Acta Dermatoven APA Vol 14, 2005, No 2 49
Craniofacial cavernous hemangioma:
succesful treatment with
L. A. Dourmishev and A. L. Dourmishev
Systemic corticosteroid treatment is reported as effective for problematic cutaneous hemangiomas
occuring in infancy, and depend on the dose, the duration of treatment, and the age at which the course
of drugs is initiated.
A 7-month-old female infant with extended cavernous hemangioma on the left part of forehead, face,
and neck which appeared 15 days after birth is presented. She was successfully treated with oral
methylprednisolone (initial doses of 3 mg/kg/daily and reduced in steps over 6 months) with significant
involution of the lesions and with good aesthetic results.
The oral corticosteroid treatment is an efficient medical therapy for common extended cavernous infan-
tile hemangiomas with accelerated growth if initiated early in the proliferative phase.
W O R D S
S U M M A R Y
Hemangiomas of the skin are the most common
benign tumors of childhood, characterized by a unique
natural history of growth in early infancy, followed by
slow involution in the following years (1–3). The large
forms of infantile cavernous hemangiomas are visually
displeasing, frequently have accelerated growth, and
may cause significant functional disturbances and exis-
tential complications. Several noncontroversial indica-
tions that demand treatment include hemangiomas af-
fecting vision or producing serious permanent disfig-
urement (that is unlikely to resolve on its own and cause
long-term adverse psychological consequences), or
other problematic symptoms that depend on the ana-
tomic location (3, 4). Different methods of treatment
including cryotherapy, sclerosing solutions, surgical re-
moval and laser therapy have been used with differing
results (5–7). The conservative approach that leaves
the lesions untreated is widely accepted, because the
results of the destructive treatment are usually worse
than those that result from spontaneous involution (3,
8). The mainstay of medical therapy are corticosteroids
(9) but the recommended dosage, duration of treat-
ment, recommended monitoring during and after treat-
ment, and methods of tapering vary widely (4). Corti-
costeroids have been used for decades (10–23) in the
treatment of infantile hemangiomas that cause func-
tional impairment of vital organs, due to the location
and dimension of the hemangioma.
The conservative approach of leaving such lesions
untreated is still widely accepted, because the results
of destructive treatment may in some cases be worse
than those from spontaneous involution (3, 8).
We present a 7-month-old girl with a craniofacial
cavernous hemangioma that was successfully treated
C a s e r e p o r t Craniofacial cavernous hemangioma
Acta Dermatoven APA Vol 14, 2005, No 2
with oral methylprednisolone, and that resulted in sig-
nificant involution of the lesions and good aesthetic re-
A 7-month-old female infant with an extended cav-
ernous segmental hemangioma on the left part of the
forehead, face, occipital area and neck was presented
to the dermatological outpatient service. The cavern-
ous tumor on her face had appeared 15 days after birth
and rapidly progressed. During the fourth month of age
oral treatment with prednisone (1 mg/kg/24 h) had
been initiated for one month without effect.
Physical examination revealed a large deep heman-
gioma on the left forehead, face, eyelids, nose, and
upper lip that measured 14 x 12 cm (Figure 1) affect-
ing vision in the left eye as well as several aberrant
vascular tumors of the skin in the occipital area and
over the left sternocleidomastoideus muscle.
Laboratory investigation, the complete blood count
and routine blood chemistry were within normal ranges
and no lesions in the brain and leptomeninges were
found by CT.
Treatment with 3 mg/kg/daily methylprednisolone
for 1 month was initiated, the dose was reduced gradu-
ally over the the next 5 months. At the end of the first
month, during steroid therapy the child developed
acute stomatitis (with fever and regional lymphaden-
opathy) which disappeared 2 weeks after therapy with
nonsteroid anti-inflammatory drugs (NSAID). At this
time the steroid treatment was not interrupted. At the
end of the third month of therapy the aesthetic results
were estimated as good with significant involution of
the hemangiomas (Figure 2).
Seventy percent of hemangiomas initially appear in
the first several weeks of life. Rapid growth during the
neonatal period is the historical hallmark of hemangio-
mas (2). It is known that hemangiomas of infancy occur
more frequently among female infants (male / female
ratio is 1:3) (3) and are most commonly located on the
head and neck (< 60% of cases) (1, 2). The enlargement
of these lesions during the first phase of the neonatal
period is a result of rapidly dividing endothelial cell prolif-
eration. The proliferation phase occurs during the first six
months of life. Takahashi et al. hypothesized that during
the third trimester of foetal development, immature en-
dothelial cells coexist with immature pericytes which
maintain their proliferative capacity for a limited period
during postnatal life (24). Angiogenic peptides, such as α
fibroblast growth factor (α-FGF), vascular endothelial
growth factor (VEGF) and prolifarating cell nuclear anti-
gen (PCNA), induce proliferation of these immature cells,
resulting in the development of the hemangioma2. As
the endothelial cells differentiate, an influx of mast cells
and tissue inhibitors of metaloproteinases (TIMPs) oc-
curs. TIMPs, along with interferons and TGF produced by
the mast cells terminate the endothelial cell proliferation
and passively induce involution by senescence of the
endothelial cells (2).
