Transcript Venous malformations

Vascular Head and Neck lesions:
Imaging and Management Essentials
Sean Woolen, MD, Ashok Srinivasan, MD
Department of Radiology
University of Michigan Health System, Ann Arbor
Disclosures
No relevant financial disclosures
Purpose
Classification and terminology
Role of imaging
Management
Mulliken and Glowacki Classification
(based on endothelial cell turnover)
Arteriovenous M.
Vascular
malformations
NORMAL
endothelial
cell turnover
Venous M.
Lymphatic M.
Capillary M.
Paraganglioma
Vascular tumors
HIGH
endothelial
cell turnover
Hemangioma
Nasopharyngeal
angiofibroma
New ISSVA Terminology
Cavernous hemangioma
Liver hemangioendothelioma
Infantile hemangioma
Port wine stain
Capillary malformation
Hepatic/vertebral/orbital
hemangiomas
Venous malformation
Lymphangioma
Cystic hygroma
Lymphatic malformation
Role of Imaging
 Anatomic localization
 Assessing extent
 Often no specific features
Vascular Malformations
Birth
4 years
15 years
Venous
malformation
Lymphatic
malformation
Arteriovenous
malformation
Growth is PROPORTIONATE to body size
AV Malformations - Clinical Picture
• Commonly manifest later in childhood or
adolescence
• Pulsatile lesions that can cause clinical
bruit, pain, bleeding, and ulceration
AV Malformations - Imaging
CT and MRI
Large dilated vascular structures with arteries and
veins being usually indistinguishable
Contrast enhanced CT
demonstrates abnormal
nidus of vessels along the
right face with dilated
vascular channels, likely
draining veins
AV Malformations - Imaging
ANGIOGRAPHY
GOLD STANDARD
Early venous shunting, arterial feeders, venous drainage
Lateral Projection
- ECA Injection
Early Arterial
Mid Arterial
AV Malformations - Staging
Stage
Description
1
Quiescent: stable
2
Increasing in size
3
Symptomatic: pain, bleeding,
disfigurement, disruption of function
4
Decompensating: high-output
cardiac failure
Schobinger classification helps guide management
for AV malformations. Treatment of symptomatic
patient's with stage II or above AV malformations.
AV Malformations - Rx
Gold Standard
Preoperative angiography with selective
embolization of the nidus and draining veins
followed by definitive resection within 24-48 hours.
Resistant Malformations
Angiography with combination of selective
embolization of the nidus and percutaneous
sclerotherapy. With limited angiographic access to
the nidus, emerging percutaneous cryotherapy is an
option.
AV Malformations - Rx
T1 MRI with frank enhancement of mandibular AVM. This was
treated with percutaneous sclerotherapy of the hypervascular
mass with near complete devascularization.
Venous Malformations – Clinical Picture
• Soft, compressible, bluish nonpulsatile mass
• Expansion on ipsilateral jugular vein
compression, performance of the Valsalva
maneuver, or dependent positioning
Venous Malformations - Imaging
MRI (Best Imaging)
• Crucial to assess multispatial extent
• Isointense -T1-weighted
• Hyperintense - T2-weighted
• Intense enhancement
Venous Malformations - Imaging
MRI
• Distinguishing features
include DISCRETE AREAS
OF HOMOGENEOUS HIGH
SIGNAL INTENSITY (venous
lakes) and PHLEBOLITHS
T2-W
T1-W
Post Gad T1-W
Venous Malformations - Imaging
MR images demonstrate phlebolith (arrows) seen as
signal voids on both T1-W and T2-W images on the
right side of the mandible
Venous Malformations - Imaging
CT
• Muscle attenuation on non-contrast images with
variable patterns of enhancement.
• LOW-FLOW lesions can demonstrate PHLEBOLITHS.
Contrast enhanced CT image
demonstrates enhancing
venous malformation in the
right forehead
Venous Malformations - Imaging
CT
• Muscle attenuation on non-contrast images with
variable patterns of enhancement.
• LOW-FLOW lesions can demonstrate PHLEBOLITHS.
Contrast enhanced CT image
demonstrates venous
malformation with
phleboliths in the right
parapharyngeal space
Venous Malformations - Treatment
Many venous malformations do not require
specific management
• Intervention depends on
– Hematologic evaluation
– Size of the lesion
– Functional impact on the patient
Venous Malformations - Treatment
Hematologic
Evaluation
Fibrinogen and D-dimer levels can detect the
presence of localized intravascular coagulopathy,
which may progress to disseminated
intravascular coagulopathy with aggressive
therapy.
