2. Vascular Anomalies
which are classified into two distinct
entities:
1.Vascular tumors (hemangioma) with
proliferation of vascular endothelial cells.
2.Vascular malformations characterized by
the abnormal dilation of vessels without
proliferation.
5. History and clinical examination
•In most cases, an accurate history and
physical examination will help establish the
diagnosis. In cases in which the diagnosis is
equivocal a repeat assessment in 3–4 months
will greatly improve the chance of arriving
at a diagnosis and special investigations are
only occasionally necessary.
6. Magnetic resonance imaging (MRI)
• It provides accurate information about the extent
of the lesion.
• It provides better contrast between the lesion
and surrounding tissues.
• It has multiplanar capabilities.
• It can also help distinguish between the different
types of vascular anomalies
10. Angiography
• It has a specific but limited role in the diagnosis
of vascular lesions, but should not be used as a
first line investigation. It is useful for mapping
out the blood supply of the lesion and in the
assessment of the characteristics of flow of
arteriovenous malformations. Angiography is
usually reserved for therapeutic endovascular
interventions
12. Hemangioma
•It is the most common tumor in childhood
affecting about 10% of neonates.
•The head and neck region is the most
commonly involved site (60%).
•Most lesions are solitary (80%) and girls are
more affected than boys (3:1).
•Hemangiomas can be divided into two main
groups:
13. Hemangioma
1. Infantile hemangioma which appears soon after
birth.
2. Congenital hemangioma which is present at
birth, these are further subdivided into:
a) Rapidly involuting congenital hemangioma.
b) Non-involuting congenital hemangioma.
c) Partially involuting congenital hemangioma
17. Hemangioma runs in stages
1. Proliferative stage: lasts about 6-10 months after which
the tumor slows in growth and enters the second stage.
2. Involutive stage: the lesion becomes dull purple and
less firm, 50% of the lesions show complete resolution by
the age of 5 years and 90% of the cases by the age of 9
years.
3. Involuted stage: 40% of patients will have permanent
changes such as; atrophy, scarring, fibrofatty tissue or
telangiectasia.
18.
19. Hemangiomas can also be described
according to the depth of the lesion as:
• Superficial hemangiomas originate from the
papillary dermis and present as bright red macular
or papular masses.
• Deep hemangiomas originate from the reticular
dermis or subcutaneous tissues and appear as
bluish or relatively colorless masses.
• Compound hemangiomas have superficial and deep
components.
20.
21. Complications
• Ulceration with or without secondary infection.
• Bleeding.
• Periocular lesions may result in amblyopia, strabismus or
astigmatism.
• Airway obstruction in laryngeal lesions.
• Visceral involvement.
• Kasabach-Merritt syndrome characterized by the combination of
rapidly growing hemangioma, thrombocytopenia,
microangiopathic hemolytic anemia and consumptive coagulopathy
with 30%-40% mortality rate.
24. Treatment
•In the proliferative phase the aim is to
eradicate or stunt the growth:
1. Benign or Watchful neglect with education of
parents and regular follow up especially
for lesions that are not life or sight
threatening.
25. Treatment
2. Pharmacological treatment; which is the first
line of treatment in life and sight threatening
and disfiguring lesions
a) Systemic corticosteroid therapy
b) Interferon α 2A and B
c) Nonselective beta adrenergic receptor blocker
propranolol
27. Treatment
3.Laser therapy; pulsed dye laser (PDL) with
0.7-1mm penetration depth for superficial
lesions. Nd:YAG laser for lesions in the late
proliferative stage. The role of laser
therapy for the treatment of hemangioma
is controversial.
29. Treatment
4.Surgery in the proliferative stage is
reserved for the lesions that do not
respond to the non-surgical treatment.
30. Treatment
• In the involutive stage treatment is aimed to
improve function and esthetics. Corticosteroids
are not effective, the type of treatment depends
on the depth of the lesion.
• PDL and Nd:YAG laser for superficial lesions.
• Surgical resection for deep lesions.
32. Vascular Malformations
• These are errors of morphogenesis that are populated by
stable mature vascular endothelium.
• Both sexes are equally affected.
• They are always present at birth (though some may not be
apparent until a later stage) and in contrast to
hemangiomas they never proliferate or involute.
