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Review Article
Vascular lesions of the Head and Neck: A Review
Upender Malik1*, Shilpa Dutta Malik2, Shally Raina1
Department of Oral Medicine and Radiology, Seema Dental College and Hospital, Rishikesh249201, Uttarakhand, India
2 Department of Oral Pathology, Seema Dental College and Hospital, Rishikesh-249201,
Uttarakhand, India
1
Abstract
Vascular masses which are capable of causing significant abnormalities and life threatning
conditions are heterogeneous group of lesions derived from blood vessels with differing
histologies, clinical courses, imaging appearances and treatment options,with vascular
malformations always present at birth and growing in proportion to body growth and never
regress spontaneously. In the present review article , an attempt has been made to draw
attention to the current classification, terminology, clinical features, natural history,
diagnosis, various syndromes associated and management of these lesions.
Key words:
vessel.
Hemangiomas, vascular malformations, involuting, non involuting, feeder
Cite this article as:
Upender Malik1, Shilpa Dutta Malik, Shally Raina. Vascular lesions of the Head and Neck:
A Review. American Journal of Advances in Medical Science.2015; 3(4):9-20.
Introduction
Vascular masses are a heterogeneous group
of lesions derived from blood vessels with
differing histologies, clinical courses, imaging
appearances and treatment options. These
lesions are of great importance as they are
capable of causing significant abnormalities
and life threatning conditions. Before 1982,
there was a great confusion regarding the
classification of vascular lesions owing to
their complex constitution and varied clinical
features. Mulliken & Glowacki in 1982 gave a
classification for vascular lesions on the basis
of histological and clinical characteristics
which divided these lesions broadly into
Hemangiomas and malformations. [1]. Three
types of benign blood and lymph vessel tumor
(hemangiomas, lymphangioma and hygroma)
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not only have a common origin, but grow in
an identical fashion by projecting buds of
endothelial tissue. Hemangiomas are the most
prevalent benign tumors of infants and
children and are generally composed of
vascular spaces, they are known as true
Hamartomas as they arise from endothelial
cells and not by incorporation of nearby
vascular channels and hence are haratomas
rather than true neoplasms. Vascular
malformations on the other hand are thought
to result when there is interruption at the
particular stage of development of a vessel
hence its type is dependent on the stage at
which normal morphogenesis is interrupted.
As the commonest site of occurrence of these
vascular lesions are head and and neck, the
massive disfigurement caused by unusually
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9
Conflict of Interest: None declared
Page
Source of Support: Nil
10
Vascular lesions of the Head and Neck: A Review
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10
abnormal lesions are of great concern to the
patients psychologically and the presence of
these lesions at locations such as nose, eyes
and viscera’s indicates immediate treatment
in
order
to
avoid
life
threatening
complications. In this review article, clinical,
radiological, pathological and management
aspects of these lesions along with a
classification system that can be followed
clinically as well as pathologically are
discussed.
Classification of Vascular lesions
Before the 1980s, terminologies such as
(portwine stain, strawberry haemangioma,
salmon patch) were only based upon clinical
appearance and lacks correlation with the
biological behavior or natural history of these
lesions [2]. Mulliken and Glowacki developed
a system of biological classification of
congenital vascular lesions & divided these
lesions broadly into two distinct entitiesHemangiomas and vascular malformations.
Original classification as given by Mulliken
and Glowacki in 1982 as follows:
Classification of vascular lesions in infants
and children
Hemangiomas
 Proliferating phase
 Involuting phase
Malformations
 Capillary
 Venous
 Arterial
 Lymphatic
 Fistulae
Further, Hemangiomas have been categorized
into congenital (which is present at birth) and
more common (which appear after birth).
Congenital
Hemangiomas
are
further
subdivided into rapidly involuting congenital
Hemangiomas
(RICH),
non
involuting
congenital
Hemangiomas
(NICH)
and
congenital non- progressive hemangiomas.
On the basis of this further categorization an
improvement was made in the original
classification [3].
Improved classification of Hemangiomas
and vascular malformation
Hemangiomas
 Superficial (capillary hemangioma)*
 Deep (cavernous hemangioma)*
 Compound
(capillary
cavernous
hemangioma)*
Vascular malformations
 Simple lesions
 Low flow lesions
 Capillary
malformation
(capillary
hemangiomas, port- wine stain)*
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 Venous
malformation
(cavernous
hemangiomas)*
 Lymphatic malformation (lymphangioma,
cystic hygroma)*
 High flow lesions
 Arterial malformations
Combined lesions
 Arteriovenous malformations
 Lymphovenous malformations
 Other combinations
(*) old terminology within brackets.
Hemangiomas
Hemangiomas soon appear after birth,
although upto 30% may already be apparent
at birth. They typically proliferate during the
first year of life and then involute during the
childhood
years.