If elevated, should start prophylactic Lovenox.
Venous Malformations - Treatment
Sclerotherapy
The sclerosants administered are
typically 95% ethanol or sodium
tetradecyl sulfate (Sotradecol)
Sclerotherapy
+ Surgery Debulking
For large, infiltrating malformations
that are difficult to delineate,
combined sclerotherapy with ablative
and reconstructive surgery is indicated
Small & well
circumscribed
Large &
infiltrating
Sirolimus is an emerging treatment when extensive venous
malformations are refractory to other treatments.
Venous Malformations - Treatment
Sclerotherapy procedure of right facial venous
malformation using ultrasound guidance, 20 gauge
angiocatheter access, and injection of bleomycin
sclerosant.
Lymphatic Malformations Clinical Picture
Arises from sequestered portions of the embryonic
lymphatic anlage
CLINICAL EXAM
• Manifest as soft, colorless masses
• 60% apparent at birth, while 80-90% manifest by 2
years
• Head and neck are the most common site
of distribution
• Growth proportionate to body size
Lymphatic Malformations
• Type I lymphatic malformations (macrocystic)
– Generally arise below the mylohyoid and occur in the
anterior and posterior triangles of the neck.
– Historically termed cystic hygromas
– >2 cm in size
• Type II lymphatic malformations (microcystic)
– Generally located above the mylohyoid commonly
involving the tongue, floor of the mouth, cheeks, lips,
and sometimes the parotid gland
– Historically termed lymphangiomas
– <2 cm in size
Lymphatic Malformations - Imaging
• Low Density- CT
• High T2 Signal - MRI
• No significant enhancement
(Venous components of complex lesions may enhance)
Multiloculated fluid intensity lesion in a neonate with minimal enhancement
Lymphatic Malformations - Imaging
D.D.
• Branchial cleft cyst
• Thymic cyst
• Abscess
Multiloculated fluid intensity lesion in a child with rim enhancement.
The high pre-contrast T1 signal image suggests protein content.
Lymphatic Malformations - Imaging
Venolymphatic malformation
with mixed signal intensity
(fluid signal and enhancing
components).
Venous malformation in
white
*Both have phleboliths
Lymphatic Malformations - Rx
 Sclerotherapy is advocated for large macrocystic
lesions with alcohol, doxycycline, or bleomycin
 Surgical excision with careful preservation of
involved neurovascular structures and laser
photocoagulation serve as important therapeutic
adjuncts
Lymphatic Malformations -Rx
Sclerotherapy
-Ultrasound of lymphatic malformation with macrocystic and
microcystic components.
-Large macrocystic components were accessed with needle
aspirating fluid (yellow LM and red LVM)
-Following aspiration, doxycycline mixed with contrast were
injected into the lymphatic malformation.
Lymphatic Malformations - Rx
Rx
Pre and post sclerotherapy images in lymphatic malformation showed
significant reduction in lesion size one year after sclerotherapy
Hemangioma - Prototype Vascular Tumor
Infantile Hemangioma is GLUT1 positive
Proliferating and involuting phases
Involution
Proliferation
Birth
6-12 months
Up to 9 years
Typically complete resolution –
50% by 5 years; 70% by 7 years; 90% by 9 years
Hemangioma-Classification
Congenital Hemangioma is GLUT1 negative
Rapidly involuting congenital hemangioma (RICH) most
commonly involute by 14 months
Partial-involuting congenital hemangioma (PICH)
Non-involuting congenital hemangioma (NICH) can grow in
proportion the patients age or stay the same size
Hemangioma - Imaging
• Clinical history is key to recognizing the
proliferating phase
CT
• Heterogeneity and intense enhancement, often in a
delayed manner
Contrast enhanced CT in a
6 month old infant shows
enhancing right neck mass that
was progressively getting larger
since birth
Hemangioma - Imaging
• Iso to slightly hyperintense on T1-weighted images
• Progressively hyperintense on T2-weighted images
and show contrast enhancement
T2-W
T1-W
Post Gad T1-W
MR images demonstrate internal heterogeneity, increased T2
signal, internal flow voids and intense enhancement
Hemangioma - Imaging
On angiography, they are high-flow lesions that are
well-circumscribed and exhibit a lobular pattern of
intense, persistent tissue staining.
