What are the other Names for this Condition? (Also known as/Synonyms)

• SO (Sympathetic Ophthalmia)
• Sympathetic Ophthalmitis
• Sympathetic Uveitis

What is Sympathetic Ophthalmia? (Definition/Background Information)

• Sympathetic Ophthalmia (SO) is an extremely rare eye condition that takes place following a history of injury or surgery to the eye. It is a serious condition that can lead to permanent blindness, if the condition is not immediately detected and aggressive treatment is not provided promptly
• Sympathetic Ophthalmia is described as a bilateral condition, meaning it affects both the eye. However, even though only one eye was affected by the trauma (called the ‘exciting eye’), the manifestation of signs and symptoms is seen in the other eye (called the ‘sympathizing eye’). The cause of the condition is reported to be due to an abnormal immune response of the body
• Sympathetic Ophthalmia results in signs and symptoms that include reduced vision, blurred vision, and light sensitivity. It is a rapidly progressive condition and can cause irreparable damage to the eye
• Examination of the eye by a healthcare expert may reveal the formation of granulomatous inflammation (granulomatous uveitis), thickening of the choroid, swollen iris, and involvement of the optic nerve and optic disc
• Sympathetic Ophthalmia mostly results in retinal detachment, apart from other severe eye-related complications. Upon diagnosis, treatment options, such as steroids or immunomodulators, may be used to manage the condition
• The prognosis for Sympathetic Ophthalmia depends on each individual's signs and symptoms and response to therapy. However, it is a potentially vision-threatening condition that can cause permanent blindness

Who gets Sympathetic Ophthalmia? (Age and Sex Distribution)

• Sympathetic Ophthalmia can present itself at any age and a wide age range is observed
• Some reports indicate that children and slightly older adults (60 years and above) have a slightly higher incidence of the condition. This may possibly be due to eye injuries in children and ocular surgery in elderly adults being more commonly prevalent
• Both males and females are affected and no particular preference is noted
• Individuals of different racial and ethnic backgrounds can be affected
• Sympathetic Ophthalmia is a very rare condition with a reported prevalence of 1 per 3.5 million population

What are the Risk Factors for Sympathetic Ophthalmia? (Predisposing Factors)

There are 2 key risk factors for Sympathetic Ophthalmia and these include:

• Eye injury or trauma to the eye, which was not resolved completely or adequately:
  • The eye injury, which may affect the uvea, can be a penetrating wound
  • This may occur from participation in high-risk sports, violence, or eye injuries sustained even on battlefields
• A previous surgery to the eye (typically surgery to the retina):
  • The surgeries may include vitreoretinal surgery, surgery for glaucoma, cataract repair, or surgery to partially remove the iris (iridectomy)
  • Sometimes, multiple (intraocular) surgeries may have been performed involving the same eye

Note:

• The chances of Sympathetic Ophthalmia due to an eye trauma is 1 in 200-500; it is 1 in 10,000 for every intraocular surgical procedure performed
• The time interval between eye trauma/surgery and onset of the condition can vary greatly. A majority of the cases (90% of them) are seen to onset within the first 12 months, while some may take several decades for manifestation (sometimes, even over 60 years). Some case reports indicate that up to 65% of Sympathetic Ophthalmia cases occur within the first 2 weeks following injury/surgery to the eye

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Sympathetic Ophthalmia? (Etiology)

The cause of Sympathetic Ophthalmia is not clearly understood or identified.

• Some researchers believe that it may be an autoimmune disorder that is triggered by the individual’s own immune system. The immune system creates antibodies that destroys the body part, organ, or region
• The autoimmune condition leads to an inflammatory response in the eye; abnormal response of T-cells (lymphocytes or white blood cell types) have been implicated

What are the Signs and Symptoms of Sympathetic Ophthalmia?

Sympathetic Ophthalmia can have a silent and menacing onset, or the onset of signs and symptoms can be sudden and acute (which is true in many cases). Both the eyes may appear the same, although the onset is in one eye. The main affected eye is called the ‘exciting eye’, while the other eye is called the ‘sympathizing eye’, which was not affected by past trauma/surgery; hence, the condition is called Sympathetic Ophthalmia.

The signs and symptoms can range from reduced vision to permanent vision loss and may include:

• Small specks, called floaters, that may be seen moving through one's field of vision
• Blurred vision
• Reduced vision or vision loss is observed in a majority of cases
• Light-sensitivity (photophobia)
• Conjunctivitis-like symptoms including red eyes and tearing
• Some individuals may experience pain
• There may be periods when the signs and symptoms get better and then worsen; Sympathetic Ophthalmia can be a chronic condition

In rare cases, additional signs and symptoms may be present including:

• Loss of hearing
• Headaches
• Vitiligo (discoloration of skin occurring in patches)

How is Sympathetic Ophthalmia Diagnosed?

