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Dr. Mohit Goel JR III 3/07/2014
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Page 1: Orbital pathologies.pptx 1

Dr. Mohit Goel

JR III

3/07/2014

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1. Congenital anomalies

2. Infection / Inflammation

3. Tumors

4. Trauma

5. Neoplasm

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Congenital anomalies

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MIcropthalmia

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Pthisis Bulbi

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Coloboma

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Colobomatous cyst

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Ocular detachments

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Retinal detachment.

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Inflammatory conditions

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Acute inflammation of optic nerve , commonly associated with multiple

sclerosis.

Edema and inflammatory cells infiltrate the nerve resulting in uniform

swelling and focal demyelination.

Imaging : MRI is the modality of choice with hyper intense signal of

T2WI due to fluid and edema. Fat Sat contrast enhanced T1WI will show

areas of demyelination. CT relatively insensitive.

Straightening and thickening

of right optic nerve.

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Optic Neuritis. CE Fat Sat T1W axial

(B) MR images demonstrate subtle enlargement and enhancement of the left

optic nerve (curved arrow).

T 2 WI (C) demonstrates corresponding increased signal intensity (straight

arrow).

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Retinoblastoma

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Retinopathy of Prematurity

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Coat’s disease

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Primary retinal telengiectasis

Vascular anomaly of retina

Characterized by idiopathic retinal telengiectatic and aneurysmal retinal vessels

Progressive deposition of intraretinal and subretinal exudates

Leads to massive exudative retinal detachment

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Ocular astrocytic Hamartoma

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Benign yellow-white rare retinal tumor

Associated with Tuberous Sclerosis or Neurofibromatosis

Early on it may look exactly like retinoblastoma

May involve retina or optic nerve

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Juvenile Xanthogranuloma

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Benign cutaneous disorder

Affects eye and skin

Affects iris and ciliary body, choroid, retina and optic nerve

May present as a solitary orbital mass

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Optic nerve head drusen

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Tumors

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Choroidal osteoma

Benign tumor

Unilateral usually

Affects young white girls

Patients present with painless progressive loss of vision

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Malignant uveal melanoma

More common in whites

May arise from pre-existing nevi

Metastasizes hematogeneously to liver

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Orbital Pseudotumor

• The most common cause of orbital mass in adults.

• Acute form presents with pain, proptosis and

diminished ocular mobility, with histological changes

similar to vasculitis.

• Chronic form may mimic infection or lymphoma both

clinically and histologically. Unilateral presentation is

most common, but findings can be bilateral. All

compartments of the orbit may be affected.

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• Imaging -- Heterogeneous poorly marginatedincreased CT density and decreased T1 & T2 MRI signal intensity within the intraconal fat surrounding a thickened sclera or enlarged optic nerve, sometimes simulating a mass.

• The lacrimal gland may be enlarged. Enhancement occurs following contrast infusion.

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Fig A. Scleral pseudotumour. Marked thickening and irregularity of

the sclera of the right globe involves the adjacent retro-orbital fat.

Fig B. Diffuse pseudotumour. Axial MR T1WI showing a diffuse mass

in the right orbit due to pseudotumour.

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Rhabdomyosarcoma

• Rhabdomyosarcoma is the most common primary

orbital malignancy in the pediatric age group. with

most patients presenting below 6 years of age.

• Patients present with rapidly progressive

exophthalmos that may mimic orbital infection.

Spread of the tumor esp. intracranially, portends

poor prognosis.

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• Imaging : Both CT and MRI will typically show a mass involving an extra ocular muscle. Lesions are isodense on CT and isointense on T1WI when compared to muscle.

• There may be associated bony destruction and contiguous extra orbital spread. The tumor involves the globe less often. Marked enhancement throughout the mass is seen after contrast administration.

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Rhabdomyosarcoma. CECT image

(A) Orbits demonstrates right proptosis due to large, lobular, intraorbital

mass.

(B) Image at lower level demonstrates invasion of right maxillary

sinus (asterisk) as well as extension through lateral orbital wall (arrow),

consistent with the aggressive nature of this tumor.

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• Patients with abnormalities of the optic nerve and

its covering present with proptosis, visual loss and

papilloedema.

• Expansion of the tubular shaped optic nerve and

sheath is well demonstrated on CT and MRI.

