Transcript Lacrimal gland disease

M Haider, A Mohandas, T Khairalsaeed, K Shah
Detroit Medical Center/ Wayne State University
eEdE#: eEdE-125
Disclosures
• Authors
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Maera Haider – Nothing to disclose
Aravind Mohandas – Nothing to disclose
Tagwa Khairalseed – Nothing to disclose
Kamran Shah – Nothing to disclose
Objectives
• Assessing normal globe positioning on imaging
• Review various causes of exophthalmos with typical findings on CT and/or
MR imaging.
• Review etiologies of enophthalmos with typical imaging findings
Measuring Orbital Position on Imaging
• Perpendicular distance from
the interzygomatic line (IZL) to
the posterior margin of the
globe
• 14% greater upper limit of
normal for African Americans
5-12.8mm for Caucasians
with
Measuring position ..contd
• Less commonly used method: Globe protrusion anterior to the IZL on axial CT
imaging at level of the lens
• ≥ 21mm  proptosis
• Persons of African ancestry may have an upper limit of 14.5% higher than
Caucasian counterparts
• Clinically, the Hertel exophthalmometer most commonly used for
measurements, however CT demonstrates better accuracy
Exophthalmus - Causes
• Most common cause: Thyroid orbitopathy
• Infectious
• preseptal vs orbital cellulitis
• fungal
• Inflammatory conditions
• Pseudotumor
• Vasculitis
• Lacrimal gland disease
• Orbital tumors
• Vascular malformations
• Varices
• Carotid-cavernous fistulae
Thyroid disease
Exophthalmos
• Most commonly occurs with Graves disease. Hashimotos is also an association
• Usually bilateral and symmetric
• Inflammation and hypertrophy of the EOM with sparing of tendinous insertion
• Likelihood of muscle involvement: Inferior rectus > Medial rectus > Superior rectus >
Lateral rectus
• Increased orbital fat due to edema and inflammation  reduced attenuation
• Size of muscle correlates with severity of disease
• May result in mass effect on optic nerve
• On MR, EOM may appear T2 intense when actively inflamed; enhancement
may or may not be present
Thyroid disease
Exophthalmos
72 yo man with long-standing history of Graves disease and
thyroid orbitopathy now resulting in restrictive myopathy,
underwent forced duction and medial rectus recession bilaterally
Inferior  Medial
 Superior  Lateral  Obliques
Infection: Pre-septal & Orbital Cellulitis
Exophthalmos
• Accounts for majority of primary orbital disease
• Presents with proptosis, painful ophthalmoplegia, reduced visual acuity
• Source: contiguous spread from teeth, face, or paranasal sinuses
• Most common: ethmoid sinus
• Imaging findings on CT:
• Loss of fat planes with stranding
• Edema of surrounding soft tissue as well as intra-orbital structures
• +/- abscess formation
• MR imaging is rarely necessary. Will demonstrate high T2 and low T1 signal,
diffusion restriction and peripheral enhancement in case of abscess
• Complications: cavernous sinus thrombosis, meningitis, thrombosis of superior orbital
vein, abscess formation, vision defects
Orbital cellulitis
13yo girl with headache, fever, left eye
Exophthalmos
swelling greater than right, and AMS. Pre and post septal edema
involving intra and extraconal
regions with L>R proptosis
Diffuse opacification and restricted
diffusion of sphenoid & ethmoid
sinuses indicating acute disease
with purulent material
T2WI
T1WI enhanced
T1WI
Pachymeningeal enhancement around temporal and frontal
convexities - meningitis
Bilateral nonenhancing cavernous sinuses are hypointense on T1
and T2 with diffusion restriction indicating purulent material
Lacrimal gland disease
Exophthalmos
• Lacrimal gland is composed of epithelial and lymphoid tissue, predisposing to
corresponding pathologic processes
• Bilateral disease
• Inflammatory: sarcoidosis, histiocytosis
• Malignancy: lymphoma, leukemia
• Unilateral disease
• Infection
• Malignancy: primary or metastatic such as adenocarcinoma
Lacrimal gland disease
48 yo man with right proptosis and
pre-orbital swelling with blurry
vision for past 3 years.