Vast
majority
of
Hemangiomas are found deep in or just deep
to the skin, those orginating in papillary
dermis stretch the overlying skin as they
proliferate and present as a bright red
macular or papular mass. Lesions originating
within the reticular dermis or subcutaneous
tissue sometimes expand the overlying skin
and separate it from the epidermis by a layer
of collagen, imparting a bluish hue to the
epidermis or appear colorless. On occasion
both superficial and deep components will be
present, and the resultant lesion will have
elements of both capillary hemangiomaand a
cavernous hemangioma. These lesions are
known as ‘capillary cavernous’ hemangiomas.
Strawberry
and
capillary
cavernous
Hemangiomas are in reality superficial and
cavernous Hemangiomas are deep. The term
compound hemangiomas is used for both
capillary cavernous types. During the
proliferative phase, tubules of plump
proliferating endothelial cells with frequent
mitosis are a dominant histological feature.
As the end of proliferation draws near, mast
cells become less active, and, as this
happens, they progressively flatten until,
during involution, hemangiomas are made up
of flat, inactive, normal appearing endothelial
cells, surrounding large ecstatic vascular
channels in a matrix of fibro-fatty tissue.
Natural history of Hemangiomas
They are the most common tumors in infants
with very rare incidence among Asian and
black children (0.8% - 1.4%). Familial
tendency although not present still 10% of all
white infants are affected, (1 out of every 10
children with hemangiomas will have a
positive family history) [4]. Although 30% are
present at birth, the majority present during
the first weeks of life showing an earliest
lesion as a blanched macule which increases
in contrast when the child cries. Blanching
seen in early lesion is due to the over activity
of endothelial cells resulting in plumping of
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Vascular lesions of the Head and Neck: A Review 11
Figure-2: Rapidly proliferating superficial
(capillary) Hemangiomainvolving the upper
lip showing ulcerations in one year old
female patient
Most hemangiomas are solitary focal lesions,
and the head and neck is the most common
site (60%) of involovement [5]. In the head and
neck, focal lesions may appear anywhere
along a line starting in the middle of the
cheek and passing lateral to the eyebrow,
then over the top of the eyebrow to the
glabella. From there the line passes medial to
the medial canthus, down to the paranasal
sinus area, and then across the alar groove to
the nasal tip and columella. The midline of
the upper lip and the lateral aspect of the
lower lip are also the common and most
involved sites (Figure-1). Exact reason for site
predominance is not known, but can be
contributed to the appearance of lesions
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Figure-3: Portwine stains involving the
trigeminal dermatome
Proliferation
History of proliferation generally during the
first year of life is present, but lesions fully
grown at birth also are seen which involute
rapidly within few months of life [6]. Highly
variable rate and timing of growth are present
usually during neonatal and early infancy
and a growth spurt in between 4 and 6
months of age with ultimate size of the lesion
depending upon the degree and the duration
of proliferation. Features seen in such lesions
are characterized by plump proliferating
endothelial cells [1]. In the early stages,
sheets of proliferating endothelial cells are
evident with apparent disorganization and an
absence of vascular channels. Mast cells
occur in the early and middle of involuting
phases [2]. The physical features of the
hemangioma depend on its depth with respect
to skin, its size, and its stage in involution. A
superficial hemangioma will be present as a
bright red mass, whereas a deep lesion will be
bluish or colorless, the main difference being
the depth of the lesion with respect to the
collagen layer of the papillary dermis. Since
collagen scatters visible light and red light
penetrates deeper than the blue light, red
blood vessels appear bluish through collagen
in the same way a deep nevus made of dark
brown pigment, will appear blue through
collagen. During involution superficial lesions
will turn a more dusky color, and, by late
involution, they will have assumed a
decidedly purple color. Hemangiomas range in
size from a pin head to the size of infants
head. Proliferating heamngiomas are firm in
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Figure-1: (A) sites of predilection of focal
Hemangiomas of the face. (B) An
alternative patten with diffuse
involvement tends to follow trigeminal (V)
dermatomes.
corresponding with lines of embryological
fusion. Perhaps, totipotential cells at these
sites retain their ability to develop into
endothelial cells and /or pericytes, and,
under
the
influence
of
angiogenesis
stimulation, they develop into blood vessels. A
less common, alternative pattern of diffuse
involvement tends to follow the facial
dermatome distribution.
Page
the cells causing temporary block in the
internal vascular space, leaving little or no
room for red blood cells. Continued
proliferation eventually increases the vessel
diameter, thereby allowing more room for
whole blood and thus eliminates the blanched
appearance. As the process continues, an
increase in vessel number will give rise to the
appearance of discrete telangiectasia, which,
by sheer force of vessel number, eventually
coalesece to form red macule.