The PHACE syndrome (25), including posterior fossa
brain malformations, segmental cervicofacial hemangio-
mas, arterial anomalies, cardiac defects, coarctation of the
aorta and eye anomalies was suspected, but clinical ex-
amination, laboratory findings and CT failed to confirm
the presence of any such abnormalities except cervico-
The choice of therapy in hemangiomas depends on a
Craniofacial cavernous hemangioma C a s e r e p o r t
Figure 2. Results after three months of methyl-
prednisolone therapy of a left-side segmental
hemangioma resulting in normalized left eye vision
and a cosmetically acceptable appearance.
Figure 1. Extended craniofacial cavernous heman-
gioma before treatment.
Acta Dermatoven APA Vol 14, 2005, No 2 51
careful assessment of the factors (anatomic location, ac-
celerating growth, significant functional disturbances,
unaesthetic markings) along with a comparison of the
risks and benefits of the treatment (3). Different meth-
ods of treatment as well as systemic corticosteroids,
intralesional corticosteroids, interferons, sclerosing solu-
tions, cryotherapy, surgical removal and laser therapy
have been used with uncertain results (2, 3, 5-7, 23).
Systemic corticosteroid treatment has been estab-
lished as the most efficient medical therapy for common
cutaneous infantile hemangiomas (4, 8). It is appropriate
to administer the steroid therapy during the proliferative
phase, and it will have only a negligible effect on involut-
ing hemangiomas (2). There is a wide variation in re-
sponse rates, from less than 40% to greater than 90%,
depending on dose, duration of treatment, and age at
which corticosteroids are initiated (2). According to vari-
ous clinical studies, the efficacy of steroid therapy in pa-
tients with cutaneous hemangiomas in infancy has been
evaluated at 30% (14,18) , 53% (17), 83% (11), 86% (15),
93% (20), and 100% (10, 12). The meta-analysis of litera-
ture shows that the average cumulative response rate is
84% after 2-months therapy with standard doses of oral
corticosteroids (4). Usually, higher doses of corticoster-
oids in the treatment of hemangiomas has been associ-
ated with a better response rate (4,20), although others
attribute better response to early therapy and to the size
of the tumour, regardless of the dosage of steroids (22).
The oral route is generally preferred over intralesional
therapy (4). High-risk lesions (i.e. large lesions in
prognostically unfavourably locations, likely to leave per-
manent disfigurement or causing functional impairment)
are generally given high doses (3-5 mg/ kg/d predniso-
lone) of systemic corticosteroids (2, 16). It has been dem-
onstrated that higher doses (5 mg/kg/d) have a greater
response rate than moderate doses (3 mg/ kg/d) (4, 20).
Our result confirmed that oral corticosteroids give good
aesthetic results and initiate a significant involution of
extended cavernous hemangiomas with accelerated
growth. Steroid therapy of hemangiomas is not recom-
mended in patients over 2 years-old (16).
The precise mechanism by which steroids act upon
hemangiomas has not been elucidated completely; how-
R E F E R E N C E S
ever, corticosteroids appear to act by: (i) inhibiting the
proliferative capacity of immature pericytes; (ii) intensi-
fying the vasoconstrictive effects of epinephrine and
norepinephrine on vascular smooth muscle; (iii) block-
ing of the estradiol receptors in hemangiomas; and (iv)
inhibiting angiogenesis (8, 24, 26).
If the lesion involves the eyelids and periorbital tis-
sues as in our case, a visual obstruction should be consid-
ered. Hemangiomas on the eyelids and periorbital area
can lead to visual deprivation amblyopia by one of the
following three separate mechanisms: (a) physical ob-
struction of the visual axis, (b) astigmatism from direct
pressure on the anterior segment from eyelid involve-
ment, and (c) unilateral myopia (2). Systemic corticoster-
oids in combination with other medical, surgical or laser
modalities have also been used in periorbital or periocu-
lar infantile hemangiomas to prevent deprivation amblyo-
pia with variable effectiveness (27, 28).
The adverse effects of corticosteroid treatment of in-
fantile hemangiomas in the vast majority of reported cases
were transient and did not necessitate cessation of the
therapy (4, 8). In our patient gingivostomatitis (herpes
simplex infection) appeared as a side effect of steroid
therapy and this did not enforce the discontinuation of
the steroid treatment. Potential adverse reactions as hy-
pothalamic-pituitary-adrenal axis suppression and hyper-
tension are are to be mentioned but were neither ob-
served nor reported during treatment (29).
An involutional phase of most hemangiomas occurs
often between the fifth and the ninth year: 50 % by the
age of five, 70 % at seven years, and by the age of nine
years the recovery can be 90 %. The process may be
rapid or prolonged.
Our result shows that oral corticosteroids are the
method of choice in the treatment of extended cavern-
ous hemangiomas occurring in infancy. Oral steroid treat-
ment is an efficient medical therapy for common cavern-
ous infantile hemangiomas with accelerated growth if is
initiated early in proliferative phase.
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Lyubomir A. Dourmishev MD, PhD, Department of Dermatology and
Venereology, Medical University of Sofia, 1 St. George Sofiiskyi Str., 1431
Sofia, Bulgaria, e-mail: L_Dourmishev@yahoo.com
Assen L. Dourmishev MD, same address
Craniofacial cavernous hemangiomaC a s e r e p o r t
A U T H O R S '
A D D R E S S E S