DR.
ESSAM EL-KADY---FRCS
Vascular Malformations
Dr.Essam El-Kady FRCS England
Head of Vascular Department
Maadi Armed forces Hospital
DR.ESSAM EL-KADY---FRCS
INTRODUCTION
Vascular anomalies are among the most
common congenital abnormalities observed
in infants and children. Unfortunately, these
lesions are also among the most confusing
and misunderstood conditions, largely
because of a history of inconsistent
terminology used for classification.
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CLASSIFICATION
Hemangiomas
Vascular Malformations:
High-flow Vascular Malformation
AVMs arteriovenous malformations
AVFs arteriovenous fistulas
Low-flow Vascular Malformation
Venous Malformations
Lymphatic Malformations Malformations
Lymphatic Venous Malformations
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PATHOPHYSIOLOGY
Hemangiomas:
Hemangiomas are benign tumors of infancy are
grouped as
infantile hemangiomas.
rapidly involuting congenital hemangiomas.
noninvoluting hemangiomas.
intramuscular hemangiomas.
Kaposiform hemangioendothelioma (KHE).
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PATHOPHYSIOLOGY
Vascular Malformations:
High-flow Vascular Malformations
Arteriovenous malformations are considered to be
congenital vascular anomalies, but are usually first
noted several years after birth or after certain
triggering changes such as trauma or the hormonal
changes of puberty or pregnancy.
Arteriovenous fistulas (AVFs) are simple
arteriovenous connections. Most AVFs are
secondary to penetrating injuries after birth,
although some are believed to be congenital.
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PATHOPHYSIOLOGY
Vascular Malformations:
Low-Flow Vascular Malformations
Venous malformations are spongy, masslike lesions
composed of abnormal veins, ie, veins with a
relative lack of smooth muscle cells in their walls
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PATHOPHYSIOLOGY
Vascular Malformations:
Low-Flow Vascular Malformations
Lymphatic malformations (Classified by MRI to)
Micro cystic lymphatic malformations consist of mass like
soft-tissue abnormalities.
Macro cystic lymphatic malformations consist of visible
cystic spaces that contain lymphatic fluid.
Mixed
Lymphatic venous malformations are a combination of
abnormal lymphatic and venous channels.
Capillary malformations (port-wine stains) are common
birthmarks and involve only the superficial tissues (skin)
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OVERGROWTH SYNDROMES:
Klippel-Trnaunay syndrome which is a low-flow
combined vascular anomaly (capillary-lymphatic-
venous malformation) usually associated with
marked overgrowth of the leg and capillary stains.
Parkes-Weber syndrome consists of an AVM-like
high-flow malformation that involves the entire
extremity (usually a lower limb), and it is usually
associated with a capillary malformation over the
enlarged limb.
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Klippel-Trnaunay syndrome
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SIGNS AND SYMPTOMS
Hemangiomas
usually first appear a few weeks after birth and
affect the head and neck in most patients. The
trunk and extremities are less commonly involved.
Hemangiomas look like red, flat or raised, patches
or plaques with or without a cluster of superficial
veins.Hemangiomas are generally firm and
rubbery to the touch.
DR.ESSAM EL-KADY---F
Hemangiomas
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SIGNS AND SYMPTOMS
Vascular Malformations:
High-flow Vascular Malformations
Arteriovenous malformations (AVMs) are generally present in
neonates at birth, but they often suddenly become obvious
when the patient is older because of various stimuli such as
trauma, pregnancy, or puberty. There are four recognized
stages of AVMs:
Stage I lesion has a pinkish-bluish stain and warmth.
Stage II, the lesion has pulsations, thrill, and bruit.
Stage III, the patient has dystrophic skin changes,
ulceration, bleeding, and pain.
Stage IV, the patient has high-output cardiac failure.
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High-flow Vascular Malformations
DR.ESSAM EL-KADY---F
SIGNS AND SYMPTOMS
Vascular Malformations:
Low-Flow Vascular Malformations
Venous malformations: are congenital lesions but usually
become symptomatic in older children or young adults,
with bluish skin discoloration, local swelling, and pain.
Although venous malformations are considered benign
entities, some extensive venous malformations can result
in significant morbidity, particularly those in the head and
neck (eg, with airway involvement). Extremity venous
malformations may be associated with a limb-length
discrepancy, particularly if the malformation is large.
