vascular anomalies presentation
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Vascular Anomalies -A Review
Division of Oral and Maxillofacial Surgery
College of Dentistry
King Saud University.
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Introduction : Vascular anomalies
Vascular lesions in the head and neck region
can result in significant cosmetic problems
for the patient, and some may lead to even
serious life threatening hemorrhage.
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Vascular anomalies
In the past, there has been confusion regarding the propernomenclature for vascular lesions.
In 1982, Mulliken and Glowacki biologically classifiedthe vascular anomalies of the maxillofacial region basedon their clinical behavior and endothelial cell
characteristics into two groups: hemangiomas andvascular malformations.
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Hemangiomas
Hemangiomas, are the most common tumors ofthe head and neck in infancy and childhood,
comprising approximately 7% of all benign soft
tissue tumors .
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Development :Hemangiomas
The hemangioma is a true vascular tumorthat results froma overgrowth of normal vascular tissue .
It exhibits relatively rapid early growth untilapproximately 6 to 8 months of age (proliferative phase),followed by regression by 5 to 9 years of age (involutory
phase).
It grows by endothelial proliferation. During the rapidgrowth phase, an increased number ofmast cells is seen
within the endothelial wall.
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Development :Hemangiomas
The majority of the hemangiomas in infants are noted bythe parent within the first month of life.
Hemangiomas are initially noticed as an erythematous,
macular patch, which progresses through a rapidproliferative phase whereby it changes its color and growsfaster than the commensurate growth of the child.
By the time the patient is 12 months of age mosthemangiomas have shown signs of involution. Theprocessof involution is normally slow and will not be completeduntil the age of5 to 9 years.
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Clinical presentation :Hemangiomas
Hemangiomas are found in the
superficial tissue, the deep tissue, or
both and may affect organ systems
such as the liver, lung, spleen, and
gastrointestinal tract.
Most superficial hemangiomas can be
diagnosed by clinical examination and
a detailed and accurate history.
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Clinical presentation :Hemangiomas
Deephemangiomas involve muscle or visceral organs and,
are more difficult to diagnose. Therefore, further
diagnostic studies are required.
Intra-osseous hemangiomas are extremely rare. However
the soft tissue lesion may deform the underlying skeleton.
The predilection for females is approximately a 3 :1 ratio.
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Clinical presentation :Hemangiomas
On examination, the superficial
hemangioma usually consists of a raised,
reddish to purple tumor with a distinct
margin.
In contrast, deep subcutaneous
hemangiomas often have a deep bluish
hue with normal overlying skin, making
diagnosis more difficult.
Both the lesions are firm to palpation and
do not pulsate or exhibit any thrills or
bruits.
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Investigations :Hemangiomas
Computed tomography (C. T .Scan) and Magneticresonance imaging ( M. R. I) imaging techniques are used
as diagnostic aids to document the extent of the deep
hemangiomas.
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Investigations :Hemangiomas
Arteriographyis rarely indicated for thediagnosis of a hemangiomas.
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Management :Hemangiomas
Observation and parental support are the initial approachesin the management of maxillofacial hemangiomas.
If functional compromise such as visual change, airway ormasticatory compromise, bleeding, ulceration, or infection
occurs intervention is necessary.
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Management :Hemangiomas
This may initially involve cortico-steroids for rapidly
proliferating lesions or therapy with interferon alfa-2a.
Surgery is generally reserved for small lesions and as a
secondary procedure after initial therapy and involution.
Treatment modalities include routine excision, injection ofsclerosing agents, cryotherapy, and ablation using an argon
laser.
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Vascular Malformations
Vascular malformations are present at birth and unlike
hemangiomas, do not go through a a rapid proliferativephase and they do not involute.
They grow commensurately with the patient.
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Vascular Malformations- Types
Vascular malformations may be capillary, venous, arterial,
or combinations of these.
Approximately 31% of these malformations are found in
the head and neck region.
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Vascular Malformations
Vascular Malformations are divided into two categories:
Low-flow and High-flow lesions.
Capillary, venous, and lymphatic malformations exhibit
low flow lesions.
Arterial and arterio-venous malformations exhibit highflow and are capable of severe hemorrhage with
significant morbidity.
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Development :Vascular Malformations
Vascular malformations are thought to result when thereis
interruption at a particular stage of development of a vessel.
The type of vascular malformation that results depends on
the stage at which normal morphogenesis is interrupted.
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Development :Vascular Malformations
Thus, vascular malformations are sub-classified by tissue
type into capillary, venous, arterial, lymphatic, andcombinations thereof, with the type of malformation that
develops depending on the type of tissue affected during
abnormal development.
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Development :Vascular Malformations
Trauma, infection, and hormonal fluctuation(pregnancy or
puberty) may stimulate increased growth of the vascular
malformation.
The mechanism of growth is not increased endothelial
proliferation - which is within a normal range in theselesions, as is the number of mast cells but alteration in
the flow dynamics within and around the lesion.
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Development :Vascular Malformations
This results in recruitment of collateral vessels and
dilatation of involved vessels.
Unlike the hemangioma, the vascular malformation
exhibits a steady increase in growth, without signs ofinvolution.
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Clinical presentation :Vascular Malformations
In many cases, the diagnosis of a vascular malformation
can be made from the patients history.
Although present at birth, these lesions are often not
identified immediately, but only later on when the lesion
enlarges enough to be clinically identifiable. This isparticularly true forintra-bony lesions.
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Venous Malformations - Low-flow lesions
Venous malformations are bluish, softand easily compressible, andauscultation reveals no bruits.
These malformations can vary fromsuperficial, localized, mucosal spongyectasis to complex invasive lesions that
permeate tissue planes and alter the
regional anatomy.
