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

• Birdshot Chorioretinopathy
• Vitiliginous Chorioretinitis

What is Birdshot Retinochoroidopathy? (Definition/Background Information)

• Birdshot Retinochoroidopathy is defined as a rare and chronic type of posterior uveitis affecting both the eyes. It presents characteristic findings when examined under a fundoscope by a healthcare specialist. Birdshot Retinochoroidopathy falls into a category of disorders known as white dot syndromes
• Uveitis is inflammation of the uvea, which is a part of the eye structure. The uvea is a pigmented layer that consists of the iris, ciliary body, and choroid. Posterior uveitis is a condition involving the inflammation of the choroid
• Birdshot Retinochoroidopathy results in signs and symptoms that include eye floaters, reduced vision, and color perception changes. It is a generally progressive condition that is observed in middle-aged and older adults
• The cause of Birdshot Retinochoroidopathy is not well-known. However, it is known to be associated with certain conditions that include hypertension, glaucoma, skin cancers and skin disorders, and hearing loss
• A healthcare provider can use various physical (eye) exams, blood tests, and imaging studies to diagnose Birdshot Retinochoroidopathy. Upon diagnosis, treatment options, such as steroids or immunomodulators, can be used to treat the condition. Some cases are known to get better on their own, even without treatment
• The prognosis for Birdshot Retinochoroidopathy depends on each individual's signs and symptoms and may be assessed on a case-by-case basis. The condition can lead to permanent blindness, if it is left undiagnosed or untreated. Complications, such as glaucoma or choroidal neovascularization, can worsen the prognosis

Who gets Birdshot Retinochoroidopathy? (Age and Sex Distribution)

• Birdshot Retinochoroidopathy can present itself at any age, but is more likely to occur between the ages of 40 to 60 years
• Both males and females are affected, although a female predominance is noted
• Individuals of different racial and ethnic backgrounds can be affected. However, Caucasians are affected more than other races
• It is a rare condition; only 1% of all uveitis is Birdshot Retinochoroidopathy

What are the Risk Factors for Birdshot Retinochoroidopathy? (Predisposing Factors)

The risk factors for Birdshot Retinochoroidopathy include:

• Family history of the condition
• The presence of HLA-A29 genetic make-up indicates an increased risk; its presence is known to increase the relative risk from between 50-225 times

The following conditions have been associated with Birdshot Retinochoroidopathy, although the cause-effect relationship is not definitely proven:

• Increased blood pressure
• Hearing loss
• Pigmented skin disorders, such as vitiligo
• A variety of skin cancers
• Glaucoma
• Psoriasis
• Sarcoidosis

Note: An association means that there is an increased incidence of the condition that is present with Birdshot Retinochoroidopathy. The presence of an associated condition does not mean that it is the cause of Birdshot Retinochoroidopathy.

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 Birdshot Retinochoroidopathy? (Etiology)

• The cause of Birdshot Retinochoroidopathy is not clearly understood or identified. It is an eye disorder that is associated with many conditions
• 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
• Birdshot Retinochoroidopathy is a type of white dot syndrome; white dot syndromes are a group of eye disorders that involve inflammation of the retina and choroid

What are the Signs and Symptoms of Birdshot Retinochoroidopathy?

The signs and symptoms of Birdshot Retinochoroidopathy may vary between individuals. It is a progressive condition in 4 out 5 cases, where the condition gets worse and the vision is affected. In some, mild features are seen and the condition may go undiagnosed for a long time period.

The signs and symptoms of Birdshot Retinochoroidopathy may include:

• Small specks, called floaters, that may be seen moving through one's field of vision is observed in one-third of the individuals
• Reduced vision is observed in a majority of cases (around 65% of them)
• Light-sensitivity is observed in 20% of the cases, and a quarter of the individuals have night vision difficulties
• Alteration of color perception
• Blurred vision
• Flashes of light in the eye, or photopsia
• Birdshot Retinochoroidopathy is usually a painless condition and a majority of individuals do not experience any pain
• Chronic signs and symptoms develop slowly over a period of time
• The condition is bilateral and both eyes are affected. Also, the signs and symptoms may be asymmetrical, which means that:
  • The combination of signs and symptoms in each eye may be different
  • The rate of progression of the signs and symptoms of each eye may be different
  • The severity of signs and symptoms may vary from eye to eye

The presentations of any underlying condition may also be observed.

