pattern is
visualized by Doppler US.
Computed tomography (CT) can delineate the extent and
involvement of the hemangioma and can also be useful in
differentiating hemangiomas from lymphatic anomalies, but
can only indicate qualitative differences in blood flow. In
the proliferative phase, a hemangioma appears as a well-
circumscribed tumor with homogeneous parenchymatous
density and intense enhancement. In the involutive phase, it
appears heterogeneous with distinct lobular architecture and
large draining veins in the center and the periphery.
MRI and MRA are particularly useful for craniofacial
lesions, revealing both the extent of involvement with tissue
planes and rheologic characteristics. Although conventional
angiography can provide information about the size of le-
sions and feeding vessels and can also distinguish between
hemangiomas and other vascular malformations, it is re-
served for patients in whom embolization or surgical inter-
vention is contemplated.
Treatment
As discussed, many hemangiomas involute spontaneously,
leaving little or no functional disability or cosmetic defect.
It is thus prudent to see patients periodically, taking photo-
graphs so to monitor signs of change and tumor regression.
Management decisions are based on: (1) lesion size and
location, (2) the presence of complications at the time of
diagnosis, (3) the age of the patient, and (4) the phase of
growth at the time of evaluation. Large segmental heman-
giomas or lesions that interfere with the function of vital
structures are likely to require immediate treatment. The
possibility of permanent disfiguration or scarring is also an
indication for early intervention.
Major therapies include pharmacotherapy, chemother-
apy, laser therapy, and surgical excision.
Pharmacotherapy
Corticosteroids
Corticosteroids are the first line of therapy for the treatment
of complicated hemangiomas; they may be administered
topically, intralesionally, or orally.
High-potency topical steroids such as clobetasol propi-
onate (0.05%, applied twice daily) have been reported to
have some success in smaller localized lesions, especially
128
Figure 2 This small, deep hemangioma of the upper eyelid had a profound effect on vision and required systemic prednisolone therapy
and patching of the contralateral eye to preserve vision (A). At age 3, he had only minimal ptosis and good vision (B). (Color version of
figure is available online.)
around the face.16 Topical application appears to accelerate
the involution process and is particularly useful in treating
problematic localized craniofacial lesions. Although such
topical ointments have some degree of systemic absorption,
they are not associated with significant adrenal pituitary axis
inhibition, as are orally administered systemic corticoste-
roids. As such, they offer an attractive alternative for local-
ized problematic lesions.
Intralesional corticosteroids are best used for treating
smaller localized, problematic lesions rather than larger
segmental hemangiomas. They are especially effective in
the periorbital region. Some surgeons and ophthalmologists
recommend using general anesthesia, whereas others per-
form the procedure on an outpatient basis using a topical
anesthetic. A long-acting steroid (eg, triamcinolone ace-
tonide) can be combined with a short-acting steroid (eg,
cortisone acetate or betamethasone acetate), in a total vol-
ume that generally does not exceed 2.5 mL. The dose and
volume are individualized according to the size of the le-
sion. A 26- to 30-gauge needle is used and injections are
made directly into the hemangioma in different directions
through the same needle hole. Following this, direct pres-
Seminars in Pediatric Surgery, Vol 15, No 2, May 2006
sure is applied for 2 to 10 minutes to prevent bleeding.
There is a 70% to 90% response rate.
One of the known complications of this approach is the risk
of retinal artery embolization and thrombosis, with resulting
blindness. To diminish this risk, fundoscopic retinal examina-
tion should be performed as the lesion is slowly injected so to
minimize risk of retinal artery embolization and recognize
early signs of retinal arterial flow interruption.
Oral corticosteroids are generally used to treat significant
life- or function-threatening lesions, and are associated with
a 70% to 90% response rate.17,18 The mechanism of action
of corticosteroids is poorly understood, but is believed to be
an antiangiogenic effect that decreases endothelial cell pro-
liferation and causes endothelial cell apoptosis. Hasan and
coworkers19 studied the histologic and molecular changes in
the proliferating hemangioma following steroid therapy and
found increased numbers of mast cells, decreased transcrip-
tional expression of cytokines, and enhanced transcription
of mitochondrial cytochrome B gene.
