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Control #: 1737
Title: Mimics of Nontraumatic Solid
Orbital Tumors: More Than Meets
the Eye
eEdE#: eEdE-119 (Shared Display)
No Disclosures
Mimics of Nontraumatic Solid
Orbital Tumors: More Than Meets
the Eye
Rajput, Anuj MD, Ahluwalia, T. MD, Rajput, Aikta,
Bangiyev, L. DO
Stony Brook University Hospital, NY
Purpose
To review the incidence, clinical presentation,
and significance of several non-traumatic orbital
pathologies with our main focus on the
thrombosed orbital varix.
Familiarity with appearance of the various
orbital lesions will help the radiologist to
correctly diagnose a thrombosed orbital varix,
exclude other differential diagnosis, and provide
appropriate recommendations.
Orbit Anatomy
(Superior to inferior)
Superior
Ophthalmic
Vein
Superior
Rectus
Muscle
Orbit Anatomy
Extraconal
Space
Intraconal
Space
Lamina
Papyracea
Orbit Anatomy
Medial Rectus
Muscle
Lateral Rectus
Muscle
Globe
Optic Nerve
Superior
Orbital
Fissure
Orbit Anatomy
Anterior Chamber
Posterior Chamber
Lens
Sclera
Inferior
Rectus
Muscle
Orbit Anatomy
Ophthalmic
Artery
Superior
Rectus
Muscle
Superior
Oblique
Muscle
Optic
Nerve
Inferior
Rectus
Muscle
Lateral
Rectus
Muscle
Medial
Rectus
Muscle
CASE
History of Present Illness
• 69 year old female presents to the ED with chief
complaint of bilateral eye swelling and vomiting,
worse on the left. Patient states she woke up with
bilateral eye pain, worse with lateral and medial
gaze. She had intermittent episodes of blurry
vision and multiple episodes of vomiting. Patient
stated that when looking straight ahead, she has
no pain or vision abnormality. She denies prior
episodes. There were no relieving factors.
• Otherwise, review of systems was noncontributory
Medical History
•
•
•
•
•
PMHx: HTN, HLD, hypothyroidism
FMHx: None
SHx: Denied alcohol, drugs, tobacco
SurgHx: Hysterectomy
Allergies: NKA
Physical Exam
Vitals: Temp: 38.6*C,HR: 85, BP: 132/89, Pulse Ox: 94%
General: Alert, no acute distress.
Skin: Warm, intact.
Head: Normocephalic, atraumatic.
Neck: Supple, trachea midline.
Eye: PERRLA, EOM intact, Visual acuity: OU 20/ 20; Slit-lamp
exam: Viewed with fluorescein, questionable circular stromal
lesion/infiltrate at approximately 6 o'clock which does not uptake
fluorescein, similar lesion on right, not clearly ulcerative and
appear almost crystalline. Ant. Chamber quiet. No photophobia
IOP: OD: 15 OS: 18,
ENT: Within normal limits
Neurological: A&O X3, normal sensory, motor 5/5, normal
speech, cranial nerves intact.
Laboratory Values
• General Chemistry Panel
– unremarkable
• TSH
–
within normal limits
• General Hematology Panel
– unremarkable
• Coagulation
– Antithrombin III and Protein C were elevated
– Otherwise within normal limits
CT Orbits C-
Following CT C-, an MRI with
contrast of orbit and brain
were performed
Differential Diagnosis?
Axial CT C- of the orbits
demonstrates a left
intraorbital high density
circumscribed mass (arrow)
with Hounsfield units
compatible with blood
products.
Coronal CT C- of the orbits shows
left orbital intraconal mass with
associated mass effect and
displacement of the left optic
nerve (blue arrow).
Axial T2 MR image
demonstrates low signal
intensity mass in the left
orbit (arrow).
Axial T1 precontast MR
image demonstrates iso to
low signal intensity of the
mass in the left orbit.
Axial T1 post contrast MR image
demonstrates thin rim enhancement
around the left orbital lesion (arrow). A
smaller homogeneously enhancing
lesion is also present within the right
orbit (yellow arrow)
Coronal T1 post contrast MR image again
demonstrates nonehancing left intracornal
mass with mass effect on the optic nerve
(red arrow). The smaller right intraconal
mass is homogeneously enhancing
without significant mass effect (yellow
arrow).
Differential Diagnosis of Solid
Orbital Mass
• Neoplastic - lymphoma, multiple myeloma,
meningioma, metastasis, optic glioma
• Inflammatory – Pseudotumor, thyroid
ophthalmopathy
• Granulomatous – Sarcoidosis, Wegner’s
granulomatosis
• Congenital - infantile hemangioma
• Vascular - orbital varix, venolympathic
malformation
• Infectious
Diagnosis
Thrombosed Orbital Varix
Background
• Rare < 1.3% of all orbital tumors
• Most common cause of spontaneous orbital
hemorrhage.
• Abnormally enlarged vein - single vessel with
saccular or segmental dilatation or tangled plexus
of venous channels.
