and Ocular Disorders


Prevalence:   3.5÷100,000; 1–2% of orbital childhood masses; 4–8% of expanding pediatric orbital lesions; 25% of vasculogenic orbital lesions

Origin:   arises from a pluripotent venous anlage as a malformation (NOT neoplasm of proliferating cells!)

Path:   unencapsulated diffuse multicompartmental lesion often with intra- and extraconal components insinuating between normal orbital structures

Histo:   dilated lymphatics, dysplastic venous vessels, smooth muscle, areas of hemorrhage

(a)  simple / capillary lymphangioma

=  lymphatic channels of normal capillary size

(b)  cavernous lymphangioma (most common)

=  dilated microscopic channels

(c)  cystic hygroma

=  macroscopic multilocular cystic mass

May be associated with:  intracranial vascular anomalies (70%): developmental venous anomalies (61%), AVM, cerebral cavernous malformation, sinus pericranii

Age:   1st decade or later (mean age of 6 years); M÷F = 1÷1; 43% before age of 6 years; 60% before age of 16 years

•  proptosis:

•  slowly progressive proptosis with restriction of eye movement, optic nerve compression, vertical displacement of globe

•  sudden proptosis from intratumoral hemorrhage

=  CARDINAL FEATURE; often occurring spontaneously / after minor trauma / during upper respiratory infection

•  worsening proptosis from intralesional proliferation of lymphocytes during viral infection with subsequent resolution

•  associated with lesions on lid, conjunctiva, cheek:

•  eyelid fullness from birth

•  purple discoloration of skin, usually in superomedial orbit

•  vesicles in conjunctiva, facial skin, oral mucosa

•  restricted ocular motility (50%)

•  growth with patient’s growth ± accelerated growth in response to hormonal changes (during puberty / pregnancy)

Location:   unilateral; usually medial to optic nerve with intra- and extraconal component, crossing anatomic boundaries (conal fascia / orbital septum)

Site:   superficial (conjunctiva + eyelid) in anterior orbit (79%); deeper orbit (67%); pre- and postseptal orbit (73%)

√  poorly defined infiltrating multilobulated heterogeneous lesion

√  single / multiple cystlike areas with rim enhancement (after hemorrhage):

√  solid-appearing microcystic component

√  macrocystic component = cysts of 1–2 cm in size

√  blood cyst = “chocolate cyst” associated with multiple recurrences

√  variably mild patchy low-flow enhancement (= venous channels) / ring enhancement (after hemorrhage)

√  rarely contains phleboliths (DDx: hemangioma, orbital varix)


√  heterogeneous echotexture with ill-defined borders:

√  area of predominantly cystic low-level internal echoes with infiltrative borders

√  hyperechoic intracystic clot


√  poorly marginated lesion insinuating itself between normal structures:

√  well visualized ← inherent contrast between malformation + orbital fat

√  venous / solid components slightly hyperattenuating relative to brain tissue

√  macrocystic lymphatic components similar in attenuation to vitreous of globe

√  mild to moderate expansion / remodeling / hyperostotic / lytic lesion of bony orbit

√  ± widening of superior / inferior orbital fissure

√  frequent thickening of upper eyelid

MR (modality of choice):

√  iso- to slightly hyperintense relative to brain on T1WI + very hyperintense relative to brain on T2WI:

√  T1WI for lymphatic / proteinaceous fluid

√  fat-suppressed T1WI for blood / blood products

√  fat-suppressed T2WI for nonhemorrhagic fluid

√  fluid-fluid levels of hemorrhages of various ages / T2 shading in multiple cysts (almost PATHOGNOMONIC)

√  NO enlarged feeding vessels / flow voids

Prognosis:   no involution; aggressive behavior with continued enlargement (in 64%) + recurrence after treatment; eventually loss of vision (in 40%); progression slows with termination of body growth

Rx:   observation; intralesional injection with sclerosing agents (tetradecyl sulfate or OK-432) / steroids; surgery in case of optic nerve compression + for relieve of pain and for cosmetic improvement

DDx:   orbital varix (affected by postural changes, communication with systemic circulation)


Usually presents without evidence of systemic disease; subsequent development of systemic disease frequent

