Transcript Cavernous Sinus Syndrome Imaging: A Myriad of

Cavernous Sinus Syndrome Imaging:
A Myriad of Etiologies
Aleema Patel, Wilson Altmeyer, Cedric W. Pluguez-Turull, Bundhit
Tantiwongkosi, Achint K. Singh
eEdE-34
Disclosures
• None of the authors have any financial
relationships to disclosure
Educational Objectives
1. Review cavernous sinus syndrome
2. Familiarize with cavernous sinus anatomy
3. Describe the common causes of cavernous
sinus syndrome
4. Discuss imaging appearances of different
pathologies associated with cavernous sinus
syndrome
Cavernous Sinus Anatomy
Cavernous Sinus
Oculomotor Nerve (III)
Hypophysis (pituitary gland)
Internal Carotid Artery
Trochlear Nerve (IV)
Optic Chiasm
Abducens Nerve (VI)
Internal Carotid Artery
Ophthalmic Nerve (V1)
Maxillary Nerve (V2)
Sphenoid Sinus
Cavernous Sinus Anatomy
• Paired collection of thin walled veins between the
endosteal and meningeal dural layers located at
the center of the head which drains intracranial
blood
– Anterior Border = Superior orbital fissure
– Posterior Border = Petroclinoid fold and Clivus dura
matter
– Inferolateral Border =Inner middle cranial fossa
– Lateral Border = Outer dural meningeal layer with
deeper inner nerve containing layer
– Medial Border = Sella turcica
Cavernous Sinus Anatomy
• Venous Flow
– Receives venous tributaries from the superior and
inferior ophthalmic veins and superficial cortical
veins
– Drains into the superior and inferior petrosal
sinuses
– Connected to the venous basilar plexus posteriorly
Cavernous Sinus Anatomy
• Nerves
– Lateral wall, superior to
inferior
• CN III: Occulomotor nerve
• CN IV: Trochlear nerve
• CN V1: Ophthalmic nerve,
the V1 branch of the
trigeminal nerve
• CN V2: Maxillary nerve,
the V2 branch of CN V
– Paralleling internal
carotid artery
• CN VI: Abducens nerve
• Artery
– Internal carotid artery
• Bends as the cavernous,
C4 segment
• Bifurcates
– Meningohypophyseal
trunk & Inferolateral
trunk
Imaging Anatomy of Cavernous Sinus
Axial T1-W FS post gad MR
Coronal T1-W FS post gad
MR
Normal thin wall
enhancement of
cavernous sinus
Internal Carotid Arteries
Cavernous Sinus Syndrome
• Definition
– Syndrome caused by thrombosis of the cavernous
sinus
• Symptoms
– Edema of the eyelids and conjunctivae
– Paralysis of extra-ocular muscles supplied by the
CN III, CN IV, and CN VI
Tumors
Carotid
Artery
Aneurysm
Tolosa-Hunt
Syndrome
Cavernous
Sinus
Lesions
Thrombosis
Carotid
Cavernous
(C-C) Fistula
Cavernous Sinus Tumors
•
Most common etiology of cavernous sinus syndrome
•
Primary tumors
– Schwannoma
– Neurofibroma
– Meningioma
– Hemangioma
– Lymphoma
•
Secondary involvement/Metastatic disease
– Pituitary Adenoma
– Nasopharyngeal carcinoma
– Perineural spread of tumor through neural foramina
– Base of skull tumor
• Chondrosarcoma
• Osteosarcoma
Pituitary Macroadenoma
• May grow laterally and invade the cavernous
sinus
• Cavernous Sinus Invasion
– Encasement of the intracavernous internal carotid
artery by >30% of its diameter or tumor extension
lateral to the top (12 o’clock) of the internal carotid
artery
• Does not narrow internal carotid artery unlike meningiomas
– Presence of abnormal soft tissue between the lateral
wall of the cavernous sinus and the internal carotid
artery
Pituitary Macroadenoma with
Cavernous Sinus Invasion
Sagittal T1-W post gad MR
Coronal T1-W post gad MR
Coronal T2-W MR
Large enhancing pituitary
mass extending to the
right cavernous sinus and
encasing the right ICA
Axial T1-W post gad MR
Meningioma
• Most cavernous sinus meningiomas arise from the lateral dural wall
– Sometimes arise exclusively inside the cavernous sinus
• Imaging:
– Hypo- to isointense with respect to gray matter on all MR imaging
sequences
– Intense enhancement
– Dural tail frequently can be seen extending away from the edge of the
tumor and often into the ipsilateral tentorium
– Constrict the lumen of the ICA