Because there are no prospective randomized controlled
studies that have investigated dosing or efficacy, current
dosing strategies are based on retrospective studies. The
Adams and Lucky Cervicofacial Vascular Anomalies
general recommendation for oral steroids is a starting dose
of 2 to 5 mg per kg daily of prednisolone as a single
morning dose. Typically, an assessment of response is per-
formed 2 weeks after this regimen is initiated. If the re-
sponse is positive, this high dose is maintained for 4 to 6
weeks and then tapered over 4 to 6 months. Ranitidine is
given concomitantly for gastric irritation. For patients who
undergo steroid therapy over an extensive period of time,
some clinicians prescribe trimethoprim/sulfamethoxazole to
prevent Pneumocystis carinii.
Short-term side effects of steroids include irritability,
gastric irritation, diminished linear growth and weight gain,
nonsystemic fungal infections, and Cushingoid appearance
and suppression of the hypothalamic pituitary axis. Boon
and coworkers20 evaluated 62 patients receiving systemic
corticosteroid therapy for problematic infantile hemangio-
mas and found: (1) Cushingoid facies in 71% of patients, (2)
personality changes in 21% of patients, (3) gastric irritation
in 21% of patients, (4) fungal infections in 6% of patients,
and (5) reversible myopathy in 1 patient. Diminished lon-
gitudinal growth was seen in 35% of patients and dimin-
ished weight gain in 42%; however, catch-up growth oc-
curred in most patients. Potential side effects of steroid use
include immunosuppression, hypertension, significant but
reversible suppression of the hypothalamic pituitary adrenal
axis, hyperglycemia, ophthalmologic changes, myositis, os-
teoporosis, cardiomyopathy, and neurologic changes. The
systemic effects of intralesional and topical steroid therapy
have not been well studied.
Patients undergoing systemic glucocorticoid therapy
should be monitored for potential side effects. Close mon-
itoring of height and weight, blood pressure, developmental
milestones, and adrenal suppression at the end of treatment
should also be done. Live vaccines should not be given to
these patients. If exposure to varicella occurs, a physician
should be called immediately and the administration of
VZIG should be considered. Patients should be seen for
significant fevers and followed regularly. They should be
instructed to increase their dosages during stresses related to
illness or surgical procedures.
Chemotherapy
Vincristine
Chemotherapy with vincristine is an option for patients who
have failed or cannot tolerate other medical therapies. Vin-
cristine interferes with mitotic spindle microtubules and
induces apoptosis in tumor cells in vitro. It has a known side
effect profile that includes peripheral neuropathy, constipa-
tion, jaw pain and irritability, electrolyte disturbances, and
neurological problems. Because it is a vesicant, vincristine
is best delivered through central venous access. To date,
there have been only two retrospective studies21,22 of pa-
tients with life-threatening hemangiomas who received vin-
129
cristine following failed steroid therapy or significant com-
plications from steroid therapy. In one study, vincristine
was initiated at an average of 8 months of age and continued
for mean duration of 6 months. Nine of 10 patients re-
sponded and 1 had a partial response with an average time
to response of 3 weeks. A similar result was found in the
second study.22
Interferon
As a treatment of last resort, interferon-␣ is used. Interfer-
on-␣ is an antiangiogenic agent that decreases endothelial
cell proliferation by down-regulating basic fibroblast
growth factor (bFGF). Numerous studies have reported the
efficacy of interferon alpha-2a and interferon alpha-2b,
showing a complete response rate ranging from 40% to 50%
with doses between 1 and 3 million Units/m2 daily. 23,24
Neurotoxicity resulting in spastic diplegia and other motor
developmental disturbances has, however, been reported
with all preparations of interferon in 10% to 30% of pa-
tients; some patients have experienced permanent spastic
diplegia.25 Clinicians should thus conduct neurologic exam-
inations before therapy and every month during the course
of therapy. Laboratory evaluations should be done twice a
month.
Laser therapy
Laser treatment of hemangiomas remains controversial. Al-
though there is no substantial body of evidence indicating
that pulsed-dye laser (PDL) therapy prevents the growth of
hemangiomas or causes involution to occur significantly
faster, it is commonly used to treat small lesions. The results
of a prospective, randomized controlled study conducted by
Batta and coworkers26 has shed light on this debate. Authors
investigated outcomes with PDL laser therapy versus ob-
servation alone. They found that the number of children
whose lesions showed complete clearance or minimum re-
sidual signs at 1 year was not significantly different from the
number in PDL-treated and observation groups. Moreover,
PDL-treated infants were more likely to have subsequent
skin atrophy and hypopigmentation. The only objective
measure of resolution with PDL treatment was a more rapid
decrease in hemangioma redness.