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Vascular lesions of the Head and Neck: A Review
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12
consistency and will expand with raised
intravascular pressure (Figure- 2).
Involution
By the end of the first year of life involution
begins which is clinically evident as a definite
diminution in the rate of growth. In time,
hemangiomas will feel less tender to palpation
and less tense. Eventually the lesion will
soften and begin to shrink from its center to
its periphery in a radial pattern. Only 40% of
hemangiomas involute with acceptable result.
Therefore 60% of the patients require some
kind of corrective surgery.
Complications of Hemangiomas
Ulceration
It is one of the most common complications
and occurs in up to 5% of cases.. Two
possibilities which may explain this are,
firstly rapid proliferation may distend the
overlying skin, which eventually reaches its
elastic limit, splits, and, in doing so, forms an
ulcer. Secondly, because of the typical
hemangioma derive its blood supply from a
circumferential network that in turn gives off
feeding branches at right angles to the
network,
a
rapidly
proliferating
hemangiomamay well outstrip its blood
supply and necroses at a point most distant
from the blood supply (i.e. the center).
Airway Obstruction
emangiomas obstructing both nasal passages
can cause serious problems in infants.
Laryngeal hemangiomas always pose a life
threatening problem. Cough, cyanosis, and
occasionally hoarseness may be present in
about 50% of all the A large cervical
parapharyngeal or palatal hemangiomamay
also encroach on the upper airway and cause
acute or sub-acute obstruction.
Auditory obstruction
Parotid hemangiomas may obstruct the
external auditory canal.
Visual Obstruction
Periorbital hemangiomas may obstruct the
visual axis and this in turn results in
stimulus deprivation amblyopia, and may
lead to blindness if not aggressively treated
[7]. Even in the absence of obstruction,
anisometric (astigmatic) amblyopia may occur
in association with a hemangiomaof the upper
eyelid. Paralytic strabismus and strabismus
secondary to ambylopia may be found [8].
Haemorrhage
Trauma to the lesion mostly occurs in lesions
which are in exposed locations. Usually 10-15
minutes of pressure stops the bleeding.
Hemorrhage is of a great concern in lesions
associated with bleeding disorders such as
Kassabach-Merrit syndrome where it becomes
challenging to stop the bleeding.
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Congestive Heart Failure
High output cardiac failure is a serious
complication and carries a significant
mortality rate. High cardiac output failure is
much more seen in multiple cutaneous and /
or visceral and / or hepatic lesions. This
complication occurs in rapidly proliferating
stage
(early
infancy),
with
presenting
symptoms of congestive cardiac failure,
anemia and hepatomegaly.
Skeletal Distortion
This is more common due to mass
displacement
effect,
includes
anterior
displacement of the mandible by massive
parotid lesion, outer calvarial changes of the
skull, flattening of the nasal pyramid by large
glabellar lesion and orbital expansion.
Systemic Hemangiomas
Multiple cutanoeus Hemangiomas cases
should be investigated for the potentiality of
systemic involvement. This condition is
known as systemic Hemangiomatosis. An
abdominal ultrasound or MRI scan is usually
diagnostic.
Vascular malformations
Vascular
malformations
are
true
developmental anomalies that were believed
to result from sporadic, non familial
developmental error in the formation of
vascular tissue. Genetic background involves
mutation at position R894W resulting in
increased activity of tyrosine kinase TIE2 in
two families with venous malformations.TIE2
is essential for early vascular development,
and an increase in TIE2 activity may lead to
abnormal sprouting, branching and modeling
of
the
primary
vascular
plexus
[9].
Abnormality in vascular neural malformation
also leads to at least two types of
malformations [10]. Relative or absolute
deficiency in autonomic innervations of the
post capillary venular plexus can be sited as a
reason for Venular malformations, and
arteriovenous malformations can occur due to
the same deficiency but at the level of
precapillary
sphincters
[3].
Venous
malformations may seem to be due to
uncoupling of proliferating endothelial cells
and smooth muscle cells [11]. Involvement of
collecting system vessels (i.e. capillaries,
venules, veins and lymphatic vessels) and not
arteries
is
common
to
all
vascular
malformations. Vascular malformations are
always present from birth, even though they
may not be obvious and only manifest at a
later date [2]. Sexual and racial predilection is
not present. Rate of endothelial turn over is
normal hence, neither proliferates nor
involute. A slow steady increase in size
and/or thickness of the lesion will take place
due to hyperplasia. With advancing age
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Vascular lesions of the Head and Neck: A Review 13
progressive ectasia (dilatation) of the vascular
component will lead to expansion of the lesion
and in some instances nodularity [12].
ISSVA Class
Trauma, sepsis, hormonal modulation or
changes in blood or lymph pressure are
factors associated with rapid increase in size.
