Imaging in orbital pathology by Dr. Amit rauniyar

1. IMAGING IN ORBITAL
PATHOLOGIES
• Nirmal Prasad Neupane
1
DR. AMIT RAUNIYAR
MD RESIDENT
RADIODIAGNOSIS & IMAGING

2. Bony boundaries
• Roof
• - orbital plate of frontal
bone (anteriorly)
• - lesser wing of sphenoid
bone (posteriorly)
• - separates the orbit from
the anterior cranial fossa
• Floor
• - separates the orbit from
the cavity of the maxillary
sinus
• - maxillary bone (mainly)
• - zygomatic bone
(anterolaterally)
• - palatine bone
(posteromedially)
2

3. Medial wall
- separates the orbit from the
nasal cavity, sphenoid and
ethmoid air cells
- ethmoidal labyrinth, frontal
process of maxilla, lateral
surface of lacrimal bone, body
of sphenoid bone
Lateral wall
- frontal process of zygomatic
bone anteriorly
- greater wing of sphenoid
bone posteriorly
- separates the orbital cavity
from the temporal bone
3

4. • Base (orbital opening)
• - frontal bone
• - zygomatic bone
• - maxilla
• Apex situated at the posterior end of
orbit (optic foramen opens and
transmits the optic nerve and the
ophthalmic artery from the optic canal)
4

5. Contents of the orbit
1. The Globe
2. Muscle cone
3. Optic nerve sheath complex
4. Lacrimal apparatus
5. Vascular and nerve
structures
6. Orbital fat
5

6. The Globe
• Cornea
• Anterior chamber
• Lens
• Vitreous
• Retina- sclero-choroid complex
6
• Embedded in fat, separated from it
by membranous sac - Tenon's
capsule or fascia bulbi
• Transparent anterior segment -
1/6th of eye ball.
• Opaque posterior segment - 5/6th
• Optic axis - central point of ant
curvature to post pole
• Normal axial length - 24 mm

7. Muscle Cone
•Divides the orbit into
intraconal & extraconal
spaces
•- 7 extra-ocular muscles
•Superior rectus
•Inferior rectus
•Lateral rectus
•Medial rectus
•Superior oblique
•Inferior oblique
•Levator palpebrae
superioris
7

9. Optic nerve sheath complex
• Optic nerve (diameter upto 4 mm is normal)
• Meningeal sheath
• CSF (SA space)
• - Optic nerve takes a sigmoid course from the apex to the
globe, so coronal imaging is essential to assess nerve –
sheath complex.
9

10. Lacrimal gland
Lacrimal gland: located in the
superolateral aspect of the orbit
•Secretary ducts
•Puncta & Canaliculi: superior
& inferior
•Lacrimal sac
•Nasolacrimal duct
10

11. Orbital fat
• All the remaining spaces are occupied by the
orbital fat which provides the natural contrast
between it & the other orbital structures.
11

12. Major orbital foramina
•Optic canal: optic nerve &
ophthalmic artery
•Superior orbital fissure: lies
at posterior part, junction
between roof and lateral wall
•3rd, 4th & 6th cr. nerves,
ophthalmic nerve (V1) &
vein, lacrimal & frontal
nerves
•Inferior orbital fissure: lies at
junction between lateral wall
and floor.
•maxillary nerve (V2),
zygomatic nerve &
infraorbital vessels
12

13. APPROACH TO ORBITAL PATHOLOGY
• The first thing is to decide whether it is an
ocular lesion or a non-ocular lesion, i.e. is it
involving the globe or involving the structures
outside the globe.
If it is a non-ocular lesion, the next question is
whether the lesion is located within the
intraconal space, i.e. within the space
bounded by the cone formed by the
extraocular muscles, or whether it is located
within the conal or extraconal space?

14. Contd
Once we have decided where the lesion is
located, consider the differential diagnostic
possibilities using the mnemonic VITAMIN C
and D.
We will first describe the anatomic spaces of the
orbit and summarize the pathology within these
spaces, even if some of these pathologies are
not visible radiologically.
Then we will discuss the radiological findings in
certain orbital diseases.

15. Contd
Ocular space
The eye has the following well defined anatomic
spaces:
Anterior chamber
When we move from anterior to posterior the first
area is the anterior chamber.
It is bounded by the cornea anteriorly and the lens
and iris posteriorly.
Specific pathologies within the anterior chamber
are:
Rupture of the globe
Hemorrhage: also known as anterior hyphema
Cataract
Keratitis: inflammation of the cornea
Periorbital cellulitis
Posterior chamber
This is a very small area posterior to the iris, which

16. Contd
Vitreous body
The larger area posterior to the lens is the
vitreous body.
Specific pathologies within the vitreous body are:
Rupture
Hemorrhage
CMV infection: especially in HIV
Persistent Hyperplastic Primary Vitreous (PHPV):
primary vitreous failed to develop into secondary
clear vitreous leading to blurred vision
Reinflation procedures for detachments leading to
different densities within the vitreous body

