vascular anomalies presentation

7/30/2019 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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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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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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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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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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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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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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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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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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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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vascular anomalies presentation

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