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Infantile
Hemangioma
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Infantile hemangioma is a benign
neoplasm that commonly develops in
neonates within their first few months
of life. These birthmarks are more
common in whites, and girls are three to
five times more likely than boys to have
a hemangioma. Most infantile hemangiomas
undergo rapid initial proliferation,
with a subsequently plateau in infants
aged about 9-10 months; finally, they
become involuted. The involuting phase
extends from 1 year until 5 to 7 years
of age.
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Photo#1:
Typical infantile hemangioma or
birthmark in the forehead. Photo#2
and 3 show another infantile
hemangioma (birthmark)
in the lower lip. Discoloration
which has sharp margins is clearly
seen. The lesion is soft, but not
easily compressible.
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Hemangiomas
generally affect the head and neck
(80-85%). The trunk and extremities are
less commonly involved.
Some
hemangiomas are easily noticeable
(superficial) usually bright red, flat or
raised patchs on the skin, whereas others
are deeper and may have healthy overlying
skin or only slightly bluish skin
discoloration. Hemangiomas are usually
firm, warm to the touch and can swell when
the baby is upset or sick. Most
hemangiomas are easily diagnosed without
any additional diagnostic tests (eg, MRI).
Deeper hemangiomas or questionable
superficial lesions, however, may require
imaging studies to confirm the diagnosis
and to evaluate their extent. US may be
used during the initial assessment or in
place of MRI if it is unavailable.
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Photo#1
shows a superficial hemangioma or
birthmark, photo#2 shows a large
deep hemangioma, the skin is intact.
Photo#3 shows a small hemangioma
lesion or birthmark in the mid neck
area. Infantile
hemangiomas are occasionally seen in
the extremities. This is a typical
hemangioma lesion in the foot
(photo#4).
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During
the involutional phase, decreasing
vascularity, decreasing enhancement, and
progressive fibro-fatty replacement of the
tumor is present. As the hemangioma
involutes, fatty replacement can easily be
recognized on MRI, which demonstrate fat
signal intensity with some residual
prominent vessels. Lesions adjacent to
bone may cause a smoothly marginated
erosion of the bone. Phleboliths and
calcifications are not features of
hemangiomas.
Some
hemangiomas completely disappear, while
others not and may require corrective
surgery. During the involution phase,
hemangiomas become softer and less tender
to the touch.
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If
the patient is older than 5 years of age,
another type of tumoral mass (eg,
fibrosarcoma) should be favored in the
differential diagnosis, and a biopsy may
be required. When a high-flow lesion is
noted on noninvasive studies, a biopsy
should be performed carefully because the
risk of significant bleeding is high.
Angiography may be necessary for
embolization of associated spontaneous
hemorrhage.
Image#1
is an MRI image obtained following
intravenous contrast administration.
The lesion enhances (bright) and
there are vessels within the
periphery of the lesion, as well as
near the lesion, which is typical
appearance of infantile hemangioma
on MR. Image#2 is also a
post-contrast MRI with fat
suppression clearly outlines the
margins of this tumoral mass
(infantile hemangioma)
On
angiograms, hemangiomas appear as
well-circumscribed masses that have a
lobular architecture with intense and
persistent tissue staining. Prominent and
early draining veins may be present. Less
intense tissue staining is evident with
involuting lesions.
The
differential diagnosis includes other
neonatal and infantile tumors,
particularly soft-tissue sarcomas. In
general, hemangiomas are characterized by
discrete margins, relatively homogenous
signal intensity, and homogenous contrast
enhancement, whereas sarcomas tend to be
heterogeneous.
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Diagnosis
& Treatment |
Diagnostic
Workup ? |
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Treatment
/ Management ? |
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Abnormalities
associated with
hemangiomas? |
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PHACE sydrome: Group
of findings including
posterior fossa
malformations, hemangioma,
arterial anomalies,
coarctation of the aorta and
cardiac anomalies, eye
anaomalies and sternal
cleft. Affected patients
(nearly always girls)
usually have one or two of
the associated abnormalites
rather than all components. |
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Spinal Abnormalities:
Association with spinal
abnormalities in hemangiomas
overlying the lumbosacral
spine. The most common
malformation is a tethered
cord, often accompanied by
an occult lipomeningocele.
