Exophthalmos is defined in Dorland's Medical Dictionary as an "abnormal protrusion of the eyeball; also labeled as proptosis.

" Proptosis in the same reference is defined as exophthalmos. Henderson reserves the use of the word exophthalmos for those cases of proptosis secondary to endocrinological dysfunction.[1 !herefore" this dictum will be followed" and non#endocrine$ mediated globe protrusion will be referred to as proptosis and exophthalmos will be reserved for protrusion secondary to endocrinopathies.

%ilateral exophthalmos and upper lid retraction secondary to &raves disease.

Pathophysiology
!he etiological basis of proptosis can be inflammatory" vascular" or infectious. 'n adults" thyroid orbitopathy is the most common cause of unilateral and bilateral exophthalmos. (ther causes include such neoplasms as cavernous hemangiomas" lymphangiomas" lymphomas" )egener granulomatosis" and orbital cellulitis. 'n children" unilateral proptosis is often due to an orbital cellulitis#type picture" and" in bilateral cases" neuroblastoma and leu*emia are more li*ely. +or instance" lymphangiomas" by their histologic nature" can increase in si,e during viral illnesses and result in an increase in orbital volume. - ruptured lymph hemangioma can enlarge due to its rupture and se.uestering of heme" which pathologically is described as a chocolate cyst. (rbital varices can result in proptosis with increased venous pressure in the orbit as seen with a /alsalva maneuver or change in postural position. !he etiology of the thyroid$related orbitopathy is an autoimmune$mediated inflammatory process of the orbital tissues" predominantly affecting the fat and the extraocular muscles. 0ymphocytes" plasma" and mast cells are the cellular constituents in this process. !he deposition of glycosaminoglycans and the influx of water increase the orbital contents. (bstruction of the superior ophthalmic vein with resultant diminished venous outflow also contributes to the orbital engorgement. 1unery has segregated patients with thyroid$related orbitopathy into type ' and type ''.[2 !hose with type ' do not have restrictive myopathy" whereas those with type '' do. !ype ' was believed to be caused by a profundity of hyaluronic acid manufactured by the orbital fibroblasts" stimulating lipoid hyperplasia and edema. Patients with type '' experience restrictive myopathy and have diplopia within 234 of fixation. (rbital emphysema can be a significant cause of proptosis and re.uires emergency treatment.

1o matter what the etiology may be" globular protrusion is secondary to the increase in volume within the fixed bony orbital confines. 5ince the orbit is widest at its anterior aspect" the orbital contents are displaced anteriorly" resulting in proptosis and exophthalmos.

Epidemiology
Mortality/Morbidity

Proptosis due to any cause can compromise visual function and the integrity of the eye.

A proptotic eye not adequately protected by the lids, as with lagophthalmos, can develop exposure punctuate keratopathy. Such disruption of the finely orchestrated homeostatic mechanism to protect the eye will result in corneal compromise, epithelial death, ulceration, and possible corneal perforation in severe cases. At a minimum, the disruption of the tear film layer and incomplete moisturizing of the eye will adversely affect vision and ocular comfort. Proptosis secondary to a space occupying process can result in a compressive optic neuropathy. !mpeded optic nerve blood flow results in irreversible neuronal death and diminished optic nerve function. Such manifestations as depression of visual and color acuities, pupillary dysfunction, and constriction of visual field can occur. Proptotic compressive effects are remedied initially by forward protrusion of the eye, thereby reducing the compressive effect within the orbit. "owever, the eye can extend only so far, and severe stretching can adversely affect the eye and compromise the optic nerve.

Race

!n adult #aucasian males, the average distance of globe protrusion is $% mm, and, in adult African American males, it is $& mm. 'emales also show racial variation. A difference of more than $ mm between the $ eyes of any given patient is considered abnormal.

Sex

!hyroid orbitopathy has a female preponderance with a female$to$male ratio of 671.

Age

Proptosis occurs in both adults and children at any age. !hyroid orbitopathy and the resultant exophthalmos show a predilection for females aged 83$63 years.

History
- meticulous history of the patient9s ocular and systemic systems is *ey in establishing a diagnosis.

!he ophthalmic history should address the duration and the rate of onset of the proptosis. !he patient should be .ueried about pain" change in visual acuity or refraction" diplopia" and decreased fields of vision. !ransient visual loss or blac*out periods may signify optic nerve compromise and may call for rapid intervention. :omplaints of foreign body sensation or dry gritty eyes are symptoms that may indicate corneal decompensation. 'n performing a thorough medical history and a review of systems" the ophthalmologist should consider orbital involvement secondary to systemic pathology. Past trauma and family history also may aid in the diagnosis.

