Orbital Ddx

7/31/2019 Orbital Ddx

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Kansas City March 2010

Orbital Differential Diagnoses

Michelle A. Michel, M. D.Professor of Radiology and Otolaryngology

Medical College of Wisconsin, Milwaukee, WI

Illustrations courtesy Amirsys, Inc.


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Objectives To review basic orbital

anatomy

To discuss imagingmodalities for evaluating theorbit

To describe usefulapproaches for distinguishing

orbital lesions

To review the more commonorbital differential diagnoses


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Orbital Differential DiagnosesNormal Anatomy Bony Orbit

Bones

6 bones contribute

Foramina/Fissures Optic canal

CN2, ophthalmic artery

Superior orbital fissure

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

V2, PP ganglion, IOV,pterygoid plexus

GWS LWS


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Orbital Differential DiagnosesNormal Anatomy - Globe

Layers

Outer cornea (anterior)/sclera(posterior)

Uvea (choroid, iris, ciliary body) Retina

Anterior segment

Anterior and posterior

chambers Posterior segment

Vitreous chamber


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Orbital Differential DiagnosesNormal Anatomy Optic Nerve & Sheath Complex

CN2 is a CNS tract

Segments

Orbital

Canalicular Intracranial

Sheath

Meningeal layers

Subarachnoid spacecontinuous withsuprasellar cistern


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Orbital Differential DiagnosesNormal Anatomy Anterior Structures &

Lacrimal Apparatus

Anterior structures

Orbital septum

Fascia from periosteal margin

Inserts into lid fascia at tarsalmargins

Tarsal plates

Lacrimal apparatus

Gland

Puncta > canaliculi > sac >NLD > inf meatus www.fotosearch.com

*


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Orbital Differential DiagnosesImaging Techniques CT, MR, or US?

US

Evaluation of intraocularlesions

Non-invasive, readilyavailable

CT & MR

Complementary Both indicated for complex

lesions


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Imaging Techniques CT

Excellent for bony orbit

Good intrinsic contrast(fat, bone, air, softtissues)

Detects Ca++

Rapid > motion-free Avoids pediatric sedation


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Imaging Techniques - MR Optimal soft tissue

contrast

Globe

Optic nerve

Lacrimal gland

Intracranial disease

Improved image quality(faster gradients, surfacecoils, fat-saturation)


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Orbital Differential DiagnosesApproach to Orbital Lesions

Diverse pathology

Similar appearances Pseudotumor and

lymphoproliferativelesions on many DDxs

Limited patterns ofdisease and key DDxs

Helpful clinical info Age

Duration of symptoms

Presence of pain

Rhabdomyosarcoma

Cavernous Hemangioma

Courtesy Deborah Shatzkes MD


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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


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Orbital Differential DiagnosesApproach to Orbital Lesions Age

Child Retinoblastoma

ROP

PHPV

Coat disease Rhabdomyosarcoma

Histiocytosis

Metastasis

Neuroblastoma, sarcomas

Leukemia

Fibro-osseous lesion

Expansile SN process

Adult Pseudotumor

Thyroid orbitopathy

Uveal melanoma

Metastasis Lung, breast, melanoma,

renal

Multiple myeloma

Meningioma

Lymphoma Fibro-osseous lesion

Expansile SN process


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Orbital Differential DiagnosesApproach to Orbital Lesions Symptoms

Painful, rapid

Infection, inflammation

Painless, rapid Malignancy, vascular

Painless, slow/stable

Benign neoplasm,

congenital, indolent SNprocess (polyposis,mucocele), depositiondisease Courtesy C. Douglas Phillips MD


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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


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Intraocular lesion Ocular calcification Optic nerve-sheath

complex lesion

Intraconal lesion Extraconal lesion Extraocular muscle

enlargement

Ill-defined/infiltratingorbital lesion

Lacrimal gland lesion


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Orbital Differential DiagnosesIntraocular Lesion

Hemorrhage

Retinal detachment

Retinoblastoma PHPV, ROP, Coats

Choroidal osteoma

Uveal melanoma

Metastasis


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Orbital Differential DiagnosesIntraocular Lesion Uveal Melanoma

Most common primaryintraocular tumor in adults

5% of melanomas

Clinical signs andsymptoms Blurred vision or loss of vision

Visual field deficit or floaters

Asymptomatic

Retinal detachment isfrequently present


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Sites of origin Anterior uvea (iris)

Posterior uvea (choroid &ciliary body)