Syndromes associated with vascular lesions [22]
Associated
Clinical findingsLINICAL FINDINGS
conditions
Tumor
Kaposiform
Haemangiond
othelioma
Tufted
angioma
Spindle
cell
Haemangiond
othelioma
Diffuse
neonatal
haemangiom
as
Lumbosacral
haemangiom
as
KasabachMerit
Syndrome
KasabachMerit
Syndrome
Maffuci
Syndrome
Mutifocal cutaneous
involvement [24]
Hemangiomas
with
possible
visceral
Associated with tethered cord imperforate anus renal anomalies
and bony abnormalities of the sacrum
Thrombocytopenia,
Hypofibrinoginemia
congulopathy
in
association with vascular tumor and occasionally in association
with lymphatic malformation [25]
thrombocytopenia, Hypofibrinoginemia congulopathy in association
with vascular tumor and occasionally in association with lymphatic
malformation [25]
Multiple hemangioendotheliomas endochondromas associated
malignancy [26]
Venular malformations
Midline Venular Malformations are commonly referred to as salmon patch, stork bite or angels
kiss (older terminologies). Lesions are often transient and fade within first year of life, often
involve midline structures with nape of the neck is the common site followed by anterior midline
lesions involving upper eyelids, forehead, glabellum, nasal alae, and philtrum of the upper lip plus
the lumbar sacral area. Anterior midline lesions involving forehead and glabellum are typically V
shaped and correspond with the distribution of the supra trochlear and supra orbital nerves.
Involvement of the nose is typically in the supra alar crease, and the lip involvement is typically in
the upper two thirds of the philtrum. These lesions presents as light pink macule, this may be
confluent with distinct border or non confluent. Anterior midline lesions are non confluent where
as posterior lesions are confluent.
Venular malformations (Portwine stains)
These are true malformations of post capillary venules within the papillary plexus. Etiology
involves sick dermatome theory in which there is an absolute or relative deficiency of vascular
autonomic and sensory vascular innervations as the underlying pathology. Lesions with an
absolute deficiency will progress more rapidly and early hypertrophy with cobblestoning is likely,
whereas a relative deficiency of autonomic innnervation will give rise to slower progression. Males
and females are affected equally with lesion presenting as a flat, pink macule mostly on the head
and neck. Involvement of sensory dermatome i.e. trigeminal dermatome completely or partially
can be seen. V2 dermatome is most commonly involved followed by mandibular V3 and then
ophthalmic V1 dermatome (Figure-3) [13]. Mucosal involvement is common, especially if midfacial
V2 lesion is present where vermillio-cutaneous border, lip mucosa, and maxillary gingival may be
involved. These lesions progress with advancing age and by puberty reaches to a dark deep red
lesion which soon progress to dark purple, thick, cobblestoned portwine stain by 30 years of age.
The natural history relates to sick dermatome theory according to which early and more rapid
progression of the lesion is due to relative or absolute deficiency in the innervations of the venular
plexus. Early darkening, thickening, and cobblestone formation can be expected. Patients in
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13
Haemangioma
of infancy
Posterior fossa malformations. Haemangioma of the face, arterial
Anomalies (especially the cerebral Arteries, cardiac anomalies and
coarctation of the aorta. Eye abnormalities (such as
microphthalmia.Homer syndrome, cataract increased retinal
vascularity). PHACE may also be associated with sternal cleft,
supraumbilical raphe and a variety of other brain anomalies.
Represents a spectrum of findings. Not all features need be present
to diagnose PHACE syndrome marked predominance in females
[23])
Page
PHACE
Syndrome
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Vascular lesions of the Head and Neck: A Review
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14
whom there is a relative deficiency will, on the other hand, experience slow progression of their
lesion. Skeletal changes are sometimes associated in the form of bony hypertrophy. This is
especially true of V2 lesions that extend onto mucosal and gingival surfaces of the maxilla.
Localized hypertrophy of the underlying bone, increased spacing between the teeth and a
prominence of the upper lip, due in part to underlying hypertrophy of the alveolar ridge is seen.
While soft tissue hypertrophy is common, especially in older patients, the role of bony
hypertrophy is often unrecognized, and can lead to unnecessary and ineffective surgical debulking
of the lip. Struge-Webber syndrome in which dermal vascular plexus involvement along with
choroid involvement and ipsilateral meningeal invovlment is associated with portwine stains.
Patients with this syndrome are present with glaucoma and sequelae of neural involvement such
as seizures, cerebral atrophy, degeneration and subdural hemorrhage, the cutaneous component
of strugeweber syndrome is similar to venular malformation accept it involves both sides of face.
Hypertrophy of subjacent maxilla and mandible and soft tissue hypertrophy of the skin and
appendages are also more common.
