Pediatric
Vascular Anomalies
Mark Ferguson, M.D.
Thursday, August 23, 12
Disclosure
• I have no financial disclosures
• I will discuss off-label
use of gadolinium contrast
Thursday, August 23, 12
Objectives
• review classification scheme
and appropriate terminology of
vascular anomalies
• review imaging characteristics of
common anomalies
Thursday, August 23, 12
Classification
1996 International Society for the Study of Vascular Anomalies
Vascular
Tumors
Vascular
Malformations
plump,
proliferative
endothelium
thin,
dysplastic
endothelium
Vaidya S, et al. Seminars in Interventional Radiology 2008; 25(3):216-33
Thursday, August 23, 12
Case
Ultrasound
T1 + GAD
Thursday, August 23, 12
Time-resolved
MRA
Question - not SAM
Imaging findings are most
consistent with which anomaly?
• A. Arteriovenous malformation
• B. Congenital hemangioma
• C. Venous malformation
• D. Infantile Hemangioma
Thursday, August 23, 12
Venous Malformation
US Findings
• tangle of tubular
hypoechoic
structures
• often monophasic
slow flow on
Doppler
• occasionally
(~15%) no detected
flow due to stasis
Thursday, August 23, 12
Venous Malformation
STIR
MR
Findings
Thursday, August 23, 12
T1 + GAD
Time-resolved MRA
• serpigenous T2 hyperintense, T1
intermediate intensity venous channels
• venous phase enhancement on TR-MRA
Venous Malformation
• Pathology: cluster of abnormal venous channels
with dysplastic endothelium, variable smooth
muscle, and absent valves
• Time Course: present at birth, but may not become
clinically apparent until later in life
• Notable: repeated cycles of thrombosis and
thrombolysis can eventually calcify, resulting in
phleboliths
• Treatment: sclerotherapy
Thursday, August 23, 12
Case
STIR
Thursday, August 23, 12
T1 + GAD
Question - not SAM
Often associated with Down, Turner, and
Noonan syndromes, the demonstrated
lesion is a/an …
• A. Lymphatic malformation
• B. Infantile Hemangioma
• C. Venous malformation
• D. AVM
Thursday, August 23, 12
Lymphatic Malformation
MR Findings
STIR
T1 + GAD
Macrocystic
• cysts >2 cm
• no central
enhancement
• thin septal
enhancement
Microcystic
• cysts <2 cm
• conglomeration of
enhancing septa
can give the
appearance of an
infiltrative mass
Thursday, August 23, 12
Lymphatic Malformation
US Findings
Macrocystic
• discreet anechoic
cyst-like spaces
without color flow
• separated by thin
septae
Microcystic
• infiltrative soft
tissue mass
• multiple tiny
unresolvable cysts
result in diffusely
hyperechoic tissue
Mixed Micro- and Macrocystic Lymphatic Malformation
Thursday, August 23, 12
Lymphatic Malformation
• Pathology: cluster of abnormal, dilated,
lymphatic channels
• Time Course: present at birth and typically
enlarges proportionally with child
• Notable: can come to clinical attention due to
rapid enlargement with hemorrhage or infection
• Treatment: sclerotherapy or surgery
Thursday, August 23, 12
Mixed Venolymphatic
Malformation
Disparity
between
STIR signal
abnormality
and venous
enhancement
suggests the
macrocystic
lymphatic
portion of a
mixed
venolymphatic
malformation
STIR
Thursday, August 23, 12
T1 + GAD
Time-resolved MRA
Case
STIR
Thursday, August 23, 12
T1 + GAD
Time-resolved MRA
Question - not SAM
Capillary malformation plus demonstrated findings
are consistent with which syndrome (note lack of
high flow component)?
