DISORDERS OF THE ORBIT

ORBIT-- Pain, proptosis ORBIT Pain, proptosis

Six Ps: pain, proptosis, progression, palpation, pulsation,
and periorbital changes and periorbital changes.
Pain: inflammatory, infection, oribital hemorrhage,
li t l i l l d t d NPC malignant lacnimal gland tumors and NPC
Proptosis--Hertel exophthalmometer
Axial proptosis-an intraconal space-occupying lesion,
Eccentric proptosis--- an ant.space-occupying lesion.
ORBIT-- Progression ORBIT Progression
Days to weeks--idiopathic orbital inflammatory dis.,
cellulitis, hemorrhage, thrombophlebitis, thyroid
ophthalmopathy neuroblastoma metastatic tumors ophthalmopathy, neuroblastoma, metastatic tumors,
rhabdomyosarcoma, or granulocytic sarcoma.
O th t d id b i i d t Over months to years-dermoids, benign mixed tumors,
neurogenic tumors, cavernous hemanglomas, lymphoma,
fibrous histiocytoma, or osteomas.
ORBIT-- Palpation ORBIT Palpation
Superonasal quadrant--- mucoceles, mucopyoceles,
encephaloceles neurofibromas or lymphoma encephaloceles, neurofibrom as,, or lymphoma.
Superotemporal quadrant--- dermoids, prolapsed
lacrimal gland, lacrimal gland tumors, lymphoma,
or idiopathic orbital inflammation disease p
ORBIT-- Pulsation ORBIT Pulsation
Without bruits-neurofibromatosis,
i h l l l f h meningoencephaloceles, or removal of the
orbital roof. orbital roof.
With or without bruits-carotid cavernous
fistulas, dural arteriovenous fistulas, and
orbital arteriovenous fistulas orbital arteriovenous fistulas.
Clinical evaluation of orbital disease
1. History: (1)pain, (2)mode of onset of proptosis
2 Exclusion of pseudoproptosis: 2. Exclusion of pseudoproptosis:
(1) large ipsilateral globe,
(2) facial asymmetry (2) facial asymmetry,
(3) contralateral enophthalmos (blow-out fracture)
3. Evaluation of ocular motility: restrictive
myopathy (thyroid blow-out fracture) myopathy (thyroid, blow out fracture)
4. Visual acuity
5. Dynamic properties -valsalva maneuver or jugular
compression pulsation bruit compression,,pulsation, bruit
6. Forced duction test
7. Differential IOP test
Laboratory studies Laboratory studies
thyroid function (T3,T4, TSH); antithyroglobulin,
antimicrosomal antibodies(60%-70% of patients
with Graves disease) with Graves disease)
antineutrophil cytoplasmic antibody (ANCAs),
serum assay, Wegener granulomatosis
Dysthyroidophthalmopathy y y p p y
Classification
Eyelid retraction
Soft tissue Soft tissue
involvement
Proptosis
Optic neuropathy Optic neuropathy
Restrictive myopathy
Pathogenesis Pathogenesis
Hypertrophy of EOM
Cellular infiltration
Proliferation Proliferation
Management Management
Systemic steroids
Radiotherapy
Orbital decompression Orbital decompression
Orbital infection and inflammations
Preseptal cellulitis: affects children. secondary to
lid infection such as hordeolum, skin laceration or
an insect bite .
Examination: periorbital swelling, tenderness
Treatment: oral antibiotics Treatment: oral antibiotics
Orbital cellulitis: ethmoidal sinusitis, maxillary
sinusitis, dacryocystitis, dental infection, post-trauma.
Most common causative organisms: streptococcus
pneumonia, streptococcus aurcus, streptococcus
pyogenes;( age of 5 years--Haemophilus influenzae),
aspergillosis.
Examination--proptoisis, eyelids-swollen, erythematous,
warm, tender. EOM--restricted and painful. CBC, CT,
bl d d l lt blood and nasal cultures.
Treatment--- bacteria-systemic antibiotics; fungus--iv
h t i i B id i i amphotericin B, wide excision.
Orbital pseudotumor Orbital pseudotumor
idiopathic, non-specific, inflammatory, orbital disease
Presentation--20-50 years of age. Painful lid edema,
EOM--limitation proptosis. Children: 1/3 bilateral p p /
involvement.
Adult: bilateral--- associated with systemic disease Adult: bilateral associated with systemic disease.
Treatment--observation, systemic steroids, radiotherapy,
t t i d cytotoxic drugs.
Orbital myositis Orbital myositis
Unilateral ptosis, diplopia, redness and pain over
the involved muscle.
Examination-pain on attempted gaze into the Examination pain on attempted gaze into the
field of action of the involved muscle.
CT f if l t f th i l d l CT: fusiform enlargement of the involved muscle.
Treatment-systemic steroids or NSAID. y
Tolosa-Hunt syndrome
involve the orbital apex, sup. Orbital fissure, such as
inflammation vascular and traumatic lesions and tmuors inflammation, vascular and traumatic lesions, and tmuors.
Presentation-ipsilateral periorbital or hemicranial pain.
E i ti i l i l l i Examination--proptosis,ocular cranial nerve palsies,
pupillomotor dysfunction. Sensory loss of 1 and 2
divisions of V nerve.
Treatment---systemic steroids. y
Orbital tumors
Classification
V l Vascular
capillary haemangioma
h i cavernous haemagnioma
lymphagioma
li venous anomalies
Lacrimal gland
pleomorphic adenoma
(mixed-cell tumor) ( )
mailgnant
Lymphoproliferative Lymphoproliferative
Rhabdomyosarcoma
Cystic lesions
dermoid cyst--the common benign tumor
blood cyst
mucocele
Neural
optic nerve glioma
optic nerve sheath meningioma
Metastatic
Tumor its vasion from adjacent structures j
Capillary haemangnioma p y g
the most common primary benign tumor of the orbit
and periorbital areas of childhood. and periorbital areas of childhood.