Exophthalmos
Asymmetric thickening and infiltration of right eyelid, preseptal soft tissue, lacrimal gland and mild enlargement of EOM
Differentials include
• Chronic adenitis with overlying cellulitis
• Pseudotumor involving lacrimal gland and EOM
• Lymphoma
• Sarcoid thought unlikely due to unilaterality
On biopsy of lacrimal gland: Non-caseating
granuloma – sarcoidosis
On body imaging, found to have pulmonary fibrosis
but no lymphadenopathy or heaptosplenomegaly
Orbital varices
• Secondary: due to acquired increased flow
• Atriovenous malformation
• Carotid cavernous fistula (CCF)
• Primary: congenital and confined to orbital cavity; rare
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Usually not symptomatic until 3rd decade of life
‘Intermittent’ proptosis or diplopia with Valsalva phenomenon/straining
Thin walled and distensible
Eventually may cause atrophy of orbital fat resulting in enophthalmos
Diagnosis: Color flow sonogram, MRI and CECT – with and without Valsalva
CT findings
• Phleboliths (on non-contrast)
• Ehancement of varix
• MR will show T1 and T2 hypointensity when no thrombosis
• Heterogenous hyperintensities when thrombosed
Exophthalmos
Carotid cavernous fistula
82 yo woman post MVA with nondisplaced C2
fracture, developed AMS day after event
Significant flow related enhancement
ICA shunting
of cavernous sinuses, superior orbital
veins and retro-clival venous plexus.
Retro-orbital fat stranding is present.
Cerebral angiogram performed with
plan
to embolize any
Orbital
congestion
amenable lesion. Imaging revealedwith
bilateral
transected internal
associated
of segments with
carotid arteries at petrocavernousenlargement
and cavernous
the EOM
direct CC fistula. Determined too extensive
for safe endovascular
DWI and ADC: Extensive cerebral watershed distribution of infarctions
treatment.
secondary to hypoperfusion from the CCF.
Made hospice, patient expired soon after.
Left: Image from angiogram on the left demonstrates attenuated flow
distal to the cavernous sinus
Exophthalmos
Sphenoid wing dysplasia
Exophthalmos
• High association with neurofibromatosis type 1
• Present in up to 10% of NF-1
• Diagnostic criteria for NF-1
• Imaging Findings:
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Absent or hypoplastic sphenoid wing
Widened superior orbital fissure
Expansion of middle crania fossa, often associated with arachnoid cysts
Herniation of intracranial structures into the orbital cavity results in proptosis, and
occasionally pulsating proptosis
Sphenoid wing dysplasia
25 yo woman with known NF1
Enhancing soft tissue mass involving left
temporalis muscle and periorbital
structures infiltrating fat – consistent with
plexiform neurofibroma
Inferior herniation of temporal lobe. If
Deformity of left middle cranial
herniation occurs into the orbital cavity, it
fossa secondary to sphenoid wing
may result in pulsatile proptosis
dysplasia and calcified proptosis
Elongation of
left globe axis
Exophthalmos
Orbital Pseudotumor
• Usually unilateral in adults, bilateral in children
• Diagnosis of exclusion
• Can involve: extra-ocular muscles (most commonly), other retrobulbar structures,
sclera, uvea, lacrimal glands
• Findings:
• Moderate diffuse irregular enlargement of structures with enhancement
• Slight hyperintensity on T2 and STIR
• Relative hypointensity to other orbital lesions suggests fibrosis
• In chronic disease, decreased intensity suggests sclerotic variant and therefore worse prognosis
• If only EOM involved, may resemble thyroid orbitopathy, however tendons are
involved in case of pseudotumor and presentation is typically painful
• Extensive differentials: sarcoidosis, systemic lupus erythematosus, dermatomyositis,rheumatoid arthritis,
lymphoproliferative conditions, Wegener’s, cellulitis
• Tolosa-Hunt is a variant that involves the cavernous sinus
Pseudotumor
T2 hyperintensity and
enhancement showing
edema and inflammatory
infiltration extending along
the right optic nerve sheath
with involvement of the
intraconal fat, medial and
superior rectus muscles
Exophthalmos
T1WI
Fat suppressed T1WI with contrast - axial
T2WI
56 yo woman with 1 year history of proptosis and swelling.
Negative work-up for inflammatory and malignant
etiologies. Diagnosis: Idiopathic Orbital Inflammatory
Syndrome
Fat suppressed T1WI with contrast - coronal
Orbital tumors
Exophthalmos
• Primary – intraconal vs extraconal
• Nerve /sheath tumors – low T1 and high T2 signal typically with enhancement
• Neurofibroma
• Solitary – no association with NF-1, easily resectable. Undistinguishable from schwannoma on imaging.