17. Contd
The vitreous body is surrounded by the membranes
of the retina, the choroid and the sclera.
Retina pathology:
Retinoblastoma: a common tumor in children
Hemangioblastoma: most common retinal tumor in
the adult and associated with von Hippel Lindau
disease
Detachment: most common retinal lesion mostly
seen in diabetic retinopathy
Choroid pathology:
Melanoma: choroid contains the melanin cells
Metastases: choroid is the most vascular structure in
the eye
Detachment: usually post-traumatic
Sclera pathology:
Infection: either due to sinusitis or viral
Pseudotumor
Detachment

18. Contd
Intraconal space
The ocular muscles within the orbit form a
muscle-cone.
These ocular muscles are connected via the
annulus of Zin, which is a fibrous connective
tissue sheet and together they form the conal
space.
It separates the intraconal from the extraconal
space.
Intra-orbital pathology which is non-ocular is
either in the intraconal, conal or extraconal
space.
Intraconal space pathology:
Venous vascular malformation
Capillary hemangioma
Optic nerve lesions
Optic neuritis
MS

19. Contd
• Conal space
The conal space is formed by the ocular
muscles and an envelope of fascia.
• Conal space pathology:
• Thyroid eye disease; usually enlargement of
the inferior and medial rectus
• Pseudotumor: idiopathic orbital inflammation
• Adjacent inflammation: sinusitis
• Uncommon causes of enlargement of the
extra-ocular muscles are glycogen storage
disease and lymphoma.

20. Contd
Extraconal space
The extraconal space is the area outside the
muscle cone.
Extraconal space pathology:
Abscess due to sinusitis
Schwannoma of the V1 and V2 branches of the
trigeminal nerve
Bone lesions:
Fibrous dysplasia of the sphenoid wing
Metastases
Multiple myeloma
Diseases of the orbital appendages

21. Contd
Orbital appendages
The lacrimal gland is located superolaterally in
the orbit.
Diseases of the lacrimal gland can be divided into
granulomatous, glandular and developmental
(see Table).
Secretions go medially across the globe and are
collected in the punctum and then go into
the lacrimal sac.
From the lacrimal sac secretions travel inferiorly
to the nasal lacrimal duct, which drains under the
inferior terminate into the nose.
In children congenital obstructions of the valves
in the lacrimal duct can lead to cystic areas
medially in the orbit also known as
dacryocystoceles.
In adults obstruction is more often due to
strictures from ethmoid sinusitis or stones
blocking the nasolacrimal duct.

22. Techniques of examination
• Imaging imp- pathology if not detected and treated –
loss of vision
1. Plain x-ray- orbital trauma, foreign bodies, PA , lateral
and half axial townes view
2. Dacryoocystography – congenital and acquired
disorders of lacrimal drainage apparatus.
3. Angiography – rare , done in suspected or proven
vascular anomalies of the orbit or middle cranial
fossa- carotico-cavernous fistula or dural
arterovenous malformation
4. Usg- evaluate the globe for intrinsic pathology-
remainder is not seen.

23. CT
• Intraorbital fat- good contrast
• Ct vs mri- osseous structure, calcification, less imaging time(less
sensitive to motion of globe and eyelid)
• Contiguous thin section – 2-3mm(axial) and 4-5 mm (coronal)- bone
and soft-tissue windows
• Coronal or oblique sag projection- can be reformatted from axial
sequences.
• Orbital apex evaluation- very thin 1.5 mm is taken in coronal plane
• Most imp image – coronal image- ONS , vessels and globe
• Contrast study- inflammatory , infectious , neoplastic and vascular
lesion.
• Orbital varix- suspected- CT done without and with valsalva
maneouvre. If valsalva cannot be performed- imaging done in prone
position.

24. • MRI : axial orbital roof to floor, coronal back of pons through
globe, T1 pre-contrast axial and coronal and post contrast with
fat saturation and and T1 C+
• Optimal soft tissue contrast for evaluation of globe, optic nerve,
orbital structures and intracranial extension

25. ORBITAL PATHOLOGIES
• Infections
• Inflammatory conditions
• Vascular orbital abnormalities
• Retinal and choroidal detachments
• Calcifications
• Trauma to orbit
• Neoplastic
• Lacrimal gland pathologies
25

26. PROPTOSIS VS EXOPHTALMUS
• Proptosis abnormal protrusion of globe. Exophthalmus- abnormal
prominence of globe, severe proptosis(>18mm of proptosis).
• CT
• Assessment of proptosis on cross-sectional imaging is difficult and
dependant on the study being acquired in the correct plane:
• the plane of the study must be parallel to the head of the optic
nerve and the lens
• the patient must have their eyes open and be looking forward with
no eye movement
• The reference line for measurement of proptosis is
the interzygomatic line (a line is drawn at the anterior portions of
the zygomatic arches:
• the distance from this line to the anterior surface of the globe
should be less than 23 mm