Signal depends on %melanin T1 hyperintensity

T2 hypointensity

Diffuse contrast enhancement

Signal of subretinalexudates variable

Orbital Differential DiagnosesIntraocular Lesion Uveal Melanoma


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Orbital Differential DiagnosesIntraocular 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


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Orbital Differential DiagnosesOcular Calcification

Drusen

Arcus senilis Phthisis bulbi

Retinoblastoma

Choroidal osteoma


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Courtesy Richard Wiggins MD


Orbital Differential DiagnosesOcular Calcification - Retinoblastoma

Most common ocular tumorof childhood

25-30% bilateral Trilateral with pineal

neuroblastic tumor Ca++ in 90-95%

Infiltrating form Rare, older children, no Ca++

Intraocular lesions haveexcellent prognosis

Spread beyond globe 10-15%

Courtesy Richard Wiggins MDCourtesy Gregory Katz MD


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Orbital Differential DiagnosesOcular Calcification Choroidal Osteoma

Benign ossifying tumor

Usually in youngfemales

Located near optic disc

Calcified plaque on CT +/- retinal detachment

Courtesy Mauricio Castillo MD


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Orbital Differential DiagnosesOptic Nerve-Sheath Complex Lesion

Optic neuritis

Perioptic meningioma

Optic glioma Pseudotumor

(perineuritis)

Sarcoidosis

Lymphoproliferativelesion

Metastasis


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Orbital Differential DiagnosesOptic Nerve-Sheath Complex Lesion Optic Neuritis

70% unilateral

Intraorbital segment mostcommon

> 50% of ON patients haveMS

> 70% of MS patients haveON

Other causes: viral, CVD,sarcoidosis, XRT

Nerve enhancement withminimal enlargement


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Orbital Differential DiagnosesOptic Nerve-Sheath Complex Lesion

Perioptic Meningioma

5% of 1 orbital tumors

90% secondarily involveorbit

Painless, progressivevision loss

F:M 2-4:1

Tram-track appearance Intense enhancement

Ca++ (< 50%) & perioptic

cysts


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Orbital Differential DiagnosesOptic Nerve-Sheath Complex Lesion Optic Glioma

Childhood, benign, 30-40% with NF1

Adult, typically malignant

Fusiform enlargement ofnerve and chiasm

Kinking of nerve Variable enhancement


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Orbital Differential DiagnosesIntraconal Lesion

Cavernous hemangioma

Lymphatic malformation

Venous varix Schwannoma

Pseudotumor

(tumefactive) Lymphoproliferative

lesion


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Orbital Differential DiagnosesIntraconal Lesion Cavernous Hemangioma

Venous hamartomatousmalformation withendothelial-linedcavernous spaces

80% intraconal

Extraconal, intramuscular,intraosseous variants

Round or ovoid, well-defined, T2,enhancement initiallypatchy then fills in


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Orbital Differential DiagnosesIntraconal Lesion Lymphatic Malformation

Orbital lymphatic-venous malformation(OLVM)

Hamartomatousmalformation

Present in 1st decade

Isolated from systemic

drainage Lobulated, cystic

regions, fluid-fluidlevels, rim enhancement Courtesy Mauricio Castillo MD

Courtesy Deborah Shatzkes, MD


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Orbital Differential DiagnosesIntraconal Lesion Venous Varix

Distensible low flowvenous malformation

with Valsalva

Usually retrobulbar &intraconal

Can be anywhere

Tubular, tortuous Phleboliths, thrombus

Enhance intensely


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Orbital Differential DiagnosesExtraconal Lesion

Postseptal infection

Dermoid

Rhabdomyosarcoma

Pseudotumor

Lymphoproliferative lesion

Meningioma

Orbital bone lesion

Sinonasal lesion


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Orbital Differential DiagnosesExtraconal Lesion Postseptal Infection

Orbital complicationsmost common in peds

Spread along valveless

ethmoidal veins Can progress to CST or

other intracranialcomplications

Surgical interventionusually required Endoscopic

ethmoidectomy

O bi l Diff i l Di


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Orbital Differential DiagnosesExtraconal Lesion Dermoid

Cystic mass with fat, fluid,mixed contents

Childhood-teenage years

Tethered to periosteum atsutures

Frontozygomatic (70%) or

frontolacrimal Osseous remodeling

(80%)


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Orbital Differential DiagnosesExtraconal Lesion Extrinsic Lesions

Meningioma

Secondary spread

Primary intraorbital

Fibro-osseous lesions

Surrounding bone orsinuses

Sinonasal lesions Metastases

Breast, EG


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Orbital Differential DiagnosesExtraocular Muscle Enlargement