Rarely, genitourinary
abnormalities may be
seen. Please see Case
Studies (Case#17) |
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Hypothroidism: Hypothyroidism
is a potential complication
of hemangiomas, particularly
for hepatic hemangiomatosis.
The mechanism is believed to
be tumoral production of
type 3 idothyronine
deiodinase, causing
peripheral inactivation of
thyroid hormone. |
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- This
study evaluating 62 patients
with an initial diagnosis
of infantile hepatic
hemangioma concluded that MRI
features of hepatic
hemangiomas are typical in
most patients and
certain imaging findings are
predictive of the clinical
course. MRI is the
technique of choice in
diagnosing infantile hepatic
masses >>> abstract
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- Case
presentation to report a
case of PHACE syndrome
associated with congenital
glaucoma. This report
concluded that
Ophthalmologists who examine
children with large facial
hemangiomas should consider
PHACE syndrome in the
differential diagnosis and
should obtain appropriate CNS
imaging studies and cardiac
evaluation when the diagnosis
is suspected. Congenital
glaucoma should be added to
the list of PHACE-associated
ocular anomalies >>> abstract
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- Retrospective
study evaluating 100
consecutive patients wit
parotid hemangioma concluded
that infantile hemangioma
in the parotid gland
responds to pharmacological
treatment in a similar manner
as hemangioma in other
locations. Drug therapy was
effective in the majority of
infants with parotid
hemangioma, whether given
because the tumor was large,
deforming, ulcerated, or
involved nearby structures
with functional consequences
>>> abstract
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Imaging of Hemangiomas: Where,
When, Which and Why?
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By Patricia Burrows,
MD - Infantile hemangiomas: Current Knowledge, Future
Directions, Bethesda, 2005
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While numerous imaging
modalities can be used to investigate hemangiomas, clinical
experience has shown that ultrasonography, with Doppler
interrogation, and MR imaging are two most effective modalities.
The choice will be based on the reason for studying the patient
and available modalities in each institution.
If the study is indicated
to distinguish between a deep hemangioma and other entities,
ultrasonography by a radiologist familiar with vascular
anomalies is the most noninvasive and efficient method.
Typically, sonography imaging will confirm the presence of a
solid mass containing high-flow vessels, and supplied and
drained by high-flow vessels. Limitations include operator
dependence and potential inability to predict extent and
presence or absence of associated malformative anomalies.
Doppler interrogation of the feeding and intralesional vessels
demonstrates low resistance waveforms, and draining veins show
turbulent or arterialized waveforms, consistent with
microshunting.
MR imaging is the
preferred study for patients who need documentation of the
extent of disease or determination of the presence of absence of
associated anomalies prior to the institution of
pharmacotherapy. To best determine the nature of the lesion, the
following sequences are recommended: T1W sequence without
gadolinium, T1W with fat saturation post gadolinium, T2W with
fat saturation or Inversion Recovery and gradient flow sequence
or MR angiogram. The field of view should include not only the
obvious lesion but tissues that could be involved by contiguity
with the hemangioma, or associated malformed states.
While CT is inferior to
MRI in demonstrating the morphology of hemangioma, modern
multi-detector scanners can produce high quality examinations
without requiring sedation, because of ultrashort scan times. In
certain locations, CT angiography may be more effective than MR
angiography. In general, institutional availability may dictate
the choice of examination. CT evaluation should be carried out
without and with intravenous contrast administration.
Angiography is performed only when endovascular therapy is
necessary. Embolization is indicated rarely, generally to
control high output cardiac failure or bleeding that is not
responsive to pharmacotherapy.
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Where:
Hemangioma of the Head & Neck. When:
Problematic hemangiomas that do or may require
pharmacological therapy, including hemangiomas of the
orbit and airway or involving high risk
"patterns" such as the beard or regional
distribution. Which:
MR imaging should include the affected area (e.g., orbit,
face, neck) some of the adjacent soft tissues (e.g., neck
and mediastinum) and the brain. |
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Most associated arterial
abnormalities can be identified on routine MR imaging sequences.