28. INFECTIONS
• Orbital infections represent more than half of primary orbital
disease processes.
• The location of an orbital infection is described with respect to
the orbital septum, as either preseptal (periorbital) or postseptal
(orbital).
• The orbital septum is a thin sheet of fibrous tissue that originates
in the orbital periosteum and inserts in the palpebral tissues
along the tarsal plates.
• The orbital septum and muscle cone provides a barrier against
the spread of periorbital infections into the orbit proper, although
valveless veins of face and orbit allow the spread of
thrombophlebitis across these planes.
• The distinction between periorbital and orbital processes is
clinically important because postseptal infections are treated
more aggressively to prevent devastating complications such as
cavernous sinus thrombosis and meningitis.
28

29. • Periorbital cellulitis, which is defined as a preseptal
process limited to the soft tissues anterior to the orbital
septum, most commonly arises from the contiguous
spread of infection from adjacent structures such as the
face, teeth, and ocular adnexa.
• It also may arise from local trauma.
• Cross-sectional imaging demonstrates diffuse soft-tissue
thickening anterior to the orbital septum.
• Periorbital cellulitis in adults typically is treated with
antibiotics on an outpatient basis.
29

30. Periorbital cellulitis
30

31. Orbital cellulitis
• Orbital cellulitis is a postseptal infectious process most commonly
caused by paranasal sinusitis which spreads to the orbit via a
perivascular pathway. Thus, bone destruction is not usually seen.
• Treatment of orbital cellulitis typically requires the intravenous
administration of antibiotics.
• Development of an orbital subperiosteal abscess is most
commonly associated with ethmoid sinusitis.
• Drainage of the abscess may be necessary to avoid a rapid
elevation of intraorbital pressure and resultant visual impairment.
31

32. Complications of orbital cellulitis
• Thrombosis of the superior ophthalmic vein, the
cavernous sinuses, or both
• bacterial meningitis
• epidural and subdural abscess
• parenchymal brain abscess
• Frontal sinusitis may cause periorbital cellulitis or frontal
bone osteomyelitis with a secondary extracranial abscess
known as a Pott puffy tumor
32

33. Orbital cellulitis
33

34. 34

35. 35

36. Dacryocystitis
• Dacryocystitis is inflammation and dilatation of the lacrimal
sac, which is located along the inner canthus .
• Although the diagnosis of dacryocystitis is based on clinical
manifestations, imaging may be requested to exclude
orbital cellulitis.
• The typical imaging finding is a well circumscribed round
lesion that is centered at the lacrimal fossa that
demonstrates peripheral enhancement.
36

37. 1
37

38. CMV retinitis
• Cytomegalovirus (CMV)-induced retinitis occurs in approximately one-
third of patients with acquired immunodeficiency syndrome (AIDS)
• CMV-induced retinitis is usually diagnosed ophthalmologically; however,
it may be an important incidental finding at imaging in the population
with AIDS.
• Radiologically, it manifests as uveal and retinal enhancement, retinal
detachment, and calcifications in the retina.
• CMV-induced retinitis most commonly begins in one eye and
progresses to involve the contralateral eye.
• Without treatment, CMV-induced retinitis causes permanent blindness
in most patients within 3–6 months .
38

39. 39

40. INFLAMMATIONS
40

41. Graves ophthalmopathy
• Most common cause of exophthalmos in adults.
• Orbital findings include lid retraction, proptosis,
ophthalmoplegia, conjunctivitis, and chemosis .
• In Graves ophthalmopathy, classically spindle-shaped
enlargement of the extraocular muscles is observed,
with sparing of the tendinous insertion.
• The inferior, medial, superior, and lateral rectus muscles
(listed in order of decreasing frequency of involvement)
may be involved .
• These findings are usually bilateral and symmetric;
however, they also maybe unilateral .
41

42. • Additional imaging findings include increased orbital fat, lacrimal
gland enlargement, eyelid edema, stretching of the optic nerve, and
tenting of the posterior globe.
• The presence of chronic extraocular muscle atrophy, fibrosis, and
intramuscular fat deposition may be helpful in diagnosing Graves
ophthalmopathy .
42

43. 43

44. Idiopathic orbital inflammatory
syndrome
• Idiopathic orbital inflammatory syndrome, also known as orbital
pseudotumor, is the second most common cause of exophthalmos.
• Most common cause of orbital mass in adults.
• A nongranulomatous orbital inflammatory process with no known local
or systemic cause, although autoimmune cause is suspected
• This syndrome is diagnosed by excluding other possible causes of
exophthalmos.
• Diagnosis is based on the medical history, clinical course, results of
laboratory testing, and response to steroids.
44

