Orbital DDX

Orbital Differential Diagnoses
Michelle A. Michel, M. D.
Professor of Radiology and Otolaryngology Medical College of Wisconsin, Milwaukee, WI
Illustrations courtesy Amirsys, Inc.

Kansas City March 2010

Objectives
To review basic orbital anatomy To discuss imaging modalities for evaluating the orbit To describe useful approaches for distinguishing orbital lesions To review the more common orbital differential diagnoses

Normal Anatomy Bony Orbit
Bones
6 bones contribute
Foramina/Fissures
Optic canal
CN2, ophthalmic artery
GWS
LWS
Superior orbital fissure

CN3, 4, V1, 6, SOV
Inferior orbital fissure

V2, PP ganglion, IOV, pterygoid plexus

Normal Anatomy - Globe
Layers
Outer cornea (anterior)/sclera (posterior) Uvea (choroid, iris, ciliary body) Retina
Anterior segment
Anterior and posterior chambers
Posterior segment
Vitreous chamber

Normal Anatomy Optic Nerve & Sheath Complex CN2 is a CNS tract Segments
Orbital Canalicular Intracranial
Sheath
Meningeal layers Subarachnoid space continuous with suprasellar cistern

Normal Anatomy Anterior Structures & Lacrimal Apparatus
Anterior structures
Orbital septum
Fascia from periosteal margin Inserts into lid fascia at tarsal margins
Tarsal plates
Lacrimal apparatus
Gland Puncta > canaliculi > sac > NLD > inf meatus
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Imaging Techniques CT, MR, or US?
US
Evaluation of intraocular lesions Non-invasive, readily available
CT & MR
Complementary Both indicated for complex lesions

Imaging Techniques CT
Excellent for bony orbit Good intrinsic contrast (fat, bone, air, soft tissues) Detects Ca++ Rapid > motion-free Avoids pediatric sedation

Imaging Techniques - MR
Optimal soft tissue contrast
Globe Optic nerve Lacrimal gland Intracranial disease
Improved image quality (faster gradients, surface coils, fat-saturation)


Approach to Orbital Lesions
Diverse pathology Similar appearances
Pseudotumor and lymphoproliferative lesions on many DDxs
Limited patterns of disease and key DDxs Helpful clinical info

Age Duration of symptoms Presence of pain
Rhabdomyosarcoma
Cavernous Hemangioma
Courtesy Deborah Shatzkes MD

Imaging Features
Solid vs. cystic Fluid, fat, blood, soft tissue, calcification Well-defined vs. infiltrative Homogeneous vs. inhomogeneous Bony remodeling vs. destruction Degree and homogeneity of enhancement Presence or absence of flow voids

Approach to Orbital Lesions Age
Child
Retinoblastoma ROP PHPV Coat disease Rhabdomyosarcoma Histiocytosis Metastasis
Neuroblastoma, sarcomas
Adult

Pseudotumor Thyroid orbitopathy Uveal melanoma Metastasis

Lung, breast, melanoma, renal
Leukemia Fibro-osseous lesion Expansile SN process
Multiple myeloma Meningioma Lymphoma Fibro-osseous lesion Expansile SN process

Approach to Orbital Lesions Symptoms
Painful, rapid
Infection, inflammation
Painless, rapid
Malignancy, vascular
Painless, slow/stable
Benign neoplasm, congenital, indolent SN process (polyposis, mucocele), deposition disease
Courtesy C. Douglas Phillips MD

Anatomic Approach
Intraocular vs. transcleral Optic nerve vs. nerve-sheath complex Intraconal vs. conal vs. extraconal Lacrimal gland: unilateral vs. bilateral Isolated vs. multispatial vs. trans-spatial Primary lesion vs. secondary spread

Intraocular lesion Ocular calcification Optic nerve-sheath complex lesion Intraconal lesion Extraconal lesion Extraocular muscle enlargement Ill-defined/infiltrating orbital lesion Lacrimal gland lesion

Intraocular Lesion
Hemorrhage Retinal detachment Retinoblastoma PHPV, ROP, Coats Choroidal osteoma Uveal melanoma Metastasis

Intraocular Lesion Uveal Melanoma
Most common primary intraocular tumor in adults 5% of melanomas Clinical signs and symptoms
Blurred vision or loss of vision Visual field deficit or floaters Asymptomatic
Retinal detachment is frequently present


Intraocular Lesion Uveal Melanoma
Sites of origin
Anterior uvea (iris) Posterior uvea (choroid & ciliary body)
Signal depends on % melanin

T1 hyperintensity T2 hypointensity Diffuse contrast enhancement
Signal of subretinal exudates variable


Intraocular Lesion - Metastases
Choroid most frequent site
Temporal side of macula
Most asymptomatic Breast and lung most common