Venous malformations of the gastrointestinal tract most
commonly cause chronic bleeding and anemia.
DR.ESSAM EL-KADY---F
Low-Flow Vascular Malformations
DR.ESSAM EL-KADY---F
SIGNS AND SYMPTOMS
Vascular Malformations:
Low-Flow Vascular Malformations
Lymphatic malformations commonly occur in the
cervicofacial region (approximately 75% of lymphatic
malformations). Most lymphatic malformations are
apparent in young children. These malformations appear
in various forms, such as localized small lesions or in the
diffuse involvement of an extremity or particular body part
or organ system. The overlying skin can be normal, or it
may have tiny characteristic vesicles. Lymphatic
malformations in an extremity can cause diffuse or
localized swelling with soft-tissue and skeletal overgrowth
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Lymphatic malformations
Large lymphatic malformation in the left shoulder region. The
lesion is easily compressible because it consists of
large cysts and numerous microcysts. A surgical scar
is due to a previous attempt to remove the lesion
surgically.
Figure 14
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SIGNS AND SYMPTOMS
Vascular Malformations:
Low-Flow Vascular Malformations
Lymphatic venous malformations (LVMs) consist of
mixed clinical and imaging findings of lymphatic
malformations and venous malformations.
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DIAGNOSIS
Most vascular anomalies, particularly the superficial
anomalies (eg, capillary malformations port-wine
stains) are recognized by simple clinical history and
clinical assessment and do not require any imaging
studies. However, most anomalies extending into the
deep tissues require imaging studies
To confirm the initial diagnosis,
To determine the extent of the malformation
To plan treatment
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DIAGNOSIS
MRI IS THE IMAGING STUDY OF CHOICE
Angio- MRA- CT angio: The gold standard for high-flow
anomalies is conventional arteriography,however the new
noninvasive angiographic techniques such as magnetic
resonance angiography or computed tomographic angiography
offer noninvasive assessment of the flow dynamics and
vasculature of high-flow anomalies (eg, arteriovenous
malformation, arteriovenous fistula).
Duplex ultrasonography: Portability and availability are the main
advantages of ultrasonography compared with MRI.
Ultrasonography is commonly used to quickly evaluate anomalies
during the patient's initial visit to confirm the suspected
diagnosis. It is also used to triage patients and schedule them for
appropriate treatment.
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TREATMENT
Hemangiomas:
Most hemangiomas regress gradually and require no
treatment
Surgical Treatment: excision of the localised
hemangioma
Radiotherapy/ Arterial embolization: can be used in
selected cases.
Medical Treatmen
The leading pharmacologic agents used for hemangiomas
are steroids, either by systemic use or intralesional
injection.
Angiogenesis inhibitors such as interferon, vincristine,
can be used in selected cases.
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Surgical Treatment of Hemangioma
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TREATMENT
Low-flow Malformations:
Surgical treatment :a few patients with venous
malformations can be treated with a simple surgical
excision especially if small and dose not involve vital
structure.
Sclerotherapy: most patients with venous malformation
are dependent on sclerotherapy (in which we infuse
sclerosant agent into the lesion under various imaging
guidance techniques). Currently, the most commonly used
sclerosant agent is absolute alcohol. Other, less
commonly used agents, include ethanolamine oleate
(Ethamolin) and sodium tetradecyl sulfate (Sotrecol).
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Treatment of Low-flow Malformations:
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TREATMENT
High-flow Malformations:
Surgical treatment: Small, superficial
arteriovenous malformations can be removed
surgically. However,
Embolization: It has been the only feasible
treatment option for most arteriovenous
malformations. Embolization, which closes off
the arterial feeders of the malformation, is
generally effective in arteriovenous
malformations to stabilize the malformation.
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OUTCOMES
Although they often cause significant psychosocial stress for
parents and potentially for children, most vascular anomalies are
benign conditions and do not require diagnostic tests or
treatments.
However, some (eg, arteriovenous malformations or large venous
malformations) are quite problematic, causing significant
discomfort or disability, and they may worsen.
Unfortunately, misclassifications or incorrect diagnoses are
common and usually a result of the limited experience of the
clinicians or radiologists involved in the diagnosis and
management.
With the appropriate diagnostic workup and therapeutic
management, even rapidly progressing malformations can be
managed successfully.
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DR.ESSAM EL-KADY---F
