Presentation on theme: "Updated ISSVA (International Society for the Study of Vascular Anomalies) Department of diangosis imaging Children’s Hospital 2."— Presentation transcript:
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.
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
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
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
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