Transcript Progressive, painless visual loss

Doctor, What is Wrong with My Eye?
Common and Uncommon Extra-ocular Lesions
A Case-Based Tutorial
eEdE-111
Jason B. Mueller, M.D.1 Lindell R. Gentry, M.D.2
Deborah L. Reede, M.D.3 Wendy R.K. Smoker, M.D.1
1University
of Iowa Hospitals and Clinics, Iowa City, IA; 2University of Wisconsin
Hospital and Clinics, Madison, WI; 3SUNY Downstate Medical Center, Brooklyn, NY
Financial Disclosure
Learning Objectives
•
Learn the most common extra-ocular lesions
of the orbit
•
Become familiar with important, but less
common orbital lesions that must also be
considered in the differential diagnosis
•
Learn the discriminating imaging features of
these entities
Introduction
Case 1
“Eyebrow mass”
Diagnosis?
1. Dacryocystocele
2. Dermoid cyst
3. Lacrimal cyst
4. Abscess
• Represent ectodermal inclusion cysts.
• Often contain primarily lipid, but may also
contain fluid and solid tissue, as in this
case
• Most common location = superolateral
orbit
• May produce mass effect on intraorbital
contents and even cause bone
remodeling
Case 2
22F with decreased vision OU
Diagnosis?
1. Perioptic meningioma
2. Optic neuritis
3. Optic nerve schwannoma
4. Optic pathway glioma
(OPG)
2/3 of all optic nerve tumors;3% of orbital tumors
OPGs : meningiomas = 4:1
Present with proptosis and decreased visual acuity
Typically slow growing; >90% before age 20
Fusiform enlargement of the optic nerve with
variable enhancement
• CSF is still visible within the ON sheath. There is
also posterior extension to the optic chiasm in this
case
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Case 2 continued
22F with decreased vision OU
Of the following, this
patient is most likely to
have:
1. Tuberous sclerosis
2. Neurofibromatosis I
3. Neurofibromatosis II
4. Sturge-Weber
Syndrome
• 10-35% of patients with optic
gliomas have NF1
• Conversely, 15% of NF1 patients
will have optic pathway gliomas
Case 3
Diagnosis?
1. Meningioma
2. Venous varix
3. Cavernous
malformation
4. Abscess
44F with painless proptosis
• Most common adult intraconal lesion
• Erroneously called “cavernous
hemangiomas”,--NOT true neoplasm
• Present with painless proptosis that
slowly progresses.
• T1=isointense ; T2=hyperintense +/hypointense fibrous pseudocapsule
• Patchy CE on arterial phase, which
homogeneously fills in on delayed
images
T1 pre-contrast
Immediate post-contrast
T2
One hour delay
Cavernous Malformation
companion case
• Phleboliths are not typical, but
helpful when present
• Cavernous malformations require
no treatment if asymptomatic
• Surgical resection is often curative if
lesion is causing visual
• disturbances or significant mass
effect
• Pseudocapsule facilitates removal
of the lesion
T2
Immediate post
Delay
Courtesy V Runge, MD
Case 4
6 month old with “eyelid swelling”
Diagnosis?:
1. Venous varix
2. Orbital cellulitis
3. Cavernous malformation
4. Capillary hemangioma
• AKA infantile hemangioma-most common
orbital vascular TUMOR of infancy
• Growth phase (1-2 years); involutional
phase (after 2 years); 50% involute by
age 5.
• Steroid therapy is very effective and
indicated for decreased vision/cosmesis.
• T1 isointense, T2 hyperintense
• Homogeneous enhancement; flow voids
may be present
Case 5
Positional proptosis
Diagnosis?
1. Venous varix
2. Orbital cellulitis
3. Cavernous
malformation
4. Capillary hemangioma
• Result from dilation of orbital veins
secondary to weakness of vessel wall
• Most common cause of spontaneous orbital
hemorrhage.
• May be occult on imaging without
provocative maneuvers. The abnormal veins
become engorged during Valsalva or prone
positioning
• Fluid-fluid levels may be present.
• MR signal is variable on depending on the
age of blood products
Supine
Prone
Case 6
Progressive, painless proptosis
Diagnosis?
1. Carotid-cavernous fistula
2. Orbital cellulitis
3. Meningioma
4. Lymphatic malformation
• Multilobulated, trans-spatial, enhancing
mass composed of abnormal, dilated
lymphatic channels
• Multiple fluid-fluid levels are characteristic
• May hemorrhage rapid increase in size
• Observation is the mainstay of treatment;
corticosteroids can be used for swelling/pain
• Surgical resection difficult; high recurrence
rate
Case 7
Chemosis, proptosis, and bruit
Diagnosis?