45. • The symptoms include unilateral painful
proptosis and eyelid swelling, typically with a
sudden onset, and, occasionally, associated
diplopia and decreased vision.
• Although unilateral presentation is most
common, may be bilateral.
• All compartment may be affected including
lacrimal gland.
• The radiographic features of idiopathic orbital
inflammatory syndrome vary widely and
include orbital fat stranding, myositis , a focal
intraorbital mass, lacrimal gland inflammation
and enlargement, diffuse orbital involvement,
and involvement of the optic nerve sheath
complex, uvea, and sclera.
45
• Acute forms present
with pain, proptosis
and diminished ocular
mobility, with
histological changes
similar to vasculitis.
• Chronic form may
mimic infections and
lymphoma, both
clinically and
hisologically.

46. • In idiopathic orbital inflammatory syndrome, unlike Graves
ophthalmopathy, there is tendinous involvement of the extraocular
muscles.
• Steroid therapy classically results in rapid improvement - helpful in
confirming the clinical and radiological diagnosis.
• Biopsy is reserved for those patients with atypical features such as
bony involvement or lack of response to medication; most often
lymphoma will be diagnosed in these cases.
46

47. 47

48. Features Graves’ Pseudotumor
1. Extent of
involvement
Bilateral, symmetrical
Enlargement of
extraocular muscles
(common), infiltration
of retro-orbital fat (less
common)
Unilateral
Involvement of
extraocular muscles,
lacrimal apparatus,
sclera, optic nerve
sheath, lid, fat & diffuse
involvement
2. Extraocular muscle
involvement
Only bellies involved,
tendons spared
Bellies as well as
tendons involved
3. Scleral thickening Absent Present
4. Order of involvement I M SLow No specific order
48

49. OPTIC NEURITIS
• Optic neuritis, which refers to inflammation or demyelination
of the optic nerve, often manifests with unilateral eye pain and
visual loss .
• Optic neuritis is often associated with multiple sclerosis, but
some occurrences have been described as idiopathic or as
associated with other processes (including systemic lupus
erythematosus, viral infection, and radiation therapy, infection)
.
• At T2-weighted MR imaging, acute optic neuritis typically is
manifested as hyperintense signal in an enlarged, enhancing
optic nerve, whereas chronic optic neuritis is classically
characterized by T2 signal hyperintensity in an atrophic,
nonenhancing optic nerve .
49

50. 50

51. Perineuritis
• Perineuritis, which is defined as inflammation of the optic nerve
sheath, may mimic optic neuritis clinically with orbital pain,
decreased visual acuity, and optic disc edema .
• At imaging, perineuritis is characterized by thickening and
enhancement of the optic nerve sheath.
• Because similar imaging findings may be seen in patients with
dissemination of tumor cells in the cerebrospinal fluid along the optic
nerve sheath, a careful clinical evaluation is essential for accurate
diagnosis.
51

52. 52

53. • Vascular abnormalities
53

54. Carotid-cavernous fistula
• A carotid cavernous fistula is an abnormal connection between the
carotid arterial system and the cavernous venous sinuses.
• This aberrant connection may result from trauma, surgery, or dural
sinus thrombosis; however, a cause is not always identifiable, and
some cases are idiopathic.
• Spontaneous development of a carotid cavernous fistula has been
reported in the setting of atherosclerotic disease, Ehlers-Danlos
syndrome, and osteogenesis imperfecta
• The cavernous sinus transmits arterial pressure to the ophthalmic
veins, producing pulsatile exophthalmos with an auscultable bruit,
conjunctival chemosis, venous engorgement, optic nerve stretching,
cranial nerve deficits, and visual disturbances.
• Imaging findings include proptosis, engorgement of the superior
ophthalmic vein , cavernous sinus distention, and abnormal flow
voids within the cavernous sinuses on MR images.
54

55. • Conventional angiography is necessary to identify the exact
location of the carotid cavernous fistula so as to plan definitive
treatment .
• Complications include vision loss and, in rare cases, ischemic
ocular necrosis
55

56. 56

57. Superior ophthalmic vein thrombosis
• Superior ophthalmic vein thrombosis is most commonly associated
with an infectious process such as paranasal sinusitis.
• Contrast-enhanced CT and MR images demonstrate filling defects
within the superior ophthalmic vein, often with associated
enlargement of both the superior ophthalmic vein and the cavernous
sinus, engorgement of the extraocular muscles, exophthalmos, and
periorbital edema.
• Potentially devastating complications of superior ophthalmic vein
thrombosis include vision loss, thrombosis of the cavernous sinuses,
and, if the cause of thrombosis is infection, sepsis.
57