• Imaging of the intracranial space is required

because the optic nerve and its coverings are

continuous with the brain and dura mater.

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Occur in children

Are low grade astrocytomas.

Associated with NF-1

Imaging:- optic nerve may expand uniformly and diffusely. Plain

films will show asymmetric widening of the optic canal. Post

contrast show uniform enhancement.

Optic nerve glioma.

Enhanced coronal CT image

demonstrates homogeneous

enhancement of enlarged right

optic nerve.

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Optic nerve glioma. Enhanced fat-saturated axial T1W image (A)

demonstrates mild enhancement and enlargement of intraorbital

and canalicular segments of left optic nerve.

Coronal image (B) confirms enlargement of nerve and surrounding perioptic

space.

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• Meningiomas are dense fibrous tumors. Calcification is

common.

• Plain films may show widening of the optic canal, or

hyperostosis of the sphenoid wing.

• CT generally shows a dense, sharply defined tubular mass

surrounding and paralleling the course of the optic nerve, with

marked enhancement after contrast administration ('tram

track'). Kinking of the nerve may be seen.

• MRI will show a homogeneous mass of decreased T1 and T2

signal intensity, with strong enhancement, especially with the

use of fat suppression sequences.

• Coronal images are preferred, as the encased optic nerve will

be seen in relief against the densely enhanced tumor.

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Fig A. Optic nerve Meningiomas. CT -- Enhancement of thickened right

optic nerve with elevation of optic disc (arrowhead).

Fig B. Axial T1-weighted post contrast fat-saturated image (B) demonstrates

peripheral enhancement of the thickened right optic nerve sheath.

Nonenhancing soft tissue within represents the encased optic nerve.

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Trauma

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Miscellaneous

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Thyroid ophthalmopathy.

Unenhanced axial (A) and coronal (B) CT images demonstrate massive

enlargement of the rectus muscles, including fusiform enlargement of the

lateral rectus with relative sparing of the distal muscle insertion.

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Lymphoma. T1WI (A) demonstrates proptosis of right globe due to a large

intermediate signal intensity lesion that involves the lacrimal fossa and the

right lateral rectus muscle (arrow), with extension posteriorly in the

extraconal compartment.

Post contrast image (B) demonstrates homogeneous enhancement.

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Cavernous Hemangioma. T1-weighted axial

(A) and sagittal (B) MR images demonstrate proptosis of right globe due

to well circumscribed, mid to high signal intensity intraconal mass.

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Lymphangioma.

Axial T,-weighted (A) and T2 -weighted (B) MR images demonstrate mild right

proptosis due to complex, multi loculated, cystic, extra-axial lesion in the

superomedial aspect of the right orbit.

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Encephalocele. Axial T1W MR image demonstrates marked

proptosis of right globe with stretching of attenuated right optic

nerve (arrowhead) due to herniation of dura and temporal lobe

through a large sphenoid defect in this patient with

neurofibromatosis.

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Plexiform neurofibroma. T1WI(A) and T2WI (B) MR images show extensive

left temporal scalp lesion with extension to left orbit resulting in mild

proptosis. MR also demonstrates ectatic left optic nerve (arrow).

CT image at bone windows (C) demonstrates associated bony defect of left

lamdoid suture.

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Metastases to the orbit may occur from systemic primaries,

particularly neuroblastoma and leukaemias in children, and breast, lung,

prostate and stomach cancer in adults. These lesions are poorly defined,

infiltrative and demonstrate marked contrast enhancement

on CT and MRI.

Prostatic Ca: Axial CT (A) shows small lytic lesion of left lateral orbital wall.

Soft-tissue windows (B) demonstrate contiguous extension of soft tissue into

lateral extraconal compartment (asterisk) with medial displacement of the

lateral rectus muscle.

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These are congenital lesions that result from sequestration of primitive

ectoderm in the region of the orbit, usually presenting during childhood as a

discrete mass, located near the lacrimal fossa or nasal bone and are

homogeneous in appearance. The presence of fat is clearly seen on CT and

MRI. They do not enhance.

Lacrimal gland dermoid.

Coronal T 1WI demonstrate a well-

circumscribed lesion located in the

upper outer quadrant of left orbit.

High signal intensity is consistent with

fat.

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Thank You