Transcript Interactive Neuropathology Modules

Orbit Lesions: Differential
Diagnosis by Location
James G. Smirniotopoulos, MD,
and,
Neel Vaidya, MD,
Joel McFarland, MD,
Fletcher Munter, MD
National Library of Medicine
Bethesda, MD
Looking from Lateral to Medial
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Courtesy: Patrick J. Lynch, illustrator; C. Carl Jaffe, MD
Sectional Anatomy
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Left Figure Courtesy: Patrick J. Lynch, illustrator; C. Carl Jaffe, MD
Radiologic Anatomy
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MR: T1-weighted image
MR: T2-weighted image
Radiologic Anatomy
Extraconal
– Medial
 Ethmoid
 Lacrimal sac
– Lateral
 Lacrimal gland
 Muscle cone
 Intraconal
– Optic nerve
– Vessels & nerves
– Orbital fat
 Globe
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Radiologic Anatomy: Measurements
3.9
3.6
3.6
3.0
3.5
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ON *
2.2 – 5.2
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±
±
±
±
±
.3
.25
.2
.15
.3
Neuroradiology. 2003 Mar;45(3):184-7. || *Br J Radiol. 2009 Jul;82(979):541-4
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Inferior
Medial
Superior
Sup Obl
Lateral
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Orbit Lesion Navigator
Move the cursor over the orbit
– then click for the differential
diagnosis.
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Normal Anatomy
Author Credits
Normal Orbit
T1 – Fat is white
T2 – Water is white
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Radiologic Anatomy
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MR: T1-weighted image
MR: T2-weighted image
Orbit Anatomy
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Ant Clinoid & Optic Canal
Axial section schematic
Medial
ethmoid
pituitary
brainstem
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Lateral
Orbit Lesion Navigator: Intraconal ON
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Normal Anatomy
Author Credits
Intraconal – Optic Nerve Lesions
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Optic Neuritis
 Optic Nerve Glioma
 Optic Nerve Meningioma
 Dilated Optic Nerve Sheath
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Axial T1W MR w/Gadolinium (fat
suppressed)
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Optic Neuritis
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Axial T1W MR w/Gadolinium
(fat suppressed)
Optic Neuritis
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Axial T1W MR w/Gadolinium
(fat suppressed)
Optic Neuritis
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Who: Adults < 45yo, women >> men
Why: Multiple sclerosis, ocular infection, degeneration,
ischemia, meningitis
Symptoms: onset of unilateral vision loss over hours
to days, with painful eye movements
CT: Normal to mildly enlarged optic nerve and chiasm,
may enhance
MR: mild enlargement, enhancement of optic
nerve
Prognosis: spontaneous improvement, 1-2 weeks
Optic Nerve Glioma
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Optic Nerve Glioma
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Optic Nerve Glioma
Coronal: T1W w/contrast, fat-suppressed
Axial: T1W w/contrast, fat-suppressed
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Optic Nerve Glioma
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– Adult form – diffuse astrocytoma, may be fatal
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Who: 80% < 10yo, M<F, peak 5 yo, extremely rare
variant in 6th decade
Why: Associated with NF in 10-50%, especially
children (50-80%)
Symptoms: Decreased visual acuity, minimal proptosis
CT: fusiform enlargement of optic nerve, posterior
extension along optic tracts in 2/3, slight
enhancement, rarely calcification
MR: T2 bright, enhancement, more sensitive for
intracranial extent
Prognosis: Pediatric – grows slowly, if at all
Optic N. Glioma vs. Meningioma
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Optic Nerve Meningioma
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Optic Nerve Meningioma
Coronal: T1W non-contrast
Coronal: T1W w/contrast, fat-suppressed
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Optic Nerve Meningioma
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Who: children, mid-age & elderly women,
3:1 Women to men
Why: arises from arachnoid rests in meningeal
investiture of optic nerves, occasionally seen in NF2
Sx: loss of visual acuity over months, proptosis
CT/MR: calcifications strongly suggestive, tubular
thickening of nerve, with enhancing “tram tracks” on
axial view; and, ”ring” on coronal view around
nonenhancing optic nerve
Prognosis: In one series, 87% 5-year and 58% 10year survival
Bilateral Intraconal Lesions: Meningioma
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Dilated Optic Nerve Sheaths
(Pseudotumor cerebri in a child)
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Courtesy Mauricio Castillo, M.D. UNC
Dilated optic sheaths
Dysplasia of dura
– Marfan, Ehler-Danlos
Arachnoid hypertrophy + dural dysplasia: NF-1
 Increased intracranial pressure
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– Lead, Vitamin A, venous thrombosis or narrowing
(skull base dysplasias), pseudotumor (rare in
children)
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Clinical mimicker: Drusen bodies
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Orbit Lesion Navigator: Intraconal non-ON
Move the cursor over the orbit
– then click for the differential
diagnosis.