58. 58

59. Orbital varices
• Orbital varices, the most common cause of spontaneous
orbital hemorrhage, are slow-flow congenital venous
malformations characterized by the proliferation of venous
elements and by massive dilatation of one or more orbital
veins
• Most orbital varices have a large communication with the
venous system, resulting in orbital varix distention and
increased proptosis during the Valsalva maneuver or postural
change.
• Prone to thrombosis and hemorrhage.
• Imaging findings of orbital varices may be subtle, and imaging
during the Valsalva maneuver may be necessary to elicit the
characteristic appearance. The lesions usually enhance
intensely after a contrast material is administered .
59

60. 60

61. Venous lymphatic malformations
• Venous lymphatic malformations are low-flow vascular abnormalities
that usually manifest in childhood .
• They appear as unencapsulated, multilobulated masses consisting of
vascular and lymphatic channels.
• Observations of an absence of communication with the systemic
circulation and presence of lesional stability during postural changes
help differentiate venous lymphatic malformations from orbital
varices.
61

62. 62

63. • Retinal and choroidal detachments
• Recognition of retinal and choroidal detachments
encountered in the emergent setting is crucial to patient
care, not for the evaluation of the detachment itself but
rather for the detection of a more ominous underlying cause
such as an intraocular tumor.
63

64. Retinal detachment
• A retinal detachment is a full-thickness tear of the retina with
subsequent movement of liquefied vitreous into the subretinal
space.
• Retinal detachments have a characteristic V shape, with the
apex of the detachment at the optic disc on cross-sectional
images.
64

65. 65

66. Choroidal detachment
• Choroidal detachment is defined as fluid accumulation in the
subchoroidal space, a condition that may occur after ocular surgery,
trauma, or an inflammatory choroidal process (uveitis).
• Choroidal detachment spares the region of the optic disc, in the
posterior third of the globe, because of the anchoring effect of short
posterior ciliary arteries, veins, and nerves in the ciliary body, where
choroidal arteries pierce the sclera.The sparing of this region gives
choroidal detachment its characteristic imaging appearance.
66

67. 67

68. Calcifications
• In adults the most common intraorbital
calcifications occur at the tendinous insertion of
the ocular muscles.
These are usually asymptomatic, but when the
ophthalmologist inspects the eye, there is the
impression of papilledema, i.e. pseudo-
papilledema.

69. In children calcifications in the globe means
retinoblastoma until proven otherwise even if
it is bilateral.
On the left an image of an adolescent with
bilateral retinoblastoma.

70. •
• Orbital calcifications are common incidental findings
that occur in characteristic locations, which helps
distinguish them from radiopaque intraorbital foreign
bodies. Frequently encountered calcifications include
trochlear calcifications, scleral plaques, optic drusen,
and phthisis bulbi.
70

71. Trochlear calcification
•Trochlear calcifications
may occur in adults as
aging-related normal
variants or may be seen
in young patients with
diabetes mellitus.
•They typically have a
superomedial location
within the orbit.
71

72. Scleral plaques and
pthysis bulbi
•Scleral plaques are
most commonly seen in
elderly patients and are
located at the insertion
sites of the medial and
lateral rectus muscles .
•Phthisis bulbi, a
shrunken globe with
ocular calcification or
ossification, is the
sequela of a wide variety
of pathologic ocular
processes, including
infection, inflammation,
and trauma.
72

73. Optic drusen
• Optic drusen are
typically seen in
patients with age-
related macular
degeneration
however, they also
may be seen in
relatively young
patients.
73

74. • Orbital Trauma
74

75. Orbital blow out fractures
• Orbital floor or medial wall fracture resulting from impact
of blunt object of diameter greater than orbital aperture
• Pure: Without orbital rim fracture
• Impure: With orbital rim fracture
75

76. • Bone CT
• Simple or comminuted fracture of orbital floor/medial wall,
with or without
• Herniation of orbital contents (fat, EOMs)
• Fracture through infraorbital canal
• Injury to orbital soft tissues (globe rupture, retrobulbar
hematoma)
• Significant orbital emphysema more common in medial wall
fractures
• Stretching/compression of optic nerve may occur
• Related air-fluid level or sinus opacification may be noted
• May occur in combination with other facial fractures, e.g.,
nasal, transfacial (LeFort), zygomaticomaxillary complex
(ZMC)
76

77. 77

78. • Globe injury:
• Laceration and rupture well seen on CT as deformation
of globe with decreased ocular volume
• Enucleation more common with lateral wall fracture
• Lens dislocation
• Hemorrhage: amorphous soft tissue density within
anterior or posterior chambers or in the orbital fat
• Retinal and choroidal detachment -
78

79. • Optic nerve injury
• May be seen as discontinuity
• More often inferred from clinical findings and presence of
perineural hematoma
• MRI may show focal injury as T2 high signal, which may
enhance on post contrast study
• Foreign bodies
• Xray may demonstrate, but CT more helpful for detection of
smaller fragments and their relationship to globe and optic
nerve
• Density varies with the nature of foreign body
• Metallic foreign bodies must be excluded before undergoing
MRI
79

80. NEOPLASTIC LESION

81. • Schematically, orbital tumors can be classified
based on origin:
1)primary lesions, which originate from the orbit
itself;
2) secondary lesions, which extend to the orbit
from neighboring structures and include such
lesions as intracranial tumors and tumors of the
paranasal sinuses that, by contiguity, extend to
involve the orbit; and
3) metastatic tumors.

