Congenital Vascular Malformations

Chelsey Tinder, MD
USF Department of vascular and endovascular surgery
Congenital Vascular Malformations
Malformed vessels secondary to arrested
development during various stages of
embryogenesis, most occur between the 4
th
and 10
th

week of embryogenesis
Grow as the child grows, do not regress
Incidence up to 15% of live births
Wide range of presentation with an unpredictable
course
88% asymptomatic
Can involve any organ system, most commonly
pelvis, extremities, intracranial circulation
Mulliken Classification
Fast flow lesions
Low flow lesions
Hamburg Classification
Predominately arterial
Truncular
Hypo-, a-, hyperplasia, obstruction, membrane, spur, dilatation
Extratruncular
Infiltrating or limited
Predominately venous
Truncular
Hypo-, a-, hyperplasia, obstruction, membrane, spur, dilatation
Extratruncular
Infiltrating or limited
Predominately AV shunting defects
Truncular
Deep AVF, superficial AVF
Extratruncular
Infiltrating or limited

Hamburg Classification
Combined vascular defects
Truncular
Arterial and venous, hemolymphatic
Extratruncular
Infiltrating hemolymphatic, limited hemolymphatic
Predominately lymphatic defects
Truncular
Hypo-, a-, hyperplasia, obstruction, membrane, spur,
dilatation
Extratruncular
Infiltrating or limited


Classification
Extratruncular Lesions
Arrested development early in the embryo
Arise and are composed of mesenchymal cells
Growth influenced by hormones, trauma
Higher rate of recurrence
Compress surrounding structures
Truncular lesions
Occur later in embryogenesis, cells differentiated
More hemodynamically significant
Types of CVM
Capillary malformation
Dilated capillary vessels in the
dermis, asymmetric overgrowth of
the involved limbs, and
sometimes multiple soft tissue
tumors
Venous Malformation
Bluish swelling under the skin
Compressible, enlarge with
dependence
Increased incidence of
thrombosis/PE
Low flow lesions
Lymphatic Malformation
Diffuse (lymphedema) or localized
(lymphangioma) swelling
Types of CVM
Arteriovenous malformation
Usually extratruncular
Initially present as local swelling +-
thrill, bruits, local hyperthermia
Develop symptoms of shunting skin
necrosis, distal gangrene, high output
cardiac failure
Nidus present central area of AV
connection (no capillaries)
High flow lesions
Develop dilated, thickened, tortuous
vessels, arterialized veins (medial
thickening and fibrosis)
Most morbid, highest rate of
recurrence

Noninvasive imaging
MRI
Duplex
Air plethysysmography
Volumetry
Whole-body blood pool scintigraphy
Uses tagged RBCs to detect
abnormal blood collections
Radioisotope lymphoscintigraphy
Transarterial lung perfusion
scintigraphy
Uses albumin to calculate shunting
percentage
CT
Uterine AVM

Invasive imaging
Angiography
Venography
Lymphangiography
Indications for therapy
Absolute
Hemorrhage
High output cardiac failure
Ischemia
Chronic venous hypertension
Threat to life or vital function
Relative
Disabling pain
Functional impairment
Cosmetically severe deformity
Vascular bone syndrome
High risk of complications
Lymph leak
Therapy
High flow lesions Endovascular therapy
Direct puncture, transarterial, or transvenous
approach
Superselective catheterization of feeding vessels
until identification of nidus
Embolization with polymerizing agents, ethanol
(toxic,) detachable balloons, coils can be used
definitively
Nitinol mesh plug successful with large AVMs
Temporary occlusion with foams or collagen can
be used for preoperative devascularization
Complications include end organ ischemia,
embolization, tissue destruction by ethanol,
severe swelling with progression to compartment
syndrome, PE, arterial emboli, MI from ethanol
AVM embolization

Coil embolization of pulmonary AVM

Therapy
High flow lesions - open surgical therapy
Often not possible or horribly morbid operations due to
complexity and location of lesions
Most suitable for well circumscribed lesions affecting the
Should involve ligation of all feeding vessels and entire
malformation unless doing so would result in end organ
ischemia, the only potential for cure is complete
eradication of the nidus
Incomplete resection can lead to recurrence or block
access from an endovascular approach
Therapy
Low flow lesions
Sclerotherapy with ethanol (toxic,) sodium tetradecyl
sulfate, polidocanol, ethanolamine oleate, or foam
sclerotherapy
Induces direct damage to endothelium resulting in
fibrosis and thrombosis
Large lesions require multiple treatments
Complications include tissue destruction by ethanol,
nerve damage from slerosing agents, severe swelling with
progression to compartment syndrome
Congenital Vascular Malformations
Complex problem with a wide array of manifestations
Therapy often ineffective or palliative, many
interventions may be required - complete lesion
eradication in only 15% of patients