Combined malformations
Capillary Heperkera
Venous
totic
cutaneous Cerebral capillary malformations [Eerola et al, 2000]
capillary
Venous
malformat
ions
Klipped
Capillary, deep and superficial venous and arterial malformations
Trenaunay with limb asymmetry [Berry etal. 1998] Complex malformation and
CapillaryLym syndrome
overgrowth syndrome with vascular and or lymphatic malformation
phatic[Biesecker et al 1999]
Venous
Proteus
Similar to Klippel Trenaunay syndrome with AV fistula
syndrome
Capillary
Parkessimilar to Klippel Trenaunay syndrome with AV fistula and possible
lymphatic
Weber
associated high output congestive heart failure Schachnerand and
venous
syndrome
Hansen, 1996].
arterial
Venous malformations
Venous malformations may be superficial or
deep, localized, multicentric or diffuse. The
overlying skin or mucosa varies in color
depending on the depth and degree of ectasia
of the underlying malformation. Because
expansion invariably progresses along planes
of least resistance, superficial malformations
will expand toward the surface or the mucosal
surface
and
impart
a
deep
purple
discoloration. Deeper malformations impart
varying degrees of bluish hue-the deeper the
lesion, the less is the discoloration. Very deep
lesions such as buccal fat space lesions
appear as flesh color mass. The overlying skin
is not discolored and is usually soft and
compressible. Expansion of the lesion occurs
on crying due to increase in venous pressure.
Phlebothrombosis occurs with a greater
frequency in ectatic lesions and phleboliths
are consider the hallmark of venous
malformations. These lesions may be unifocal
or multifocal and certain sites such as buccal
mucosa, the tongue, the oral commisure, the
lower or the upper lip, the buccal pad fat
space and neck are more commonly involved
in head and neck region. Anatomical borders
are not a limitation for venous malformations
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and hence, muscle, salivary glands and skin
infiltration are common and on occasion,
lesions may be entirely intraosseous.
Mandible is most commonly involved followed
by maxillary nasal, parietal and frontal
malformations [14]. Mandibular lesions
usually appear as painless, slow growing
masses. Increased spacing between teeth and
increased mobility of the teeth due to the
expansion of the buccal cortical plate and
bleeding around the gingival necks of the
teeth are all the manifestations [15].
Uncontrolled and massive bleeding from the
tooth socket immediately after extraction may
be the first sign of an intraosseous venous
malformation.
Other
than
this,
bony
hypertrophy and/distortion along with soft
tissue hypertrophy can also be evident.
Lymphatic malformations
All congenital malformations of the lymphatic
system that results in a contiguous mass of
dilated lymphatics are known as lymphatic
malformations.
In these malformations the primary defect is
believed to be at the level of the efferent
channels. Obstruction at this level, whether
relative or absolute, will result in dilation of
the proximal channels that form the mass
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Vascular lesions of the Head and Neck: A Review 15
[16]. Although present by birth, some lesions
manifest at a later stage. 65% to 75% are
diagnosed at birth, and 80 to 90% are
Capillary
Venous
Cutis
marmorata
telangiectatica
congenital
Phakomatosis
pigmentovascu
laris
Robert-SCPhocomelia
WiedemannBeckwith
syndrome
Hereditary
neurocutaneo
us
angioma
Blue-rubber
Bleb Nevus
syndrome
Glomangiomas
Capillary (or mixed malformations) of the skin associated with
vascular malformations of the central nervous system. [Zaremba et.
al 1979; M1M106070] (33)
Cutaneous venous malformations and venous malformations of
gastrointestinal tract and other sites. Some cases have autosomal
dominant inheritance pattern.
Cutaneous venous anomalies with glomus cells [34]. Some cases
have autosomal dominant inheritance pattern.
Traditionally, these malformations are divided
into
capillary
lymphangioma
(smallest
lymphatic channel malformation), cavernous
(intermediate
lymphatic
channel
malformation) & cystic hygroma (dilated
macrocystic lymphatic malformations). Head
and neck region is the most common site for
lymphatic malformations, and over 90% are
found in the neck. The complexity of the
cervical lymphatic system has been offered as
a
possible
explanation
[17].
Clinical
manifestations vary in accordance with the
extent and depth of the lesion as well as the
degree of fibrous reaction around it. Mucosal
and cutaneous involvement usually results in
the formation of multiple cutaneous or
mucosal fluid filled vesicles. These vesicles
may be connected to larger, deeper lymphatic
cisterns lying within the subcutaneous or
submucosal tissues. The involvement of
deeper tissues follows one of the two patterns,
either massive generalized oedema with poorly
defined borders (the diffuse microcystic
variety) or a localized area of multilocular
cysts (the macrocystic variety). Cervical
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lesions (previously known as cystic hygromas)
tend to be localized, macrocystic and thus
amenable to complete surgical resection. The
rate of growth varies the earlier the diagnosis,
the more aggressive the lesion. Low grade
lesions present later and are less inclined to
result in complications. Sudden or rapid
expansion may result from sepsis and
spontaneous
or
traumatic
interlesional
hemorrhage. Both these complications are
frequent, and their abrupt onset may
precipitate a life threatening emergency due
to airway obstruction. Cervical lesions may
displace and eventually compress the the
pharynx, and mediastinal extension may
compress the trachea [17].
