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

Slide1

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

Department of diangosis imagingChildren’s Hospital 2

Classification of vascular anomalies

Slide2

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.

Slide3

Traditional classification

Capillary hemangioma

Strawberry hemangioma

Strawberry nevus

Port wine stain

Flame nevus

Cavernous hemangioma

Venous

angioma

Lymphangioma

Arteriovenous malformation

Slide4

CLASSIFICATION(ISSVA)

Slide5

Updated ISSVA classification of vascular anomalies.

Infantile hemangiomasCongenital hemangiomas (RICH and NICH)Tufted angioma (with or without Kasabach-Merritt syndrome)Kaposiform hemangioendothelioma (with or without Kasabach-Merritt syndrome)Spindle cell hemangioendotheliomaOther, rare hemangioendotheliomas (epithelioid, composite, retiform, polymorphous, Dabska tumor, lymphangioendotheliomatosis, etc.)Dermatologic acquired vascular tumors (pyogenic granuloma, targetoid hemangioma, glomeruloid hemangioma, microvenular hemangioma, etc.)

1..Slow-flow vascular malformations:Capillary malformation (CM)Port-wine stainTelangiectasiaAngiokeratoma Venous malformation (VM)Common sporadic VMBean syndromeFamilial cutaneous and mucosal venous malformation (VMCM)Glomuvenous malformation (GVM)(glomangioma)Maffucci syndromeLymphatic malformation (LM)2. Fast-flow vascular malformations:Arterial malformation (AM)Arteriovenous fistula (AVF)Arteriovenous malformation (AVM)3.Complex-combined vascular malformations:CVM, CLM, LVM, CLVM,AVM-LM, CM-AVM

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

Vascular tumors

Vascular malformations

Slide6

Differentiating Features

Hemangioma

Vascular Malformations

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

No tumor, Comprised of dysplastic vessels

1:1

female:male

Present at birth

Growth proportional to child

never disappear

Diagnosis:

MRI,Doppler

ultrasonography,angiography

Slide7

Comparison of Previous Terminology and New ISSVA Terminology

Previous

ISSVA

Capillary or cavernous Hemangioma of any organ

Infantile hemangioma

Infantile hemangioendothelioma of the liver

Hepatic or infantile hemangioma

hepatic

hemangioma

, cavernous

hemangioma

Venous malformation

Lymphangioma,Cystic

hygroma

Lymphatic Malformation

Port-wine stain,Capillary Hemangioma

Capillary Malformation

Slide8

Key Imaging Features of the Most Common Pediatric Vascular Anomalies

Slide9

Differentiation between Hemangioma and Hemangioendothelioma of the liver

Slide10

Classification of Vascular Tumors

Benign tumors and tumor-like conditions

Hemangiomas

Spindle cell hemangioma (‘hemangioendotheliomas’)

Epithelioid hemangioma

Low-grade malignant tumors

Retinform hemangioendotheliomas

Composite hemangioendotheliomas

Polymorphous hemangioendotheliomas

Kaposiform hemangioendotheliomas

Malignant tumors

Epithelioid hemangioendotheliomas

Angiosarcoma

Slide11

HEMANGIOMA

congenital hemangioma

Arterio-Venous Malformation(AVM)

Infantile hemangioma

Slide12

HEMANGIOMA

Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon

Slide13

Hemangioma

Benign endothelial cell tumor

2 main types

1. Infantile Hemangioma

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

Slide14

Hemangioma(cont)

2. Congenital Hemangioma

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

Slide15

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

Slide16

Venous Malformation (VM)

Present at birth

skin discoloration, local swelling, and pain

Thin-walled, dilated veins:Inadequate smooth muscle layer

Complications: Thrombosis, bleeding

Slide17

Venous Malformation (VM)

Slide18

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, high-outputcardiac failure

Slide19

Arterio-Venous Malformation (AVM)

Slide20

AVM

Slide21

Summary of Regional and Diffuse Syndromes Associated With Vascular Malformations

Regional syndromes with associated vascular malformationsSturge–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 malformationsProteus 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 malformationsHereditary 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.

Slide22

OVERGROWTH SYNDROMES:

Klippel-Trénaunay 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.

Slide23

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)

Slide24

Complex malformations

regional

Sturge-Weber syndrome

Klippel-Trénaunay syndrome

F. P. Weber syndrome

diffuse

Maffucci syndrome

Solomon syndrome

Proteus syndrome

Slide25

Arteriovenous malformations (AVMs)

Slide26

Low flow malformations

Lymphatic Malformations

-Microcystic

-Macrocystic

Venous Malformations

Capillary Malformations

Combined types

Slide27