Frequency:   in 8% of leukemia; in 3–4% of lymphoma; 67–90% of orbital lymphoproliferative tumors; 24% of all space-occupying orbital tumors in patients > 60 years of age

Age:   50 years on average

Type:   usually non-Hodgkin B-cell lymphoma; Burkitt lymphoma with orbit as primary manifestation; Hodgkin disease rare

•  palpable mass, mildly restricted ocular motility

•  painless swelling of eyelid (pain is uncommon)

•  proptosis (late in course of disease)

◊  3rd most common cause of proptosis (after orbital pseudotumor + cavernous hemangioma)

Location:   extraconal (especially lacrimal gland, anterior extraconal space, retrobulbar) > intraconal > optic nerve-sheath complex; unilateral (76%)

◊  The lacrimal gland is a common site for leukemic infiltrates + lymphoma (40%)!

Growth types:

(a)  well-defined high-density mass (most commonly about lacrimal gland)

√  smooth circumscribed mass (50%)

(b)  diffuse infiltration (tends to involve entire intraconal region)

√  diffuse ill-defined lesion (50%)

√   tendency to mold to orbital structures (globe, optic nerve, orbital wall → ± bone remodeling

√  slight to moderate uniform enhancement

  √  osseous erosion (rare)

√  isointense relative to muscle on T1WI

√  hyperintense relative to orbital fat on T2WI


√  solitary / multiple hypoechoic homogeneous masses with infiltrative borders

DDx:   pseudotumor (infiltration / thickening of ocular muscles, mass commonly T2-isointense relative to orbital fat, high ADC values, acute onset of pain)


=  DIKTYOMA = TERATONEUROMA [diktyon, Greek = net]

=  rare embryonal malignant (most) / benign intraocular neoplasm

Origin:   primitive medullary epithelium in ciliary body

Histo:   folded cords + sheets resembling a fisherman‘s net (diktyomatous pattern) surrounding fluid collections predominantly composed of hyaluronic acid; heteroplastic components of hyaline cartilage, rhabdomyoblasts, neuroglia, sarcomatous elements (a) teratoid (30–50%) (b) nonteratoid (50–70%)

Mean age:   5 years; M=F

•  poor vision ← lens subluxation, lens notching, glaucoma, cataract formation, retinal detachment

•  pain; leukokoria; mass of iris / ciliary body

•  exophthalmos, buphthalmos. strabismus, ptosis

Location:   ciliary body (common); optic nerve head / retina (rare); usually unilateral

√  dystrophic calcifications (in hyaline cartilage component) in 30%


√  echogenic irregularly shaped /ovoid mass


√  dense irregular mass

√  moderate to marked enhancement


√  slightly to moderately hyperintense to vitreus on T1WI

√  hypointense on T2WI

√  marked homogeneous enhancement / heterogeneous (← cystic components)

Prognosis:   local recurrence common; metastases rare


Prevalence:   1–13% of orbital tumors

Origin:   source known in only in 50%

adults:   carcinoma of breast + lung

children:   neuroblastoma > Ewing sarcoma, leukemia, Wilms tumor

Frequency of metastases to orbit:

breast cancer (48–53% of orbital metastases) > prostate carcinoma > cutaneous melanoma > lung cancer

•  proptosis, motility disturbance, pain, diplopia, ↓ vision

•  paradoxical enophthalmos (10% of orbital lesions)

=  posterior globe retraction ← infiltrative + fibrotic contraction of orbital fat ← scirrhous breast cancer (most common) / scirrhous gastrointestinal carcinoma

Location:   12% intraorbital, 86% intraocular; bilateral in ⅓

Preferential site by type of metastasis:

›  breast cancer → orbital fat + muscle

›  prostate cancer → bone

›  melanoma → muscle

√  diffuse enhancement of retrobulbar fat with abnormally heterogeneous hypointensity on T1WI + T2WI ← fibrotic infiltration

DDx:   thyroid ophthalmopathy (bilateral, sparing tendinous insertions); orbital pseudotumor (typically painful involving tendinous insertion); sarcoidosis

Choroidal Metastasis

[choroid = posterior portion of uvea]

◊  Most common ocular malignancy in adults!