• Helps differentiate from pituitary adenomas
– Extend inside the cavernous sinus and the Meckel cave and via the
porous trigeminus into the prepontine cistern
– Often difficult to distinguish from schwannomas
Meningioma
Axial T2-W MR
Axial T1-W post gad MR
Coronal T1-W post gad MR
T2 isointense left cavernous sinus meningioma with narrowing of the left ICA
Trigeminal Nerve Schwannoma
• Commonly involves the cavernous sinus
– Multiple cavernous sinus schwannomas and bilateral vestibular schwannomas
are seen in patients with neurofibromatosis type 2
– 50% have dumbbell-shape with bulky tumor in the Meckel cave and the
prepontine cistern and a waist at the porous trigeminus
– May also be found only involving the Meckel cave
• Imaging
–
–
–
–
–
–
–
Solid, variable cystic, or hemorrhagic components +/- fluid levels
Small tumors  homogeneous
Large tumors  heterogeneous in appearance
T1 isointense-to-hypointense
T2 hyperintense
Enhance with contrast
Follow the expected course of the nerves from which they arise
• May arise from other cranial nerves in the cavernous sinus, especially cranial nerve III
Trigeminal Nerve Schwannoma
Axial T1-W post gad MR
Coronal T1-W post gad MR
Sagittal T1-W MR
T2 hyperintense mass in
the right cavernous sinus
with retrograde
extension along the
cisternal segment of
right CN V
Axial T2-W MR
Plexiform Neurofibromas
• Most commonly involve the trigeminal nerve,
typically the first and second branches
• Seen in 30% of patients with neurofibromatosis
type 1 but are extremely rare outside this disease
• Imaging:
– Tortuous or fusiform enlargement of the nerves that
exhibit heterogeneous signal intensity
– Neurofibromas are less likely to extend to the Meckel
cave unlike schwannomas
Plexiform Neurofibromas
Axial T2-W MR
Axial T1-W post gad MR
Axial T2-W MR
Multiple plexiform neurofibromas in a patient with NF-1 with one
of the neurofibromas extending into the right cavernous sinus
Nasopharyngeal Carcinoma
• Most common primary malignant extracranial neoplasm to
invade the cavernous sinus
– Intracranial extension directly via the skull base erosion or by
perineural spread along branches of the trigeminal nerve
– Can extend through the petro-occipital synchondrosis and
foramen lacerum into the inferior cavernous sinus or via the
carotid canal to gain access to the cavernous sinus without
destroying bone
• Imaging:
– Hypointense to iso-intense relative to muscles on T1-weighted
images
– T2 hypointense
– Moderate-to-intense contrast enhancement
Nasopharyngeal Carcinoma
Axial T1-W FS post gad MR
Coronal T1-W FS post gad MR
Left nasopharyngeal
mass with extension in
the left cavernous sinus
Axial T2-W MR
Axial T1-W post gad MR
Cavernous ICA Aneurysm
• Composes 5% of giant aneurysms (>2.5 cm in
diameter)
• Most of these aneurysms are idiopathic,
occasionally traumatic or mycotic
– More frequent in the elderly population
• Produces cavernous sinus syndrome by mass
effect, inflammation, or rupture into the CS, with
subsequent development of a carotid cavernous
fistula
• Can present with an indolent ophthalmoplegia
– Endovascular occlusion is often successful treatment
Cavernous ICA Aneurysm
• Imaging:
– Patent aneurysm
• Signal-intensity void on spin-echo MR
– Partially thrombosed giant aneurysms
• Mixed signal intensities
– Due to clot in lumen or in walls secondary to chronic
dissections
• Flowing blood through the patent portion of the lumen
appears as a signal-intensity void on spin-echo images
and high signal intensity on gradient techniques
Cavernous ICA Aneurysm
CTA
CTA: Coronal MIP
Carotid Cavernous (C-C) Fistula
•
•
Abnormal connection between the carotid artery and the cavernous sinus
Four Types
–
Type A (Direct): High-flow communication between the internal carotid artery and cavernous sinus
secondary to trauma or a ruptured aneurysm of the cavernous internal carotid artery
•
–
Types B-D (Dural CCFs): Low-flow fistulas occurring between meningeal branches of the carotid
