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Case Report
The star‑crossed curse of childhood: A case of infantile
hemangioma
Geon Pauly N, Nidhin Kurian1, Roopashri Rajesh Kashyap, Raghavendra Kini,
Prasanna Kumar Rao, Ann Thomas1
Departments of Oral Medicine and Radiology and 1Pedodontics, A.J. Institute of Dental Sciences, Mangalore, Karnataka, India
ABSTRACT
Vascular lesions are indeed very widespread, with
vascular tumors numbering the most common
tumors in childhood. Researchers and authors
frequently use the idiom “hemangioma” to portray
or describe vascular malformations and a potpourri
of vascular anomalies. Infantile hemangioma, a
type of hemangioma, is one of the most common
benign vascular tumors in infancy and childhood.
As hemangioma could regress spontaneously,
it generally does not require treatment unless
proliferation interferes with normal function or
gives rise to the risk of serious disfigurement and
complications unlikely to resolve without treatment.
Various methods for treating infant hemangiomas
have been documented, including wait‑and‑see
policy, laser therapy, drug therapy, sclerotherapy,
radiotherapy, and surgery. However, none of
these therapies can be used for all hemangiomas.
Hence, to obtain the best treatment outcomes, the
treatment protocol should be individualized as per
the case.
KEYWORDS: Infantile hemangioma, orbital tumor,
palate, vascular malformation
Introduction
Hemangioma is a kind of endothelial malformation
and can cause significant morbidity and mortality
in children and adults. [1] It is a benign vascular
abnormality characterized by rapid endothelial
cell proliferation and does not undergo malignant
transformation. [2] Although a few cases are
congenital, most develop in childhood with
rarely older individuals been affected. Literature
proves that they tend to have a higher female
predilection. [3]
Hereby, we present a case of a 3‑year‑old female
patient who visited our department with an unusual
extensive growth across the palate.
© 2019 Journal of Indian Society of Pedodontics and Preventive Dentistry | Published by Wolters Kluwer - Medknow
Address for correspondence:
Dr. Geon Pauly N,
Department of Oral Medicine and Radiology, A.J. Institute
of Dental Sciences, Kuntikana, NH‑66, Mangalore ‑ 575 004,
Karnataka, India.
E‑mail: geonpauly@gmail.com
Access this article online
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DOI:
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Case Report
A  3‑year‑old female patient  was referred to our
department with a chief complaint of a growth inside the
mouth in the palatal region. Her parents gave a history of
a having first noticed a growth of such nature when she
was 3 months old. They also gave a history of the growth
having phases of spontaneous remission and regression
over time and were positive that it had never completely
resolved. She was moderately built and nourished for her
age and had no associated complaints or lack of appetite.
However, the recurrence after previous remission which
dated 1  week prior had resulted in profuse bleeding
while having food and, to the best of their knowledge,
had been the most sizeable form they had even seen.
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How to cite this article: Pauly GN, Kurian N, Kashyap RR,
Kini R, Rao PK, Thomas A. The star‑crossed curse of childhood:
A case of infantile hemangioma. J Indian Soc Pedod Prev Dent
2019;37:209-13.
209
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Pauly, et al.: Infantile hemangioma
On clinical examination, there was a visually evident
diffuse asymptomatic swelling on the left side of the
face resulting in asymptomatic narrowing of the left
eye with no impairment in vision  [Figure  1a and b].
On intraoral examination, multiple solitary/coalescing
bluish purple pinpoint lesions with overlying elevated
yellowish slough having irregular margins and
surrounded by erythematous borders were seen across
the hard and soft palate, predominantly on the left side
extending onto the left alveolus distal to the primary
second molar and crossing the midline onto the right
side of the palate [Figure 2a and b]. It bled profusely
to touch.
A provisional diagnosis of infantile hemangioma (IH)
was given based on the patient’s history and clinical
presentation. An occlusal radiograph was not possible
as it bled acutely on coming in contact with the film
at the alveolus region. Hence, a magnetic resonance
imaging  (MRI) and a computed tomography  (CT)
scan, plain and contrast, were advised. MRI and CT
images revealed alarmingly extensive ill‑defined areas
of altered intensity/density involving the hard palate,
pterygopalatine fossa, infratemporal fossa, and left
orbit, encasing the medial rectus muscle suggestive
of hemangioma  [Figure  3a and b]. The patient was
referred to a vascular surgeon where they were advised
to wait and watch for possible spontaneous regression
with age as she was only 3 years old on date. The first
periodic visit after 3 months revealed slight regression
of the lesion [Figure 2c].
a b
Figure 1: (a and b) Facial asymmetry on the left side with narrowing
of the left eye
a b
Figure 2: (a and b) Bluish purple pinpoint lesions on the palate and alveolar mucosa with overlying yellowish slough. (c) Slight regression of
the lesion after 3 months
210 Journal of Indian Society of Pedodontics and Preventive Dentistry | Volume 37 | Issue 2 | April-June 2019 |
Discussion
The term “hemangioma” is a generic one used
to describe congenital hamartomas and vascular
malformations. Although Hemangiomas’ are
overwhelmingly benign, they still remain under the
scanner on whether they represent a true neoplasm,
malformation, or hamartomas, and thus have been
the subject of continuous debate, and use of the terms
hemangioma and malformation can be confusing,
which could well be applied to our case.[4,5] The
etiopathogenesis of hemangioma of infancy had
remained surrounded by speculations and beliefs.