Prostate, renal, thyroid, GI
Unknown primary in 18% Increasing in frequency


Ocular Calcification Drusen Arcus senilis Phthisis bulbi Retinoblastoma Choroidal osteoma

Ocular Calcification - Retinoblastoma
Most common ocular tumor of childhood 25-30% bilateral
Trilateral with pineal neuroblastic tumor
Ca++ in 90-95% Infiltrating form

Rare, older children, no Ca++
Courtesy Richard Wiggins MD
Intraocular lesions have excellent prognosis Spread beyond globe

10-15%
Courtesy Gregory Katz MD Courtesy Richard Wiggins MD


Ocular Calcification Choroidal Osteoma
Benign ossifying tumor Usually in young females Located near optic disc Calcified plaque on CT +/- retinal detachment
Courtesy Mauricio Castillo MD

Optic Nerve-Sheath Complex Lesion
Optic neuritis Perioptic meningioma Optic glioma Pseudotumor (perineuritis) Sarcoidosis Lymphoproliferative lesion Metastasis

Optic Nerve-Sheath Complex Lesion Optic Neuritis
70% unilateral Intraorbital segment most common > 50% of ON patients have MS > 70% of MS patients have ON Other causes: viral, CVD, sarcoidosis, XRT Nerve enhancement with minimal enlargement

Optic Nerve-Sheath Complex Lesion Perioptic Meningioma
5% of 1 orbital tumors
90% secondarily involve orbit
Painless, progressive vision loss F:M 2-4:1 Tram-track appearance Intense enhancement Ca++ (< 50%) & perioptic cysts

Optic Nerve-Sheath Complex Lesion Optic Glioma
Childhood, benign, 3040% with NF1 Adult, typically malignant Fusiform enlargement of nerve and chiasm Kinking of nerve Variable enhancement

Intraconal Lesion
Cavernous hemangioma Lymphatic malformation Venous varix Schwannoma Pseudotumor (tumefactive) Lymphoproliferative lesion

Intraconal Lesion Cavernous Hemangioma Venous hamartomatous malformation with endothelial-lined cavernous spaces 80% intraconal
Extraconal, intramuscular, intraosseous variants
Round or ovoid, welldefined, T2, enhancement initially patchy then fills in


Intraconal Lesion Lymphatic Malformation
Orbital lymphaticvenous malformation (OLVM) Hamartomatous malformation Present in 1st decade Isolated from systemic drainage Lobulated, cystic regions, fluid-fluid levels, rim enhancement
Courtesy Deborah Shatzkes, MD
Courtesy Mauricio Castillo MD

Intraconal Lesion Venous Varix
Distensible low flow venous malformation
with Valsalva
Usually retrobulbar & intraconal

Can be anywhere
Tubular, tortuous Phleboliths, thrombus Enhance intensely


Extraconal Lesion
Postseptal infection Dermoid Rhabdomyosarcoma Pseudotumor Lymphoproliferative lesion Meningioma Orbital bone lesion Sinonasal lesion

Extraconal Lesion Postseptal Infection
Orbital complications most common in peds Spread along valveless ethmoidal veins Can progress to CST or other intracranial complications Surgical intervention usually required
Endoscopic ethmoidectomy

Extraconal Lesion Dermoid
Cystic mass with fat, fluid, mixed contents Childhood-teenage years Tethered to periosteum at sutures
Frontozygomatic (70%) or frontolacrimal
Osseous remodeling (80%)


Extraconal Lesion Extrinsic Lesions
Meningioma
Secondary spread Primary intraorbital
Fibro-osseous lesions
Surrounding bone or sinuses
Sinonasal lesions Metastases

Breast, EG

Extraocular Muscle Enlargement
Thyroid orbitopathy Pseudotumor Lymphoproliferative lesion Metastases Trauma Cellulitis Sarcoidosis Rhabdomyosarcoma
Carcinoid Metastases
Courtesy Laurie Loevner, MD

Extraocular Muscle Enlargement Thyroid Orbitopathy
Graves disease, endocrine ophthalmopathy Autoimmune, lymphocytemediated inflamm of EOMs, fat, connective tissue Most common cause of adult proptosis in myasthenia gravis, Addison disease, pernicious anemia Spares tendinous insertions

Extraocular Muscle Enlargement - Pseudotumor
Non-specific inflammation involving any area of the orbit Pain, edema, inflammation 3rd most common ophthalmologic disorder Myositic most common pattern Bilateral in 25% Tolosa-Hunt intracranial variant Diagnosis of exclusion; steroid responsive

Pseudotumor The Great Mimic #1
Myositic
Lacrimal
Anterior
Tolosa-Hunt

Extraocular Muscle Enlargement Lymphoproliferative Lesion
60-90% are NHLs (monoclonal); 10-40% reactive or atypical lymphoid hyperplasia (polyclonal) Lacrimal predilection; muscle involvement mimics thyroid orbitopathy Pliable, homogeneous, enhancing mass Excellent XRT response