artery and cavernous sinus
•
•
•
Milder symptoms with more insidious presentation than direct fistulas
Often resolve spontaneously
Treated by endovascular occlusion techniques by interventional radiology
–
•
Present acutely with pulsating exophthalmos, chemosis, visual loss, and ophthalmoplegia
Occasionally, surgical treatment with carotid ligation is necessary; this sometimes is preceded by a
superficial temporal-to-middle cerebral bypass operation to ensure cerebral circulation after carotid
ligation
MR Imaging Findings:
–
–
Dilated cavernous sinus with multiple signal-intensity void structures
Proptosis & enlarged superior ophthalmic vein
–
Flow-related enhancement in the cavernous sinus on TOF MR angiography suggests the diagnosis in the right clinical
setting
–
–
“Dirty” appearance of the retro-orbital fat & enlargement of the extraocular muscles
Enlargement of bilateral cavernous sinuses in setting of very high-flow fistulas
Carotid-Cavernous (C-C) Fistula
CTA
CTA
Enlarged right superior ophthalmic vein with early arterial
enhancement of the right cavernous sinus suggestive of carotid
cavernous fistula
Cavernous Carotid (C-C) Fistula: Case 2
Axial CTA
Axial T2-W MR
Early arterial enhancement,
flow voids and arterial signal
on the TOF MR of the
cavernous sinus suggestive of
cavernous carotid fistula
Axial TOF MR
Axial CTA
Cavernous Carotid (C-C) Fistula: Case 2
Post gad MRA: Oblique Coronal MPR
Arterial enhancement of
the cavernous sinus
Cavernous Sinus Thrombosis
• Often secondary to infection of the sinonasal cavities, orbits, and/or
the middle 1/3 of face
• MR Imaging:
– Changes in signal intensity and/or in the size and contour of the
cavernous sinus
• Enhancement of the peripheral margins of an enlarged cavernous sinus
• Subacute thrombus demonstrates high signal intensity on all pulse sequences
 easy to recognize
• Acute thrombosis often isointense  difficult to diagnose
– Indirect signs
• Dilation of the superior ophthalmic veins
• Exophthalmos
• Increased dural enhancement along the lateral border of cavernous sinus and
ipsilateral tentorium
– Diagnosis confirmed by presence of sinusitis and clinical symptoms
Cavernous Sinus Thrombophlebitis
Coronal T1-W FS post gad MR
Axial T1-W FS post gad MR
Enlarged nonenhancing left
superior
ophthalmic vein
(compared with
right)
Enlarged left
cavernous sinus
with subtle
central
hypointensity
Enlarged
extraocular
muscles
Coronal
CT
Axial CT
Cavernous Sinus Thrombosis: Case 2
Axial CT
Axial CT
Acute fulminant invasive mucormysosis with sphenoid
sinusitis complicated by acute cavernous sinus thrombosis
Tolosa-Hunt Syndrome
• Retro-orbital pseudotumor extending to the cavernous sinus
• Clinical triad
– Unilateral ophthalmoplegia
– Cranial nerve palsies
– Dramatic response to systemic corticosteroids
• Usually unilateral but may be bilateral (5%)
• Histopathology = low-grade nonspecific inflammatory process with
lymphocytes and plasma cells
• MR Imaging
– Enlarged cavernous sinus containing abnormal soft tissues that are
isointense to muscle on T1-weighted images and dark or bright on T2weighted images and display contrast enhancement with focal
narrowing of the ICA
Tolosa-Hunt Syndrome
Axial T2-W MR
Axial T1-W FS post gad
T2 iso-hypointense signal in the left cavernous sinus with
enhancement
Enhancement is also seen in the left superior orbital fissure
Summary
• Cavernous sinus syndrome is a complex
constellation of symptoms caused by a broad
range of pathology
– Pathology is shaped by variety of anatomic
structures within and adjacent to the cavernous
sinus
Conclusions
• Careful examination of the cavernous sinus by
radiologists is imperative to ensure detection
of lesions
References
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sinus, cerebello- pontine angle, and skull base. BMJ Group.
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