How is Birdshot Retinochoroidopathy Diagnosed?

A healthcare professional may diagnose Birdshot Retinochoroidopathy using the following tests and procedures:

• Physical examination and analysis of previous medical history
• 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
• 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)
  • Testing to determine the presence of HLA-B27 genetic make-up
• Rarely, a biopsy of the choroid may be performed to help with the diagnosis
• Other tests to determine any underlying condition causing Birdshot Retinochoroidopathy

If individuals have other signs and symptoms, then the following tests may be performed:

• Chest X-rays
• Neuroimaging studies including MRI scan of brain
• Lumbar puncture: In this procedure, the cerebrospinal fluid is collected and analyzed

Note:

• In some cases, because of the gradual onset of (or mild) signs and symptoms, there can be a delay in diagnosis that can result in irreversible harm to the eye
• In general, there is a set of diagnostic criteria proposed by eye experts to diagnose Birdshot Retinochoroidopathy. The healthcare provider will determine if the individual meets the diagnostic criteria

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 Birdshot Retinochoroidopathy?

Complications of Birdshot Retinochoroidopathy may lead to serious vision abnormalities and may include:

• Choroidal neovascularization (CNV): An abnormal development of blood vessels in the choroid, which if left untreated can result in permanent loss of vision
• Glaucoma: A condition that can cause blindness due to higher intraocular pressure
• If left undiagnosed and/or untreated, the condition can lead to permanent blindness
• Kidney damage, high blood sugar, and high blood pressure due to steroid treatment
• Recurrent Birdshot Retinochoroidopathy: The condition may recur if the underlying cause is not identified and treated appropriately. With each recurrence, the signs and symptoms may get more severe

How is Birdshot Retinochoroidopathy Treated?

Early diagnosis and treatment of Birdshot Retinochoroidopathy is very crucial in preventing complications that may result in irreversible damage to the eye. With appropriate treatment, the chance of recurrence decreases. Also, with suitable treatment, the vision abnormalities and other signs and symptoms either remain stable or become better, but do not deteriorate/worse.

The treatment of Birdshot Retinochoroidopathy may include the following measures:

• Oral medications (including pain medications)
• Oral corticosteroids are generally effective and may be given to control inflammation. Steroids may also be administered intravenously depending on the underlying cause
• Dark glasses may be prescribed for light-sensitivity
• Addressing underlying conditions that may be contributing to progression of the disorder
• If the underlying cause is unknown, then decreasing inflammation is the main step towards treating Birdshot Retinochoroidopathy
• Administration of immunomodulators, which are medications to control dysfunctional immune system
• If choroidal neovascularization is observed, then laser therapy may be helpful
• In case of chronic cases that fails to get better with medication, surgery (such as vitrectomy) to treat the condition may be considered
• Around 20% of the cases are known to get better on their own, without the need for any intense treatment

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

How can Birdshot Retinochoroidopathy be Prevented?

It may be difficult to prevent Birdshot Retinochoroidopathy, but the risk for the condition may be lowered through the following measures:

• Avoiding stress and taking suitable steps to maintain blood pressure within normal ranges
• Educating the individual and family members about the underlying cause and preventing the development of any modifiable risk factors associated with the underlying condition (if possible)
• Treating any underlying infections and immunologic conditions, as early as possible
• Maintaining long-term follow-up checkups to watch out for any recurrences

What is the Prognosis of Birdshot Retinochoroidopathy? (Outcomes/Resolutions)

• The prognosis of Birdshot Retinochoroidopathy generally varies between individuals. It depends upon the severity of the signs and symptoms
• In a majority of individuals (80% of them), the condition is progressive in nature and the symptoms get worse with time. Birdshot Retinochoroidopathy can result in permanent blindness
• In about 1 in 5 cases, the condition is known to resolve spontaneously on its own, without the need for treatment

Additional and Relevant Useful Information for Birdshot Retinochoroidopathy:

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

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