There are a number of selective indications for PDL
treatment. PDL is advocated for the treatment of ulcerated
hemangiomas, decreasing pain and promoting more rapid
reepithelialization. In most cases, this is appropriate but in a
very small number of patients, particularly those with seg-
mental lesions, ulceration may worsen after laser treatment.
The PDL is also effective for treating the residual telangi-
ectasias left after involution.
Since the neodymium:yttrium-aluminum-garnet (Nd:
YAG) laser system has a greater depth of penetration than
the PDL, several authors have reported its successful use in
130
treating the deeper component of hemangiomas. The Nd:
YAG laser is also used for intralesional photocoagulation.
Both the carbon dioxide (CO2) laser and the erbium (ER:
YAG) laser are useful for resurfacing wrinkled and scared
skin following involution.
Surgical excision
If feasible, surgery is indicated in a number of clinical
circumstances: (1) when hemangiomas present a threat to
function or are associated with complications (eg, persistent
ulceration, significant hemorrhage, or infection) and do not
respond to pharmacotherapy or other less invasive alterna-
tives; (2) when they have a high probability of leaving a
bag-like fibrofatty residuum, such as seen in pedunculated
hemangiomas; (3) when required to revise residual scars
after lesions have involuted; (4) when lesions persist beyond
a reasonable period of time or grow atypically; and (5) when
there is a perceived emotional burden on the child or family
and the lesion can easily be removed, leaving no significant
cosmetic deformity. The timing of surgery remains a con-
troversial subject, particularly in regard to early surgery on
hemangiomas that are disfiguring but in which the ultimate
results of spontaneous involution are difficult to predict. In
such cases, the potential risks must be weighed against
numerous factors, including the likely prognosis, the rate of
involution, and parental expectations and desires. For ex-
ample, nasal tip hemangiomas commonly require surgical
intervention. They are usually medically managed in in-
fancy with intralesional steroids for smaller lesions and
systemic steroids for larger lesions. Staged resection should
be contemplated in early childhood. If surgical excision is
required to avoid the psychosocial consequences of a visible
hemangioma, the operation should be performed during the
preschool period, when the child is beginning to develop a
defined body image.
The surgical approach is highly dependent on the size
and location of the hemangioma. Although lenticular exci-
sion enclosure is an established approach for localized le-
sions, the length of scarring is a major drawback. A partic-
ularly useful technique for localized lesions in most
anatomic sites is circular excision and pursestring closure
(Figure 3A and B).27 This technique can be used at any
phase in the clinical course of a lesion. It reduces both the
longitudinal and transverse dimensions and converts a large
circular lesion into a small ellipsoid scar. No other excision
and closure technique results in a smaller scar. Because
normal skin and subcutaneous tissue are relatively lax, this
technique is unlikely to result in significant distortion of
surrounding structures. In addition, the scar can be readily
revised and made even smaller or placed in desirable skin
crease if necessary.
Cryosurgery is widely used in Europe, but there is little
experience with this modality in the United States because
of the potential for scarring.
Seminars in Pediatric Surgery, Vol 15, No 2, May 2006
Ulceration complications
Ulceration occurs in 10% of hemangiomas and is a common
complication of lesions of the head and neck, especially
those involving the nose, lips, and periorbital region. It can
lead to pain, infection, anemia, and scarring and disfigure-
ment and, as such, requires immediate treatment. Topical or
systemic antibiotics are frequently used. Appropriate dress-
ings that cover the ulceration securely (eg, Duoderm or
Mepilex) can provide immediate relief of pain and promote
healing. Newer products such as imiquimod, which stimu-
lates endogenous interferon production, and becaplermin
gel, which promotes wound healing, require further study to
assess their usefulness in treating ulcerated hemangio-
mas.28,29 Surgical excision of ulcerated lesions often pro-
vides rapid relief of pain and bleeding and in areas such as
the scalp and neck can result in an excellent cosmetic
outcome.
Summary
Unless hemangiomas are associated with significant com-
plications and disfigurement, they are often best left un-
treated. Early recognition and treatment of complications is
imperative, and optimally, this should be done in an inter-
disciplinary setting. For patients in whom treatment is pru-
dent, there are a number of well-established medical and
surgical options, and the clinical decision as to which treat-
ment or combination of treatments to use should be made on
an individual basis. When intervention is warranted, phar-
macotherapy with topical, oral systemic, or intralesional
corticosteroids are the first line of treatment. Chemotherapy
with vincristine is an effective treatment in complicated
hemangiomas, though it requires further evaluation through
prospective clinical trials. Because of its neurotoxicity, par-
ticularly in children younger than age 1 year, interferon
should be used as a medical therapy of last resort. Laser
therapy remains controversial, though it may be useful for
the treatment of ulcerated hemangiomas. Surgical excision
is indicated when lesions do not respond to nonoperative
approaches and when lesions are life or function threaten-
ing.
