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• Capillary hemangioma• Strawberry hemangioma• Strawberry nevus• Port wine stain• Flame nevus• Cavernous hemangioma• Venous angioma• Lymphangioma• Arteriovenous malformation

CLASSIFICATION(ISSVA)

Updated ISSVA classification of vascular anomalies.

• Infantile hemangiomas• Congenital hemangiomas (RICH and NICH)• Tufted angioma (with or without Kasabach-

Merritt 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.)

1..Slow-flow vascular malformations:• Capillary malformation (CM)

– Port-wine stain– Telangiectasia– Angiokeratoma

• Venous malformation (VM)– Common sporadic VM– Bean syndrome– Familial cutaneous and mucosal venous

malformation (VMCM)– Glomuvenous malformation (GVM)

(glomangioma)– Maffucci syndrome

• Lymphatic 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

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

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

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– Retinform hemangioendotheliomas– Composite hemangioendotheliomas– Polymorphous hemangioendotheliomas– Kaposiform hemangioendotheliomas

• Malignant tumors– Epithelioid hemangioendotheliomas– Angiosarcoma

HEMANGIOMA

congenital hemangioma

Arterio-Venous Malformation(AVM)

Infantile hemangioma

HEMANGIOMA

Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon

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 year2. Involuting - few years3. Involuted - most resolved by age 10

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

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, high-outputcardiac failure

Arterio-Venous Malformation (AVM)

AVM

Summary of Regional and Diffuse Syndromes Associated With Vascular Malformations

• Regional syndromes with associated vascular malformations– 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– 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– 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 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.

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