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Vascular Malformations, Skeletal Deformities Including Macrodactyly, Embryonic Veins

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Vascular Malformations, Skeletal Deformities Including Macrodactyly, Embryonic Veins 1.) Give a general classification and nomenclature to think about when evaluating these patients 2.) Share some helpful tips to narrow the differential in a minute or less of interaction 3.) Discuss some helpful imaging recommendations focusing on ultrasound Vascular Anomalies Tumors: Malformations: Hemangiomas: Low flow: Infantile Hemangioma (IH) Capillary malformation (CM) Rapidly involuting congenital hemangioma (RICH) Non-involuting congenital hemangioma (NICH) Venous malformation (VM) Kaposiform Hemangioendothelioma Lymphatic malformation (LM) (KHE) High flow: Arteriovenous malformation (AVM) Tufted Angioma (TA) Combined including syndromic VA. Other rare tumors www.issva.org • 2014 ISSVA classification is now 20 pages long • The key is that the imaging characteristics have not changed • Rapidly growing field • Traditionally, options were always the same – Surgery – Do nothing • With the increase in awareness and research as well as the development of the specialty of vascular anomalies: New Treatment Options Available – Treatment directly linked to diagnosis • Today, we have: – Interventional catheter based therapies – Laser surgery – Ablation technologies: Cryo, RFA, Microwave, etc. – Direct image-guided medications to administer – Infusion medicines – Oral medicines – Surgery- although much less common – Do Nothing- a VERY important alternative • Survey sample of 100 Referred patients – 47% wrong Dx – 35% wrong Tx • 14% wrong Tx with correct Dx • VAC – 14% indeterminate or wrong Dx – only 4% leave with no Tx plan • Important because different diagnosis have different treatments! • How to navigate the EMR – Previous surgery or treatment, biopsy report • Family history • Right protocol was done to answer question • Clinical questions • Correct diagnosis • Intralesional clotting • Angioarchitecture • Anatomic space involvement • Hamartomatous component • Single or multifocal, accurate size • Presence of overgrowth • “I have some crazy vascular malformation thing I need you to scan, not sure what I am looking at” • Questions: – How long has it been there, since birth? – Anybody in family have similar? – When did it start hurting or was noticed? – Is it growing fast or with patient? • The glance: – Is there a scar? – Is there skin discoloration? – Is there vesicles? Is there blebs? – Are there hard pebbles? • This is the first fork in the road, very important one • Four helpful differentiators: – Cystic, solid or bundle of vessels – Skin involvement – When did symptoms start – Growth in proportion or out of proportion to patient growth Vascular Anomalies Tumors: Malformations: - Cell proliferation - Normal cell turnover - Can spontaneously involute - No spontaneous involution - Small or absent at birth - Usually seen at birth - Rapid growth during infancy - Growth proportional to patient • Most common vascular tumor • MASS is NOT present at birth, a pink stain in location of future infantile hemangioma can be • Many of these will not be imaged • Proliferation and then involution natural history PHACES LUMBOSACRAL AND MIDLINE NECK/BACK -35-51% of associated deep involvement -Spinal dysraphism, deep involvement -PELVIS, SACRAL, LUMBAR all discuss dysraphism, anogenital, renal, urologic, arterial and bony anomaly syndromes Post Gad • Most clinically significant we see are congenital hemangiomas (RICH and NICH), KHE and Verrucous Hemangioma (unclassified) • Kaposiform Hemangioendothelioma (KHE) clinically significant because of Kasabach-Merritt and Tx. • Congenital Hemangiomas due to bleeding and Tx. • Maximum growth percentage wise at birth • RICH rapidly involutes, see auto infarct signs of cystic/necrotic change and calcification, bleeding • In liver known as Infantile Hemangioendothelioma • Mild Thrombocytopenia (Not like KHE), Cardiomegaly, Heart Failure • NICH grows in proportion to patient, superficial always a bleeding risk, arterial tumor • No medicine works for RICH or NICH, commonly tried • Surgery with or without embolization standard – With RICH, conservative if possible, less is more • Be aware of mimickers: Infantile Fibrosarcoma, KHE infancy 1 year 4 years -observe the veins,Ca++ • KHE locally aggressive vascular neoplasm of children • Kasabach-Merritt phenomenon (KMP) – up to 71% report to have thrombocytopenia • High mortality rate : reported at 24 %, better today • Strong lymphatic component, watch for spread Treatment: – Medical first • mTor inhibitors, namely Sirolimus, now first line, oral • Vincristine has long track record of success also, needs port – Surgery if resection possible – There have been reports of spontaneous involution Behr GG, Johnson CM. Vascular anomalies: hemangiomas and beyond—part 1. Fast-flow lesions. AJR 2013 Feb;200(2):414-22. A B C • Different Type of Lesion, also called Angiokeratoma – Officially “unclassified” in ISSVA, GLUT-1 positive • Must clinically suspect to manage properly • Keratotic, wart-like lesion with red to purple color • Does not involute, with continue growth • Treatment is surgical or CO2 laser • Needs biopsy • No medical therapy successful, many tried • Is it lymphatic malformation? • Is it venous malformation? • Is it arteriovenous malformation? • Cause painful flares, classified macrocystic and microcystic • Fluid/fluid levels of hemorrhage, surrounding inflammation of superinfection, size change • Anatomic location, especially with airway association • Atypical features: calcification, thick walls, nodules • Location, location, location • Outflow size and connection • Clotting, most common with spongiform “grapes” type • Classic misses at VAC: Plexiform N., synovial sarcoma • No mass on imaging despite clinical appearance of mass from edema and eschar • Associated CM suggest autosomal dominant RASA1 • These classically become more symptomatic with puberty and pregnancy, hormonally responsive – Common teenage presentation • The clinical key is steal phenomenon, despite increased flow, decreased oxygenation • Angioarchitecture description key- feeding arteries and draining veins, dominant/codominant • Treatment includes embolization and surgery, no medical therapy proven successful - Four clinical stages: 1. Quiescent 2. Expansion – classic at puberty 3. Destruction (ulceration, bleeding and pain) 4. High outflow heart failure (rare) 16 hours of embo before • Dramatically different management and complications • Should be taken care of by multidisciplinary team when possible • Entities include Klippel Trenaunay (KTS), Parkes Weber (PWS), CLOVES, Proteus and many more • Need to know embryonic anatomy – Lateral Marginal, Sciatic, other – Size, course and outflow communication key • Need to know any macrocystic or focal spongiform VM components and compartments contained • Pay attention to bladder wall, uterus and colonic wall, invasion can be subtle and hugely important • Purely high-flow overgrowth, no masses or slow flow component – Familial seen, CM-AVM, RASA1 mutation • Clinically will look like arterial insufficiency in elderly, AV shunting causes relative distal ischemia • Typically at angio have AVF’s and AVM’s • Complex overgrowth syndrome- Congenital Limb Overgrowth, Vascular Malformation, Epidermal Nevus and Spinal Anomalies/Scoliosis • Significant overlap with KTS- embryonic vein findings most clinically important, can have chest wall and upper extremity • Second and third ray upper/lower macrodactyly The E in cloves • Mosaic, progressive and sporadic – Nature of the syndrome is essential diagnostic criteria – Historically, like KTS, most named Proteus not Proteus • Cerebriform connective tissue nevus is pathognomonic • Hemihypertrophy, kyphoscoliosis, epidermal nevus, vascular malformations, skeletal deformities including macrodactyly, embryonic veins .
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