Other benign vascular tumors
Kaposiform hemangioendothelioma and tufted
angioma
Kaposiform hemangioendothelioma (KHE) and tufted an-
gioma were first described in 1940 by Kasabach and Merritt
when they reported a case of an infant with thrombocyto-
penic purpura due to what they believed was a giant capil-
lary hemangioma.30 Thereafter, the association of a capil-
lary hemangioma with thrombocytopenia was called
Adams and Lucky Cervicofacial Vascular Anomalies
Figure 3 The vascular component of this focal hemangioma
resolved, leaving a fibrofatty mass (A). A pursestring excision
yielded a more acceptable cosmetic result (B). (Color version of
figure is available online.)
131
Kasabach–Merritt syndrome; it is presently referred to as
Kasabach–Merritt (K-M) phenomenon. The clinical and he-
matologic features of K-M phenomenon include an enlarg-
ing vascular lesion, profound thrombocytopenia, a microan-
giopathic hemolytic anemia, and a mild consumptive
coagulopathy. The phenomenon has been associated with a
mortality rate as high as 20% to 30%.31
In 1997, two groups of investigators demonstrated that
these lesions were not true hemangiomas, but rather were
associated with vascular lesions that were diagnosed histo-
logically as kaposiform hemangioendotheliomas (KHE) or
tufted angiomas.32,33 The clinical profile of these lesions
differs from that of hemangiomas. Although they have a
predilection for the upper trunk and extremities, thigh, sa-
crum, or retroperitoneum, they can occur in the head and
neck area. They are warm, firm indurated purpuric lesions.
They are unifocal and are usually present at birth; however,
recently they have been identified later in childhood. They
affect both sexes equally. MRI imaging scans have demon-
strated that KHE and tufted angioma invade the skin and
subcutaneous fat and muscle.
A number of therapies have been reported in the treat-
ment of K-M phenomenon, but none have been uniformly
effective.31 Therapies involve the use of steroids, interferon,
antifibrinolytic agents, and chemotherapy, including cyclo-
phosphamide, vincristine, and actinomycin. These lesions
often prove to be a dilemma for clinicians in that they
present with a wide clinical spectrum and response to ther-
apy. They should not be confused with hemangiomas and
require supervision by an interdisciplinary team. Some of
these lesions are not associated with coagulopathy.
References
1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations
in infants and children: a classification based on endothelial charac-
teristics. Plast Reconstr Surg 1982;69:412-22.
2. Mulliken JB, Enjoras O. Congenital hemangiomas and infantile hem-
angioma: missing links. J Am Acad Dermatol 2004;50:875-82.
3. Leon-Villapalos J, Wolfe K, Kangesu L. GLUT -1: an extra diagnostic
tool to differentiate between hemangiomas and vascular malforma-
tions. Br J Plast Surg 2005;58:348-52.
4. North PE, Waner M, Mizeracki AM, et al. A unique microvascular
phenotype shared by juvenile and human placenta. Arch Dermatol
2001;137:559-70.
5. Fishman SJ, Mulliken JB. Vascular anomalies: a primer for pediatri-
cians. Pediatr Clin North Am 1998;45:1455-77.
6. Enjolras O, Mulliken JB. Vascular tumors and vascular malformations
(new issues). Adv Dermatol 1997;13:375-423.
7. Drolet B, Esterly N, Frieden IJ. Hemangiomas in children. N Engl
J Med 1999;341:173-81.
8. Cohen RC, Myers NA. Diagnosis and management of massive hepatic
hemangiomas in childhood. J Pediatr Surg 1986;21:6-9.
9. Enjolras O, Riche MC, Merland JJ, et al. Management of alarming
hemangiomas in infancy: a review of 25 cases. Pediatrics 1990;85:
491-8.
10. Berenguer B, Mulliken JB, Enjolras O, et al. Rapidly involuting
congenital hemangioma: clinical and histopathologic features. Pediatr
Developmental Pathol 2003;6:495-510.
132
11. Frieden IJ, Reese V, Cohen D. PHACE syndrome: The association of
posterior fossa brain malformations, hemangiomas, arterial anomalies,
coarctation of the aorta and cardiac defects, and eye abnormalities.