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Normal Anatomy
Author Credits
Intraconal lesions w/o Optic Nerve
Involvement
Cavernous Hemangioma
 Orbital Varix
 Lymphangioma
 Pseudotumor
 Lymphoma
 Metastases
 Cavernous Carotid Fistula
 AVM
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Cavernous Hemangioma
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Cavernous Hemangioma
Who: middle-aged adults, F:M is 5:1
Why: large dilated endothelial lined spaces surrounded by
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CT/MR:
fibrous tissue.
 Sx’s: slow progressive proptosis, extraocular muscle and visual
impairment
– Sharply demarcated mass in superior-temporal portion of the
conus (66%)
– Expansion of bony orbit
– Heterogeneous enhancement
– NO flow voids on MR
 Prognosis: slowly progressive, may rapidly enlarge during
pregnancy.
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Orbital Varix
Without Valsalva
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With Valsalva
Orbital Varix
Who: Anyone
 Why: Intermittent proptosis with straining
 Sx’s: Retrobulbar pain
 CT: Enhanced CT with and w/o Valsalva
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– Enhancing, well-defined mass w/o internal
septations
– Enlarges with Valsalva
– May produce bony erosion
– Involve superior or inferior orbital vein
Lymphangioma
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Lymphangioma
Who: Children
 Why: Bulky, arise from lymphoid follicles
 Sx’s:
 CT/MR:
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– Clear fluid channels
– Enhance less often and less intensely than
hemangioma
– Infiltrative, lack defined capsule
– Can hemorrhage
Capillary Hemangioma
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Who:
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Sx’s:
– proptosis exaggerated by crying
– Associated with skin angioma
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CT/MR:
– NOT intraconal
– Mass with enhancement equal to or greater than orbital muscle.
– Flow voids on MR
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Prognosis: Spontaneous involution within 1-2 years.
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– Children (95% are less tjan 6 months of age)
– M<F
Orbit Lesion Navigator: Globe
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Normal Anatomy
Author Credits
Globe Lesions
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Neoplastic
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Non-Neoplastic
– Large Globe
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Coloboma
Staphyloma
Sturge-Weber
Neurofibromatosis Type 1
– Normal Globe
– Small Globe
 Persistent Hyperplastic Primary Vitreous
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– Retinoblastoma
– Melanoma
– Metastasis
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Retinoblastoma
Retinoblastoma
Coronal: T1W non-contrast, fat suppressed
Coronal: T2W non-contrast
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Orbit - Prosthetic eye
Post Tx w/enucleation
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Dx: Retinoblastoma
Retinoblastoma
Who: noninherited in 2/3, Heritable in 1/3
 Heritable:
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Noninherited:
– Who: sporadic mutation, 23 months average age
– Why: sporadic somatic mutation, subsequent generations
unaffected
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– Who: sporadic heritable form in 25%, 12 months avg age
– Familial retinoblastoma in 8%, aut dominant with 95%
penetrance, present 8 months, bilateral in 2/3, may be
tri/quadrilateral
More Retinoblastoma
Sx: “cat’s eye” leukocoria in 60%, decreased visual
acutiy, strabismis
 CT: lobular hyperdense mass, orbital calcifications
characteristic, enhances
 MR: iso to mildly hyperintense on T1, marked
enhancement
 Prognosis: calcifications are favorable, enhancement is
not, <10% mortality if optic nerve spared, 65%
mortality if choroidal invasion
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Primary Ocular Melanoma
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Melanoma
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Who: whites of northern European descent, highest
incidence at 55 y.o.
