OPTOM FASLU MUHAMMED

Orbital tumours` include tumour like

conditions such as inflammatory lesions,

cysts and lymphoid hyperplasia

A 70% of all orbital tumours originate from

orbital tissues while 30% invade the orbit

from adjacent structures or metastasis from

distant primary foci.

Space occupying lesions, involving the orbit,

produce symptoms and signs by

compression, infiltration and or infarction of

orbital structures .

In the final analysis, the clinical presentation

will result from displacement and or

dysfunction of the globe, optic nerve,

oculomotor nerves and blood vessels.

It is defined as forward displacement of the

eyeball beyond the orbital margins.

It is a common feature of all orbital tumours,

though it`s degree may vary.

Those within the muscular cone usually

produce axial proptosis.

Those outside the muscular cone tend to

push the eye outwards and in a direction

opposite to that lesion (eccentric)

Optic nerve involvement may result in :

a) Progressive visual loss associated with edema of disc

- in many patients visual loss may be minimal and of delayed onset . In such cases testing of colour vision may reveal subtle defects.

a) Unilateral transient visual loss which may occur in certain positions of gaze and clears when the direction is changed

c) A specific triad may develop in chronic

compression of the optic nerve

1. Loss of vision

2. Swelling of disc which resolves into optic

atrophy

3. Appearance of optociliary shunt veins

The tumours located in the orbital apex may

involve oculomotor nerves in early stage

even before causing proptosis.

Some tumours may involve one or two

muscles, till late in the disease .

Diplopia produce by orbital masses may be

neurogenic or myogenic and rarely it may be

a combination of both.

The mechanical restoration of ocular mobility

can be confirmed by performing certain

tests.

Forced duction or traction test

IOP increases on looking in the direction of

gaze limitation

Most of the lesions are painless

Pain is more frequent with malignant

tumours.

Lesions that involve cavernous sinuses and

paranasal sinuses are usually painful.

Abnormalities can occur depending upon the

involvement of the parasympathetic or

sympathetic nerves, but this is often marked

by involvement of oculomotor nerve palsy.

The presence of Marcus Gunn pupil is

suggestive of optic nerve compression.

DEVELOPMENTAL LESIONS

Pulsating exophthalmos results due to

defective development of sphenoid wing and

roof of the orbit.

These tumours result from embryonic ectodermal sequesrations.

These are rare , found inside the orbit, and most of them are located in the periorbitalregion.

Dermoids and epidermiods have the same histological features as seen elsewhere in the body.

They are invariably benign and can be excised with ease

It is a type of congenital lesion composed of

tissues normal to the location.

One type of the tissue may be predominantly

seen such as vascular tissue.

It is a non neoplastic disease of the bone

that affects children and young adults.

Craniofacial fibrous dysplasia is a benign

condition forming about 3% of all bone

tumours.

Dysplasia of frontal, sphenoid, ethmoid,

zygomatic and maxillary bones may involve

the orbit causing visual symptoms.

These lesions is usually painless

INFLAMMATORY LESIONS

Orbital cellulitis can remain as a diffuse inflammation or progress to loculation to form an abscess.

There is usually profound disturbance of oculomotor function , pain and constitutional symptoms.

Occasionally , the abscess may become chronic and lesion may manifest like benign space occupying lesion.

Pathology reveals diffuse suppurativeinflammation composed of neutrophils.

Orbital pseudo tumour or idiopathic

inflammatory pseudo tumour of the orbit is

the most common cause of an intra orbital

mass.

Pseudo tumour can occur throughout the

orbit from the region of lacrimal gland to the

orbital apex and thus produce varied clinical

presentations.

Swelling or puffiness of the eyelids, proptosis, orbital pain, restricted ocular movements, diplopia, chemosis and redness.

Most cases are unilateral, although both sides may be involved occasionally.

The condition typically affects individuals between 40 and 50 years; however, age is no bar.

Spontaneous remissions after a few weeks are known in pseudo tumour.

Recurrences are also common. In some patients severe prolonged inflammation may cause progressive fibrosis of the orbital tissues leading to a frozen orbit with visual impairment.

Orbital infections

1. Aspergillosis

2. Tuberculosis

3. cysticercosis

It is a fungal disease caused by aspergillus

fumigatus , the most common species.

Infection usually begins in the sinuses and

erodes into the orbital cavity.

The organisms have a tendency to invade

vessels and cause ischemic necrosis.

A necrotizing reaction destroys muscles,

bone and soft tissue

patients are present with Pain and proptosis

Necrotic areas with black eschar formation

may be seen on the mucosa of palate,

turbinates and nasal septum and skin of

eyelids

Diagnosis is made clinically and confirmed

by biopsy of the involved area and finding of

nonseptate broad branching hyphae.

Orbital tuberculosis is rare

Etiology shows caseating and non caseating

coalescing granulomas.

There may be variable degree of fibrosis.