Arch Dermatol 1996;132:307-11.
12. Reese V, Frieden IJ, Paller AS, et al. Association of facial hemangi-
omas with Dandy-Walker and other posterior fossa malformations.
J Pediatr 1993;122:379-84.
13. Burrows PE, Robertson RL, Mulliken JB, et al. Cerebral vasculopathy
and neurologic sequelae in infants with cervicofacial hemangioma:
report of eight patients. Radiology 1998;207:601-7.
14. Orlow SJ, Isakoff MS, Blei F. Increased risk of symptomatic heman-
giomas of the airway in association with cutaneous hemangiomas in a
“beard” distribution. J Pediatr 1997;131:643-6.
15. Kushner B. Infantile orbital hemangiomas. Int Pediatr 1990;5:249-57.
16. Cruz OA, Zarnegar SR, Myers SE. Treatment of periocular capillary
hemangioma with topical clobetasol propionate. Ophthalmology 1995;
102:2012-5.
17. Bartoshesky LE, Bull M, Feingold M. Corticosteroid treatment of
cutaneous hemangiomas: how effective? A report on 24 children. Clin
Pediatr (Phila) 1978;17:625,629-38.
18. Brown SH Jr, Neerhout RC, Fonkalsrud EW. Prednisone therapy in the
management of large hemangiomas in infants and children. Surgery
1972;71:168-73.
19. Hasan Q, Tan ST, Gush J, et al. Steroid therapy of a proliferating
hemangioma: histochemical and molecular changes. Pediatrics 2000;
105:117-20.
20. Boon LM, MacDonald DM, Mulliken JB. Complications of systemic
corticosteroid therapy for problematic hemangioma. Plast Reconstr
Surg 1999;104:1616-23.
21. Enjolras O, Breviere GM, Roger G, et al. Vincristine treatment for
function- and life-threatening infantile hemangioma. Arch Pediatr
2004;11:99-107.
22. Adams DM, Orme L, Bowers D. Vincristine treatment of complicated
hemangiomas. 14th International Workshop on Vascular Anomalies,
Netherlands, June 2002
Seminars in Pediatric Surgery, Vol 15, No 2, May 2006
23. Ezekowitz RA, Mulliken JB, Folkman J. Interferon alfa-2a therapy for
life-threatening hemangiomas of infancy. N Engl J Med 1992;326:
1456-63.
24. Chang E, Boyd A, Nelson CC, et al. Successful treatment of infantile
hemangiomas with interferon-alpha-2b. J Pediatr Hematol Oncol
1997;19:237-44.
25. Barlow CF, Priebe CJ, Mulliken JB, et al. Spastic diplegia as a
complication of interferon Alfa-2a treatment of hemangiomas of in-
fancy. J Pediatr 1998;132:527-30.
26. Batta K, Goodyear HM, Moss C, et al. Randomised controlled study of
early pulsed dye laser treatment of uncomplicated childhood haeman-
giomas: results of a 1-year analysis. Lancet 2002;360:521-7.
27. Mulliken JB, Rogers GF, Marler JJ. Circular excision of hemangioma
and purse-string closure: the smallest possible scar. Plast Reconstr
Surg 2002;109:1544-54.
28. Hazen PG, Carney JF, Engstrom CW, et al. Proliferating hemangioma
of infancy: successful treatment with topical 5% imiquimod cream.
Pediatr Dermatol 2005;22:254-6.
29. Metz BJ, Rubenstein MC, Levy ML, et al. Response of ulcerated
perineal hemangiomas of infancy to becaplermin gel, a recombinant
human platelet-derived growth factor. Arch Dermatol 2004;140:867-
70.
30. Kasabach H, Merritt K. Capillary hemangioma with extensive purpura:
report of a case. Am J Dis Child 1940;59:1063-70.
31. Haisley-Royster C, Enjolras O, Frieden IJ, et al. Kasabach-merritt
phenomenon: a retrospective study of treatment with vincristine. J Pe-
diatr Hematol Oncol 2002;24:459-62.
32. Sarkar M, Mulliken JB, Kozakewich HP, et al. Thrombocytopenic
coagulopathy (Kasabach-Merritt phenomenon) is associated with Ka-
posiform hemangioendothelioma and not with common infantile hem-
angioma. Plast Reconstr Surg 1997;100:1377-86.
33. Enjolras O, Wassef M, Mazoyer E, et al. Infants with Kasabach-
Merritt syndrome do not have “true” hemangiomas. J Pediatr 1997;
130:631-40.