Updated ISSVA
(International Society for the Study of Vascular Anomalies)
Department of diangosis imaging
Children’s Hospital 2
INTRODUCTION
• Vascular anomalies are among the most common congenital
abnormalities observed in infants and children.
• Older nomenclature continues to cause confusion,
misunderstood diagnoses, and potential mismanagement
• In 1982, Mulliken and Glowacki proposed a classification
system for vascular anomalies based on their clinical
behavior and endothelial cell characteristics into two groups:
hemangiomas and vascular malformations.This system,
which was adopted by the ISSVA, has since been expanded
and is now widely accepted.
• Radiologists can use the ISSVA classification system by
correlating imaging findings with patient history and physical
findings. Consistent use of this system will help patients
receive the correct diagnosis and treatment.
Traditional classification
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Capillary hemangioma
Strawberry hemangioma
Strawberry nevus
Port wine stain
Flame nevus
Cavernous hemangioma
Venous angioma
Lymphangioma
Arteriovenous malformation
CLASSIFICATION(ISSVA)
Vascular anomalies
Vascular tumors
Infantile hemangioma
Vascular malformations
Slow-flow
Fast-flow
vascular malformations vascular malformations
Capillary malformation(CM)
Venous malformation(VM)
Lymphatic malformation(LM)
Arterial malformation(AM)
Arteriovenous fistula(AVF)
Arteriovenous malformation(AVM)
Updated ISSVA classification of vascular anomalies.
Vascular tumors
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Infantile hemangiomas
Congenital hemangiomas (RICH and NICH)
Tufted angioma (with or without KasabachMerritt syndrome)
Kaposiform hemangioendothelioma (with or
without Kasabach-Merritt syndrome)
Spindle cell hemangioendothelioma
Other, rare hemangioendotheliomas
(epithelioid, composite, retiform,
polymorphous, Dabska tumor,
lymphangioendotheliomatosis, etc.)
Dermatologic acquired vascular tumors
(pyogenic granuloma, targetoid
hemangioma, glomeruloid hemangioma,
microvenular hemangioma, etc.)
Vascular malformations
1..Slow-flow vascular malformations:
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Capillary malformation (CM)
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Port-wine stain
Telangiectasia
Angiokeratoma
Venous malformation (VM)
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Common sporadic VM
Bean syndrome
Familial cutaneous and mucosal venous
malformation (VMCM)
Glomuvenous malformation
(GVM)(glomangioma)
Maffucci syndrome
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Lymphatic malformation (LM)
2. Fast-flow vascular malformations:
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Arterial malformation (AM)
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Arteriovenous fistula (AVF)
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Arteriovenous malformation (AVM)
3.Complex-combined vascular malformations:
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CVM, CLM, LVM, CLVM,
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AVM-LM, CM-AVM
C:capillary; V:venous; L:lymphatic; AV:arteriovenous; M:malformation.
RICH:rapidly involuting congenital hemangioma; NICH:noninvoluting congenital hemangioma.
Differentiating Features
Hemangioma
•True tumors, with proliferation
of the vascular endothelium
• >3:1 female:male
•Small or absent at birth
•Rapid growth during infancy
•“self-limited”
•Diagnosis:Clinical history+
appearance
Vascular Malformations
•No tumor, Comprised of
dysplastic vessels
•1:1 female:male
•Present at birth
•Growth proportional to child
• never disappear
•Diagnosis: MRI,Doppler
ultrasonography,angiography
Comparison of Previous Terminology and New ISSVA
Terminology
Previous
ISSVA
•Capillary or cavernous Hemangioma of
any organ
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•Infantile hemangioendothelioma of the
liver
•Hepatic or infantile hemangioma
•hepatic hemangioma, cavernous
hemangioma
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Infantile hemangioma
Venous malformation
• Lymphatic Malformation
•Lymphangioma,Cystic hygroma
•Port-wine stain,Capillary Hemangioma
• Capillary Malformation
Key Imaging Features of the Most Common
Pediatric Vascular Anomalies
Differentiation between Hemangioma and
Hemangioendothelioma of the liver
Classification of Vascular
Tumors