82. Retinal origin
• Retinoblastoma: a common tumor in children
• Hemangioblastoma: retinal tumor in the adult and
associated with von Hippel Lindau disease.
Choroidal origin
•Melanoma: choroid contains the melanin cells
•Metastases: choroid is the most vascular structure in the eye

83. Intraconal space
•Cavernous hemangioma
•Capillary hemangioma
•Lymphoma
•Metastasis
•Rhabdomyosarcoma (children)
•Hemangiopericytoma
•Neurofibroma/schwannoma
(cranial nerve III, IV, VI)
•Ectopic meningioma

84. Optic Nerve Sheath Lesions
• Optic nerve glioma
• Meningioma
• Neurofibroma
• Schwannoma
• Lymphoma/leukemia
• Metastasis
• Hemangioblastoma
• Hemangiopericytoma

85. Extraconal space tumours:
Metastasis
Primary malignancy from adjacent
structures
Benign mixed tumor (lacrimal gland)
Adenoid cystic carcinoma (lacrimal
gland)
Non-Hodgkin's lymphoma
Rhabdomyosarcoma (children)
Schwannoma of the V1 and V2
branches of the trigeminal nerve
Bone lesions:
Fibrous dysplasia of the sphenoid wing
Multiple myeloma

86. Retinoblastoma
• Retinoblastoma is one of the common tumors in the first year of life
(11% of cancers in the 1st yr of life).
• Incidence: 1:17000-24000 live births worldwide.
• Bilateral in 25-30% of cases
– 10 % are inherited
– All hereditary tumors associated with tumor suppressor gene Rb(13q14).
• 90% calcify
• Presents with leucokoria

87. • Trilateral retinoblastoma (6%)= bilateral
retinoblastoma + pinealoblastoma
• Quadrilateral retinoblastoma = trilateral +
suprasellar or parasellar tumor.
• 1/5th of treated patients develop secondary
neoplasm, especially at the radiation site.

88. • USG-Intraocular masses of varying size & echogenicity with
calcification.
• CT diagnostic procedure of choice - detects calcification (only
10% lacks calcification), delineates mass, extraorbital
extension.
• Any calcification within the globe in CT in pediatric age group
should be considered as retinoblstoma until proven
otherwise.
• MRI :detect extension into the region of optic canal as well as
parenchymal lesion in brain. Superior in evaluation of
transcleral, or perineural spread.
T1W & PD- mildly hyperintense to extraocular muscle.
Mod- marked enhancement of noncalcified soft tissue.
T2W- increased signal intensity.

89. Retinoblastoma with intracranial extension. Axial CECT reveals a
left intraocular mass with multiple foci of calcification with
extension along the optic nerve (a) to the suprasellar area (b)

90. • Retinoblastoma.
MRI reveals a left
intraocular mass
which is isointense
on T1W (a) and
markedly
hypointense on
T2W (b) images
showing moderate
contrast
enhancement (c)

91. Trilateral retinoblastoma. Axial CECT shows
bilateral intraocular masses with calcification (a)
with a separate intensely enhancing mass in the
pineal location (b)

92. • Always examine the brain in these patients
and remember that at the age of 0-4 years,
which is the peak age for retinoblastoma, the
pineal gland does not calcify, so any
calcification in this region is suspicious of
retinoblastoma

93. • Small retinoblastomas are treated with different
kinds of therapy (cryoablation, laser
photocoagulation, chemothermotherapy,
brachytherapy, plaque radiotherapy) in order to save
the eye and avoid enucleation.
• If the patent is treated with radiation, there is a 30%
chance of a second malignancy within the radiation
field, due to the radiation and the deficient tumor
suppression gene.

94. • Outside the radiation field there is an 8%
chance of malignancy.
In order of frequency: Osteosarcoma >
other sarcoma > melanoma > carcinoma

95. • Unilateral
retinoblastoma. Axial
CECT shows a right
intraocular mass with a
large chunk of
calcification. There is
no evidence of
retrobulbar spread.
• When a retinoblastoma
occupies more than
half of the globe, as in
this case, the eye has
to be enucleated.