• A. Parks-Weber
• B. Klippel-Trenaunay
• C. Sturge-Weber
• D. PHACE
Thursday, August 23, 12
Klippel Trenaunay Syndrome
STIR
T1 + GAD
Time-resolved MRA
Left lower extremity hypertrophy with mixed venous and
lymphatic malformation and large lateral draining vein (arrow)
Thursday, August 23, 12
Klippel Trenaunay Syndrome
• Pathology: low flow vascular malformations and limb
hypertrophy with cutaneous capillary malformation
• Time Course: presents in the newborn
• Distribution: typically involves a single extremity,
most often a lower extremity
• Notable: often a persistent large lateral marginal
vein (vein of Servelle) which is incompetent
Thursday, August 23, 12
Parkes Weber Syndrome
STIR
Mild left lower
extremity
hypertrophy
Subtle STIR
hyperintensity
represents mixed
vascular
malformation
Time-resolved MRA
Similar to Klippel Trenaunay syndrome, but with high flow
components as well
Thursday, August 23, 12
Parkes Weber Syndrome
STIR
Early draining
vein reflects high
flow component
Mild left lower
extremity
hypertrophy
Subtle STIR
hyperintensity
represents mixed
vascular
malformation
Time-resolved MRA
Similar to Klippel Trenaunay syndrome, but with high flow
components as well
Thursday, August 23, 12
Case
Time-resolved MRA
Thursday, August 23, 12
Question - not SAM
The demonstrated lesion likely represents…
• A. Infantile Hemangioma
• B. AVM
• C. AVF
• D. Venous malformation
Thursday, August 23, 12
Arteriovenous Malformation
Time-resolved MRA
Angiographic
Findings:
Thursday, August 23, 12
• coiled nest of vessels with arterial phase
enhancement (central nidus)
• early opacification of draining veins
Arteriovenous Malformation
Companion Case
CTA
Angiographic
Findings:
Thursday, August 23, 12
Catheter Angiogram
• coiled nest of vessels with arterial phase
enhancement (central nidus)
• early opacification of draining veins (arrow)
Arteriovenous Malformation
• Pathology: tangle of vessels without intervening
capillary bed, characterized by a central nidus
• Time Course: present from birth
• Notable: arteriovenous shunting results in spin echo
T2 flow voids and arterialization of draining vein
on US (or high peak venous velocities)
• Treatment: embolization of feeding arteries (coils) or
across the nidus (glue); surgery may be required
Thursday, August 23, 12
Case
Thursday, August 23, 12
Question - not SAM
The demonstrated lesion likely represents…
• A. Hemangioma
• B. AVM
• C. AVF
• D. Venous malformation
Thursday, August 23, 12
Congential Arteriovenous
Fistula
Arterialization of portal
flow
Yin-Yang pattern
Similar to arteriovenous malformation, but without central
nidus of vessels. Tend to embolize with coils.
Thursday, August 23, 12
Congential Arteriovenous
Fistula
Arterial phase CT
Angiogram
Similar to arteriovenous malformation, but without central
nidus of vessels. Tend to embolize with coils.
Thursday, August 23, 12
AVF with VM
STIR
T1+GAD
Time-resolved MRA
High and low flow vascular
malformations can
commonly coexist
Thursday, August 23, 12
AVF with VM
Direct high flow
component
STIR
Low flow venous
malformation
Time-resolved MRA
Time-resolved MRA
High and low flow vascular
malformations can
commonly coexist
Thursday, August 23, 12
Case 5
STIR
TR-MRA
STIR
T1 + GAD
Thursday, August 23, 12
Question - not SAM
The demonstrated lesion likely represents…
• A. Infantile Hemangioma
• B. AVM
• C. Lymphatic malformation
• D. Venous malformation
Thursday, August 23, 12
Infantile Hemangioma
TR-MRA
STIR
•
•
MR
Findings: •
Thursday, August 23, 12
well defined mass lesion
hyperintense on T2,
hypointense flow voids (arrows)
homogeneous arterial phase
enhancement
Infantile Hemangioma
US Findings
• High vessel density (>5/cm2)
• High peak arterial Doppler shift (>2kHz)
• Solid tissue component distinguishes a
hemangioma from vascular malformations
• Together findings suggest hemangioma
• sensitivity: 84%
• specificity: 98%
•No significant difference in vessel density or
peak arterial velocity between hemangioma
and AVM, but greater than low flow lesions
Dubois et al. 1998
Paltiel et al. 2000
Thursday, August 23, 12
Infantile Hemangioma
• Pathology: benign vascular neoplasm,
proliferative endothelium, GLUT-1 positive
• Time Course: typically absent at birth, with rapid
growth (most in first 4-6 months) followed by
involution (70% gone by 7 years)
• Notable: no perilesional edema, if atypical in imaging
appearance, consider biopsy to evaluate for
kaposiform hemangioendothelioma or
angiosarcoma
• Treatment: typically untreated; can use beta-blockers,
steroids, pulsed-dye laser or surgical resection if
located in a critical area
Thursday, August 23, 12
Case
T1 + GAD
At Birth
Thursday, August 23, 12
T1 + GAD
At 11 months
T1 + GAD
At 23 months
Question - not SAM
The demonstrated lesion likely represents…
• A. Infantile Hemangioma
• B. Rapidly involuting Congenital Hemangioma (RICH)
• C. Non-involuting Congenital Hemangioma (NICH)
• D. Venous malformation
Thursday, August 23, 12
Rapidly Involuting Congenital
Hemangioma (RICH)
T1 + GAD
At Birth
T1 + GAD
At 11 months
T1 + GAD
At 23 months
Similar imaging features as infantile hemangiomas, RICH are fully
formed at birth and rapidly involute over the first two years of life
Thursday, August 23, 12
Non-involuting Congenital
Hemangioma (NICH)
16 year old female with left arm mass since birth
Though also
fully formed
at birth,
NICH
demonstrate
little or no
involution
over time, in
contrast to
their rapidly
involuting
counterparts
STIR
Thursday, August 23, 12
T1 + GAD
TR-MRA
Congenital Hemangioma
• Pathology: benign vascular neoplasm,
plump endothelium, GLUT-1 negative
• Time Course: fully formed at birth, will rapidly involute
over the first two years of life (RICH) or never
involute (NICH)
• Notable: vascular aneurysms, intravascular thrombi,
and arteriovenous shunting are some features
that can help to distinguish congenital from infantile
hemangiomas by imaging (?)