• Diffuse – may be associated with NF-1, extensive involvement of intraorbital structures may be present
• Plexiform – pathognomonic for NF-1
• Schwannoma
• Paraganglioma
• Optic nerve glioma
• Usually low grade, especially in children <5yrs, likely pilocytic, often bilateral
• High grade in adults, likely glioblastoma multiforme or anaplastic
• On MRI: fusiform enlargement of optic nerve; isointense on T1 and T2 when restricted to orbit, higher
signal when involves optic chiasm
• Meningioma
• Circumferential thickening of nerve sheath with uniform enhancement. ‘Tram track’ sign with nonenhancing central nerve
Orbital Tumors: continued
• Primary contd
• Mesenchymal
• Cavernous hemangioma: most common benign orbital tumor. Well-delineated and enhancing on
CECT, low signal on T1, high signal on T2. Rarely recur after resection
• Lymphangioma: hemorrhage often hence heterogenous T1 and T2 signals with minimal
enhancement
• Hemangiopericytoma, rhabdomyosarcoma, fibrous histiocytoma
• Rare: fibroma, fibrosarcoma, lipoma, leiomyoma etc
• Secondary – contiguous spread from adjacent structures
• Osteoma, ossifying fibroma, aneurysmal bone cyst
• Metastasis
• Represent 2.5 – 3.7% of all orbital tumors
• Most common sources are breast, lung and prostate
Orbital tumor – optic glioma
Exophthalmos
2 yo boy with right proptosis and optic
cupping on clinical examination
Images on the right
demonstrate a T2
hyperintense mass
expanding the optic sheath
with small amount of
peripheral fluid preserved.
Normal appearance of
optic chiasm.
T2WI
Images on the left depict
the marked optic edema in
the right eye compared to
the left.
No other stigmata of NF1 were present in this patient.
Pathology revealed: Pilocytic astrocytoma WHO grade
1
T1WI
Sphenoid wing meningioma
Exophthalmos
30 yo man with history of right sphenoid wing meningioma for which
he underwent debulking and radiation therapy. Now with visual
disturbance and proptosis
MRI shows a large lobulated
Remote post-operativeheterogenous
craniotomy extraaxial mass in
middle cranial fossa involving
changes with extensivertsclerosis
greater
and hyperostosis of skull
base and
and lesser wing of
sphenoid. Transcranial spread to
sphenoid wing due to osseous
right infratemporal fossa
infiltration
A large enhancing intra- and
extracranial meningioma is
visualized encroaching into the
cavernous sinus, right superior
and inferior orbital fissures.
Crowding at the apex results in
mass effect on optic nerve.
Metastasis of Ovarian Origin
Exophthalmos
61 yo woman with history of papillary serous
ovarian cancer and worsening left eye pain
with abnormal ophthalmologic exam
MRI reveals an enhancing mass in
muscle belly of left medial rectus.
Focal enhancing mass also noted in
superior rectus belly. The lateral and
inferior rectus muscles, orbital fat,
globes and optic nerves are
unremarkable
Metastasis of Nasopharyngeal Origin
Exophthalmos
56 yo M with history of swelling and ulceration along roof of mouth.
Found to have tumor along tongue and palatine tonsils as well as
multiple neck nodes. PET/CT revealed FDG avid spots on nasopharynx.
Underwent chemotherapy and radiation. Subsequent PET revealed
progression of disease. Went on to develop headache and proptosis.
Enhancement of the right temporalis on MR
suggests further contiguous infiltration
CT demonstrates a large infiltrating mass centered in the posterior right nasopharynx
extending into the right orbital apex involving the medial and inferior orbit, along
with extension to involve the sphenoid, right ethmoid and right maxillary sinuses
Pseudoexophthalmos
• Confounders to measurement of proptosis
• Axial myopia:
• Increased length of globe, unilateral or bilateral
• Risk of retinal detachment
• Congenital vs youth acquired vs adult onset
• Buphthalmos:
• Enlargement of globes without deformation or intraocular mass
• Usually due to infantile glaucoma
• Associated with neurofibromatosis type 1
• Shallow orbits: associated with multiple syndromes
• Apert syndrome, Crouzon disease, Dubowitz syndrome, Edward and Patau syndromes, bone
hyperostosis for eg. secondary to radiation injury
Apert Syndrome
• Rare; autosomal dominant and sporadic variants
• Constellation of limb and skull malformations
• Associated with CNS, GU and cardiac anomalies
• Classic features:
• Craniosyntosis
• Facial hypoplasia
• Syndactyly
• Other features:
• Harlequin eyes - elevation of the superolateral corner of the orbit
• Hypertelorism – increased distance between the orbits
Pseudo-exophthalmos
Apert syndrome – 2 month old girl
Pseudo-exophthalmos
Hypertelorism is noted with
bilateral harlequin appearance
of the orbits. The shallow
orbital cavity results in
proptotic globes
Imaging reveals increased
craniocaudal dimension with bilateral
shallow anterior cranial fossae and
frontal bossing as well as bilateral
croronal craniosynostosis.
