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

• AFS (Aniridic Fibrosis Syndrome)

What is Aniridic Fibrosis Syndrome? (Definition/Background Information)

• Aniridic Fibrosis Syndrome (AFS) is an uncommon eye condition that develops as a complication following eye surgery, which is typically undertaken for cataract correction in individuals with congenital aniridia (an absence of iris by birth)
• In general, individuals with congenital aniridia are at an increased risk for undergoing several intraocular surgeries due to poor vision. Aniridic Fibrosis Syndrome only occurs in individuals with preexisting congenital aniridia. A majority of the cases are incidentally observed in women

Who gets Aniridic Fibrosis Syndrome? (Age and Sex Distribution)

• All recorded cases of Aniridic Fibrosis Syndrome are reported in individuals with congenital aniridia
• Congenital aniridia is a rare condition with a reported incidence between 1:64,000 and 1:100,000
• Both males and females may be affected; however, many cases are observed in women
• Worldwide, individuals of all races and ethnicity are prone to developing the condition

What are the Risk Factors for Aniridic Fibrosis Syndrome? (Predisposing Factors)

The predisposing factors for Aniridic Fibrosis Syndrome may include:

• Female gender (over 85% of the cases are reported in women)
• Multiple intraocular surgeries

The condition is exclusive to individuals with congenital aniridia.

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 Aniridic Fibrosis Syndrome? (Etiology)

The cause of development of Aniridic Fibrosis Syndrome (AFS) is not well understood.

• Individuals with congenital forms of aniridia develop AFS following eye surgery (such as cataract surgery with intraocular lens implantation). Often, an individual may undergo multiple surgeries in their lifetime, thereby increasing the risk for this post-surgical complication
• Some experts believe that the irritation caused by intraocular devices on eye structures may result in a progressive fibrosis (scarring) that extends to cover the ciliary body and retina too

What are the Signs and Symptoms of Aniridic Fibrosis Syndrome?

• The signs and symptoms of Aniridic Fibrosis Syndrome (AFS) are dependent on the extent of fibrosis formation
• Some individuals report poorer vision following onset of the condition

In general, individuals with congenital aniridia have poor baseline vision. AFS develops following eye surgery in the background of congenital aniridia.

How is Aniridic Fibrosis Syndrome Diagnosed?

Aniridic Fibrosis Syndrome may be diagnosed by the following observations and tests:

• Physical examination and medical history evaluation (including establishing congenital aniridia and a history of ocular surgery)
• Assessment of signs and symptoms
• General eye exam will reveal absence of iris and associated post-surgical fibrosis
• 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

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 Aniridic Fibrosis Syndrome?

The complications of Aniridic Fibrosis Syndrome may include:

• Emotional stress
• Severe loss of vision
• Treatment complications
• Recurrence of the condition after corrective surgeries

How is Aniridic Fibrosis Syndrome Treated?

• Aniridic Fibrosis Syndrome is managed early through appropriate invasive procedures such as membranectomy via penetrating keratoplasty
• Close follow-up is recommended to evaluate treatment response and prevent any “further” post-surgical complications

How can Aniridic Fibrosis Syndrome be Prevented?

Currently, there are no methods to prevent Aniridic Fibrosis Syndrome.

• Individuals with congenital aniridia may need to be made aware of surgical risks and complications
• Healthcare providers are informed to adhere to best medical practices while performing cataract extraction surgery with intraocular lens implantation (IOL), particularly in such individuals

What is the Prognosis of Aniridic Fibrosis Syndrome? (Outcomes/Resolutions)

• The prognosis of the Aniridic Fibrosis Syndrome (AFS) depends on the severity of the condition and timeliness of surgical management
• In order to prevent further worsening of the condition and restore (previous) baseline vision, prompt recognition of AFS is important

Additional and Relevant Useful Information for Aniridic Fibrosis Syndrome:

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

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