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) Vascular anomalies Vascular tumors Infantile hemangioma Vascular malformations Slow-flow vascular malformations Capillary malformation(CM) Venous malformation(VM) Lymphatic malformation(LM) Fast-flow vascular malformations Arterial malformation(AM) Arteriovenous fistula(AVF) Arteriovenous malformation(AVM)

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 HemangiomaVascular 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 PreviousISSVA 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 year 2. Involuting - few years 3. 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 fistulae –arteriovenous malformations (with a nidus of multiple shunts)arteriovenous malformations

Complex malformations regional –Sturge-Weber syndromeSturge-Weber syndrome –Klippel-Trénaunay syndromeKlippel-Trénaunay syndrome –F. P. Weber syndromeF. P. Weber syndrome diffuse –Maffucci syndromeMaffucci syndrome –Solomon syndromeSolomon syndrome –Proteus syndromeProteus syndrome

Arteriovenous malformations (AVMs)

Low flow malformations Lymphatic Malformations -Microcystic -Macrocystic Venous Malformations Capillary Malformations Combined types