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Imaging of Vascular Anomalies

Radiology Res Peds

Don Garbett

on 2 April 2013

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Transcript of Imaging of Vascular Anomalies

Imaging of Vascular Anomalies Don Garbett, MD
Radiology Resident PGY-4
Aurora St Luke's Medical Center
Childrens Hospital of Wisconsin contact: dlgarbett@gmail.com
312-320-5352 Diagnosis Exam



Angiography Issue:
Common Confusion among the different types of
Vascular Anomalies
Capillary or Cavernous Hemangioma Infantile Hemangioma
of any organ

Infantile Hepatic or infantile
hemangioendothelioma of liver Hemangioma

Adult hepatic hemangioma Venous Malformation
Adult vertebral hemangioma
Adult orbital cavernous hemangioma OLD ISSVA Example of Difficulty Correct Diagnosis
1. Determine Treatment
2. Prevent adverse physical / emotional sequela
for the child and family Determine Treatment Vascular Malformations Vascular Neoplasms Slow flow (simple)

High flow (combined)
Arteriovenous malformation
Arteriovenous fistula
Any combination of the above Hemangiomas
Congenital: RICH/NICH
Spindle cell
Dermatologic acquired vascular tumor International Society for the study of Vascular Anomalies (ISSVA)
Adopted from Mulliken and Glowacki 1982 Increased endothelial cell turnover Oma = tumor?
In the past, the suffix “oma” was used to described neoplasms and malformations. However, because malformations are not neoplasms, words such as “lymphangioma” are misnomers, and the terminology is being abandoned. some of what we used to call hemangiomas are hemangiomas, although its gotten more complicated

other times they're actually venous malformations Moral
Don't name your hemangiomas before they've hatched How do we image?
Ultrasound - field of view.
MRI and MRA - cost, sedation, time Example Protocol Vascular malformations are not neoplasms but, rather, structural anomalies that result because of a localized error in vascular morphogenesis that can affect arteries, veins, capillaries and lymphatics
Not present at birth, (prev names - strawberry, capillary, juvenile, and nascent hemangioma)
most common, 3-10% of infants
they evolve and resolve
proliferate - grow from 2 w - 2 m and peak at 1 year of age
involute - over time
fibrose - eventually with only a fibro fatty remnant (scar)

If atypical Glut-1
presence on path
is definitive. Ricardo Restrepo, PEDIATRIC MUSCULOSKELETAL IMAGING: BEYOND THE BASICS. Pediatr Radiol (2013) 43 (Suppl 1):S141–S154 References

1. Ricardo Restrepo, Multimodality imaging of vascular anomalies. Pediatr Radiol (2013) 43 (Suppl 1):S141–S154
2. Lowe, et al. Vascular Malformations: Classification and Terminology the Radiologist Needs to Know. Semin Roentgenol. 2012 Apr;47(2):106-17.
3. Google images.
4. Photos by D. Garbett and Witold Pociej Congenital Hemangioma Much less common than IH (Infantile Hemangioma)
present at birth and can be seen in utero
NICH - non involuting
grows with child
RICH - rapidly involuting
involutes much faster than CH (usually w/i 2 years, even in utero)
More common near head and extremities close to joints
glut 1 negative in contrast to IH, otherwise histologically indestinguishable Vascular Malformations note: CHs contain larger more visible vessels (compared with infantile), with evidence of micro-shunting, sometimes simulating an AVM. Other features suggestive of a CH are less-distinct borders, associated fat stranding and occasionally, calcifications. artwork by Marc Chagall (Jacob's Dream) Slow flow (simple)

High flow (combined)
Arteriovenous malformation
Arteriovenous fistula
Any combination of the above Infantile Hemangioma Hemangiomas Capillary Lymphatic Venous AV Malformation and AV Fistula How to Image? What is it? Infantile and Congenital may be indestinguishable on US and MR, so history (growth, presence or absense at birth) and path GLUT1 is key to diagnosis Thank you Where is this? Innsbruck, Switzerland Present at birth, grow in proportion to systemic growth until puberty.
Do not regress
May be discovered later in life http://www.hopkinsmedicine.org/vascular/conditions/malformations/ Macrocystic (cystic hygroma) >2 cm locules
Microcystic 1-2 cm locules
Lymphangioma Circumscriptans - thought to develop from abnormal subcutaneous lymphaticcisterns that communicate with ectopic lymphaticvessels.

VEGFR3 - expressed by lymphatic vessels etc, required for differentiation

May change throughout the day, infection, illness US - multiseptated cystic mass, anechoic to low level echoes
fluid/fluid levels/solid components if hemorrhage/infection
if inflamed may see septal vascularity
MR - minimal or no septal enhancement
if hemorrhage may be hyper to muscle on T1 Sporadic 94%
Dominantly inherited
Cutaneomucosal 1% <5cm
Glomuvenous 5% (GVM)

Walls def of smooth muscle
no valves, no internal elastic lamina
Elevated D-dimer except for GVM more infiltrative, dilated large thin walled veins with subendothelial glomus cell clusters US&MRI- Vascular mapping is important for therapy
appearance is solid to multicystic
low flow or rarely no flow
infiltrative lesions may be misdiagnosed as neoplasm
due to lack of discernable vascular spaces
phleboliths (highly specific)
hypo to hyper to muscle on T1
Extent is best assessed on T2 or STIR (fluid sens) LIC localized intravascular coagulation >40% VM
DIC may result form surgical intervention Capillary Malformation (port wine stain)
mature ectatic capillaries with disorganized surrounding collagen (usually confined to dermis)
decreased capillary tone leads to ectasia
associated with Sturge-Weber and Klippel-Trenaunay syndromes
May occur in association with AVM or AVF (Parkes Weber Syndrome) US - skin thickening
MR - Hypo T1 Hyper T2, +enhancement Usually sporadic or solitary, but can be associated with syndromes
Often diagnosed in older children (may be asymptomatic 1st 2 decades)
cutaneous blush, warm to touch, pulsation, thrill, murmur
pain, ulceration, bleeding, ischemia, CHF
May hypertrophy underlying tissue Variable combination of capillaries, venules, arterioles in fibrous or fibromyxoid stroma
arteries - enlarged, may or may not have internal elastic lamina
veins - muscular hypertrophy or fibrosis of intima, no adventitia US - ill defined mixed echogenicity, arteries with diastolic flow, pulsatile draining venous flow
MR - define extent, flow voids, no ST mass or mass effect, heterogenous enhancement
MRA - vascular map (TRICKS) Time Resolved Imaging of Contrast KineticS can be very helpful (id. nidus, draining veins)
Conventional Angio - characterization (planning) and treatment (embolization) feeding artery aneurysms 2 planes T2 fatsat or STIR T1 fatsat Precontrast MRA MRV TRICKS T1 Post Contrast 2 or 3 planes
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