Why: typically arises from melanocytes in the choroid
Sx: decreased visual acuity, floaters, painless visual
field deficit. Rarely painful.
CT/MR: Hyperdense, enhancing, T1 bright and T2
dark, good to eval extraocular extension.
Prognosis: Poor - usually fatal due to mets
Large Globe
Coloboma
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Coloboma
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Who:
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Why:
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Hereditary conditions, post-traumatic or post-surgical.
Congenital or acquired defect in any ocular structure
Results from incomplete closure of choroidal fissure
Sx’s: Blurred vision, decreased visual acuity
CT/MR:
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Findings relate to the size of the defect
Usually a cone shaped defect at the inferomedial globe
Widening of the optic nerve head & continuous with vitreous humor
No uveoscleral thinning.
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– Anyone, about 60% are bilateral
– Syndromic associations: CHARGE syndrome
Large Globe
Staphyloma
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Staphyloma
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Who:
Why: Attributed to increase in axial length of the globe.
 Sx’s: Blurred distance vision, squinting and eye strain.
 CT/MR:
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– Thinning of the posterior sclera
– Temporal side of the globe bulges
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– Anyone with axial myopia, glaucoma or trauma
– Patients with RA & other inflammatory conditions.
Small Globe
Persistent Hyperplastic Primary Vitreous
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Courtesy: Dave Yousem, MD
Persistent Hyperplastic Primary Vitreous
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Who:
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Why:
– Persistence of embryonic hyaloid vascular system
– Hyperplasia/proliferation of embryonic connective tissue
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Sx’s: Unilateral Leukokoria
CT/MRI
– Microopthalmia, increased attenuation of the vitreous
– S shaped structure at optic nerve origin, called Cloquet's Canal
 Enhancing; hypointense on MR
– Does NOT calcify
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– Anyone
– Associated with other ocular malformations (Norrie Disease)
Orbit Lesion Navigator: Muscle cone
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Normal Anatomy
Author Credits
Muscle Cone Lesions
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Thyroid Ophthalmopathy
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Orbital Pseudotumor
– Tendons affected
Orbital Cellulitis
 Lymphoma
 Varix
 Metastases
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– Tendons normal
Thyroid Ophthalmopathy
Coronal: CT non-contrast
Axial: CT non-contrast
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Muscle belly enlarged, sparing
of the tendon
Thyroid Ophthalmopathy
Pearl
Thyroid ophthalmopathy spares the muscle tendon
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Pearl
Involvement: inferior > medial > superior > lateral
Thyroid Ophthalmopathy
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Who: adults, 1:4 men to women
Why: Graves Dz – increase in orbital pressure leads to
muscle ischemia, edema, fibrosis
Sx: Proptosis – most common cause, uni- or bilateral,
lid lag, periorbital swelling
CT/MR: sparing of muscle insertion on globe,
affects I>M>S>L>O, high T2 signal in muscles due to
edema
Prognosis: 90% resolve in 3-36 months, 10% lose
visual acuity due to corneal ulcers/optic neuropathy
Pseudotumor
of
Orbit
Pearl
Orbital pseudotumor may involve the tendons
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Pseudotumor of Orbit
Coronal: MR T2-weighted
Coronal: MR T1W non-contrast
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Pseudotumor of Orbit
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http://medpix.nlm.nih.gov
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Who: young women, ¼ unilateral exophthalmos
Why: idiopathic, sarcoid/collagen d/o, infection, foreign
body
Sx: pain, proptosis, chemosis
CT: retrobulbar fat>extraocular muscle>optic
nerve. Increased density of conal fat, diffusely
thickened muscles (including tendinous insertions),
proptosis
MR: lesions isointense to fat on T2
Prognosis: dramatic and rapid response to steroids,
may have remitting or chronic and progressive course
Orbital Cellulitis
with resultant proptosis
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Orbital Cellulitis
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http://medpix.nlm.nih.gov
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Who: Children >> adults, median 7-12 y.o., twice as
common in boys, no racial predilection
Why: acute bacterial infection, often extending from
paranasal sinuses/eyelids
Sx: proptosis, scleral thickening,
CT/MR: enlarges and displaces EOM (often medial
rectus), increased density of retro-orbital fat,
associated ethmoid/max sinusitis. Contrast-enhanced
fat-suppressed images most sensitive on MR.