Presentation-during the perinatal period with a
superior peniocular swelling or proptosis or a superior peniocular swelling or proptosis, or a
combination of both.
Examination---seen in the fornices on everting the Examination---seen in the fornices on everting the
lid.Superficial straw-berry naevi may also be
found on the eyelids or other parts of the body found on the eyelids or other parts of the body.
N l d i h fi f lif Natural course---during the first year of life,
it starts to involute spontaneously Complete p y p
resolution
Occurs in about 75% of cases by age 7 Occurs in about 75% of cases by age 7.
Association-subglottic haemangiomas,
thrombocytopenia, high-output cardiac
failure.
Indications for Treatment Indications for Treatment
Threat of visual impairment: Threat of visual impairment:
(1) most commonly secondary to induced
astigmatismand anisometropia astigmatism and anisometropia.
(2) optic n. compression or
(3) exposure keratopathy.
A severe cosmetic blemish A severe cosmetic blemish
Severe necrosis of infection
High output cardiac failure High-output cardiac failure
Methods of treatment Methods of treatment
(1) Steroid injection,
(2) Systemic steroids,
(3) Local resection (3) Local resection,
(4) Radiotherapy
C h i Cavernous haemangnioma
the most common primary benign orbital tumor in
adults adults.
Presentation--during middle age with a
l l i il t l t i I th th t slowly progressive unilateral protosis. In women the growth rate
may be accelerated by pregnancy.
Examination--axial proptosis, may be
associated with optic disc edema and chorioretinal folds.
Treatment --surgical excision.
Lymphangnioma Lymphangnioma
benign vascular tumor
tendency to recurrent hamorrhage, poorly
circumscribed---frustrating for surgeons. circumscribed frustrating for surgeons.
Presentation---in childhood or early adult life
E i ti A t T ti Examination--Ant. Tumor: cystic
conjunctival component in the upper nasal quadrant.
Post. Tumor: sudden onset of painful protosis and spontanous
hamorrhage within the tumor--chocolate cysts. hamorrhage within the tumor chocolate cysts.
Treatment---surgery is difficult. Chocolate
t d i d C b di id l bt t ll i i cysts-drained. Carbon dioxide laser--subtotally excision.
Orbital venous anomalies Orbital venous anomalies
Arteriovenous malformations (AVM): dilated
corkscrewepiscleral vessels corkscrew episcleral vessels.
Treatment-selective occlusion of the feeding vessels,
followed by surgical excision followed by surgical excision.
Arteriovenous fistulas (AVF):
Cause: (1) Basal skull fracture-most commonly cause Cause: (1) Basal skull fracture most commonly cause.
(2) degeneration--H/T. atheroselerosis.
Carotid cavernous fistula--AVF of the orbit and
cavernous sinus.
Orbital varices:
Cause: dilation of preexisting venous channel.
Valsalva manuver---proptosis p p
Treatment---conservation
Pleomorphic lacrimal gland adenoma Pleomorphic lacrimal gland adenoma
most common epithelial turnor-benign mixed-cell tumor.
Presentation-adult life with painless smooth firmnon-tender Presentation-adult life with painless, smooth, firm non-tender,
slowly growth in the upper outer quadrant ) 1 year's duration.
Examination--most cases, arsing from the orbital portion of
the lacrimal gland. CT scan bony excavation of the lacrimal gland g y g
fossa.
Treatment-- lateral orbitotomy Treatment-- lateral orbitotomy
Malignant lacrimal gland tumor Malignant lacrimal gland tumor
adenoid cystic carcinoma
pleomorphic adenocarcinoma pleomorphic adenocarcinoma
mucoepidermoid carcinoma
Presentation incomplet or piecemeal excision of a benign Presentation-incomplet or piecemeal excision of a benign
pleomorphic adenoma; rapidly growing lacrimal gland mass.
Examination--CT, MRI, biopsy, nerological , , p y, g
assessment.
Treatment Treatment
Radical surgery: orbital exenteration or
mid facial resection; mid-facial resection;
the prognosis-extremely poor
Radiotherapy:
Rhabdomyosarcoma Rhabdomyosarcoma
most common primary malignant orbital tumor in
children.
P t ti th fi t d d f W ith idl Presentation-the first decade of We with a rapidly
progressive proptosis
Examination-a mass in the upper part of the orbit Examination a mass in the upper part of the orbit.
Systernic investigations-evidence of metastatic spread:
CT of the chest, abdomen, and pelvis; liver function tests; lumbar
b b d k l l d h puncture; bone marrow biopsy. and skeletal radiographs.
Treatment--radiotherapy and chemotherapy.
Exenteration: Exenteration:
recurring or radiotherapyresistant tumor.The survival rate is
90%--confined to the orbit and 65% in the presence of bony
d t ti d t i destruction and extension.
Dermold cyst Dermold cyst
most commn orbital tumors of childhood; benign
cystic teratoma (choristoma). cystic teratoma (choristoma).
Lined by keratinized stratified squamous epith.,
has a fibrous wall and contain dermal appendages has a fibrous wall and contain dermal appendages
such as sweat glands, sebaceous glands and hair
f lli l follicles.
Epidermoid cyst do not contain adnexal structures.
Examination -upper temporal orbit.
Treatment-cosmetic indications Treatment cosmetic indications
Optic nerve glioma Optic nerve glioma
l i t i l 25 50% f

