Updated ISSVA (International Society for the Study of Vascular Anomalies) Children’s Hospital Department of diagnosis imaging Dr Lien Bang INTRODUCTION     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        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 Slow1 Slow-flow vascular malformations:  Capillary malformation (CM)  PortPort-wine stain  Telangiectasia  Angiokeratoma  Venous malformation (VM)  Common sporadic VM  Bean syndrome  Familial cutaneous and mucosal venous malformation (VMCM)  Glomuvenous malformation (GVM)(glomangioma) GVM)(glomangioma)  Maffucci syndrome  Lymphatic malformation (LM) FastFast-flow vascular malformations:  Arterial malformation (AM)  Arteriovenous fistula (AVF)  Arteriovenous malformation (AVM) 3.Complex3.Complex-combined vascular malformations:  CVM, CLM, LVM, CLVM,  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 # 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  main types Infantile Hemangioma • • • • Most common tumor of infancy/childhood Usually has overlying patch of redness Appears weeks/months after birth Natural course - stages Proliferating - first year Involuting - few years Involuted - most resolved by age 10 HEMANGIOMA (cont) Congenital Hemangioma • • • • Present at birth Rare (compared to infantile) Blue/gray hue, pale halo (skin) types   Non Involuting Congenital Hemangioma (NICH) - persistent Rapidly Involuting Congenital Hemangioma (RICH) resolved by 1-2 yrs Arterio-Venous Malformation (AVM) Arterio-Venous Malformation (AVM) Vascular Complex malformations  Regional    Sturge-Weber syndrome Klippel-Trénaunay syndrome F P Weber syndrome  Diffuse    Maffucci syndrome Solomon syndrome Proteus syndrome Summary of Regional and Diffuse Syndromes Associated With Vascular Malformations    Regional syndromes with associated vascular malformations  Sturge– Sturge–Weber: facial capillary malformation with intracranial capillary malformation, malformation, venous malformation, or AVM  Klippel - Trenaunay : limb/trunk capillary venous lymphatic malformations with overgrowth overgrowth  Parkes Weber: CAVM with overgrowth; lymphatic malformation Diffuse syndromes associated slowslow-flow malformations  Proteus syndrome: vascular malformations (capillary or venous), hamartomatous syndrome with overgrowth(hemihypertrophy and macrodactyly), macrodactyly), lipomas, lipomas, pigmented nevi  Blue rubber bleb nevus (Bean) syndrome: syndrome: multiple cutaneous, 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– Bannayan–Riley– Riley–Ruvalcaba syndrome: syndrome: vascular malformations (cutaneous (cutaneous,, intracranial), macrocephaly, macrocephaly, ectodermal dysplasia, dysplasia, lipomatous masses, and intestinal hamartomatous polyps, PTEN suppressor gene mutation association Diffuse syndromes associated fast flow malformations  Hereditary hemorrhagic telangiectasia (Osler– Osler–Weber– Weber–Rendu): Rendu): telangiectasias (skin, mucous membranes, gastrointestinal mucosa) and AVMs (lungs, liver, 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 Klippel-Trénaunay syndrome Klippel-Trénaunay syndrome Klippel-Trénaunay syndrome Proteus syndrome Proteus syndrome Differentiation between Hemangioma and Hemangioendothelioma of the liver Key Imaging Features of the Most Common Pediatric Vascular Anomalies Comparison of Previous Terminology and New ISSVA Terminology Previous Capillary or cavernous Hemangioma of ISSVA  Infantile hemangioma any organ Infantile hemangioendothelioma of the Hepatic or infantile hemangioma liver hepatic hemangioma, cavernous hemangioma Lymphangioma,Cystic Port-wine  Venous malformation  Lymphatic Malformation  Capillary Malformation hygroma stain,Capillary Hemangioma 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  [...]... 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) NORMAL AVM Arterio-Venous Malformation (AVM) Arterio-Venous Malformation (AVM) Vascular Complex malformations... 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      Collection of lymph filled channels/ cysts Present... 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... Sturge-Weber syndrome Klippel-Trénaunay syndrome F P Weber syndrome  Diffuse    Maffucci syndrome Solomon syndrome Proteus syndrome Summary of Regional and Diffuse Syndromes Associated With Vascular Malformations    Regional syndromes with associated vascular malformations  Sturge– Sturge–Weber: facial capillary malformation with intracranial capillary malformation, malformation, venous malformation,... nevus syndrome (Solomon syndrome): vascular malformations (intracranial AVM), epidermal nevi, various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular, and urogenital systems  Bannayan– Bannayan–Riley– Riley–Ruvalcaba syndrome: syndrome: vascular malformations (cutaneous (cutaneous,, intracranial), macrocephaly, macrocephaly, ectodermal dysplasia, dysplasia, lipomatous... 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... Head/neck  Extremities/axilla  Trunk 2 type:  Microcystic: multiple small vesicles  Macrocystic: Few large septaled cysts Complications:    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...  Proteus syndrome: vascular malformations (capillary or venous), hamartomatous syndrome with overgrowth(hemihypertrophy and macrodactyly), macrodactyly), lipomas, lipomas, pigmented nevi  Blue rubber bleb nevus (Bean) syndrome: syndrome: multiple cutaneous, cutaneous, musculoskeletal, and gastrointestinal tract venous malformations  Epidermal nevus syndrome (Solomon syndrome): vascular malformations