• 2nd and 3rd decade of life M=F
• Primary or secondary
• Intermittent diplopia or proptosis during of
straining or prone.
• Hemorrhage or thrombosis are possible
complications can cause acute orbital pain and
decreased visual acuity.
Background
Primary Form
• Congenital venous
malformation.
• May manifest itself during
infancy or delayed until
young adulthood.
• Transient exophthalmos
induced by alsalva due to
communication with the
systemic venous system.
Secondary Form
• Acquired due to
increased blood flow as a
result of intracranial AVM,
CC fistula or dural AVF
which drain vial the orbit.
• Associated with vein of
Galen malformation and
occasionally with venous
angiomas.
Clinical Presentation
• Presenting symptoms include intermittent
diplopia or proptosis during episodes of
straining or prone positioning.
• Hemorrhage or thrombosis are possible
complications that can cause an acute
onset of retro- orbital pain and decreased
visual acuity.
Work up & Treatment
• Difficult to diagnose without provocative
examination with valsalva
• Large may present as a hyperdense orbital
mass on CT, especially if thrombosed
• Thrombosed orbital varix show complex MR
signal of blood products and/or fluid levels
• Treatment ranges from observation with
mild symptoms to embolization or surgery
with intractable pain or threatened vision.
Case Review of Relevant
Differential Diagnosis
Ocular Lymphoma
• B-cell mucosa-associated lymphoid tissue (MALT)
are the most common14,15
• 5-10% of orbital masses
• Associated with systemic collagen vascular
diseases and immunocomprised patients.
• Indolent course and painless mass
• Key imaging features - intermediate or low T2,
homogeneous enhancement, ↑ DWI and ↓ ADC24
• Favorable prognosis.
• Sensitive to radiation16,17
• High grade lesions may require systemic
chemotherapy.
Ocular Lymphoma
A
B
C
D
FLAIR (A), T1 Post-Gd (B), B1000 (C), ADC (D) demonstrate a mildly enhancing extraconal mass
in the superior medial right orbit with a small central focus of irregular, spiculated T2 and FLAIR
hyperintensity and more intense enhancement. It restricts diffusion. This is displacing the
medial rectus as well as the optic nerve laterally. There is remodeling medial deviation of the
inner wall of the right orbit suggesting a benign or long-standing lesion.
Multiple Myeloma
• Clonal proliferation of malignant plasma cells
in the bone marrow leading to multiorgan
failure13
• Hypercalcemia, renal dysfunction and bone
lesions13
• Rarely presents as orbital lesion9
• Solitary extramedullary orbital plasmacytomas
have been reported as the initial presention 11
• Treatment include systemic chemotherapy
and local12
Multiple Myeloma
Figure 1: Prominent soft tissue
mass associated with bone
destruction located in the left
superior orbital fissure region.
(arrow). The left optic canal is
fully destroyed and occupied
by tumor.
Figure 2: Numerous lytic
lesions in the calvarium. (blue
arrows)
Figure 1
Figure 2
Meningioma
•
•
•
•
Benign mass, presents in 4th or 5th decade of life.
Painless eye mass, vision loss, and proptosis 24
Unilateral. Bilateral in neurofibromatosis type 2. 24
Primary and Secondary:
– Primary arises from capillary cells of the arachnoid around
the intraorbital or intraconal portions of the optic nerve.25-26
– Secondary arises intracranially and invades the optic canal
and orbit by extension25-26
• Imaging – mass surrounding and constricting the optic
nerve; calcification on CT; ‘‘tram track’’ or ‘‘target’’ like
enhancement.24
• Fractionated stereotactic radiotherapy is first line therapy
Meningioma
A
B
C
Axial T1 C+ (A) demonstrates an enhancing mass surrounding
the optic nerve in a “tram-track” pattern. Coronal T2 (B) and
Coronal C+ (C) MR images demonstrate T2 hyperintensity and
contrast enhancement surrounding the optic nerve creating a
target-like pattern.
Ocular Metastasis
• Carcinomatous origin in adults. Sarcomatous
or neural embryonal tumors in children.27
• Account for 1–13% of all orbital tumors.28
• Typically occur in the anterior orbit.29
• Most frequent: breast, lung, prostate,
melanoma, carcinoid, GI, renal cell,
neuroblastomas and rhabdomyosarcomas.28
• Imaging findings vary widely. Globe
retraction is highly suspicious for scirrhous
breast cancer metastasis.24
Ocular Metastasis
A
B
C
Bilateral lobular metastasis with heterogeneous signal on T1WI (A) and
T2WI (B) with diffuse enhancement (C). More advanced on the right
demonstrated by right globe retraction. Findings consistent with
schirrous breast metastasis.
Optic Glioma
• Indolent and low grade tumors
• Typically associated with NF-1 and found
in mostly in children.
• Key features: Smooth, homogeneous
spherical mass with hypointense T1
signal, and T2 hyperintense signal with or
without enhancement.