Endolaryngeal cystic mucosal blebs may also
embarrass and on occasions, totally obstruct
the airway. Soft tissue and skeletal distortion
are
extremely
common.
Macroglossia,
macrotia and macrochelia are sequelae.
Mandibular hypertrophy will give rise to
prognathism and malocclusion. Due to the
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15
Sturge-Weber
syndrome
Simple malformations
Capillary or mixed malformation overlying spine(sometimes
extending to leg) with associated vascular malformation in the
spinal cord within one or two spinal segments [27]
Capillary malformation of the face with eye disease (choroidal
vascular malformation glaucoma buphthalmos) and neurologic
disease (leptomeningeal vascular malformation seizures hemiparesis
etc) [28]
Reticulated capillary malformation or mixed malformation may be
associated with many anomalies especially port wine stain,
cutaneous atrophy and or ulceration, macro cephaly, ocular
abnormalities neuromotor symptoms limb asymmetry etc. [29]
Association of capillary malformation and dermal melanocytosis
nevus spilus or the other pigmentary anomalies. May have
extracutaneous abnomalities such as psychomotor retardation
epilepsy intracranial calification and eye abnormalities [30]
Sever tetraphocomelia cleft lip and palate mental retrardation poor
survival. Midfacial capillary malformation in many cases. [31]
Abdominal wall defects overgrowth facial anomalies with mid facial
capillary stain [32]
Page
Cobb
Syndrome
diagnosed by the end of the second year of
life. The remainder may manifest as late as
the first pregnancy or even later.
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Vascular lesions of the Head and Neck: A Review
establishment of venous lymphatic shunts
spontaneous regression has been reported.
Arterio-Venus malformations
The term arteriovenous malformation refers to
a congenital malformation made up of
multiple fistulous tracts communicating
between arteries and veins resulting from the
abnormality at pre capillary sphincters. These
lesions are present by birth but clinical
presentation usually is delayed with third,
fourth, or fifth decade of life may be the time
when these are evident. The most common
manifestation
of
an
arteriovenous
malformation is a mass complex comprised of
dilated
tortuous
arteries
and
veins,
occasionally with an overlying portwine stain.
Ulceration of the overlying skin and skeletal
hypertrophy are also seen. AV malformations
are firmer to palpation than venous and do
not empty as readily as venous malformations
on compression. Once compressed, they
rapidly refill.
Pulsation or fluid thrill is
uncommonly associated. These malformations
are firm vascular lesions surrounded by
hypertrophied arteries and dilated veins
becoming prominent with advancing age. An
increased circulating blood volume will
counter the effect of decreased arterial
pressure and in turn lead to an increased
stroke volume, tachycardia and cardiomegaly.
High output cardiac failure is rare, but has
been reported in infants with large
arteriovenous malformations [18]. Indeed, an
otherwise healthy infant can adequately
compensate for an increased cardiac output
for many years before decompensating [19].
The primary abnormality could be due to an
ecstatic capillary bed. An absence of
autonomic nerve supply to the sphincters, an
absence of actual sphincters, or some
deficiency at the neuroreceptors resulting in
the free flow across that particular capillary
bed could be the cause if ecstatic capillary
bed. In time, the vessels in the bed dilate, and
eventually the area supplying the arteries
hypertrophy and vein dilate. The variation in
the age of presentation and speed of
progression is due to absolute or relative
absence of capillary sphincter control.
Ligation of the feeding artery is therefore in
effective in both above and below the
arteriovenous malformation.
A proximal arteriovenous malformation with a
significant shunt may cause an increase in
cardiac output that in turn may lead to high
output cardiac failure. Distal shunting on the
other hand has the propensity to significantly
reduce the flow rate beyond shunt and so
induce peripheral ischaemia (steal syndrome)
without adversely affecting cardiac output.
Because of the presence of high to low
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pressure shunt, an increase flow in the
afferent artery may be seen due to the
decreased peripheral resistance leading to
dilatation and tortuosity of the afferent artery
with subsequent thickening of the wall due to
hypertrophy in the media. In the face of a
large
shunt
across
an
arteriovenous
malformation, the arterial flow distal to the
shunt may reverse direction, thus causing
distal ischaemia, the so called steal
syndrome. The decrease in the arterial
pressure also encourages the development of
an extensive collateral circulation. An
increased flow through the arteriovenous
malformation may also dilate the efferent
venous system with consequent thickening of
the wall due to hypertrophy of the media. As
the lesion matures, the degree of ectasia
increases, and the development of venous
dilatation and arterial hypertrophy becomes
apparent.