Galen, the Greek physician, thought emotions of the
mother to be the cause of vascular birthmark. The
word “hemangioma” is derived from the Greek word
“haima,” meaning blood, while the term “angioma”
was designated by Virchow to refer to all vascular
anomalies.[6]
The prevalence of hemangiomas is estimated to be
around 2%–3% in neonates, 10%–12% below 1  year
of age, and 22%–30% found among babies who at the
time of birth weigh <1000 g.[7] These lesions represent
the most common growths in infancy and childhood,
and may vary from small innocent birthmarks to large
disfiguring tumors. IHs usually appear 2–4 weeks after
birth; grow rapidly till the age of 6–8 months and then
slowly develop. By the age of 5–8 years, they start to
involute and spontaneously regress in 70% of the cases
and so were advised to observe as in our case.[8]
Historically, hemangiomas have been classified in a
variety of ways. According to Enzinger and Weiss,
hemangiomas are broadly classified into capillary,
cavernous, and miscellaneous forms such as
verrucous, venous, and arteriovenous hemangiomas.
An important descriptive classification is related
to the depth of soft‑tissue involvement given as:
superficial, deep, and mixed and as per location as
focal or segmental.[9] Our case would clearly fall under
deep and segmental types based on the classification
charts.
Although hemangiomas are the most common
benign tumors of the head and neck in children, their
occurrence on the palatal mucosa is extremely rare.
c
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In 80% of cases, hemangiomas are known to be seen
as single lesions. They usually, but not invariably,
follow a benign course. Although the exact cause is
unknown, some authorities believe that this lesion is
not a true lesion, but rather a developmental anomaly
or hamartoma. It has also been hypothesized that
angiogenesis likely plays a role in the vascular excess
a b
Figure  3:  (a) Axial slice view on magnetic resonance imaging.
(b) Coronal slice view on magnetic resonance imaging
Figure 4: Algorithm for the management of infantile hemangioma
Journal of Indian Society of Pedodontics and Preventive Dentistry | Volume 37 | Issue 2 | April-June 2019 |
Pauly, et al.: Infantile hemangioma
present. Cytokines, such as basic fibroblast growth
factor and vascular endothelial growth factor, are
known to stimulate angiogenesis. Excesses of these
angiogenic factors or decreases of angiogenesis
inhibitors have been implicated in the development of
hemangiomas.[10]
Hemangiomas have also been seen to be associated
with cerebrovascular and facial arterial anomalies.
It was first noticed by Pascual‑Castroviejo. Facial
hemangiomas were first described by  Reese et al.
The acronym PHACE, which stands for posterior
fossa brain malformation, hemangioma, arterial
lesion, cardiac abnormalities and eye abnormalities,
was first proposed by  Frieden et al.  However, its
occurrence is very rare. Since PHACE syndrome has
a multisystem involvement, a thorough evaluation
prior to surgery will help rule out the possibility of
such a condition.[11]
Hemangiomas may mimic other lesions clinically,
radiographically, and histopathologically. The
differential diagnosis of hemangiomas includes
pyogenic granuloma, peripheral giant cell granuloma,
peripheral ossifying fibroma, chronic inflammatory
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Pauly, et al.: Infantile hemangioma
gingival hyperplasia  (epulis), epulis granulomatosa,
varicocele, telangiectasia, and squamous cell
carcinoma.[12]
In our case, the hemangioma was seen extending up
to the medial rectus muscle of the left eye. Literatures
have shown that of the entire eyelid and orbital
tumors seen in childhood, IHs are the most common.
The incidence of IH is referenced in the literature
between 3% and 10%. Although they are considered
benign lesions that have a generally self‑limited
course, in the periocular region, they have the
potential to cause amblyopia, strabismus, and severe
disfigurement.[13]
As far as management is concerned, there is no “gold
standard” treatment applicable to all patients, and
multidisciplinary management is often needed for
best efficacy. Based on the articles published by Zheng
et al. in 2009 and 2013, we have charted an algorithm
to devise an effective treatment plan for treating IH
[Figure 4].[13,14]
Usually, only 10%–20% requires treatment because
of their size, location, or their behavior since the
vast majority undergo spontaneous regression.
Although different therapeutic procedures including
microembolization, radiation, cryotherapy, sclerosing
agents, corticosteroids and, recently, laser therapy
have been reported, complete surgical excision of these
lesions, if possible, offers the best chance of cure.[10]
The advent of neodymium: yttrium aluminum garnet
laser, in particular, which can be used in a contact
mode with fiber‑optic hand piece, makes it the most
suitable surgical option. The principal advantages
are immediate hemostasis and little damage to the
surrounding tissues, and so no postoperative pain. In
addition, a sterile field is obtained, so there is no risk
of secondary infection and no need for postoperative
periodontal dressing.[15] The prognosis of hemangioma,
in general, is excellent since it does not tend to recur or
undergo malignant transformation following adequate
treatment.[10]
However, one of the great difficulties in the
management of IH lies within the decision to initiate
therapy. Since many IHs may resolve spontaneously
and interventions carry risk, the need to intervene
is not always obvious. Parents and physicians alike
approach the decision to intervene with anxiety and a
degree of uncertainty.[16]
Conclusion
Benign tumors like IH are highly unpredictable in
most cases. Although they tend to involute in time,
so wait and watch would be the wise and advisable
choice. But on the flip side, few isolated cases do not
involute and may turn threatening based on the extent
and sites of involvement. Thus, it indicates the need
212 Journal of Indian Society of Pedodontics and Preventive Dentistry | Volume 37 | Issue 2 | April-June 2019 |
for timely and accurate diagnosis of cases of IH at
an early age, followed by a tremendously important
sequence of mandatory periodic recalls and finally
an essential need for extensive precautions to be
undertaken for surgery, for there is adequate chance
of heavy bleeding which could turn life‑threatening if
not curtailed.
Declaration of patient consent
The authors certify that they have obtained all
appropriate patient consent forms. In the form the
patient(s) has/have given his/her/their consent for
his/her/their images and other clinical information
to be reported in the journal. The patients understand
that their names and initials will not be published and
due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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