Cysticercosis of orbit is very rare

They shows fibro cellular reaction with

pallisading histiocytes around the parasite

Faciomaxillary injuries may involve the orbit

by means of fractures of the orbital walls or

haemorrhages within the orbit.

Blowout fractures’ mainly involve orbital floor

and medial wall.

NEOPLASTIC LESION

1.Cavernous haemangioma

2.Capillary haemangioma

3.Lymphangioma

4.Meningiomas

It is the commonest benign orbital tumour among adults.

The tumour is usually located in the retrobulbar muscle cone. So, it presents as a slowly progressing unilateral axial proptosis in the second to fourth decade.

It may occasionally compress the optic nerve without causing proptosis.

Surgical excision of the tumour is undertaken since the tumour is well encapsulated, complete removal is generally possible.

It is generally single,bright red and smooth.

A benign tumour manifests usually in the first

five years of life and tend to regress

thereafter

Histologically , capillaries of small size are

found closely packed with no smooth muscle

in between.

It is an uncommon tumour presenting with

slowly progressive proptosis in a young

person.

It often enlarges because of spontaneous

bleed within the vascular spaces, leading to

formation of ‘chocolate cysts’ which may

regress spontaneously.

They are three types based on their origin

1. Intracranial meningiomas

- Meningiomas of middle cranial fossa

especially those of shenoid ridge are

notorious to cause proptosis

- Meningiomas of the anterior cranial fossa

may invade the orbital roof.

- Ectopic meningiomas from frontal sinus

may encroach into the orbit

2.Primary intraorbital meningiomas

- Are rare tumours which arise from the optic

nerve.

- This is most difficult to treat and has been

called the impossible meningioma.

3.Those which have no apparent connection

with optic nerve are occasionally found in the

orbit

1. Rhabdomyosarcoma

2. Adenoid cystic carcinoma

3. Lymphoma

4. Histiocytoma

5. Metastasis

6. Optic glioma

It is a highly malignant tumour of the orbit arising from the extraocular muscles.

It is the most common primary orbital tumour among children, usually occurring below the age of 15 years (90%).

It classically presents as rapidly progressive proptosis of sudden onset in a child of 7-8 years.

Massive proptosis due to rhabdomyosarcoma located in the superonasal quadrant (mimmicking acute inflammatory process).

Histologically, the tumours are of three types

1.Embyonal 2.adult pleomorphic 3.alveolar

Embryonal rhabdomyosarcoma is the most

common type of malignant tumour.

They are round, oval or stellate rhabdo

myosarcoma with mitosis in loose syncitium.

Cells contain dense eosinophilic cytoplasm

with striations.

It occurs in adults of either sex in the fourth

decade of life.

The lesion is remorselessly progressive ,

invading the adjacent tissues with a

characteristic tendency to spread along

perineural lymphatics.

They are usually found in the anterior orbit.

These may involve orbit, lacrimal glands,

lids and subconjunctival tissue and produce

varied clinical features.

They are essentially B cell lymphomas and

orbital lymphomas may be the first

manifestation of systemic lymphomas

This is a group of diseases characterised by

an idiopathic abnormal proliferation of

histiocytes with granuloma formation.

These diseases primarily affect children with

an orbital involvement in 20 % of cases.

These involve the orbit by haematogenous spread from a distant primary focus and include the following:

1. Neuroblastoma — from adrenals and sympathetic chain.

2. Nephroblastoma —from kidneys.

3. Carcinoma — from lungs, breast, prostate, thyroid and rectum.

4. Malignant melanoma — from skin.

5. Ewing’s sarcoma —from the bones.

6. Leukaemic infiltration.

It is a benign tumour arising from the

astrocytes.

It usually occurs in first decade of life

It may be intraorbital or intracranial

1.CAROTID CAVERNOUS FISTULA

2.AV MALFORMATION

It is a common cause of pulsating

exophthalmos.

This is quite often triggered by head injury,

but may spontaneous due to ruptured

intracavernous aneurysms.

Symptoms may be characterised by painful

pulsating proptosis with congestion and

edema of conjunctiva and eyelids.

Arteriovenous shunts are rare in the orbit

and mostly occur with whyburn mason

syndrome.

They present as pulsating exophthalmosis or

subarachnoid haemorrhage.

These as well as venous angiomas cause

proptosis that is affected by position.

GRAVE`S DISEASE

This term is coined to denote typical ocular

changes which include lid retraction, lid lag,

and proptosis .

It is the most common causes of proptosis

which mostly affects females.

Graves’ ophthalmopathy has an autoimmune

etiology.

Mucoceles are para nasal sinuses which

seen more frequently at frontal sinus.

Which can cause periorbital swelling , pain

and displacement of globe .

Sphenoidal sinus mucoceles cause

headache, peri or retro-orbital pain and

ophthalmoplegias due to extension into the

orbital apex and cavernous sinus.

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