A healthcare professional may diagnose Sympathetic Ophthalmia using the following tests and procedures:

• Physical examination and analysis of previous medical history (including history of any eye injury or eye-related surgical procedure)
• Eye examination by an eye specialist
• Fundoscopic (ophthalmoscopic) examination by an eye specialist, who examines the back part of the eye (or the fundus)
• Visual acuity test using a special and standardized test chart (Snellen chart)
• Slit-lamp examination: Examination of the eye structure using a special instrument called a slit-lamp. In this procedure, the pupils are dilated and the internal eye structure is examined. Slit-lamp exams indicate large inflammatory cell clumps on the cornea, called ‘mutton-fat' keratic precipitates, which is a characteristic feature of the condition
• Tonometry: Measurement of intraocular pressure or eye fluid pressure, especially to detect conditions such as glaucoma
• Fundus fluorescein angiography (FFA): In this technique, the eye blood vessels are examined using a fluorescein dye
• Fundus autofluorescence (FAF) imaging: It is a diagnostic technique to examine the fundus of the eye using a fluorescent dye
• Indocyanine green (ICG) angiography: It is used to examine the blood vessels of the choroid using a dye, called indocyanine green, particularly to study the choroid
• B-scan ultrasonography: Special ultrasound scan of the eye through a non-invasive diagnostic tool, to assess health of the eye structures
• Electroretinogram (ERG): It is a technique to measure electrical activities in the retinal cells
• Optical coherence tomography (OCT) of eye: Radiological imaging technique to visualize the eye structure
• Blood tests that include:
  • To check for the presence of antibodies in blood
  • Blood culture for infections
  • Complete blood count (CBC) with differential
  • Erythrocyte sedimentation rate (ESR)
• Rarely, a biopsy of the choroid may be performed to help with the diagnosis
• Other tests to determine any underlying condition causing Sympathetic Ophthalmia

Note:

• A differential diagnosis to eliminate the following conditions is crucial in establishing a diagnosis:
  • Vogt-Koyanagi-Harada syndrome
  • Phacoanaphylactic endophthalmitis (choroid inflammation with lens damage)
  • Sarcoidosis
• In majority of the cases, a delay in diagnosis can result in irreversible harm to the eye

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Sympathetic Ophthalmia?

Complications of Sympathetic Ophthalmia may lead to serious vision abnormalities and/or SO may be associated with the following conditions:

• Choroidal neovascularization (CNV): An abnormal development of blood vessels in the choroid, which if left untreated can result in permanent loss of vision
• Macular edema (fluid in the macula) and papillary edema (swelling of the optic disc)
• Glaucoma: A condition that can cause blindness due to higher intraocular pressure
• Cataracts: When the lens of the eye becomes clouded and cause vision loss
• Retinal detachment is observed in most cases: It is an eye condition wherein the retina gets separated from the eye structures that holds the retinal layers together
• Poliosis: A condition causing decreased melanin pigment in the eyebrows and eyelashes
• Severe inflammation causing complete loss of vision
• If left undiagnosed and/or untreated, the condition usually leads to permanent blindness
• Kidney damage, high blood sugar, and high blood pressure due to steroid treatment

How is Sympathetic Ophthalmia Treated?

A prompt diagnosis and emergency treatment of Sympathetic Ophthalmia is extremely crucial in preventing complications that can result in permanent damage to the eye. With immediate and aggressive treatment, a complete loss of vision may be avoided.

The treatment of Sympathetic Ophthalmia may include the following measures:

• Oral medications (including pain medications)
• Use of cycloplegic eye drops for controlling the signs and symptoms (through a mechanism known as ciliary muscle paralysis)
• High dose corticosteroids may be given to control inflammation. Steroids may also be administered intravenously
• Dark glasses may be prescribed for light-sensitivity
• Administration of immunomodulators, which are medications to control dysfunctional immune system
• If choroidal neovascularization is observed, then laser therapy may be helpful
• Very close observation and monitoring of the condition is essential and highly recommended
• For milder conditions, topical steroid applications and medications that cause papillary dilation may be recommended. Extremely rarely, with mild cases, self resolution is known to occur

It is important to note that steroids may not be used in all cases, since it can worsen the condition (especially if there is an infection). A healthcare provider will provide the best treatment options based upon each individual’s specific circumstances.

How can Sympathetic Ophthalmia be Prevented?

Currently, it is very difficult to prevent Sympathetic Ophthalmia. However, prompt and adequate treatment of any eye injury is very important and crucial in preventing vision loss. Some healthcare experts advocate removal of the eye affected by injury/trauma (the ‘exciting eye’), through a procedure known as enucleation of the eye.

Enucleation of the eye: It is recommended only when all attempts by the healthcare experts at salvaging or saving the eye is proved unsuccessful.

• Removal of the affected or exciting eye, for severe trauma and when the eye is potentially blind, or show severe signs and symptoms and pain
• This has to be performed immediately following eye trauma (within a period of 7 days)
• This can help prevent the other eye from being affected

What is the Prognosis of Sympathetic Ophthalmia? (Outcomes/Resolutions)

• The prognosis of Sympathetic Ophthalmia generally varies between individuals and depends on the severity of the signs and symptoms and response to treatment
• Up to 35% of the individuals may become blind; 50% are known to have severe vision abnormalities
• A rapid detection and treatment is very important in preventing irreversible damage to the eye accompanied by vision loss

Additional and Relevant Useful Information for Sympathetic Ophthalmia:

Please visit our Eye & Vision Health Center for more physician-approved health information:

http://www.dovemed.com/health-topics/vision-center/