Transcript Spine Interventions for Pain Management

Diagnosis and Management of a Rare
Sphenoid Wing Mass with Clinical
Findings Mimicking a Carotid
Cavernous Sinus Fistula
Melissa Chen, MD Stephen Chen, MD Vivek Sahani, DO
Control #: 2805 Poster # EE-25
Disclosures
•
None
Purpose
•
To describe an unusual presentation of vision loss from a rare
sphenoid wing hemangioendothelioma and present imaging
findings that is crucial for neurosurgical planning.
History
• 37 year old Hispanic female from El Salvador
• Gradual onset of mild left eye pain and left forehead
pain.
• Initially seen in a community
clinic and prescribed eye
drops for the pain.
• Presented to Emergency room 2 x over course of next
month with severe blurring of vision, new onset
tinnitus, worsening headache and sharp stabbing left
eye pain.
Physical exam
• Left orbital proptosis and chemosis
• Left pupil is non-reactive to light
• Visual acuity 20/400 in left eye
Unenhanced CT Head
On soft tissue window, a mass
(red arrow) erodes the adjacent
sphenoid wing.
The left superior ophthalmic
vein (blue arrow) is enlarged.
Enhanced CT Face
Contrast enhanced CT
demonstrates avid contrast
enhancement of a lobulated 2.8
cm extra-axial mass (red arrow)
that destroys the adjacent
bone.
The enlarged superior
ophthalmic vein (blue arrow)
enhances early, suggesting
arterialization. Note that there
is no enhancement of the
contralateral vein.
Enhanced CT Face
The left middle meningeal (blue
arrow) artery is enlarged
adjacent to the tumor, which
suggests that it supplies the
tumor.
Enhanced CT Face
Coronal image demonstrates a
similarly enlarged left internal
maxillary artery (blue arrow)
also supplying the highly
vascular tumor.
Atypical enlargement and early
enhancement is seen in both
facial veins (red arrows),
indicates high flow AV shunt
physiology within the mass.
External Carotid DSA
Angiogram confirmed a
hypertrophied middle
meningeal artery (red
arrow) supplied the tumor,
the dominant feeder.
Secondary feeder from a
large posterior deep
temporal artery (blue
arrow).
Lateral
External Carotid DSA
Mass demonstrates rapid
arterial venous shunt
pathology (red arrow).
Drainage is through the
superior ophthalmic vein
(blue arrow).
Secondary drainage through
inferior ophthalmic vein
(yellow arrow).
Early filling of enlarged facial
vein (dark blue arrow).
Lateral
MRI Head
Axial T2 image demonstrates
intermediate inhomogeneous
signal within the mass (red).
Flow voids (blue) are visible
within the mass which can also
be a sign of a highly vascular
tumor.
The left extraocular muscles
(yellow) are enlarged with
increased T2 signal, likely
sequela of venous
hypertension.
Embolization
Tumor was catheterized
through the middle
meningeal artery (red
arrow) with a 1.5 Fr.
Marathon microcatheter
(ev3).
Lateral
Embolization
Tumor was then embolized
with a liquid embolic,
Onyx18 (ev3)
The posterior deep temporal
artery was also catheterized
and embolized.
This tantalum opacified cast
fills the tumor and feeding
vessels.
Lateral
Post Embolization
Absent filling of the distal
internal maxillary artery (red
arrow) due to vasospasm.
A small residual collateral
(blue arrow) from the
proximal middle meningeal
artery remained present,
but vision and proptosis
improved immediately.
Lateral
MRI Head
Axial T1 fat saturated image
with contrast demonstrates
avid enhancement of the
infiltrative mass (red arrow) in
the sphenoid wing.
Asymmetric increased
enhancement is also seem in
the left extra-ocular muscles
(yellow arrow)
Dx: Epithelioid Hemangioendothelioma
Clinical Diagnosis
• Vascular lesions involving the orbit are often
misdiagnosed as conjunctivitis, uveitis and
scleritis by clinicians.
• Arterial venous fistula
involving the eye should
be considered when patients present with:
 Orbital pain with proptosis
 Chemosis
 Orbital bruit
 Tinnitus
 Vision loss
Chen, SR (2016) Endovascular Approaches to Orbital Vascular Lesions and Carotid Cavernous Sinus Fistulas In Yen MT. Vascular Lesions of the Orbit
and Face (pp 63-87) Switzerland, Springer International Publishing.
Epithelioid Hemangioendothelioma
• Rare malignant, vascular
tumor
• Incidence 1:1,000,000
• Slow growing tumors,
with
intermediate malignancy
pattern between benign
hemangioma and high
grade angiosarcoma.
(hematoxylin and eosin, 10x)
Weiss SW, Enzinger FM (1982) Epithelioid hemangioendothelioma a vascular tumor often mistaken for a carcinoma. Cancer 50:970–981.
Epithelioid Hemangioendothelioma
• Accounts for only < 0.02%
of intracranial tumors,
where it arises from the
dura or the bone.
• Histologically, primitive
thick walled vascular
channels are seen within
cords and nests of spindle
and epithelioid cells.
(hematoxylin and eosin, 10x)
Tian W-Z, Yu X-R, Wang W-W, et al (2016) Computed tomography and magnetic resonance features of intracranial hemangioendothelioma: A study of
7 cases. Oncol Lett 11:3105–3110.
Discussion
• Although the pathologic diagnosis and clinical
presentation in this case are extremely rare,
identification of key imaging findings and correlation
with clinical signs are widely applicable.
• Severe intraoperative hemorrhage during tumor
resection can be avoided if the need for pre-operative
embolization is identified prospectively, avoiding intraoperative complications, shortening operative times,
and reducing repeat surgery.
Diagnostic imaging clues
• High arterial flow
 Enlargement of arteries adjacent to a tumor
 Early avid enhancement
 Flow voids on MR T2 weighted images
• Arterial venous shunting
 Early venous enhancement: arterialization of the veins
 Venous engorgement in draining vessels
Conclusion
•
Optic nerve compression is not the only source of vision loss.
Arterial venous shunting around the orbit can result in venous
hypertension and retinal ischemia. Embolization can immediately
restore and preserve vision.
•
Radiologists should be able to recognize imaging findings of high
arterial flow into a tumor and recommend pre-operative
embolization to prevent excessive blood loss and surgical
complications.
•
By recognizing these imaging findings, neuroradiology can offer a
more complete patient care pathway encompassing both
diagnosis and therapy.