Optic Glioma
A
C
B
T1 hypointense (B,E), T2
hyperintense (C),
heterogenously
enhancing left intraconal
mass (A,D) associated
with the left optic nerve.
D
E
Thyroid Ophthalmopathy
• Most common cause of proptosis in adults.35
• Optic nerve dysfunction affects ~5% of patients.34
• Enlargement of the ocular muscles by edema and
infiltration of inflammatory cells.35
• Imaging features enlargement of the extra ocular bellies (T1
Isointense, T2 Hyperintense in acute and iso-hypointense in
chronic stages), with sparing of the myotendinous junction.
• Lateral rectus muscle is the last extra ocular muscle
affected.
Thyroid Ophthalmopathy
A
B
C
D
T1WI (A), T2WI (B), T1WI C+ (C), and Axial T1WI C+
images demonstrate enlargement of the ocular muscles with
sparing of the lateral rectus muscle and myotendinous
junction. There is slight proptosis of the left orbit.
Pseudotumour (Idiopathic Orbital Inflammation)
•Unknown etiology of orbital inflammation that mimics a tumor mass
•Histology demonstrates lymphocitic infiltration.36
•Affects adjacent structures such as the lacrimal gland, mimicing a
mass.
•Rapid onset and produce symptoms similar to thyroid ophthalmopathy
•Involve the myotendinous junction, unlike thyroid ophthalmopathy.
•Key findings include enlargement of the bellies of the extraocular
muscles including the myotendinous junction, T1 iso/hypointense, T2
hypointense, and diffuse enhancement with C+ imaging.
Pseudotumour (Idiopathic Orbital Inflammation)
A
B
C
Coronal T1WI (A), T1WI C+ (B), T2WI (C), and Axial
T2WI (D) demonstrate enhancing T1 hypointense, T2
hyperintense signal adjacent to the right superior
rectus muscle with inferior displacement of the muscle
and the optic nerve with proptosis in the patient with
eye pain and visual disturbance compatable with
orbital pseudotumor.
D
Sarcoidosis
• Multisystem granulomatous disease.
• Ocular involvement usually includes uveitis and
conjunctival granulomas.
• Orbital tissue involvement is rarely reported, varying
from lacrimal gland infiltration to optic nerve sheath
involvement.5
• Pathology reveals non-caseating granulomas that
infiltrate orbital fat.
• Laboratory values demonstrate elevated blood and
CSF ACE levels.
• Additional imaging may reveal hilar lymphadenopathy
on a chest x-ray or chest CT.
• Responds well to systemic steroids.
Sarcoidosis
A
D
B
C
E
FIGURES A-C: Multiple coronal
MR images demonstrate a T1
hypointense (A), T2 hyperintense
(B), and homogenously enhancing
mass of the R orbit (C), with
medial displacement of the optic
nerve.
FIGURES D&E: Bilateral hilar
lymphadenopathy; typical
findings of Sarcoidosis
Infantile Hemangioma
• The most common tumor in infancy and appear within
the first 6 months of life.18-20
• A proliferative phase occurs up to 10 months, followed
by a slow involution phase for up to 10 years.21-23
• Diagnosis is established clinically, however, contrast
enhanced MRI is performed to evaluate the extent of
the lesion and possible mass effect.
• Key distinguishing imaging features include bright T2
signal, lobular borders, fine internal flow voids,
extraconal location, and intense homogeneous
enhancement.24
Infantile Hemangioma
A
B
C
Multiple MR images demonstrate a
lobulated mass within the right orbit with
FLAIR hyperintense signal (B), T1
isointense signal (A,D) with intense
homogeneous enhancement (E), and
internal flow voids (C).
D
E
Venolympathic Malformation
•
Benign vascular malformations commonly affecting children and rarely
involving the orbit.
•
Unencapsulated and composed of fibrous material including endothelial
lacunae filled with blood or serious fluid.30
•
Symptoms include swelling, intraorbital hemorrhage, ocular proptosis,
blepharoptosis and cellulitis.
•
MR demonstrates vascular components, and fluid filled levels or menisci
can be seen.31
•
T1 isointense, T2 hyperintense relative to the brain with internal
septations.31
•
Venous component of a lesion will enhance, whereas the lymphatic
component will only show fine enhancement of septations. 24
Venolympathic Malformation
A
B
C
T2 MR images (A,D) demonstrates a heterogeneous
mass in the left orbit with T2 hyperintense components
and fluid-filled levels. T1 C- (B,C) and C+ (E)
demonstrate a hypointense mass with homogenous
enhancement, encasement of the optic nerve, and
proptosis of the left eye.
D
E
Conclusion
• Orbital varix is a rare – 1% of all orbital masses.
• Presents with intermittent diplopia or proptosis
during episodes of straining or prone positioning.
• Hemorrhage or thrombosis can cause an acute
onset of pain and decreased visual acuity.
• Review of this educational exhibit will enabled a
radiologist to be familiar with this entity, consider
associated complications, narrow relevant
differential diagnoses, and offer appropriate
recommendations.
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