Oral
and
maxillofacial
hemangiomas and vascular malformations
are congenital lesions with various clinical
characteristics, manifestations, indications,
and possibilities for treatment. Diagnosis is
made on the basis of clinical examination and
patient’s history. However, the clinical
examination
underestimates
the
deep
extension of the lesion. The feasibility of
treatment and the plan of approach depend
on the accurate delineation of the extent, size
and location of the lesion, Therefore,
complimentary radiographic studies are
essential for treatment [20].
The physical examination of the child with a
vascular lesion typically reveals a soft, raised,
nontender, well circumscribed lesion that
varies in color from blue to red or may have
no color variation from the overlying skin or
mucosa. It may blanch and fill with digital
pressure if it is superficial and contains
blood. A bruit and pulse may be present in
the case of a high-flow arterio-venous
malformation, and it should transilluminate if
it is a macrocystic lymphatic malformation. A
direct flexible laryngoscopy is often very
helpful in evaluating for a possible subglottic
hemangioma. Because it is a dynamic
examination in an awake child, other
anatomic and neurogenic problems can be
ruled out including laryngomalacia, vocal
cord paralysis, reflux laryngitis, and laryngeal
web. The subglottic hemangiomas may be
visualized as a raised pale to reddish lesion
most commonly on the left and posterior, but
it can be in any position and bilateral.
Because of inherent limitations in flexible
laryngoscopy, direct laryngoscopy under
general anesthesia may be required, but
biopsy should not be needed. If the overlying
skin is involved, contrast imaging should be
Vol-3: No-4: 2015
American Journal of Advances in Medical Science
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patient sedation. Gray-scale US is good for
identifying the extent of the lesion and
echogenicity, which relates to cystic versus
solid features. When color flow Doppler
capabilities and spectral analysis are added,
the direction and velocity of flow of blood
within the lesion can be assessed, which can
differentiate between high and low-flow
lesions. Recently it was demonstrated that US
can differentiate a Hemangiomafrom a
vascular malformation and there are also
reports of identification of 48 of 49
Hemangiomas
based
on
their
solid
appearance
on
gray-scale
US.
The
nondiagnostic
Hemangiomawas
isoechoic
with surrounding tissue and could not be
identified, presumably because it was
involuted.
Thirty-eight
vascular
malformations were also studied which
showed Characteristics of color Doppler US
and spectral analysis to be useful in
determining the presence of arteries within
the Hemangiomas and in defining the nature
of each vascular malformation. Other imaging
studies useful in evaluating vascularlesions
include plain radiographs, CT scanning, and
MRI. Plain films are of value in determining
bone or joint involvement, growth disturbance
or presence of phleboliths.CT is useful in
imaging the detail of underlying bone. MRI is
superior in defining soft-tissue differences
and vascular anatomy. An MRI protocol
suggested by Fordham et al. included T2weighted sequences, T1-weighted images with
and
without
gadolinium,
gradient-echo
sequences, and MR angiography or MR
venography
as
needed.
Also,
MR
lymphangiography may be used to evaluate
lymphatic malformations. In fact, MRI has
replaced CT as the imaging study of choice in
evaluating these lesions. Dubois and Garel
designed a diagnostic algorithm for detecting
Hemangiomas and vascular malformations in
which lesions are generally categorized as
typical or atypical. Typical lesions that do not
need to be treated require no investigation,
whereas pretreatment investigation should be
done for the other typical and all atypical
lesions. In this algorithm, US is used as the
initial imaging study for all atypical lesions
and biopsy is recommended for moderate flow
atypical lesions.
Tatsurou Tanaka et al, described fusion of 3D
phase contrast MRA (3D- PCMRA) images
without contrast using heavy T2 weighted
images with 3D- FASE sequences in patients
having head and neck Hemangiomas and
concluded that the fused images allowed for
the non invasive assessment of the
relationship between the Hemangiomas and
the arterial system feeding it [21].
Vol-3: No-4: 2015
Page
done to ascertain whether there is contiguity
with the subglottic lesion.