Physical
Evaluation of the patient with exophthalmos begins with a thorough ophthalmic and medical history. )hen concomitant sinus disease or an intranasal source is suspected" a speculum or endoscopic intranasal examination is warranted. 5pecial emphasis on the duration and rate of progression of the patient9s signs and symptoms is essential. Pain" diplopia" pulsation" change in effect or si,e with position or /alsalva maneuver" and disturbance of visual acuity are symptoms that should be explored.

- complete ophthalmic examination is paramount. Periorbital changes can be noted easily on gross examination in a well$illuminated examination room. o Hypertelorism" exorbitism" eyeball protrusion ;proptosis<" eyelid lesions or edema" chemosis" and engorged con=unctival vessels are several periorbital signs.
o

%lepharoptosis" lagophthalmos ;incomplete lid closure<" and interpalpebral fissure distance are additional signs to be considered during the examination.

Palpation of the anterior orbit can assess the level of tenderness" texture" and mobility of the mass.
o

!enderness may denote an inflammatory process or neural invasion by a neoplasm. -ttention should be paid to regional lymph nodes. !actile inspection of the globe may reveal pulsations secondary to arteriovenous communications or physiological intracranially pulsations transmitted through a bony defect of the orbit" such as an encephalocele.

o o

Protrusion of the eye is an important clinical manifestation of orbital disease. 'n addition to proptosis" one should note the displacement of the eye in planes other than the anteroposterior dimension ;eg" downward" lateral<.
o

Hertel exophthalmometry is a well$accepted tool to .uantitate proptosis. !he base is determined by the interlateral canthal space. !he transection of the central cornea by the premar*ed millimeter ruler records the amount of anterior

displacement of the globe. 'ts use re.uires intact lateral orbital rims. 'f the rim is not intact" a 0uedde exophthalmometer can be used.
o

>elative protrusion can be observed by simply standing behind a seated patient and ga,ing downward toward the chin from the forehead to assess the displacement of one globe as compared to the contralateral side.

-uscultation of the orbit may detect a high flow state in the orbit or intracranially. !he bell is useful for this examination. 'f a high$flow lesion is suspected ;eg" carotid cavernous fistula<" arteriography should be sought to further .ualify these lesions. 't is important to have the contralateral eye remain fixated on a target while auscultating the orbit. ?ecreased visual acuity" change of refraction" and pupillary abnormalities should be noted. Extraocular motility dysfunction and diplopia should be carefully assessed and documented. +orced duction testing may .ualify the dysfunction as restrictive or neurogenic in nature. 'ntraocular pressure may be elevated" and slit lamp examination can discern chemosis and engorged or sentinel vessels. ?ilated funduscopic examination may reveal optic disc edema or pallor" retinal detachment" choroidal folds" vascular engorgement or shunt vessels" or indentation of the posterior pole.

Causes
Proptosis can be the result of a myriad of disease processes resulting from primary orbital pathology or systemic disease processes. !he list below is not comprehensive but can help in forming a differential diagnosis. !he list only consists of adult causes since a fair amount of overlap exists in the differential diagnosis of exophthalmos in adults and children.

Proptosis in adults o 'nfectious

o

(rbital cellulitis @ucormycosis :oncurrent sinus disease

'nflammatory

(rbital inflammatory syndrome ;orbital pseudotumor" benign orbital inflammation< !hyroidopathy

/asculitis

)egener granulomatosis :hurg$5trauss syndrome

1eoplastic

0acrimal 0ymphoma 0eu*emia @eningioma &lioma (ssifying fibroma[8 @etastatic ;breast in women" lung and prostate in men" gastrointestinal" *idney<

(rbital vascular disease

(rbital varix ;venous malformation< (rbital arteriovenous malformation ;carotid$cavernous sinus fistula" arteriovenous malformation<

!rauma

!raumatic or iatrogenic orbital hemorrhage (rbital fractures +acial fractures

Pseudoproptosis ;pseudoexophthalmos<

%uphthalmos :ontralateral enophthalmos 'psilateral lid retraction -xial myopia :ontralateral blepharoptosis

