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

• AZOOR (Acute Zonal Occult Outer Retinopathy)

What is Acute Zonal Occult Outer Retinopathy? (Definition/Background Information)

• Acute Zonal Occult Outer Retinopathy (AZOOR) is defined as a rare type of eye condition that affects a region of the retina. It presents characteristic findings when examined under a fundoscope by a healthcare specialist. AZOOR falls into a category of disorders known as white dot syndromes
• The condition results in signs and symptoms that include vision abnormalities, blurred vision, and flashes of light in the eye perception. Acute Zonal Occult Outer Retinopathy is generally observed in young to middle-aged adults and is associated with shortsightedness (myopia)
• The cause of Acute Zonal Occult Outer Retinopathy is not well-known. AZOOR is seen to occur with various conditions that include Sjögren’s disease, Crohn’s disease, and diabetes mellitus
• A healthcare provider can use various physical (eye) exams, blood tests, and imaging studies to diagnose Acute Zonal Occult Outer Retinopathy. Upon diagnosis, treatment options, such as steroids or immunomodulators, can be used to treat the condition
• The prognosis for Acute Zonal Occult Outer Retinopathy depends on each individual's signs and symptoms. The prognosis is generally good, but the condition recurs in nearly one-third of the cases

Who gets Acute Zonal Occult Outer Retinopathy? (Age and Sex Distribution)

• Acute Zonal Occult Outer Retinopathy can present itself at any age, but is more likely to occur in young and middle-age adults (mid-thirties)
• Both males and females are affected, although a female predominance is noted
• Individuals of different racial and ethnic backgrounds can be affected, but Caucasians are affected more than other race/ethnic groups

What are the Risk Factors for Acute Zonal Occult Outer Retinopathy? (Predisposing Factors)

The specific risk factors for Acute Zonal Occult Outer Retinopathy may include the following:

• Young women
• Myopia or nearsightedness
• Individuals of Caucasian race

AZOOR may be associated with the following conditions:

• Multiple sclerosis
• Hashomoto’s thyroiditis
• Myasthenia gravis
• Sjögren’s disease
• Crohn’s disease
• Addison’s disease
• Insulin-dependent diabetes mellitus
• CREST syndrome

Note: An association means that there is an increased incidence of the condition that is present with Acute Zonal Occult Outer Retinopathy. The presence of an associated condition does not mean that it is the cause of AZOOR.

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 Acute Zonal Occult Outer Retinopathy? (Etiology)

• Acute Zonal Occult Outer Retinopathy is an uncommon eye condition of unknown cause. It is known to be associated with several conditions and autoimmune disorders
• AZOOR is also observed in individuals with myopia (or near vision)
• It 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 Acute Zonal Occult Outer Retinopathy?

The signs and symptoms of Acute Zonal Occult Outer Retinopathy may include:

• The presence of flu-like symptoms that is usually observed prior to the development of eye-related symptoms
• The condition can be unilateral or bilateral i.e., affecting one eye or both eyes
• Vision defects or abnormalities that include reduced vision or blurred vision
• Photopsia or flashes of light sensation
• In some individuals, the signs and symptoms progress initially and then stabilize after a certain period (about 6 months)
• 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

How is Acute Zonal Occult Outer Retinopathy Diagnosed?

A healthcare professional may diagnose Acute Zonal Occult Outer Retinopathy 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)
• Other tests to determine any condition that is associated with AZOOR

If individuals have additional 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

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 Acute Zonal Occult Outer Retinopathy?

Complications of Acute Zonal Occult Outer Retinopathy may lead to 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
• Retinal detachment: An eye condition wherein the retina gets separated from the eye structures that holds the retinal layers together
• 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 Acute Zonal Occult Outer Retinopathy: In about one-third of the cases, the condition is known to recur if it is not treated appropriately. With each recurrence, the signs and symptoms may get more severe

How is Acute Zonal Occult Outer Retinopathy Treated?

Presently, standard treatment protocols are not well-established for Acute Zonal Occult Outer Retinopathy. The following treatment measures may be considered on a case-by-case basis:

• Oral corticosteroids are generally effective and may be given to control inflammation. Steroids may also be administered intravenously depending on the underlying cause
• 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 AZOOR
• If choroidal neovascularization is observed, then laser therapy may be helpful
• Administration of immunomodulators, which are medications to control dysfunctional immune system
• Treatment of myopia by using eye glasses or contact lenses; surgical corrections using laser or lens implants

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 Acute Zonal Occult Outer Retinopathy be Prevented?

It may be difficult to prevent Acute Zonal Occult Outer Retinopathy, but the risk for the condition may be lowered through the following measures:

• Avoiding contact with individuals who have infections
• 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 Acute Zonal Occult Outer Retinopathy? (Outcomes/Resolutions)

• The prognosis of Acute Zonal Occult Outer Retinopathy generally varies between individuals. It depends upon the severity of the signs and symptoms and the individual’s response to treatment
• Individuals with mild signs and symptoms have better prognosis than those with severe conditions
• Individuals with only one episode of AZOOR generally have excellent prognoses. However, with multiple recurrences (as observed in nearly 35% of the cases), the prognosis gets worse

Additional and Relevant Useful Information for Acute Zonal Occult Outer Retinopathy:

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

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