• Benign tumors and tumor-like conditions
– Hemangiomas
– Spindle cell hemangioma (‘hemangioendotheliomas’)
– Epithelioid hemangioma
• Low-grade malignant tumors
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Retinform hemangioendotheliomas
Composite hemangioendotheliomas
Polymorphous hemangioendotheliomas
Kaposiform hemangioendotheliomas
• Malignant tumors
– Epithelioid hemangioendotheliomas
– Angiosarcoma
HEMANGIOMA
Arterio-Venous
Malformation(AVM)
congenital hemangioma
Infantile hemangioma
HEMANGIOMA
Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon
Hemangioma
• Benign endothelial cell tumor
• 2 main types
1. Infantile Hemangioma
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Most common tumor of infancy/childhood
Usually has overlying patch of redness
Appears weeks/months after birth
Natural course - 3 stages
1. Proliferating - first year
2. Involuting - few years
3. Involuted - most resolved by age 10
Hemangioma(cont)
2. Congenital Hemangioma
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Present at birth
Rare (compared to infantile)
Blue/gray hue w/ pale halo (skin)
2 types
– Non-Involuting (NICH) - persistent
– Rapidly Involuting (RICH) - resolved by 1-2 yrs
Lymphatic malformations
• Commonly occur in the cervicofacial
region, Lymphatic malformations in an
extremity can cause diffuse or localized
swelling with soft-tissue and skeletal
overgrowth
Venous Malformation (VM)
• Present at birth
• skin discoloration, local swelling, and
pain
• Thin-walled, dilated veins:Inadequate
smooth muscle layer
• Complications: Thrombosis, bleeding
Venous Malformation (VM)
Arterio-Venous Malformation
(AVM)
• Present at birth
• Reddish vascular hue (skin), often warm
pulsations, thrill, and bruit
• High-flow arterio-venous communication absence of developed capillary bed
• Complications: ulceration, bleeding,pain,
compression/displacement of organs, highoutputcardiac failure
Arterio-Venous Malformation
(AVM)
AVM
Summary of Regional and Diffuse Syndromes Associated
With Vascular Malformations
• Regional syndromes with associated vascular malformations
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Sturge–Weber: facial capillary malformation with intracranial capillary malformation, venous
malformation, or AVM.
Klippel–Trenaunay: limb/trunk capillary venous lymphatic malformations with overgrowth.
Parkes Weber: CAVM with overgrowth; lymphatic malformation.
• Diffuse syndromes associated slow-flow malformations
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Proteus syndrome: vascular malformations (capillary or venous), hamartomatous syndrome with
overgrowth(hemihypertrophy and macrodactyly), lipomas, pigmented nevi.
Blue rubber bleb nevus (Bean) syndrome: multiple cutaneous, musculoskeletal, and
gastrointestinal tract venous malformations.
Epidermal nevus syndrome (Solomon syndrome): vascular malformations (intracranial AVM),
epidermal nevi, various developmental abnormalities of the skin, eyes, nervous, skeletal,
cardiovascular, and urogenital systems.
Bannayan–Riley–Ruvalcaba syndrome: vascular malformations (cutaneous, intracranial),
macrocephaly, ectodermal dysplasia, lipomatous masses, and intestinal hamartomatous polyps,
PTEN suppressor gene mutation association.
• Diffuse syndromes associated fast flow malformations
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Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu): telangiectasias (skin, mucous
membranes, gastrointestinal mucosa) and AVMs (lungs, liver, brain, spinal cord).
AVM, arteriovenous malformation; CAVM, capillary arterial venous malformation; PTEN, phosphatase and tensin homolog.
OVERGROWTH SYNDROMES:
• Klippel-Trénaunay syndrome which is a lowflow combined vascular anomaly (capillarylymphatic-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.
Simple malformations
• slow flow
– capillary
– lymphatic
– venous
• fast flow
– arterial
• aneurysm
• coarctation
• ectasia
– arteriovenous fistulae (with one ore more shunts)
– arteriovenous malformations (with a nidus of multiple shunts)
Complex malformations
• regional
– Sturge-Weber syndrome
– Klippel-Trénaunay syndrome
– F. P. Weber syndrome
• diffuse
– Maffucci syndrome
– Solomon syndrome
– Proteus syndrome
Arteriovenous malformations
(AVMs)
Low flow malformations
• Lymphatic Malformations
-Microcystic
-Macrocystic
• Venous Malformations
• Capillary Malformations
• Combined types