orbital ddx
TRANSCRIPT
-
7/31/2019 Orbital Ddx
1/49
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.
-
7/31/2019 Orbital Ddx
2/49
Kansas City March 2010
Orbital Differential Diagnoses
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
-
7/31/2019 Orbital Ddx
3/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
4/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
5/49
Kansas City March 2010
Orbital Differential DiagnosesNormal Anatomy Optic Nerve & Sheath Complex
CN2 is a CNS tract
Segments
Orbital
Canalicular Intracranial
Sheath
Meningeal layers
Subarachnoid spacecontinuous withsuprasellar cistern
-
7/31/2019 Orbital Ddx
6/49
Kansas City March 2010
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
*
-
7/31/2019 Orbital Ddx
7/49
Kansas City March 2010
Orbital Differential DiagnosesImaging Techniques CT, MR, or US?
US
Evaluation of intraocularlesions
Non-invasive, readilyavailable
CT & MR
Complementary Both indicated for complex
lesions
-
7/31/2019 Orbital Ddx
8/49
Kansas City March 2010
Orbital Differential Diagnoses
Imaging Techniques CT
Excellent for bony orbit
Good intrinsic contrast(fat, bone, air, softtissues)
Detects Ca++
Rapid > motion-free Avoids pediatric sedation
-
7/31/2019 Orbital Ddx
9/49
Kansas City March 2010
Orbital Differential Diagnoses
Imaging Techniques - MR Optimal soft tissue
contrast
Globe
Optic nerve
Lacrimal gland
Intracranial disease
Improved image quality(faster gradients, surfacecoils, fat-saturation)
-
7/31/2019 Orbital Ddx
10/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
11/49
Kansas City March 2010
Orbital Differential DiagnosesApproach to Orbital Lesions
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
-
7/31/2019 Orbital Ddx
12/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
13/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
14/49
Kansas City March 2010
Orbital Differential DiagnosesApproach to Orbital Lesions
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
-
7/31/2019 Orbital Ddx
15/49
Kansas City March 2010
Orbital Differential Diagnoses
Intraocular lesion Ocular calcification Optic nerve-sheath
complex lesion
Intraconal lesion Extraconal lesion Extraocular muscle
enlargement
Ill-defined/infiltratingorbital lesion
Lacrimal gland lesion
-
7/31/2019 Orbital Ddx
16/49
Kansas City March 2010
Orbital Differential DiagnosesIntraocular Lesion
Hemorrhage
Retinal detachment
Retinoblastoma PHPV, ROP, Coats
Choroidal osteoma
Uveal melanoma
Metastasis
-
7/31/2019 Orbital Ddx
17/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
18/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
19/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
20/49
Kansas City March 2010
Orbital Differential DiagnosesOcular Calcification
Drusen
Arcus senilis Phthisis bulbi
Retinoblastoma
Choroidal osteoma
-
7/31/2019 Orbital Ddx
21/49
Kansas City March 2010
Courtesy Richard Wiggins MD
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
-
7/31/2019 Orbital Ddx
22/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
23/49
Kansas City March 2010
Orbital Differential DiagnosesOptic Nerve-Sheath Complex Lesion
Optic neuritis
Perioptic meningioma
Optic glioma Pseudotumor
(perineuritis)
Sarcoidosis
Lymphoproliferativelesion
Metastasis
-
7/31/2019 Orbital Ddx
24/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
25/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
26/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
27/49
Kansas City March 2010
Orbital Differential DiagnosesIntraconal Lesion
Cavernous hemangioma
Lymphatic malformation
Venous varix Schwannoma
Pseudotumor
(tumefactive) Lymphoproliferative
lesion
-
7/31/2019 Orbital Ddx
28/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
29/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
30/49
Kansas City March 2010
Orbital Differential DiagnosesIntraconal Lesion Venous Varix
Distensible low flowvenous malformation
with Valsalva
Usually retrobulbar &intraconal
Can be anywhere
Tubular, tortuous Phleboliths, thrombus
Enhance intensely
-
7/31/2019 Orbital Ddx
31/49
Kansas City March 2010
Orbital Differential DiagnosesExtraconal Lesion
Postseptal infection
Dermoid
Rhabdomyosarcoma
Pseudotumor
Lymphoproliferative lesion
Meningioma
Orbital bone lesion
Sinonasal lesion
-
7/31/2019 Orbital Ddx
32/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
33/49
Kansas City March 2010
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%)
-
7/31/2019 Orbital Ddx
34/49
Kansas City March 2010
Orbital Differential DiagnosesExtraconal Lesion Extrinsic Lesions
Meningioma
Secondary spread
Primary intraorbital
Fibro-osseous lesions
Surrounding bone orsinuses
Sinonasal lesions Metastases
Breast, EG
-
7/31/2019 Orbital Ddx
35/49
Kansas City March 2010
Orbital Differential DiagnosesExtraocular Muscle Enlargement
Thyroid orbitopathy
Pseudotumor
Lymphoproliferative
lesion Metastases
Trauma
Cellulitis Sarcoidosis
Rhabdomyosarcoma Carcinoid MetastasesCourtesy Laurie Loevner, MD
-
7/31/2019 Orbital Ddx
36/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
37/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
38/49
Orbital Differential DiagnosesPseudotumor The Great Mimic #1
Myositic
Anterior
Lacrimal
Tolosa-Hunt
Kansas City March 2010
Orbital Differential Diagnoses
-
7/31/2019 Orbital Ddx
39/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
40/49
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
-
7/31/2019 Orbital Ddx
41/49
Kansas City March 2010
Orbital Differential DiagnosesIll-Defined/Infiltrative Orbital Lesion
Cellulitis
Infantile hemangioma
Lymphatic malformation
Plexiform neurofibroma
Rhabdomyosarcoma
Pseudotumor
Lymphoproliferativedisease
Metastatic disease
-
7/31/2019 Orbital Ddx
42/49
Kansas City March 2010
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
Courtesy Richard Wiggins MD
-
7/31/2019 Orbital Ddx
43/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
44/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
45/49
Kansas City March 2010
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?
-
7/31/2019 Orbital Ddx
46/49
Kansas City March 2010
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
-
7/31/2019 Orbital Ddx
47/49
Kansas City March 2010
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
O bit l Diff ti l Di
-
7/31/2019 Orbital Ddx
48/49
Kansas City March 2010
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!
-
7/31/2019 Orbital Ddx
49/49
Kansas City March 2010
Thank You!