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Venous Malformations
Any maneuver that increases venous pressure
(e.g.Valsalva maneuver or supine positioning) can
temporarily enlarge a venous malformation.
The clinical absence of pulsations or a thrill generally
indicates a low flow Venous vascular malformation.
Phleboliths that may be noted on radiographic examinationare found only in low flow lesions.
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Venous Malformations -Phleboliths
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Capillary Malformations -Low-flow lesions
Capillary malformations may be smooth initially butbecome more pebble like as the patient grows.
Intra-orally, these lesions are often comprised of lymphatictissues and therefore take on an irregular, pebbly surface;
sometimes called as salmon eggs.
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Capillary Malformations - Low-flow lesions
Port-wine stains,
telangiectasias and
capillarymalformations may
appear pink in infancy
and darken to a deep
purple in childhood.
Port-wine stains,
telangiectasiasand
capillarymalformations may
appear pink in infancy
and darken to a deeppurple in childhood.
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Capillary Malformations:FamilialTelengectiasis
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Lymphatic Malformations -Low- flow lesions
Lymphatic malformations are normally colorless;however, combined lesions take on the hue of theadditional vessel type.
Lymphatic-venous malformations may appear deeppurple while capillary-lymphatic malformations can bepink to purple.
These malformations can become invasive by dissectingalong tissue planes and can cause bony hypertrophy,distortion or both. As these are lymphatic tissues,infections may result in rapid enlargement of these lesions.
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Lymphatic MalformationsLow- flow lesions
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Arterial / Arteriovenous Malformations -High-flow lesions
Arterial or AV Malformations are high flow lesions, which
consist of a large high-flow vessel leading into a multitude
of smaller lower-flow lesions.
It is the lower-flow vessels which induce the development
of the feeder vessels.
Thus the resulting collateral circulation makes these
malformations very difficult to manage.
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Arterial / Arteriovenous Malformations
Clinically these lesions appear stained, warm and tender topalpation. There may be swelling or asymmetry.
A bruit may be detected.
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Arterial / Arteriovenous Malformations
Often a patient presents with severe bleeding as the firstsign that a high flow-lesion is present. They may also
complain of recurrent gingival bleeding and loose or
depressible teeth.
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Investigations :Vascular Malformations
Many vascular malformations demonstrate few
radiographic signs until well into adolescence and a
significant percentage will never show any bony changes.
If the lesion involves bone, then a soap bubble or a
honeycomb appearance is the usual radiographicfinding.
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Investigations :Vascular Malformations
Magnetic resonance images (MRI) may differentiate low-flow from high flow lesions. The presence of fattydeposits, venous lakes, phleboliths in the MRI are all
indicative of low- flow lesions.
CT scans document a lesions extension into thesurrounding soft tissue.
Doppler imaging can also distinguish high flow lesionfrom low flow lesions.
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Investigations :Vascular Malformations
Arteriography cannot only delineate the size of thelesion, but can also assess the rate of flow through the
lesion.
Moreover, super-selective embolization of the selective
feeding vessels to the high flow vascular lesion can be
achieved simultaneously while performing the
arteriography procedure.
This procedure is usually done before surgery, in order to
prevent the risk of serious hemorrhage during surgery.
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Management :Capillary Malformation
In the head and neck area, the argon laser has
proven to be very effective in altering unsightlysuperficial blemishes that are caused by capillary
malformations,port-wine stains and other
telangiectasias.
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Management :Capillary Malformation
It is important to remember that port-wine stains
may occur in association with lymphatic, venousor arterial malformations. Therefore, it is
important that management of these cases be
based upon the characteristics of any deeper
malformation.
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Management :Lymphatic Malformation
Lymphatic malformations can prove to be more difficult totreat because of the poor demarcation and the infiltrativenature of the lymphatic vessels.
Therefore for the lymphatic malformations several non-surgical modalities like steroid therapy, radiation therapyand sclerotheraphy are available.
Bleomycin sclero-therapy, O.K 432 sclerotheraphy arefound to have promising results in the treatment oflymphatic malformations prior to and possibly precludingsurgical intervention.
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Management :Venous Malformation
Some of the Low-flow Venous malformations lesions may
cause bleeding during surgery, and therefore it is
important to determine the lesions size and flow dynamics
with the help ofarteriography before surgery.
The treatment of a true low flow venous malformation is
based on the size and location of the lesion.
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Management :Venous Malformation
Sclerosant theraphy alone has been shown successfulfor smaller malformations. Direct injections of sodium
morrhuate, boiling water, alcohol, and Ethibloc haveproven to be effective in fibrosing these smaller lesions.
Larger, invasive malformations have been treated with a
combination of sclerosant and surgical therapies.
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Management :Venous Malformation
Kane et al. have recently demonstrated significant patient
satisfaction with sclero-therapy with tetradecyl sulfate
(Sotrdecol) combined with conservative ablation.
It is important to know that in most cases sclerosant
therapy is purely an adjunct to proper surgical ablation.
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Management :Arterial or A-V Malformation
High flow lesions can have turbulent blood flow,
introducing the risk of consumption coagulopathies.
Therefore proper pre-operative hematological studies have
to be carried out.
Pre-ablation selective arterial embolization has improved
the surgical success rates for arterial and A-V
Malformations, especially the intra-osseous ones.
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Management :Arterial or AV Malformation
The goal of embolization is to decrease flow in themalformation while avoiding disruption of flow through
proximal feeders.
Any surgical intervention should follow within 24 to 48hours of embolization, and never later than 10 days. It is
believed that collateral flow to the malformation developssoon after embolization and that delaying surgeryincreases the possibility of intra-operative bleeding and
postoperative recurrence.
The goal of surgery should be total excision of themalformation.
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Management Flow Chart Vascular lesions
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