Additional detail is provided by MR and CT angiography of the
affected area, but these studies are not necessary for initial
screening. Why: Brain imaging should be carried out
to detect intracranial hemangiomas as well as associated
malformative anomalies including absence, stenosis, or aneurysm
formation of the cervical and intracranial arteries, cerebral
infarction, and cerebral defects such as posterior fossa cystic
anomalies, absent corpus collosum, cortical dysplasia and
hydrocephalus. Hemangiomas in a beard distribution can
involve the airway much more extensively than is clinically
evident.
Where:
Cutaneous hemangioma overlying the spine. When:
As soon as it is recognized. Which: MRI of the spine
without and with intravenous gadolinium is optimal. In the fist
three months of life, sonography can be used as a screen to rule
out intraspinal pathology. Why: Hemangiomas overlying the
spine can be associated with intraspinal extension and dysraphic
lesions including spinal dermoid, tethered cord and
lipomyeloschisis.
Where:
Multiple (more than 4) cutaneous hemangiomas. When:
When skin lesions appear to be enlarging or increasing in
number. Which: Ultrasonography of the liver and brain is
effective for screening patients with multiple cutaneous
hemangiomas. When small hepatic hemangiomas without marked
shunting are detected, monthly re-evaluation with abdominal
ultrasonography is necessary until the lesions have stabilized
or started to regress. However, in the case of extensive, high
flow or enlarging hepatic hemangiomas, MRI should be obtained to
document the extent of disease. Why: The infant with
multiple cutaneous hemangiomas (disseminated hemangiomatosis) is
at risk of having hepatic or cerebral involvement. MR imaging
without/with gadolinium is the most effective imaging method to
distinguish hemangiomas from other hepatic lesions. Multifocal
hemangiomas have characteristic, consistent imaging findings.
They appear as spherical, T2 hyperintense, enhancing masses,
with flow voids. Contrast enhancement occurs centripetally.
Involvement of adjacent organs (e.g., bowel) may also be
identified. The presence of significant shunting can be
predicted by the degree of vascularity (e.g., enlargement of the
hepatic and mesenteric feeding arteries and the hepatic veins
correlates with high flow and clinical signs of a high output
cardiac state). Abdominal compartment syndrome, resulting from
massive replacement of the liver parenchyma and compression of
venous return form the lower body can be readily predicted.
Focal lesions with large vascular structures may simulate
arteriovenous malformations; the presence of a solid parenchymal
component, sometimes not easily seen by ultrasonography,
distinguishes hepatic hemangioma from AVM.
Where:
Extensive perineal hemangioma in the presence of urogenital or
anal anomalies. When:
As soon as is feasible. Which: MRI of the pelvis and
spine. Why: Perineal hemangiomas can be associated with
structural ano-genito-urinary anomalies, such as imperforate
anus, cloaca and renal ectopia. In this situation, intraspinal
hemangioma with or without spinal dysraphism is relatively
common. Arterial anomalies have also been seen.
Where: Anywhere. When:
In the presence of severe
thrombocytopenia. Which: MRI with
gadolinium. Why: The lesion most likely represents a
kaposiform hemangioendothelioma rather than an infantile
hemangioma.
Where:
Focal cutaneous lesion with extremely high flow suggesting
arteriovenous malformation. When: Symptoms
warrant therapy. Which: MRI with MR angiography or
sonography with Doppler. Why: Most focal high flow
neonatal masses represent rapidly involuting congenital
hemangioma (RICH) rather than arteriovenous malformation. MR
imaging with MRA or sonography with Doppler are both useful to
identify the focal parenchymal mass that distinguishes RICH from
AVM. The diagnosis of RICH may obviate the need for invasive
procedures to control the high output cardiac state (e.g.,
embolization or resection).
When:
The hemangioma does not require imaging. Where:
imaging is not necessary for cutaneous hemangiomas with typical
clinical appearance and behavior that do not require
pharmacological therapy.
When:
The suspected hemangioma that presents after six months of age
or has atypical clinical appearance or imaging features.
What: Requires biopsy or excision. Why: The
differential diagnosis of atypical, hemangioma-like mass
includes KHE, other vascular tumors (e.g., angiosarcoma,
hemangiopericytoma, other hemangioandotheliomas),
rhabdomyosarcoma, teratoma, infantile fibrosarcoma and other
developmental lesions.
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