slow-growing tumor; young girls; 25-50% of

patients -associated neurofibromatosis type 1.

Presentation--visual loss in children, but this may not

apparent-until the onset of proptosis apparent until the onset of proptosis.
Examination---disc edema followed by pallor;
t i proptosis.
CT: fusiform enlargment of the optic nerve.
MRI: demonstrating the post., extent of the
tumor tumor
Treatment-
(1) observation: good vision and no tumor growth (1) observation: good vision and no tumor growth
for several year,
(2) surgical excision: vision is very poor and (2) surgical excision: vision is very poor and
growing tumor,
(3) di h d h h i i l (3) radiotherapy and chemotherapy: intracranial
extension.
Optic nerve sheath meningioma Optic nerve sheath meningioma
rare tumors; arise from the meningoendothelial cells of
thearachnoid villi.Middle-aged women. thearachnoid villi.Middle aged women.
Presentation-slowly progressive, unliateral, visual loss.
E i ti di d ti ili h t l Examination-disc edema, opticociliary shunt vessels
(capillaries connecting the central retinal vein with the
peripapillary choroidal vessels), also seen in patients with
optic nerve glioma.
CT: tubular thichening and calcfication of the optic nerve.
Treatment--
(1) b i iddl d i i h l (1)observation: middle-aged patients with slow
growing tumors because the prognosis is good.
(2)radiotherapy: with slow-growing tumors and
relatively good vision relatively good vision.
(3)surgical excision: blind of eye in young patients
ith i t with growing tumor.
Metastatic turmor Metastatic turmor
Childen: Neuroblastoma, Ewing's sarcoma
acute myeloid leukemia
Adults 25% of case the orbit is the initial Adults 25% of case, the orbit is the initial
manifestation of the tumor. Breast
(Female), bronchus (Male), prostate (Male),
skin melanoma G-I tract and kidney skin melanoma, G-I tract and kidney.
Metastatic Turnor invasion from Metastatic Turnor invasion from
adjacent structures adjacent structures
maxillary carcinoma, ethmoidal carcinoma
Congenital orbital malformations Congenital orbital malformations
Craniofacial stenoses
Crouzon syndrome-ptoptosis, corneal exposure,
hypertelorism, ET, optic atrophy.
Apert's syndrome visual loss fromstrabismic or Apert s syndrome-visual loss from strabismic or
anisometropic amblyopia, corneal exposure
or optic atrophy.
L t l f i l i t i Lateral facial microstomias
Treacher Collins syndrome-eyelid coloboma, strabismus
and amblyopia. y p
Goldenhar's syndrome--epibulbar dermoids,
microphthalmos, anophthalmos, microcomea,
tilted disc optic n hypoplasia macular hypoplasia and strabismus tilted disc, optic n. hypoplasia, macular hypoplasia and strabismus.
Hypertelorism
excessive bony tissue of the medial orbital
walls resulting in increased distance walls resulting in increased distance
between the eyes.
XT i h h l i d --XT, microphthalmos, microcomea and
optic atrophy. p p y