Origin:  lung > breast, hypervascular + hematologic malignancy

Location:   posterior half of globe near macula (access via short posterior ciliary arteries); extension along plane of choroid (in ⅓ bilateral + in ⅓ multiple)

•  often asymptomatic (unless fovea involved)

•  visual loss ← retinal detachment

√  small areas of broad-based flat thickening + increased density

√  subretinal fluid = retinal detachment

√  mild posterior choroidal thickening


√  often multiple hyperechoic posterior wall masses: usually flat / discoid + with an irregular surface

√  higher flow than melanoma at Doppler


√  iso- to hyperintense on T1WI, hyperintense on T2WI

√  heterogeneous enhancement

√  may be T1 hyperintense + T2 hypointense ← high level of protein / hemoglobin degradation products ← metastasis from mucin-producing adenocarcinoma / hemorrhagic metastasis



=  X-linked recessive disease: ? inherited form of persistent hyperplastic primary vitreus

•  seizures, mental retardation (50%)

•  hearing loss, deafness by age 4 (30%)

•  bilateral leukokoria + microphthalmia

•  cataract, blindness ← absence of retinal ganglion cells

√  microphthalmia

√  dense vitreus with blood-fluid level

√  cone-shaped central retinal detachment

√  calcifications


Frequency:   3% of all visits to Emergency Department in USA

Cause:   blunt trauma (97%)

Mechanism:   motor vehicle accident, sport-related accident, industrial accident, fall, violent trauma

Associated with:   facial fractures (up to 11%), head injury (84%)

•  clinical evaluation: testing of visual acuity, slit-lamp evaluation of cornea + anterior segment, intraocular pressure measurement, funduscopy

US (used if ocular media opaque due to vitreous hemorrhage / hyphema / traumatic cataract):

◊  Dynamic imaging improves visualization of the entire eye and depicts movement of vitreous echoes / lines.

√  may depict hyphema, lens dislocations, globe rupture, intraocular foreign body, vitreous + retinal hemorrhage

Contraindications for ocular US:

suspected traumatic globe rupture / recent surgery to prevent extrusion of ocular contents

CT (modality of choice):

√  especially useful for foreign body


√  CONTRAINDICATED with metallic foreign bodies

√  usually reserved for subtle open-globe injury / organic foreign body

Anterior Chamber Injury

1.   Corneal laceration

Cause:   usually penetrating trauma

√  decreased anterior-posterior dimension of anterior chamber compared to a normal globe on CT

Cave:   anterior subluxation of lens may mimick a decreased anterior chamber volume

Cx:   globe rupture with complete penetration of cornea

2.   Traumatic hyphema

Cause:   disruption of blood vessels in iris / ciliary body

•  blood-fluid level in anterior chamber

√  increased attenuation in anterior chamber on CT

Cave:   US NOT RECOMMENDED → excessive pressure

Lens Dislocation


(1)  Blunt trauma to eye (> 50% of all lens dislocations)

(2)  Spontaneous (and often bilateral)

associated with connective tissue: Marfan syndrome, Ehlers-Danlos syndrome, homocystinuria

Pathomechanism:   deformation of globe in anteroposterior direction → compensatory expansion equatorially → stretching + tearing of zonular attachments → dislocation of lens

Location of lens:   posterior (common) / anterior (unusual)


(a)  partial luxation = one margin of lens maintains its normal position behind iris

√  posteriorly angled position of lens

(b)  complete luxation

√  lens in dependent portion of vitreous humor

In trauma associated with:   echogenic lens (= traumatic cataract) + vitreous hemorrhage

Traumatic Cataract

Pathophysiology:   disruption of lens capsule → edema within lens → cataract

√  hypoattenuating lens compared with nonaffected lens (in acute phase)

√  hyperattenuating / calcified lens (= mature cataract)

Open-Globe Injury = Ruptured Globe

=  disruption of scleral integrity by blunt trauma / penetrating injury

Site:     behind insertion of intraocular muscles (= thinnest portion of sclera)

•  enlarged anterior chamber

US: CONTRAINDICATED in suspected globe rupture

CT (56–75% sensitive):

√  direct signs of globe injury:

√  change in globe contour (DDx: coloboma, staphyloma, posttraumatic orbital hematoma)

√  “flat tire” sign = loss of volume

√  scleral discontinuity with prolapse of vitreus

√  indirect signs of globe injury:

√  deep anterior chamber = mild posterior movement of lens (in spite of intact zonular fibers)

Mechanism:   rupture in posterior segment → decrease in pressure + volume → allows lens to sink posteriorly

√  decreased anterior chamber depth = decreased volume in anterior segment ← severe corneal laceration

One indirect imaging finding of open-globe injuries is alteration of the anterior chamber depth.