• Instead, they expand slowly and relentlessly throughout
life, in pace with the growth of the patient. Trauma,
puberty, and pregnancy can cause accelerated growth
33. These lesions are sub-classified:
According to the predominant vessel type:
1. Capillary.
2. Venous.
3. Arteriovenous.
4. Lymphatic.
5. Combined
34. These lesions are sub-classified:
According to the hemodynamic properties into:
1. High-flow.
2. Low-flow.
Unlike hemangiomas, vascular malformations are
associated with skeletal abnormalities in up to 35%
of cases.
38. Treatment
• The primary treatment of capillary malformation is the
laser application.
• Pulsed dye laser is considered the treatment of choice
for capillary malformations.
• It works on the principle of selective thermolysis which
is the ability to coagulate a target structure without
damaging the surrounding tissues
• An average of two to six treatments may be necessary
and the response rate is variable
39.
40. Treatment
•Lesions may recur after treatment, the suggested
etiology of the recurrence is that any abnormally
innervated vessels in the superficial papillary
plexus that were left behind would lead to
recurrence.
•In some cases surgical treatment is needed
followed by reconstruction with skin graft,
excision of capillary malformation can be
performed in a staged procedure
41.
42. Venous malformations
•They can be focal, multifocal or diffuse. Up to 60%
of venous malformations are located in the head
and neck area.
•The lesions are usually soft, compressible, and
enlarge in size when venous pressure is increased
due to compression of surrounding tissue or during
crying or Valsalva maneuver.
45. Treatment
• Sclerotherapy is the most widespread method.
• Sclerosing agents can be divided in the following groups:
1. Osmotic substances (hypertonic saline/salicylates)
2. Chemical substances (ethanol/iodine)
3. Anticancer substances (bleomycin)
4. Detergents (polidocanol/sodium tetradecyl
46.
47. Treatment
•Surgical treatment of venous malformation
is indicated only for small, well- defined
lesions that can be easily excised.
•Resection must be complete to prevent
recurrence and appropriate imaging is
necessary to show the full extent of the
lesion.
50. Lymphatic malformations
•Lymphatic malformations are categorized into
macrocystic (single or multiple cysts ˃2 cm3),
microcystic (<2 cm3), and mixed forms.
1. Cervical lesions are usually of the macrocystic
variety (cystic hygroma) and
2. those involving the floor of the mouth, cheek,
and tongue are more likely to be of the diffuse
microcystic variety (lymphangioma).
53. Lymphatic malformations
•Complications include
•infection, bleeding, obstruction of the airway,
disturbances of speech, and abnormal facial
growth. Hypertrophy of both soft tissue and
skeleton are common and occur in up to 80%
of cases.
54. Treatment
• Lymphatic malformations are the most difficult to
eradicate. Treatment also consists of sclerotherapy and
surgery
• All sclerosing agents are associated with complications and
multiple treatments are usually necessary.
• Macrocystic lymphatic malformations are more localized
and respect tissue planes and are more easily excised
surgically, whereas diffuse microcystic lesions are more
difficult and may require multiple operations
58. Arteriovenous malformations
• They are the least common and the most dangerous
subtype of the vascular malformations. They consist of a
nidus composed of abnormally dilated capillaries with
hypertrophied arteries and dilated veins resulting from
increased flow across the nidus.
• The lesions are often deep, but those close to the surface
may appear as a red lesion with elevated temperature and
produce a palpable thrill or pulsation.
• They are firmer than venous malformations and do not
empty readily when they are compressed
60. Treatment
• The treatment of arteriovenous malformations is
challenging and should aim to complete removal of
the arteriovenous malformation, which requires the
complete resection of the nidus.
• An incomplete removal is followed by recurrence
and may even stimulate the expansion of the
remaining part.
• The options of treatment include embolization,
resection, and combinations
64. Intraosseous arteriovenous malformations
• It is involving the jaw are rare but they can lead to
massive life-threatening bleeding commonly after tooth
extraction.
• They are more common in the mandible than the maxilla.
These lesions can remain unrecognized for long period.
• The clinical manifestations may include facial asymmetry,
unexplained dental mobility or malposition,
discolorations of skin or intraoral mucosa, palpable thrill,
spontaneous gingival bleeding, pain, or paresthesia
67. Intraosseous arteriovenous malformations
•Radiographically they appear as a
radiolucency. The diagnostic tool of choice
is arterial angiography, which provides
direct visualization of feeder arteries,
draining veins, and the collateral flow of
the malformation.