• Treatment: none for involuting subtype, surgical
resection for non-involuting subtype
Thursday, August 23, 12
Case
T1 + GAD
STIR
TR-MRA
Thursday, August 23, 12
Kaposiform
Hemangioendothelioma
T1 + GAD
STIR
MR Findings:
• Hyperintense STIR signal
• Early arterial enhancement
• Locally aggressive mass with infiltrative
margins
Thursday, August 23, 12
TR-MRA
Kaposiform
Hemangioendothelioma
• Pathology: locally aggressive vascular neoplasm
of intermediate malignant potential, rare nodal
metastases
• Time Course: can be present at birth or develop
within the first few months of life, rarely involutes
• Notable: associated platelet sequestration results
in extremely low platelet counts and fibrinogen
levels, known as Kasabach-Merritt phenomenon
• Treatment: resection and chemotherapy
Thursday, August 23, 12
Summary
Classification
Imaging
• MRI with time-resolved
MRA is the modality
of choice
• US is useful for
evaluation of flow
characteristics
Thursday, August 23, 12
References
Vaidya S et al. Imaging and Percutaneous
t
Treatment
T t
t off V
Vascular
l A
Anomalies.
li
S
Seminars
i
iin Interventional
Radiology. (2008) vol. 25 (3) pp. 216-33
Dubois J et al. Soft-tissue venous malformations in adult patients: imaging and therapeutic issues.
Radiographics 2001;21:1519–1531
Donnelly et al. Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic.
AJR Am J Roentgenol (2000) vol. 174 (3) pp. 597-608
Legiehn and Heran. Venous malformations: classification, development, diagnosis, and interventional
radiologic management. Radiologic Clinics of NA (2008) vol. 46 (3) pp. 545-97, vi
Legiehn and Heran. Classification, diagnosis, and interventional radiologic management of vascular
malformations. Orthop Clin North Am (2006) vol. 37 (3) pp. 435-74, vii-viii
Hyodoh et al. Peripheral vascular malformations: imaging, treatment approaches, and therapeutic issues.
Radiographics : a review publication of the Radiological Society of North America, Inc (2005) vol. 25 Suppl 1
pp. S159-71
Dubois et al. Vascular soft-tissue tumors in infancy: distinguishing features on Doppler sonography. AJR Am J
Roentgenol (2002) vol. 178 (6) pp. 1541-5
Dubois et al. Soft-tissue hemangiomas in infants and children: diagnosis using Doppler sonography. AJR Am J
Roentgenol (1998) vol. 171 (1) pp. 247-52
Paltiel, H. J., Burrows, P. E., Kozakewich, H. P., Zurakowski, D., & Mulliken, J. B. (2000). Soft-tissue
vascular anomalies: utility of US for diagnosis. Radiology, 214(3), 747–754.
Flors, L., Leiva-Salinas, C., Maged, I. M., Norton, P. T., Matsumoto, A. H., Angle, J. F., Hugo Bonatti, M., et al.
(2011). MR Imaging of Soft-Tissue Vascular Malformations: Diagnosis, Classification, and Therapy Followup. Radiographics : a review publication of the Radiological Society of North America, Inc, 31(5), 1321–
1340. doi:10.1148/rg.315105213
Thursday, August 23, 12