1. Carotid-cavernous
fistula (CCF)
2. Orbital cellulitis
3. Venous varix
4. Venolymphatic
malformation
Ans. 1 Carotid-Cavernous Fistula
• Congested, hypervascular
cavernous sinus
• Arterialized flow within the
superior ophthalmic vein
• CCFs may be direct (trauma
or ruptured cavernous ICA
aneurysm) or indirect ( AVM
or hypervascular tumor
draining into the cavernous
sinus)
• Worrisome signs of CCF
include intracranial
hemorrhage, cortical venous
shunting, and rapid visual loss
Carotid-Cavernous Fistula
Post-coiling
Arterialized superior ophthalmic vein
Most CCFs can be treated via an endovascular approach.
Case 8
Diabetic, fever, abrupt vision loss OD
Diagnosis?
1. Optic neuritis
2. Optic nerve glioma
3. Optic nerve infarction
4. Perioptic meningioma
DWI
Ans. 3 Optic Nerve Infarction
DWI
• Note Rt ON diffusion restriction to the chiasm
The nerve is also thickened and edematous
• Etiology of this patient’s symptoms was
invasive fungal sinusitis (in this case
mucormycosis), as evidenced by the extensive
paranasal sinus disease
• Invasion of the ICA and its branches with
resulting infarction is a known complication
Case 9
Exophthalmos
Diagnosis?:
1. Metastases
2. Thyroid associated
orbitopathy (TAO)
3. Pseudotumor
4. Sarcoidosis
Ans. 2 Thyroid Associated Orbitopathy
• Secondary to Grave’s disease
• Most common orbital disorder and most
common cause of exophthalmos in adults.
• Spindle-shaped enlargement and intense
enhancement of affected muscles
• Distribution of extraocular muscles affected is
specific and remembered with the mnemonic
“I’M SLO”; inferior, medial, superior, lateral
recti, and obliques.
• Notice tendon sparing which is characteristic
and a key distinguishing feature from
pseudotumor.
Case 10
Acute, painful proptosis OS
Diagnosis?:
1. Metastases
2. Thyroid associated
orbitopathy
3. Pseudotumor
4. Sarcoidosis
Ans. 3 Pseudotumor
• Nongranulomatous idiopathic inflammatory disorder. Myositic (EOM) pattern (this case) is
the most common
• Second most common orbital disorder after Grave’s disease; Acute, subacute, or chronic
• Extraocular muscles become enlarged without a specific predilection, unlike TAO
• Also unlike TAO, there is involvement of the muscle tendons
• Treated with corticosteroids, to which there is usually a good response
Case 11
31F with acute decreased vision and pain OD
Diagnosis?
1. Optic neuritis (ON)
2. Optic nerve glioma
3. Optic nerve infarction
4. Perioptic meningioma
• Optic neuritis is an idiopathic autoimmune condition
characterized by abrupt visual loss and orbital pain
• Corticosteroids accelerate initial recovery but do not alter
long-term vision recovery
• Key imaging features include optic nerve enhancement,
mild enlargement, and increased T2SI
Case 11 continued
31F with acute decreased vision and pain OD
What process is indicated
by the yellow arrow?
1. Chronic small vessel
ischemia
2. Acute infarction
3. Inactive demyelination
4. Active demyelination
• ON is highly associated with multiple sclerosis (MS).
• >50% of patients with ON develop MS, especially
women
• MS plaques are demonstrated on the T2WI
• Contrast enhancement demonstrated on the lower
image confirms that active demyelination is occurring
Case 12
Progressive, painless visual loss
Diagnosis?
1. Optic neuritis
2. Optic nerve glioma
3. Optic nerve infarction
4. Perioptic meningioma
Ans. 4 Perioptic Meningioma
• Typically present with slowly progressive, painless
visual loss
• Marked T2 hypointensity, a characteristic feature
• “Tram track” and circumferential intense,
homogeneous enhancement, also key findings. Note
normal optic nerve
• Calcifications are present in up to 50% of cases often
with “tram track” morphology as in bottom case
(different patient)
Case 13
Fever, proptosis
Diagnosis?
1. Orbital cellulitis
2. Uncomplicated
sinusitis
3. Mucocele
4. Subperiosteal
abscess
• A complication of acute sinusitis, particularly ethmoid
• Note opacification of maxillary sinus and ethmoid air cells, as well as dehiscence of
the lamina papyracea (often not present)
• Abscess is seen as a rim-enhancing fluid collection abutting the medial orbital wall
displacing intraorbital contents. Note medial rectus myositis
• Treatment consists of IV antibiotics and often surgical drainage, especially with
decreasing vision
Case 14
Former smoker, decreased “eye movement” OD
Diagnosis?
1. Pseudotumor
2. Thyroid associated
orbitopathy
3. Metastasis
4. Schwannoma
• Metastatic disease is favored in this case (note
history!)
• Asymmetric involvement and lack of spindleshaped EOM makes TAO less likely. Pseudotumor
is typically accompanied by infiltration of orbital fat.
A schwannoma should not be centered in an EOM.