POST
ANT
POST
ANT
POST
Lateral projection during internal maxillary artery injection
demonstrates intense and persistent tissue staining
ANT
Hemangioma -Treatment
Treatment when one of the criteria below are met:
Emergency
• Potentially
lifethreatening
complication
Urgency
• Existing or
imminent
functional
impairment,
pain, or
bleeding
Structure
Anomalies
Elective
• Identify
structural
anomalies
associated
with infantile
hemangioma
• Reduce the
likelihood of
long-term or
permanent
disfigurement
Hemangioma -Treatment
Observation remains the preferred management option
Vincristine
Beta Blockers
First line treatment
Lesions associated
with KasabachMerritt phenomenon
Surgery
Pulse dye laser
Treatment of
ulcerating lesions
Steroids, Interferon,
and Imiquimod
Failure/contraindication
to pharmacotherapy and Used less commonly
because other
favorable anatomical
treatments have
area for resection or
better safety profiles
resection of residual
fibrofatty tissue after
involution
Paraganglioma- Clinical Picture
• Jugulotympanic paragangliomas present with pulsatile
tinnitus and hearing loss. Physical exam shows a
bluish/pulsating mass behind the tympanic membrane.
• Carotid Body present as a painless, non-tender,
enlarging mass lateral to the tip of the hyoid bone.
Physical exam shows a pulsatile mass which moves
side to side, but not vertically.
• Glomus Vagale present as a painless non-tender mass
posterior to the mandible with cranial nerve
symptoms such as dysphagia (IX), hoarseness (X),
shoulder drop (XI), aspiration (XII).
Paraganglioma-Imaging
MRI
SALT AND PEPPER:
SALT– areas of subacute hemorrhage
PEPPER – hypointense serpentine flow voids,
intense enhancement, mildly T2 hyperintense
Carotid body tumor is the most common paraganglioma. The
MRI images above show typical splaying of the ICA and ECA
flow voids, heterogeneous appearance on T2, and
enhancement.
Paraganglioma-Imaging
CT
Lobular avidly enhancing mass
Glomus vagale displaces
the ECA and ICA anterior
and medial heterogeneous
enhancement on CT.
Malignant paraganglioma
encompasses the jugular
vein, ECA, and ICA.
Malignancy most common
in glomus vagale.
Paraganglioma-Imaging
Jugulotympanic is the most common
primary tumor in the middle ear.
Important to notice asymmetric
jugular foramen enlargement.
The CT image shows asymmetrical
right jugular foramen enlargement
with moth eaten appearance.
Treatment
Paraganglioma-Rx
Surgical resection + pre-op EMBOLIZATION
(with or without radiation therapy for large lesions at the skull
base and poor surgical candidates)
Angiography
Prolonged INTENSE TUMOR
BLUSH. Performed for
preoperative assessment
and embolization of
dominant arterial feeders.
Summary
• Proportionate growth to body size –
Vascular malformations
• Rapid growth from birth followed by involution –
Infantile hemangioma
• Phleboliths – Venous malformations
• Hemangiomas– Usually no Rx
• Venous and Lymphatic malformations – Sclerotherapy +/surgery
• AV malformations and Paraganglioma – Preop embolization
+ Surgery
References
• Mulliken JB et al. Hemangiomas and vascular malformations
in infants and children: A classification based on endothelial
characteristics. Plast Reconstr Surg 69:412-422, 1982
• Mulliken JB et al. Classification of pediatric vascular lesions.
Plast Reconstr Surg 70:120-121, 1982
• Lowe et al. Vascular Malformations: Classification and
Terminology the Radiologist Needs to Know. Semin
Roentgenol. 2012; 47 (2): 106-17
• Dasgupta et al. ISSVA classification. Semin in Pediatric
surgery. 23: 158-161. 2014.
References
• Fowell C et al. Arteriovenous malformations of the head
and neck: current concepts in management. Br J Oral
Maxillofac Surg. 2016. [Ahead of print].
• Nassiri N et al. Evaluation and management of peripheral
venous and lymphatic malformations. 4 (2): 257-65, 2016.
• Woolen S et al. Paragangliomas of the Head and Neck.
Neuroimaging Clin N Am. 2016. [Ahead of print].
• Darrow DH et al. Diagnosis and management of infantile
hemangioma. 136 (4): 1060-104. 2015.