Investigations
Diagnosis
in
Oral
and
maxillofacial
hemangiomas and vascular malformations is
made on the basis of clinical examination and
patient’s history. However, the clinical
examination
underestimates
the
deep
extension of the lesion. The suitability of
treatment and the plan of approach depend
on the accurate delineation of the extent, size
and location of the lesion. Therefore,
complimentary radiographic studies are
essential for treatment [20]. Clinically the
patient with a vascular lesion typically should
be examined for characteristic features such
as soft, raised, non tender, well circumscribed
lesion that varies in color from blue to red or
may have no color variation from the
overlying skin or mucosa. It may blanch and
fill with digital pressure if it is superficial and
contains blood. A bruit and pulse may be
present in the case of a high-flow
arteriovenous
malformation,
and
transillumination is present in macrocystic
lymphatic malformation. A direct flexible
laryngoscopy is often very helpful in
evaluating
for
a
possible
subglottic
Hemangioma. Because it is a dynamic
examination in an awake child, other
anatomic and neurogenic problems can be
ruled out including laryngomalacia, vocal
cord paralysis, reflux laryngitis, and laryngeal
web. The subglottic Hemangiomas may be
visualized as a raised pale to reddish lesion
most commonly on the left and posterior, but
it can be in any position and bilateral. Direct
laryngoscopy under general anesthesia may
be required in cases where there is
involvement of overlying skin. Contrast
imaging should be done to ascertain the
contiguity with the subglottic lesion.
Imaging
Imaging studies are useful in determining the
accurate diagnosis and extent of the lesions
in question. Lesions located deep to the
subcutaneous tissue appear atypical and are
difficult to assess on physical examination.
Imaging becomes useful in the diagnosis of
such cases also differentiation can be made
between Hemangiomas and other high,
moderate
and
low
flow
vascular
malformations and other lesions. Imaging
studies also document the size of the lesion
pretreatment. Imaging modalities most useful
in assessing these lesions are Plain
Radiographs, Ultrsonography (US), Magnetic
Resonance Imaging (MRI), and Computed
Tomography (CT) scans. The advantages of US
include its wide availability, low cost,
noninvasiveness, ease of use, and no need for
17
Vascular lesions of the Head and Neck: A Review 17
18
Vascular lesions of the Head and Neck: A Review
Treatment
Oral haemangiomas are quite common and do
not necessarily require treatment particularly
as they may not be visible from the outside.
However, positive therapy is indicative if the
lesion
is
aesthetically
unpleasant
or
haemangiomas
producing
episodes
of
bleeding mainly due to trauma or in
haemangiomas or lymphangiomas that are
associated with pain or discomfort.
Different modalities of treatment for the
vascular lesions are as follows:
a) Cryotherapy: The physical principle
behind cryotherapy is based on JouleThompson
expansion
which
enables
substances to undergo a drop in temperature
when moved from a high pressure area to a
lower pressure area. It involves application of
a number of agents including liquid nitrogen,
nitrogen dioxide, liquefied air, CO2 snow, dry
ice, ethyl chloride, acetone- alcohol mixture
and Freon [34]
b) Sclerotherapy: Itis a treatment of choice
for (deep) cavernous haemangiomas which
involves injection of a sclerosant, namely 3%
sodium tetradecyl sulphate into the lesion
with the patient head in the down
position(maintained for 5 minutes), this
allows sclerosant to work. Head is then
brought back into the erect position in order
to empty the lesion and pressure dressing is
applied. The whole procedure brings intimal
damage thereby initiating coagulation and
contraction using agents like invert sugar,
sodium molybdate, ethanolamine oleate and
absolute alcohol have been described.
c) Embolization: This method occludes the
blood vessels proximal to the lesion and
control lethal blood loss. The indications for
arterial embolization include the management
of acute hemorrhage, the management of
tumors, the treatment of arterio-venous
malformations etc. embolization as a single
treatment modality is rarely successful. In
high flow (arteriovenous) malformations, due
to occlusion of the proximal vessels resulting
only in stimulation of the growth of the
collateral blood supply, makes treatment
complicated. Thus multiple approaches are
usually preferred. Precutaneous embolization
using histoacrylic glue is reported in the
treatment of giant mandibular arteriovenous
malformation of the mandible [36].
d) Lasers
 CO2 lasers: functioning in the far infrared
at 10.6/- Lm may be used to remove small
haemangiomas of the cheek and tongue.
Its used to carry out excision rather than
vaporization in the band of tissue [35].
 Nd-YAG lasers: functioning in the near
infrared at 1.06/- Lm has maximal tissue
penetration
combined
with
minimal
absorption by comparison with CO2 laser.
It should be used with caution in the
region of superficial nerves as it will
destroy the integrity of the nerves.
 Tuneable dye and copper vapour lasers: it
is the method of choice for port-wine stains
of the facial region and has superseded the
earlier use of the argon laser in the visible
blue-green.
 Low intensity laser therapy: gallium
aluminum arsenide laser operating at 820
nm
is
used
to
treat
strawberry
haemangiomas (superficial) of the facial
region in infants [35]
e) Radiotherapy: This method may be
applicable in adults with intractable disease
producing major symptoms. However, it
should not be used in infants or children
where the risk of inducing eventual
haemangiosarcoma or carcinoma is possible
[35]. For haemangioma’s of bone, preliminary
embolization is a wise method. Marginal
resection of the involved area going through
uninvolved bone is the safest approach.
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