Thyroid orbitopathy

Pseudotumor

Lymphoproliferative

lesion Metastases

Trauma

Cellulitis Sarcoidosis

Rhabdomyosarcoma Carcinoid MetastasesCourtesy Laurie Loevner, MD


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Orbital Differential DiagnosesExtraocular Muscle Enlargement Thyroid Orbitopathy

Graves disease, endocrineophthalmopathy

Autoimmune, lymphocyte-

mediated inflamm of EOMs,fat, connective tissue

Most common cause of adultproptosis

in myasthenia gravis,Addison disease, perniciousanemia

Spares tendinous insertions


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Orbital Differential DiagnosesExtraocular Muscle Enlargement - Pseudotumor

Non-specific inflammationinvolving any area of the orbit

Pain, edema, inflammation

3rd most commonophthalmologic disorder

Myositic most common pattern

Bilateral in 25%

Tolosa-Hunt intracranial variant Diagnosis of exclusion; steroid

responsive


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Orbital Differential DiagnosesPseudotumor The Great Mimic #1

Myositic

Anterior

Lacrimal

Tolosa-Hunt




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Orbital Differential DiagnosesExtraocular Muscle Enlargement

Lymphoproliferative Lesion

60-90% are NHLs(monoclonal); 10-40%reactive or atypicallymphoid hyperplasia

(polyclonal) Lacrimal predilection;

muscle involvementmimics thyroid

orbitopathy Pliable, homogeneous,enhancing mass

Excellent XRT response


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Orbital Differential DiagnosesLymphoproliferative Lesion The Great Mimic #2

Intraconal/myositic Focal anterior

intraconal mass

Lacrimal gland Myositic/suprasellar

Neuro/ENT at the Beach

January 2010Kansas City March 2010


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Orbital Differential DiagnosesIll-Defined/Infiltrative Orbital Lesion

Cellulitis

Infantile hemangioma

Lymphatic malformation

Plexiform neurofibroma

Rhabdomyosarcoma

Pseudotumor

Lymphoproliferativedisease

Metastatic disease


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Orbital Differential DiagnosesIll-Defined/Infiltrative Orbital Lesion

Infantile Hemangioma

Benign, unencapsulatedendothelial cell neoplasm

50% in H&N

Most common benignorbital 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



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Orbital Differential DiagnosesIll-Defined/Infiltrative Orbital Lesion Plexiform NF

PNF pathognomonic ofNF1

Unencapsulated,

infiltrative mass Can involve multiple

orbital structures

Bag of worms texture

on palpation MR to evaluate for

intracranial extension

O bi l Diff i l Di


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Orbital Differential DiagnosesIll-Defined/Infiltrative Orbital Lesion - Metastases

To retrobulbar softtissues, globe(choroid), bony orbit Rarely to optic nerve

Breast, lung, GI, GU,neuroblastoma

Scirrhous breast

carcinoma >infiltrating lesion withenophthalmos


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Orbital Differential DiagnosesLacrimal Gland Lesion

Unilateral

Dacryoadenitis

Pseudotumor Benign mixed tumor

Adenoid cystic carcinoma

Lymphoproliferative lesion

Sarcoidosis

Bilateral

Sarcoidosis

Sjgren syndrome Pseudotumor

Lymphoproliferativelesion

Key questionIs the lesion unilateral or bilateral?


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Orbital Differential DiagnosesLacrimal Gland Lesion Salivary Neoplasms

Benign Mixed Tumor Pleomorphic adenoma 50% of all orbital neoplasms Painless inferomedial globe

displacement

Non-specific imaging; similarto 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


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Orbital Differential DiagnosesLacrimal Gland Lesion - Sarcoidosis

Non-caseating granulomatousinflammation of the orbit

20-25% of sarcoidosis ptshave ophthalmic disease

3rd 5th decades

10X in African descent

Mass-like lacrimal glandinfiltration

Can involve any intraorbitalstructure

Dedicated imaging of the brainrecommended

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

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Orbital Differential DiagnosesSummary & Key Points

CT and MRI are complementaryimaging modalities; both maybe needed for complex orbitaldisease

Orbital pathology is diverse

Pathologies may have similarimaging appearances Limited number of differential

diagnoses Clinical information and

recognition of characteristicimaging features of thecommon lesions in eachdifferential are key to makingthe correct diagnosis!


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Thank You!

Orbital Ddx

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