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

• ICE Syndrome, Cogan-Reese type
• Iridocorneal Endothelial Syndrome, Cogan-Reese type
• Iris Nevus Syndrome

What is Cogan-Reese Syndrome? (Definition/Background Information)

• Cogan-Reese Syndrome is one type of Iridocorneal Endothelial (ICE) syndrome. The ICE syndromes predominantly affect Caucasian, young to middle-aged women, and involve one eye
• While there have been some cases of Cogan-Reese Syndrome reported in children, the disease is typically observed in females in the mid-adult years
• In one study of 71 patients with ICE syndrome, the mean age at diagnosis was 51-years. Known glaucoma was present in 11 (15%) of cases
• While it is not yet known how to keep Cogan-Reese Syndrome from progressing, the glaucoma associated with the disease can be treated with medication. Additionally, corneal transplant can treat any corneal swelling

(Source: Cogan-Reese Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Cogan-Reese Syndrome? (Age and Sex Distribution)

• Cogan-Reese Syndrome is a very rare eye disorder, that primarily affects adults, although some cases have been reported in children as well
• The condition can affect both genders, although it is more prevalent in females. Most reported cases are of females in their middle adult years
• The disorder is most common among Caucasian populations

What are the Risk Factors for Cogan-Reese Syndrome? (Predisposing Factors)

The risk factors for Cogan-Reese Syndrome may include:

• Age: middle aged adults are more susceptible to this condition
• Female gender
• Being of Caucasian descent

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 Cogan-Reese Syndrome? (Etiology)

• The exact cause of Cogan-Reese Syndrome is not known at the present time
• Herpes infection and inflammation have been suggested as probable causes for the condition

What are the Signs and Symptoms of Cogan-Reese Syndrome?

The signs and symptoms of Cogan-Reese Syndrome may include:

• Nevus on the iris (which may appear like a smudge)
• Nodular iris nevi (yellow or brown clumps on the iris)
• Attachment of iris to cornea
• Corneal swelling
• Edge of pupil turning outward

How is Cogan-Reese Syndrome Diagnosed?

Cogan-Reese Syndrome is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Eye examinations
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

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 Cogan-Reese Syndrome?

The complications of Cogan-Reese Syndrome may include:

• Glaucoma
• Impaired vision and subsequent vision loss
• Side effects of medication, including eye irritation and pain

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Cogan-Reese Syndrome Treated?

The treatment for Cogan-Reese Syndrome is aimed at addressing glaucoma. The treatment options may include:

• The use of eye drops such as:
  • Prostaglandin analogues: This contains prostaglandin-like compounds as active ingredients, which help in increasing the outflow of the fluid inside the eye
  • Beta blockers: This reduces fluid production in the eye
  • Alpha agonists: This increases outflow and decreases production of fluid in the eye   

• Carbonic anhydrase inhibitors: These help in reducing the fluid production in the eye
• Cholinergic agents or miotic agents: They help in the movement of fluid out of the eye

Surgery becomes essential for patients who are non-compliant with their drops, or do not respond to the above treatments and medications. The purpose of surgical intervention is to bring down the pressure inside the eye. The surgical interventions may include:

• Trabeculoplasty: High-energy laser beams (such as argon laser trabeculoplasty and diode laser trabeculoplasty) are used to remove the blocks in the drainage canals, allowing for the free flow of liquids
• Filtering surgery (trabeculectomy): The surgeon creates an opening in the white of the eye and removes a small piece of the trabecular meshwork, which allows the eye fluid to leave through the opening. This can be performed using a surgery called Nd:YAG laser iridotomy
• Drainage implant (aqueous shunt implant): This is generally used for children or patients with secondary glaucoma. A small silicon tube is inserted into the eye to drain out the fluids

How can Cogan-Reese Syndrome be Prevented?

• The exact cause of Cogan-Reese Syndrome is not known, and therefore, no methods or guidelines are available at the present time for its prevention 
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Cogan-Reese Syndrome
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Cogan-Reese Syndrome? (Outcomes/Resolutions)

• The prognosis of Cogan-Reese Syndrome is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Cogan-Reese Syndrome:

• Cogan-Reese Syndrome is also known as Iris Naevus Syndrome
• Those with asthma or lung conditions, such as emphysema or bronchitis, may be given a different medication than the general population for the treatment of Cogan-Reese Syndrome
• Similarly, individuals with diabetes and on insulin may be prescribed alternative drugs for the treatment of Cogan-Reese Syndrome-associated glaucoma

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/