96. MALIGNANT MELANOMA
• Most common primary ocular malignancy in adults.
• Origin- choroid (85-93%)> ciliary body (4-9%)> iris (3-6%)
• Types: melanotic and amelanotic
• Almost always unilateral
• Highly invasive with high rate of recurrence when there is
extraocular spread.
• Metastasis-Hepatic (90%), Pulmonary (25%), Osseous (15%),
cutaneous, CNS

97. CT
• Homogenous dense
soft tissue mass
extending into
vitreous cavity.
• Moderate
enhancement
• Image (CECT)-
Enhancing lesion of
medial retina of left
eye with trans-
scleral invasion.

98. MR appearance
• Has dome shaped, collar
botton appearance (due
to nodular growth
through rupture Bruch’s
membrane)
• Melanotic type :
strong hyperintensity relative
to the vitreous on the T1-
weighted section (black
arrow) (due to paramagnetic
effect of melanin) and strong
hypointensity relative to the
vitreous on the T2*-weighted
section.

99. Vascular tumours
• Vascular lesions account for 5%–20% of orbital
lesions and hemangioma and lymphangioma are
the most common vascular tumours in the orbit.
• Hemangiomas can be classified into two distinct
entities:
1. cavernous and
2. capillary.

100. • Mostly located in the skin, but also seen in the
extraconal compartment of the eye.
• Hemangiomas are usually lobulated, irregularly
marginated, and heterogeneous and demonstrate
intense homogeneous enhancement at CT after
the administration of contrast material.
• Lobules with thin septa, combined with
intralesional and perilesional flow voids on MRI are
characteristic features.

102. Optic Nerve Glioma
• Actually optic nerve glioma is misnomer, it can present anywhere
along the optic tract from the occipital region to the chiasm and
the optic nerve.
• These tumors are juvenile pilocytic astrocytomas WHO type 1,
which is the most benign form of astrocytoma.
• They make up 4% of all orbital tumors.
• More than 50% of patients who have an optic nerve glioma have
NF1, but in NF1 only about 10% have optic nerve glioma.
• Bilateral mass has high degree of association with NF-1.

103. • The mean age at diagnosis is 4-5 years and only
20% of these patients have visual symptoms,
because the glioma does not affect the optic nerve
early and because these small children do not
complain of vision problems.

104. Bilateral optic nerve
glioma. T2W axial MRI
reveals thickening and
characteristic buckling of
bilateral optic nerves in a
known case of neuro
fibromatosis type 1.

105. Optic nerve glioma. Axial CECT (a) shows moderately enhancing
diffuse tubular thickening of the right intraorbital optic nerve.
(b different patient) There is marked fusiform enlargement of
the left optic nerve causing anterior displacement of the globe.

106. Nerve sheath meningioma
• Meningioma of nerve sheath is a subdural growth
leading to progressive visual loss, papilledema,
optic atrophy.
• There is a strong association with NF-2.
• The pale disk is due to venous outflow impairment.
• Calcifications are seen in 20-50%.

107. A, Coronal contrast-enhanced MR image shows
optic nerve sheath meningioma (arrow). B,
Axial contrast-enhanced MR image shows
same patient as in A with tram-track
appearance (arrow).

108. Optic nerve tram track sign
• Meningioma of the nerve sheath
• Leukemia
• Lymphoma
• Seeding into the subarachnoid space
• Sarcoidosis
• Pseudotumour

109. Optic nerve sheath glioma Vs meningioma
Features Glioma Meningioma
1. Age Children (2-6years) Middle aged (females)
2. Appearance Fusiform/ tubular
enlargement of optic
nerve
Eccentric lesion/
enlargement of the nerve
sheath
3. Calcification Rare Common
4. Enhancement
(CT/MRI)
Rare, mild enhancement. Intense, homogeneous/
Tram track enhancement
(thickened meningeal
sheath separated by
CSF).
5. Intracranial extension Common (optic chiasma
& hypothalamus)
Uncommon
6. Association Neurofibromatosis 1 NF 2

110. Rhabdomyosarcoma
• Most common mesenchymal tumor in children,
accounting for about 5% of all childhood cancers ,
and the most prevalent extraocular orbital
malignancy in children.
• Primary orbital rhabdomyosarcoma most often
occurs in the first decade of life, with a mean
patient age of 6–8 years

111. • On CT images, orbital rhabdomyosarcoma
generally appears as an extraconal, irregular, ovoid,
well-circumscribed, homogeneous mass that is
isoattenuated relative to muscle .
• In CT, the tumor can be seen to erode or thin bone
in about 40% of patients
• Moderate to marked, generalized enhancement
• Occasionally, invasion of the adjacent paranasal
sinuses or intracranial contents may be seen on MR
images
• Metastases are hematogenous, most often to the
lungs and bones .