-nophthalmos :ellulitis" (rbital

?acryoadenitis ?ermoid" (rbital ?uane 5yndrome +istula" :arotid :avernous &laucoma" Auvenile &lobe >etraction Hemangioma" :avernous Horner 5yndrome 0eu*emias @eningioma" 5phenoid )ing @ucormycosis (rbital +racture" -pex (rbital +racture" +loor (rbital +racture" @edial )all (rbital +racture" Bygomatic Ptosis" -dult Ptosis" :ongenital !hyroid (phthalmopathy

Laboratory Studies

Patients with thyroidopathy should undergo the appropriate thyroid function studies" even though some patients are euthyroid at the time of presentation with exophthalmos. -pproximately C3D of those with &raves disease manifest orbital signs within 1C months" supporting the need for ophthalmic evaluation. -ny patient suspected of having a neoplasm as the cause of the proptosis should undergo imaging studies ;see 'maging 5tudies<. !he imaging results should direct further laboratory studies. +or example" in a patient with proptosis due to lymphoma" hematologic studies" further body imaging" and a bone marrow biopsy may be indicated. 'n patients with proptosis due to orbital cellulitis" complete blood counts" blood and nasal cultures" and sinus imaging studies may be warranted.

Imaging Studies

:! scan" first used in the 1EF3s" is the product of tissue density calculations. G$rays with different vectors are emitted" penetrating through target tissues with resulting radioabsorbencies. !hese differences in radioabsorbencies are assigned value$specific gray shades to create the 2$dimensional image. :! scan can produce detailed axial and coronal views of soft tissue and bony structures. 'mage windows from 1.3$8.3 mm in thic*ness allow for detailed evaluation of orbital masses. :ontrast$enhanced images may be obtained and can help in identifying inflammatory processes" vascular tumors" and engorged vessels. :alcified lesions are discernible without the addition of contrast. @agnetic resonance imaging ;@>'< excites protons by applying a radio fre.uency with a strong magnetic field. Hydrogen nuclei emit signal intensities that are assigned specific gray tones to create an anatomical reproduction. !hree$dimensional views can be gained directly" in any anatomical plane" offering excellent spatial resolution of orbital masses and soft$tissue enhancement. @>' may provide excellent soft$tissue resolution" but :! scan is superior for gleaning details about orbital bony structures. (cular ultrasonography can be used to visuali,e anterior and middle orbital lesions. 5ound waves of 6$16 @H, breech orbital tissues that reflect echogenic energy captured by an oscilloscope. -$scan ultrasonography allows for a 1$dimensional description of echoes" while %$scan ultrasonography provides a 2$dimensional image. :$scan ultrasonography affords coronal views" and ?$scan ultrasonography creates 8$ dimensional orbital views. )ith the advent of :! scan" :$ and ?$scan ultrasonography remains unpopular. ?oppler ultrasonography may be used to evaluate orbital vasculature and blood flow.

Medical Care
@edical care for patients with exophthalmos is directed at reversing the problem and minimi,ing ocular complications.

Consultations
(nce the etiology of exophthalmos or proptosis is established" the appropriate specialists should parta*e in the patient9s care.

Medication Summary
!he goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Ocular lubricants

Class Summary

Heep ade.uate moisture in eye and prevent dryness.

(iew full drug information Artificial tears (Celluvisc, Murine, Refresh, Tears Naturale

:ontains e.uivalent of 3.ED 1a:l and maintains ocular tonicity. -cts to stabili,e and thic*en precorneal tear film and prolongs tear film brea*up time" which occurs with dry eye states.

urther Outpatient Care

Patients should be monitored in intervals tailored to the degree of exophthalmos and complications arising from this ocular malady. @easurement of exophthalmos" visual and color acuities" pupillary function" extraocular motilities" and visual fields should be obtained. 'n addition" any corneal brea*down should be assessed and remedied.