√  intraocular air (DDx: injected perfluoropropane gas for treatment of retinal detachment)

√  intraocular foreign body (DDx: metal buckle of scleral band for treatment of retinal detachment)

◊  Consider MRI when a clinically suspected open-globe injury is not identified at CT!

Cx:     blindness

Nontraumatic mimics:

coloboma, staphyloma, congenital glaucoma, elongated globe from myopia, phthisis bulbi

Altered globe contour:

mass effect from orbital mass / hematoma

Ocular Detachments / Posterior Segment Injury

1.   Traumatic retinal detachment

=  separation of retina from choroid

Mechanism:   traumatic retinal tear → accumulation of vitreous fluid + blood between retina and choroid → detachment of retina

(a)  Total retinal detachment

√  characteristic V-shaped appearance with apex at optic disc

√  retina remains bound down at ora serrata

(b)  Focal retinal detachment

√  elevated immobile line close to sclera at periphery of globe

Retinal detachments may have a characteristic V-shaped appearance with the apex at the optic disc !

2.  Vitreous detachment

=  separation of vitreous from retina

√  thin undulated mobile line moving away from posterior aspect of globe during eye motion

3.   Traumatic choroidal detachment

=  separation of choroid from sclera by fluid accumulation in potential suprachoroidal space

Mechanism:   traumatic injury → decreased pressure in posterior segment + suprachoroidal space → accumulation of fluid / blood → detachment of choroidal layer

Site:   from vortex vein to ora serrata

√  biconvex / lentiform fluid accumulation

√  lentiform / biconvex shape with sparing of posterior portion of globe

DDx:   high-attenuation silicone oil injected between vitreus and retina for treatment of retinal detachment

Choroidal detachments have a lentiform / biconvex shape and spare the posterior portion of the globe !

Intraocular Hemorrhage

1.   Vitreous hemorrhage (53%)

Mechanism:   disruption of retinal blood vessels → hemorrhage into vitreous humor of posterior segment

•  “black rain” / decreased visual acuity (frequent)


√  normal / slightly increased vitreous echogenicity (= mild acute bleeding)

√  poorly defined low-level echoes / hypoechoic clots within vitreous (= abundant hemorrhage)

√  echogenic material moving freely within vitreous chamber during eye movement

CAVE:   Severe vitreous hemorrhage may obscure other ocular findings!


√  hyperattenuating fluid in posterior segment

Cx:   retinal detachment ← vitreous traction ← fibrovascular ingrowth following hemorrhage

Prognosis:   resolution may occur within 2–8 weeks

Rx:   vitrectomy

2.   Retrohyaloid hemorrhage (2%)

√  echogenic material remaining behind detached vitreous capsule during eye movement

3.   Hematoma in retroocular space

Intraocular Foreign Body

Incidence:  penetrating eye injury in 3.1÷100,000 per year

◊  Intraorbital foreign bodies are present in 10–17% of all ocular injuries + in 41% of open-globe injuries!

Cause:  violent trauma, motor vehicle accident, recreational accident, work-related industrial accident

Location:  anywhere (commonly in posterior segment)

Type: (a)   inorganic: metal, glass, plastic

(b)  organic: wood → may cause a severe infection

Radiography (40–90% sensitive, depending on type of material)

US (95% sensitive for intraocular + 50% for intraorbital foreign body):

√  echogenic spot

DDx:  intraocular air may mimic a foreign body

CT (up to 100% sensitive):

√  metallic fragments < 1 mm can be demonstrated

√  glass foreign body: (detection depends on location, size + type of glass)

√  96% of > 1.5-mm glass foreign bodies

√  48% of 0.5-mm glass foreign bodies

√  wood splinter:

√   Wood is initially of low attenuation similar to air

√  geometric margins

√  increase in density after 1–5 days


N.B.:   Failure to detect a metallic foreign body before performing MR may result in blindness!