• Note marked enlargement, enhancement, and
hyperintense T2 signal of the inferior rectus muscle
Case 15
Painless proptosis
Diagnosis?:
1. Optic neuritis
2. Schwannoma
3. Optic glioma
4. Perioptic meningioma
Ans. 2 Schwannoma (V1)
• Relatively rare (1% of orbital tumors)
• Benign and slow-growing painless proptosis
• May be isolated or associated with NFII. Arise from
cranial nerves (excluding the optic nerve)
• Fusiform to oval shape, iso to hypointense T1 signal
and hyperintense T2 signal; they avidly enhance
uniformly
• Often displace the optic nerve (here inferomedially).
• Note extension through the superior orbital fissure
into the cavernous sinus
Case 16
Painless facial swelling; Diplopia on left lateral gaze
Diagnosis?
1. Paget disease
2. Ossifying fibroma
3. Intraosseous
meningioma
4. Fibrous dysplasia
Ans. 4 Fibrous dysplasia (FD)
• Results when normal trabecular
bone is replaced by fibrous tissue
and immature, woven bone.
• CT imaging findings include
expanded bone with intact cortex
and ground glass CT attenuation
• Classic MR features include
hypointense T1 and T2SI and
variable contrast enhancement.
• FD can be observed until there is
functional impairment. Treatment
consists of surgical excision, not
usually attempted until after
puberty
• Rarely, FD can undergo
sarcomatous degeneration
Case 17
History withheld
Diagnosis?
1. Paget disease
2. Ossifying fibroma
3. Osteoma
4. Meningioma
Ans. 3 Orbital Roof Osteoma
Mature, benign, slow-growing, osseous tumors.
Often asymptomatic and discovered incidentally.
Excision generally only required if mass effect
May be “ivory” and appear as dense, compacted
cortical bone, or “mature” with central marrow
• This is an ivory osteoma: CT-marked uniform
hyperdensity; hypointensity on T1 and T2WIs.
Gardner syndrome must be excluded if a
• Lesion displaces optic nerve inferolaterally
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•
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patient is found to have multiple osteomas!
Case 18
Periorbital pain and swelling
Most likely diagnosis?:
1. Langerhans cell histiocytosis (LCH)
2. Paget disease
3. Intraosseous meningioma
4. Fibrous dysplasia
Ans. 1 Langerhans Cell Histiocytosis
(eosinophilic granuloma)
• Rare disorder characterized by the abnormal proliferation of Langerhans cells
• Term eosinophilic granuloma is used for unifocal disease, as in this case. Two additional
syndromes, Hand-Schuller-Christian and Letterer-Siwe disease, refer to multifocal
involvement within a single organ system and widespread multi-organ system disease,
respectively
• Imaging includes “punched out” lytic lesions with associated homogeneous soft tissue
mass, often creating a “beveled edge” appearance
• Orbital LCH is a risk factor for developing diabetes insipidus
Case 19
“Raccoon eyes”, child with history of abdominal mass
Diagnosis?
1. Langerhans cell histiocytosis
2. Neuroblastoma mets
3. Intraosseous meningioma
4. Fibrous dysplasia
Ans. 2 Neuroblastoma Metastases
• Frequently metastasizes to the skull. Although rare,
bilateral metastases may produce “raccoon eyes” on
physical exam
• Result in aggressive bony destruction. Extradural,
intracalvarial extension can occur, as in this case.
• Avid contrast enhancement. There may also be
significant mass effect on intraorbital contents, also
present in this case
• Abdominal CT shows the primary tumor of
heterogeneous density with calcification, typical of
neuroblastoma
Summary
•
Extra-ocular orbital lesions may present a diagnostic
challenge to the radiologist, especially when atypical in
appearance
•
Careful inspection for characteristic distinguishing features
will often lead to the correct diagnosis or significantly narrow
the differential
•
Consider both common and uncommon entities when faced
with these cases
References
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Bahn RS. Graves' ophthalmopathy. N Engl J Med. 362(8):726-38, 2010.
Shams PN et al. Optic neuritis: a review. Int MS J. 16(3):82-9, 2009.
Smoker WR et al. Vascular lesions of the orbit: more than meets the eye. Radiographics.
28(1):185-204; quiz 325, 2008.
AnsariSA, Mafee MF. Orbital cavernous hemangioma: role of imaging. Neuroimaging Clin N Am.
2005; 15: 137–158.
Ruggieri M et al. Earliest clinical manifestations and natural history of neurofibromatosis type 2
(NF2) in childhood: a study of 24 patients. Neuropediatrics. 36(1):21-34, 2005.
Erly WK et al. Orbital histiocytosis X. AJNR Am J Neuroradiol. 16(6):1258-61, 1995.
Alexander AA et al. Paranasal sinus osteomas and Gardner's syndrome. Ann Otol Rhinol
Laryngol. 116(9):658-62, 2007.
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