Prognosis: antibiotics and steroids usually effective,
depending on extent of destruction and abscess
formation
Lymphoma
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Lymphoma
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Lymphoma
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Lymphoma
Who: 50 yo to 70 yo
 Why: usually precedes systemic lymphoma
 Sx: painless eyelid swelling, exophthalmos
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CT/MR: large homogenous enhancing mass, slightly
T2 hypointense, bone destruction is uncommon
 Prognosis: overall 5-year survival for all NHL is 55%
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http://medpix.nlm.nih.gov
– Extraconal (lacrimal gland, anterior extraconal space,
retrobulbar) > Intraconal
Orbit Lesion Navigator: Lacrimal sac
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Normal Anatomy
Author Credits
Lacrimal Sac
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Dacryocystocele
 Abscess/pyocele
 Metastasis, Lymphoma, etc.
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Dacryocystocele
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Dacryocystocele
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Who: neonates
Why: 2nd most common cause neonatal nasal
obstruction (after choanal atresia), imperforate Hasner
membrane distally, unknown why proximal duct
obstructs
Sx: tense blue-grey mass at medial canthus
CT: well-defined homogenous fluid-attenuation
mass with thin wall enhancement, may see superior
displacement of inf turbinate/contralateral nasal septal
shift
Prognosis: Good if avoid complications of
infection/periorbital cellulitis
Orbit Lesion Navigator: Lacrimal fossa
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Normal Anatomy
Author Credits
Lacrimal Fossa
Granulomatous Disease
– Sarcoid
Dermoid/Epidermoid Cyst
 Lymphoma
 Primary Neoplasms
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– Mixed tumor
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Sarcoid of Lacrimal Fossa
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Sarcoid of Lacrimal Fossa
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Sarcoid of Lacrimal Fossa
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http://medpix.nlm.nih.gov
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Sarcoid affects African-American women most
commonly, bimodal age distribution 25-35 and 45-65
y.o.
Why: Unknown
Sx: Nonspecific, can include exophthalmos, pain, visual
impairment (anterior uveitis is most common
manifestation of sarcoid in orbit)
CT/MR: Inflammatory process, tendency to extend
posteriorly along the optic nerve and involve the
chiasm, suprasellar cisterns. Can mimic pseudotumor.
Prognosis: Variable, improves with steroids but can
progress to blindness
Dermoid Cyst
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Dermoid Cyst
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Dermoid Cyst
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http://medpix.nlm.nih.gov
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Who: most common benign orbital tumor of
childhood, most common first decade
Why: usually arises in fetal cleavage planes/sutures
Sx: mass effect on EOM/globe
CT: anterior extraconal orbit, upper
temporal>>upper nasal quadrant. Well-defined cystic
mass with negative HU, nonenhancing, may have
fat-fluid level
MR: increased signal on both T1 and T2
Prognosis: good, less so if it ruptures and induces
granulomatous inflammation
Tx: Percutaneous Ablation
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Golden RP, Shiels WE, Cahil KV, Rogers GL, J AAPOS 2007;11:438-442
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US placement of angio catheter/sheath
Serial injection/aspiration of sodium tetradecyl sulfate
3% (STS)
Replace with 5F Pigtail catheter
Reinflate with saline, contrast
Reinflate (50%) with 1% Lido for 10 min.
Reinflate (50%) with STS for 2 min.
Reinflate (50%) with 98% ETOH 15 min.
Aspriate, suture cath (suction bulb) for 24 hrs.
Todah Rabah
Thank You!
Takk
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.‫لك‬
Muito Obrigado
EUXAPIΣTΩ !
SHOUKRANN
teşekkür ederim!
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Mahalo !
Dank u wel !
Merci Beaucoup
Dziekuje!
Danke Schön !
Tack
grazie!
Mil Gracias
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ありがとうございます。感謝いたします。
спасибо!
Rieska