√  more sensitive than CT in depicting organic material

Cx:     siderosis (if metallic), endophthalmitis, retinal toxicity, vision loss

DDx:   optic drusen; scleral plaques (calcifications along insertions of medial + lateral rectus mm. in elderly); calcified cataract; material for treatment of retinal detachment (scleral bands, silicon oil, gas)


[druse, German = geode]

=  accretions of hyaline material on / near surface of optic disc; often familial

Age:   patient with macular degeneration; also in young patient

•  usually asymptomatic

•  headache, visual field defects

•  pseudopapilledema

Location:   at junction of retina + optic nerve; bilateral in 75%


√  hyperechoic lesions at papilla + acoustic shadowing


√  hyperattenuating small flat / round calcification

Cx:   atrophy of optic nerve



=  isolated to single prechiasmatic optic nerve anywhere along optic tract ± extension to other optic nerve, chiasm, optic tract

◊ Most common cause of optic nerve enlargement
  primary tumor of optic nerve
  intraconal tumor of childhood
  CNS neoplasm in NF1

Frequency:   1% of all intracranial tumors; 4% of orbital masses; 80% of primary tumors of optic nerve

Path:   optic nerve is embryologically part of hypothalamus and develops gliomas instead of schwannomas

(a)  perineural spread = fusiform enlargement of optic nerve: boundary between tumor and nerve often indistinguishable, overlying dura stretched but intact

(b)  subarachnoid spread = tumor predominantly involves subarachnoid space surrounding a relatively spared nerve: infiltration through pia mater but contained by dura

Histo:   proliferation of well-differentiated astrocytes

=  low-grade glial neoplasm with cystic components

(a)  in children (most common): juvenile pilocytic astrocytoma = grade 1 WHO with spindle-shaped astrocytes having hairlike (pilocytic) processes ± eosinophilic degenerative cell processes (Rosenthal fibers)

(b)  in adults: glioblastoma

Mean age:   4–5 years; 1st decade (75%); rare in adults without NF1 (GBM); M÷F = 1÷2 to 2÷3

Associated with:   neurofibromatosis type 1 (NF1) in 10–33–50% (± bilateral optic gliomas)

◊  15–21% of children with NF1 have (often bilateral) optic nerve gliomas

◊  10% of all optic nerve gliomas are associated with neurofibromatosis

•  decreased visual acuity / visual-field deficit

•  optic disc edema, pallor, atrophy (axonal damage)

•  abnormal pupillary reflex; relative afferent pupillary defect

•  axial proptosis with larger masses (less common)

•  vision loss, strabismus

•  spasmus nutans (= high-frequency nystagmus of low amplitude associated with head nodding movement)

•  precocious puberty with accelerated growth (in 39% of only NF1 patients) ← involvement of optic chiasm + hypothalamus

Location:   any part of optic pathway (intraorbital optic nerve in 25–48%); unilateral (most common); bilateral / multifocal (PATHOGNOMONIC for NF1); involvement of chiasma (more common in sporadic cases without NF1)

Extension to:   intracanalicular + retrocanalicular optic nerve; lateral geniculate body and optic radiation (rare)

√  tubular / fusiform / excrescentic well-circumscribed homogeneous enlargement of optic nerve-sheath complex:

√  CHARACTERISTIC kinking / buckling of nerve

√  posterior extension to involve chiasm + hypothalamus in 25–60% (indicates nonresectability)

√  ipsilateral optic canal enlargement (90%) > 3 mm / 1 mm difference compared with contralateral side


√  iso- to slightly hypodense compared to normal optic nerve

√  variable contrast enhancement (less intense than meningioma)

√  calcifications (rare)


√  well-defined homogeneous mass of medium echogenicity inseparable from optic nerve

MR:   more sensitive than CT in detecting intracanalicular + intracranial extent

√  fusiform enlargement of nerve (without NF1):