Adult Dosing & ses

Relief of !ry "yes # "ye $rritation Associated %ith !eficient Tear &roduction

'nstill 1$2 gtt into eye;s< !'?IJ'? P>1; less fre.uency for some products Patients with dry eyes may use drops as fre.uently as needed (cular ointments are helpful at bedtime
Additional $nformation

@any formulations available K many manufactures are going to preservative free formulations to avoid ocular irritation" especially in fre.uent users 5ome preparations have prolonged contact time and re.uire less often dosing ;:elluvisc><

Ad!erse E""ects
're(uency Not !efined

%lurred vision Eye pain Headache

'tchingIstinging >edness in and around the eyes 5*in rash

Contraindications

Hypersensitivity to ingredients

Pharmacology
Pregnancy7 no problems reported 1o *inetic info reported
Mechanism of Action

>elieves dryness and irritation

"xo)hthalmos

Author) *ichael *ercandetti, *+, *,A, 'A#S- #hief .ditor) "ampton /oy Sr, *+

http)00emedicine.medscape.com0article0%$%1232 overview

*hat $s "xo)hthalmos+ *hat Causes "xo)hthalmos+

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Exophthalmos, or exophthalmia, is a protruding eyeball anteriorly out of the orbit >eye socket?. 8he <reek word ophthalmos means @eye@ and the <reek word ex means @out@. .xophthalmos can be either bilateral >both eyes bulge out? or unilateral >Aust one eye bulges out?. +octors use an exophthalmometer to measure the degree of exophthalmos. +epending on the severity of the exophthalmos, if it is left untreated the eye lids may fail to close during sleep, resulting in corneal dryness and eventual damage. People with exophthalmos also risk developing superior limbic keratoconAunctivitis, where the area above the cornea becomes inflamed because of the friction that occurs when the patient blinks. Some patients may experience compression of the optic nerve or ophthalmic artery, which can eventually affect the patient4s eyesight, leading to blindness. .xophthalmos is not a condition, but rather a sign of a condition, usually caused by something

wrong with the thyroid gland. According to *edilexicon4s medical dictionary, exophthalmos is "Protrusion of one or both eyeballs; can be congenital and familial, or due to pathology, such as a retroorbital tumor (usually unilateral) or thyroid disease (usually bilateral)."

#hat are the signs and symptoms o" e$ophthalmos%

A symptom is something the patient feels and reports, while a sign is something other people, such as the doctor notice. 'or example, pain may be a symptoms while a rash may be a sign. 8he most noticeable sign of exophthalmos are bulging or protruding eyeballs >either one or both?. !f the patient has <raves4 disease, a thyroid condition, the bulging develops because the tissues in the eyeballs swell, and the number of cells in the eye increases resulting in larger eyes which push forward from their orbits >sockets?. 8he human eye sockets are rigid and cannot expand to accommodate the larger eyeball. As the eyeball protrudes the eyelid is forced apart, giving the patient a staring expression with a higher than normal proportion of the whites of the eyes exposed. <raves4 disease is the most common cause of bulging eyeballs. Signs and symptoms of <raves4 disease include)

Pain in the eyes +ry eyes .ye irritation Sensitivity to light >photophobia? =acrimation eye secretions and shedding of tears +iplopia double vision caused by the weakening of the eye muscles Progressive blindness, if the optic nerve is compressed +ifficulty in moving eyes as the eye muscles weaken the patient may find it harder to move the eyes. !n some cases the eyes may turn inwards >amblyopia?.

8he following non ocular signs and symptoms are also possible with <raves4 disease) !rregular heartbeats >arrhythmia, palpitations? Anxiety

/aised appetite Sleeping problems >insomnia?

#hat are the causes o" e$ophthalmos%

<raves4 disease, an autoimmune disease that causes hyperthyroidism >thyroid gland produces too much thyroid hormone? is the most common cause of exophthalmos. 8hyroid problems generally are common causes.

-ds by &oogle
,ack Pain 8reatments +r.Prem Pillay, Singapore .xpert Spine, Pain 8herapy www.+rPremPillay.org #ancer 8reatment Are you a Bournalist or a "#P researching #ancer 8reatmentC !nfo www.thewhiteroom.info 8hyroid +isease 8reatment .ffective "erbal 8hyroid *edicine. %559 All 6atural Pills. :rder 6ow; www.<reen=ife "erbal.com The thyroid gland the thyroid gland is in the neck, below the Adam4s apple. !t produces hormones which help to regulate growth and the rate of chemical reactions >metabolism? by which the body uses energy. 8he thyroid gland produces thyroxine and triiodothyronine. Thyroid eye disease also known as 8: >thyroid orbitopathy?, is a condition in which the soft tissues and muscles surrounding the eyes become swollen and inflamed. 8his condition is closely linked to hyperthyroidism >overactive thyroid gland?, and sometimes hypothyroidism >underactive thyroid gland?. .xophthalmos does not necessarily occur during the onset of hyperthyroidism or hypothyroidism. 8he eyes may start bulging long after a thyroid problem starts- and even before.