Lymphoproliferative Lesion The Great Mimic #2
Intraconal/myositic
Focal anterior intraconal mass
Lacrimal gland
Myositic/ suprasellar
Neuro/ENT at the Beach Kansas City March 2010 January 2010

Ill-Defined/Infiltrative Orbital Lesion
Cellulitis Infantile hemangioma Lymphatic malformation Plexiform neurofibroma Rhabdomyosarcoma Pseudotumor Lymphoproliferative disease Metastatic disease

Ill-Defined/Infiltrative Orbital Lesion Infantile Hemangioma
Benign, unencapsulated endothelial cell neoplasm 50% in H&N Most common benign orbital tumor of infancy F:M 2-3:1 Growth phase (1-2 years) then involutional phase Lobulated, irregular, T2 signal, intensely enhancing
Courtesy H. C. Davidson MD

Ill-Defined/Infiltrative Orbital Lesion Plexiform NF
PNF pathognomonic of NF1 Unencapsulated, infiltrative mass Can involve multiple orbital structures Bag of worms texture on palpation MR to evaluate for intracranial extension

Ill-Defined/Infiltrative Orbital Lesion - Metastases
To retrobulbar soft tissues, globe (choroid), bony orbit
Rarely to optic nerve
Breast, lung, GI, GU, neuroblastoma Scirrhous breast carcinoma > infiltrating lesion with enophthalmos

Lacrimal Gland Lesion
Key questionIs the lesion unilateral or bilateral?
Unilateral

Bilateral
Sarcoidosis Sjgren syndrome Pseudotumor Lymphoproliferative lesion
Dacryoadenitis Pseudotumor Benign mixed tumor Adenoid cystic carcinoma Lymphoproliferative lesion Sarcoidosis

Lacrimal Gland Lesion Salivary Neoplasms
Benign Mixed Tumor
Pleomorphic adenoma 50% of all orbital neoplasms Painless inferomedial globe displacement Non-specific imaging; similar to other locations
Adenoid Cystic Ca
Most common malignant lacrimal tumor Pain and paresthesias (PNTS) Classically invasive, but can be well-circumscribed Look for bone destruction Near 100% local recurrence; 40% 5-yr survival

Lacrimal Gland Lesion - Sarcoidosis
Non-caseating granulomatous inflammation of the orbit 20-25% of sarcoidosis pts have ophthalmic disease 3rd 5th decades 10X in African descent Mass-like lacrimal gland infiltration
Can involve any intraorbital structure
Dedicated imaging of the brain recommended

Courtesy C. Douglas Phillips, MD Courtesy H. C. Davidson, MD

Summary & Key Points
CT and MRI are complementary imaging modalities; both may be needed for complex orbital disease Orbital pathology is diverse Pathologies may have similar imaging appearances Limited number of differential diagnoses Clinical information and recognition of characteristic imaging features of the common lesions in each differential are key to making the correct diagnosis!
Thank You!

Orbital DDX

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Orbital DDX

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Orbital Differential Diagnoses

Illustrations courtesy Amirsys, Inc.

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Superior orbital fissure

Inferior orbital fissure

Orbital Differential Diagnoses

Kansas City March 2010

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Improved image quality (faster gradients, surface coils, fat-saturation)

Orbital Differential Diagnoses

Limited patterns of disease and key DDxs Helpful clinical info

Kansas City March 2010

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Pseudotumor Thyroid orbitopathy Uveal melanoma Metastasis

Leukemia Fibro-osseous lesion Expansile SN process

Multiple myeloma Meningioma Lymphoma Fibro-osseous lesion Expansile SN process

Kansas City March 2010

Orbital Differential Diagnoses

Courtesy C. Douglas Phillips MD

Kansas City March 2010

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Kansas City March 2010

Orbital Differential Diagnoses

Retinal detachment is frequently present

Orbital Differential Diagnoses

Signal depends on % melanin

Signal of subretinal exudates variable

Orbital Differential Diagnoses

Most asymptomatic Breast and lung most common

Unknown primary in 18% Increasing in frequency

Orbital Differential Diagnoses

Kansas City March 2010

Orbital Differential Diagnoses

Ca++ in 90-95% Infiltrating form

Courtesy Richard Wiggins MD

Intraocular lesions have excellent prognosis Spread beyond globe

Courtesy Gregory Katz MD Courtesy Richard Wiggins MD

Kansas City March 2010

Orbital Differential Diagnoses

Courtesy Mauricio Castillo MD

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Kansas City March 2010

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Orbital Differential Diagnoses

Round or ovoid, welldefined, T2, enhancement initially patchy then fills in

Orbital Differential Diagnoses

Courtesy Deborah Shatzkes, MD

Courtesy Mauricio Castillo MD

Kansas City March 2010

Orbital Differential Diagnoses

Usually retrobulbar & intraconal