Enopthalmos: Causes
• Most common cause: Trauma
• Structural:
• Maxillary sinus disease (silent sinus syndrome)
• Bony defect (congenital, iatrogenic)
• Fat atrophy
• Age related or senile – bilateral and not uncommon
• Systemic disease (lipodystrophy, scleroderma)
• Post irradiation
• Retraction
• Metastasis (lung, gastric, scirrhous breast Ca)
• Post-inflammatory
Traumatic Enophthalmos
Enophthalmos
53 yo M with history of left ‘proptosis’; imaged
to evaluate for an orbital mass
Upon CT imaging, discovered to have old fracture
deformity of the right orbital floor and medial wall
with herniation of orbital fat through the defect
resulting in mild enopthalmos of right globe and
relative exophthalmos of left globe.
Minimal left globe proptosis due to abundant orbital
fat without enlargement of lacrimal gland or EOM
Silent Sinus Syndrome
Enophthalmos
• Severe maxillary sinusitis  centripetal collapse aka atelectasis of sinus walls
 enophthalmos and hypoglobus (downward displacement of globe)
• Often no history of sinusitis present
• Always unilateral
• Pathophysiology: negative pressure in the maxillary sinus due to resorption
of secretions with concomitant sinus obstruction
Silent Sinus Syndrome
Enophthalmos
Increased volume of orbital cavity with bowing
of the orbital floor. Often the floor is thinned,
and less frequently, can be absent
Relative posterior displacement of globe
Complete opacification of left
maxillary sinus with reduced volume
Bony defects - enophthalmos
Enophthalmos
• Most commonly, absence of greater wing of the sphenoid bone
• Associated with neurofibromatosis type 1
• May also be present with epidermoid cyst, orbital varices, or iatrogenic
• Complication: herniation of temporal lobe through the defect resulting in pulsatile
enophthalmos
• Imaging: complete or partial absence of GWS; widened superior orbital fissure;
varices; associated optic glioma or plexiform neurofibroma
• Pagets disease: enophthalmos is a rare manifestation – progressive bilateral
receding globes due to bone remodeling
Lipodystrophies
Enophthalmos
• Total vs partial vs localized
• Enophthalmos most commonly occurs with partial dystrophy
• Associations include Sjogren’s, lupus erythematosus, dermatomyositis
• Most commonly, HIV-associated lipodystrophy – can result in severe atrophy
of facial subcutaneous fat  enophthalmos. Thought to be related to
treatment rather than the disease
• Post-irradiation resulting in fat atrophy later in life, most commonly post
treatment for retinoblastoma or rhabdomyosarcoma as a child
Traction secondary to Metastasis
Enophthalmos
• 7-24% of orbital metastasis results in enophthalmos rather than
exophthalmos
• Most commonly secondary to metastatic scirrhous breast carcinoma
• Decreased ocular motility due to muscle infiltration
• Desmoplasia, fibrosis, fat atrophy  enophthalmos
• Biopsy recommended even when known hx of carcinoma for
treatment planning
• Poor prognosis
Metastatic Scirrhous Breast Carcinoma
Tends to localize to the EOM
and intra as well as extra
conal fat as demonstrated.
Presents in an irregular and
diffuse fashion, extending
along fascial planes
When bone is involved,
breast cancer tends to
be more destructive
than hyperostotic. This
patient demonstrated
both lytic and sclerotic
regions in the calvaria
Enophthalmos
Restrictive muscle conditions
Enophthalmos
• Duane retraction syndrome is most common restrictive condition, however
resultant enophthalmos is rare
• Congential EOM fibrosis: rare, non-progressive, usually bilateral, usually
familial.
• Acquired retraction syndromes: rare, usually post-trauma
Pseudoenophthalmos
• Contralateral exophthalmos - Approx 50% of time unilateral enopthalmos
initially results in investigation for ‘ contralateral exophthalmos’
• Micropthalmos
• Facial asymmetry
• Ptosis (eg. with Horner syndrome)
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The End.