112. Sagittal
T1-weighted magnetic resonance (MR) image shows a well-circumscribed extraconal mass
(arrowhead) superior to the ocular globe and isointense relative to muscle. (b) Another sagittal
MR image shows that the mass is separate from
the superior rectus muscle (arrow)

113. On the axial T2-weighted image, the mass is heterogeneous in signal intensity
and predominantly hyperintense relative to gray matter and muscle. Coronal T1-weighted
image obtained after intravenous administration of gadolinium-based contrast material
reveals intense enhancement of the tumor.

114. Lymphoma
• Lymphoma is the most common neoplasm in the
orbit, accounting for just more than half of all
cases.
• B-cell lymphomas of the non-Hodgkin's type are
by far the most common
• Usually, orbital lymphomas are primary to the
orbit, but occasionally orbital manifestation of a
systemic lymphoproliferative process is seen.
• The usual appearance is a well-defined
homogenously enhancing mass within the muscle
cone . Less frequently, extraconal masses or diffuse
infiltration of the orbital fat can be seen.

115. Figure Orbital lymphoma. A, CECT scan
demonstrates a homogeneously enhancing
intraconal mass (black arrow) adjacent to the left
optic nerve, causing medial deviation of the nerve
(white arrow). B, Axial postgadolinium fat-
suppressed T1-weighted image confirms the CT
findings (long arrow shows the enhancing mass;
short arrows show the optic nerve). C, Coronal
postgadolinium fat-suppressed images more
clearly demonstrate the enhancing mass (long
arrow) separate from the nonenhancing left optic
nerve (short arrow). M, extraocular muscles.

116. Metastatic Disease
• Secondary tumours of globe are more common than
primary malignancy in adults.
• Metastatic breast cancer is the most common type to
metastasize to the orbit, accounting for 48%–53% of
orbital metastases, followed by metastatic prostate
carcinoma, melanoma, colon and lung cancer.
• In children, most common primary lesions include
neuroblastoma, leukemia, and Ewing's sarcoma.
• Metastatic lesions may affect any of the intraorbital
structures as well as the bony orbit itself .

117. • The findings may be subtle, with small areas
of focal thickening of the globe, or large
destructive lesions.
• In addition, extension of tumor from an
adjacent structure (e.g., the paranasal
sinuses) may occur .
• Proptosis and motility disturbances are
among the most common presenting signs.
• Paradoxical enophthalmos may be present
in primary disease that is often associated
with extensive fibrous response, such as
scirrhous carcinoma of the breast.

118. Lacrimal Gland Masses
• Lacrimal gland masses represent 5%–14% of
orbital masses.
• Approximately half of these lesions are benign
and half are malignant.
• Masses of the lacrimal gland may be categorized
as epithelial or nonepithelial processes.
• Epithelial lesions compose 40%–50% of lacrimal
masses and are largely neoplastic.
• Nonepithelial lesions predominantly include
inflammatory (dacryoadenitis) and neoplastic
(lymphoproliferative disease) processes.

119. Epithelial Lesions
• PLEOMORPHIC ADENOMA.—Pleomorphic adenoma is the most common
benign neoplasm of the lacrimal gland, accounting for up to 57% of epithelial
lesions. Also called a benign mixed tumor, pleomorphic adenoma contains both
mesenchymal and epithelial elements.
• Pleomorphic adenomas are slow-growing tumors that most often manifest in
the 4th or 5th decade of life.
• At CT and MR imaging, pleomorphic adenoma appears as a well-
circumscribed, usually homogeneously enhancing mass in the superotemporal
orbit.
• Because of its slow growth, pleomorphic adenoma may demonstrate bone
remodeling, which most typically appears as a smooth concavity at the
lacrimal fossa.

120. Axial contrast-enhanced CT image of a 59-year-old man who
presented with right eye dryness shows a homogeneously
enhancing, well-circumscribed mass at the lacrimal fossa.
Rounded indentation at the zygomatic bone (arrow) reflects bone
remodeling caused by slow growth of the tumor.

121. Adenoid Cystic Carcinoma
• Most common malignancy of the lacrimal gland.
• Most patients present in the 4th decade of life.
• Adenoid cystic carcinoma is infiltrative, with a strong propensity for perineural
spread.
• . Irregular borders with distortion of the globe and orbital contents may be seen
in patients with more advanced disease. The finding of bone erosion suggests
the presence of malignancy, and calcification is also more commonly seen in
carcinoma than in benign adenomas.
• Cranial nerves, particularly the lacrimal branch of the ophthalmic nerve, should
be carefully examined for perineural invasion.

122. Adenoid cystic carcinoma in a 53-year-old woman who presented with progressive pain
and proptosis. Axial contrast-enhanced CT image shows a heterogeneous extraconal mass
at the superolateral orbit with medial displacement of the optic nerve (*) and marked
proptosis. There is erosion of the lateral orbital wall (arrowheads) and extension into
the temporal fossa (arrow).

123. • THANK YOU