Autoimmune diseases healthy people4s immune systems attack pathogens- organisms and substances that are bad for us, such as some bacteria, viruses, parasites, cancer cells and fungi. !f the person4s immune system starts attacking good tissue they have an autoimmune disease the immune system is attacking parts of the person4s body that are needed for good health. <raves4 disease is an example of an autoimmune disease. .xperts are not sure why autoimmune diseases, such as <raves4 disease occur. !f the patient4s immune system attacks the thyroid gland it often reacts by producing more hormones than normal. 8he excess thyroid hormone, as well as the autoimmune antibodies may attack the muscles and soft tissue surrounding the eyes, causing)

+ry eyes >often described as gritty eyes? /edness Puffy eyes !nflammation and swelling

Problems with eyesight .xophthalmos >bulging or protruding eyes?

nherited diseases some diseases which may affect the eyes are inherited, the patient has acquired the faulty genes from their parents or grandparents. <raves4 disease is an example of a hereditary disease. Proptosis !eyeball protrusion, one eye" the presence of something in the eye socket can cause proptosis, such as) A cancerous tumor *ucocoele >mucus filled cyst?

A blood clot 8rauma >eye inAury? Sinus infection

Anybody who notices that one or both eyes are starting to bulge should see their doctor immediately.

Ho& is e$ophthalmos diagnosed%

!n most cases the protrusion or bulging of the eyeballs from the sockets, with much more of the whites of the eyes exposed, is enough for the doctor to diagnose exophthalmos fairly easily. "owever, as mentioned earlier, exophthalmos is a sign of some disease or condition, and is not a disease in itself. 8herefore, if a <P >general practitioner, primary care physician? suspects exophthalmos the patient will probably be referred to an eye specialist an ophthalmologist. 8he ophthalmologist will most likely order further tests before confirming a diagnosis. 8he following tests may be ordered) A blood test this will probably be a thyroid function test to see whether the thyroid gland is healthy. Exophthalmometer this instrument measures the degree of eyeball protrusion as well as determining how well the patient can move their eyes. Patients with exophthalmos will be able to look upwards without moving their eyebrows. maging scans in order to examine the orbit >socket? the doctor may order a #8 >computerized tomography? scan or an */! >magnetic resonance imaging? scan. A scan may also detect a tumor, or any abnormalities in or around the eyes.

#hat are the treatment options "or e$ophthalmos%

As exophthalmos tends to be a progressive disease symptoms get worse over time the ophthalmologist will wish to monitor the patient regularly. 8reatment depends on several factors, including the cause, as well as the patient4s age and general health. Thyroid problems the doctor will treat whatever is causing the thyroid problem so that thyroid hormone levels are brought back to normal. Dsually, effective thyroid treatment results in the restoration of normal vision and the appearance of the eyes. !f the patient has <raves4 disease thyroid treatment might make no difference to the appearance of the eyes. :n occasions the affected vision and appearance of the eyes cannot be improved. !t is important to see your doctor as soon as you notice any bulging of the eyes. 8reatment is much more effective if it can begin early. #urgery if there is a problem with the connection between the arteries and veins in the eyes the patient may have to undergo surgery. !n severe cases the bony floors of the eye sockets may have to be surgically removed >surgical orbital decompression?. 8he procedure allows excess material to move down into the extra space in the antrum >maxillary sinus? by the surgical intervention. Eye drops if the patient suffers from dry eyes, eye drops will help keep the eyeballs moist it is important to keep the cornea lubricated. Eyeshades eyeshades may be required if the patient suffers from photophobia >oversensitivity to light?. Corticosteroids for patients whose eyes are especially painful or swollen >inflamed?, the doctor may prescribe corticosteroids. #orticosteroids are effective in reducing swelling and inflammation. A tumor most likely the doctor will talk to the patient about removing the tumor surgically, as well as using chemotherapy, radiotherapy >radiation therapy?, or a combination.

#hat are the possible complications o" e$ophthalmos%

!n severe cases patients may not be able to close their eyes properly, especially when they are asleep. 8his can cause the cornea to dry out and become damaged. !f the cornea dries out too much there is a much higher risk of infection or ulcers, which can damage vision. People with exophthalmos or proptosis are more susceptible to developing conAunctivitis. Eritten by #hristian 6ordqvist #opyright) *edical 6ews 8oday $ot to be reproduced %ithout permission of &edical $e%s Today

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