Classification of vascular anomalies

Updated ISSVA
(International Society for the Study of Vascular Anomalies)

Children’s Hospital 2
Department of diagnosis imaging
Dr Lien Bang


INTRODUCTION
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Vascular anomalies are the most common skin and soft
tissue lesions 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.

Vascular anomalies

Hemangioma

Vascular malformation

“…Not
“…Not every skin lesion looking like a strawberry is a hemangioma;
hemangioma; not all hemangiomas look
like strawberries…
strawberries…" J.B. Mulliken,
Mulliken, MD


Traditional classification
 Capillary hemangioma
 Strawberry hemangioma
 Strawberry nevus
 Port

wine stain
 Flame nevus
 Cavernous hemangioma
 Venous angioma
 Lymphangioma
 Arteriovenous malformation


Translation from old terminology into
classification of ISSVA

Hemangioma

Old Terminology
*Capillary
*Strawberry
*Port-wine
*Capillary-cavernous
*Cavernous
*Vernous
*Hemangiolymphangioma
*Lymphangioma
*Arteriovenous

Vascular
malformation
* CAPILLARY

* VENOUS

*LYMPHATIC
*ARTERIOVENOUS


Updated ISSVA classification of vascular anomalies.
Vascular tumors
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Infantile hemangiomas
Congenital hemangiomas (RICH and
NICH)
Tufted angioma (with or without
KasabachKasabach-Merritt syndrome)
Kaposiform hemangioendothelioma
(with or without KasabachKasabach-Merritt
syndrome)
Spindle cell hemangioendothelioma
Other, rare hemangioendotheliomas
(epithelioid,
epithelioid, composite, retiform,
retiform,
polymorphous, Dabska tumor,
lymphangioendotheliomatosis,
lymphangioendotheliomatosis, etc.)
Dermatologic acquired vascular
tumors (pyogenic
(pyogenic granuloma,
granuloma,
targetoid hemangioma,
hemangioma, glomeruloid
hemangioma,
hemangioma, microvenular
hemangioma,

hemangioma, etc.)

Vascular malformations
1..Slow1..Slow-flow vascular malformations:
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Capillary malformation (CM)
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PortPort-wine stain
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Telangiectasia
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Angiokeratoma
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Venous malformation (VM)
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Common sporadic VM
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Bean syndrome
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Familial cutaneous and mucosal venous
malformation (VMCM)
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Glomuvenous malformation
(GVM)(glomangioma)
GVM)(glomangioma)
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Maffucci syndrome
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Lymphatic malformation (LM)
2. FastFast-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.Complex3.Complex-combined vascular malformations:
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CVM, CLM, LVM, CLVM,
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AVMAVM-LM, CMCM-AVM

C:capillary; V:venous; L:lymphatic; AV:arteriovenous; M:malformation.
RICH:rapidly involuting congenital hemangioma; NICH:noninvoluting congenital hemangioma.


Vascular anormalies
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Vascular tumors

Infantile
hemangioma

NICH

Congenital
hemangioma

Vascular malformations

Slow-flow

Fast-flow

RICH

•Capillary malformation(CM)
•Venous malformation(VM)
•Lymphatic malformation(LM)

•Arterial malformation(AM)
•Arteriovenous fistula(AVF)
•Arteriovenous malformation(AVM)

•Combined types


Differentiating Features
Hemangiomas
True tumors, with

Vascular Malformations

proliferation No tumor, Comprised of
of the vascular endothelium
dysplastic vessels
 >3:1 female:male
1:1 female:male
Small or absent at birth

Present at birth
Rapid growth during infancy
Growth proportional to child
Self-limited
 Never disappear
Diagnosis:Clinical history+
Diagnosis: MRI,Doppler
appearance
ultrasonography,angiography


HEMANGOMA
 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, pale halo (skin)
2 types
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Non Involuting Congenital Hemangioma (NICH) - persistent
Rapidly Involuting Congenital Hemangioma (RICH) resolved by 1-2 yrs


Growth patterns of hemangiomas
NICH

GROWTH

RICH
IH

AGE

BIRTH

1 YR

2 YRS

RICH:rapidly involuting congenital hemangioma; NICH:noninvoluting congenital hemangioma. IH: Infantile Hemangioma


HEMANGIOMA

Infantile hemangioma

congenital hemangioma


HEMANGIOMA

Infantilehemangioma in a 4-month-old female


HEMANGIOMA

Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon


HEMANGIOMA

3 months of age

4 years of age

after 2 months of therapy with propranolol


HEMANGIOMA

Hemangioma of the parotid


Capillary malformation
 Dilated capillary channels
 Present at birth as flat, red or purple patch
 Can be associated with Hypertrophy of solf

tissues or facial skeleton, Sturge- Weber
syndrome


Capillary malformation


Lymphatic malformation
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Collection of lymph filled channels/ cysts
Present at birth , 5-6 w GA
Most common:
 Head/neck
 Extremities/axilla
 Trunk
2 type:
 Microcystic: multiple small vesicles
 Macrocystic: Few large septaled cysts
Complications:
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Infection, bleeding,
obstruction/ displacement of ogans
Overgrowth of involved tissue


Lymphatic malformation


Lymphatic malformation

Neck lymphatic malformation


Venous Malformation (VM)

 Thin-walled, dilated veins:Inadequate

smooth muscle layer
 Present at birth
 Skin discoloration, local swelling, and pain
 Complications: Thrombosis, bleeding


Venous Malformation (VM)


Venous Malformation


Venous Malformation