√  effacement of surrounding subarachnoid space

√  tortuous / kinked / buckled diffusely enlarged optic nerve (NF1 patient):

√  tumor in subarachnoid space surrounds normal-sized optic nerve

√  lesion isointense to muscle on T1WI

√  lesion heterogeneously hyperintense on T2WI

√  rim of T2-hyperintensity at tumor periphery (mimicking expanded subarachnoid space) = arachnoidal gliomatosis ← leptomeningeal infiltration + proliferation

√  ± cystic spaces

√  calcifications (rare)

√  nerve indistinguishable from tumor (DDx to meningioma)

√  variable enhancement (in 50%):

√  additional intracranial findings:

√  other gliomas

√  macrocephaly

√  hydrocephalus ← aqueductal stenosis (almost exclusive to patients without NF1)

√  neurofibromatosis spots (= foci of T2 prolongation due to myelin vacuolization)

Cx:   precocious puberty ← hypothalamic impingement

Prognosis:   slow growth / stability over time; 87–97% 5-year survival

Rx:   conservative management; chemotherapy (for young patients), radiation therapy (for patients > 5 years of age)

DDx:   optic nerve sheath meningioma (middle age, hyperattenuating mass, plaquelike calcifications, hypointense on T2WI, intense enhancement, no intracranial extension along optic pathway)

Malignant Optic Glioma of Adulthood

Prevalence:   extremely rare; 30 cases in this century

Mean age:  6th decade; M÷F = 1.3÷1.0

Histo:  anaplastic astrocytoma / glioblastoma multiforme

•  rapidly progressive monocular visual loss culminating in monocular blindness within a few weeks

•  with retrograde tumor extension: contralateral temporal hemianopia, polyuria, polydipsia

√  focal / diffuse enlargement of optic nerve

√  hypo- to isointense on T1WI + hyperintense on T2WI

√  obliteration of subarachnoid space around affected portion of nerve

√  diffuse intense enhancement of optic nerve

√  thickening + abnormal enhancement of optic nerve sheath

Tumor extension:   optic chiasm, hypothalamus, basal ganglia, brain stem, medial temporal lobes, leptomeninges, ependyma

Prognosis:  < 1-year survival despite aggressive therapy

DDx:   (1)   Optic neuritis (demyelinating plaques elsewhere)

(2)  Perioptic meningioma (hypointense on T2WI, stippled calcifications, hyperostosis)

(3)  Sarcoidosis, lymphoma, orbital pseudotumor (moderately / markedly hypointense on T2WI)



Frequency:   2% of space-occupying orbital masses; < 2% of intracranial meningiomas

◊  2nd most common optic nerve tumor!

Mean age:   49 years; M÷F = 1÷4; slightly more aggressive in children

Occasionally associated with:

neurofibromatosis type 2 (usually in teenagers)

Origin:   meningothelial cells in arachnoid sheath of optic nerve in orbit / middle cranial fossa

Histo:   syncytial growth pattern composed of meningioma cells with indistinct cytoplasmic margins ± multiple ringlike psammoma bodies (= round calcifications)

•  classic clinical triad of retinal examination:

(1)  Painless slowly progressive loss of visual acuity over months

(2)  Optic nerve atrophy → progressive loss of vision

(3)  Optociliary shunt vessels = dilated connections between ciliary circulation + central retinal vessels ← long-term compression of central retinal vein

•  proptosis


(a)  orbit = intraoptic nerve sheath meningioma

(b)  in optic canal = intracanalicular meningioma

(c)  intracranial opening of optic canal = foraminal meningioma

(d)  middle cranial fossa

√  tubular (most common) / fusiform / eccentric (excrescentic) thickening of optic nerve

√  calcifications in 20–50% (HIGHLY SUGGESTIVE)

√  sphenoid bone hyperostosis


√  hypoechoic tumor with irregular border

CECT: enhancement is the rule

√  tumor enhancement around nonenhancing optic n.:

√   “tram-track” configuration on axial CECT = enhancing tumor on either side of spared optic nerve

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Jun 29, 2017 | Posted by in GENERAL RADIOLOGY | Comments Off on and Ocular Disorders

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