Aniridia

Overview
Aniridia is the partial or complete absence of the iris, which is the colored part of your eye. The
iris regulates the amount of light that enters your eye. In low light conditions the pupil, or
opening in the center of your iris, increases in size to let in more light. Conversely, in bright
light, the pupil constricts to reduce the amount of light entering your eye.

What causes aniridia?

Sometimes the iris fails to develop properly due to a genetic defect – this is known as congenital
aniridia. Aniridia can also result from trauma to the eye, because the iris is quite fragile – like the
consistency of tissue paper.

Symptoms
Aniridia usually causes light sensitivity and problems with glare, similar to what we all
temporarily experience when we leave a movie matinee and step outside into the bright
afternoon sunshine. This is because the lack of iris tissue allows too much light into the eye. In
addition to causing light sensitivity, aniridia can also make it difficult to open the eye normally,
and it reduces the depth of field normally provided by a small or medium pupil (similar to the
aperture effect in a camera).

Treatments
Often surgeons can repair a traumatic iris defect with sutures. When iris repair is not feasible, an
artificial iris can help provide relief from light sensitivity and glare.

Dr. Francis Price and his associates at Price Vision Group have specialized in taking care of
aniridia patients, both congenital and traumatic, for over 25 years. Dr. Price is the Medical
Monitor and the Cornea Research Foundation is participating in the first USA clinical research
study of an artificial iris implant, the Ophtec Model 311. In this study, we found that 2/3 of the
patients had traumatic aniridia and 1/3 had congenital aniridia. Many congenital aniridia patients
have other associated abnormalities of the eye such as corneal scarring, glaucoma, early cataract
development, and/or improper development of the macula and retina that can lead to poor vision.
Often the attachments of the lens to the wall of the eye are abnormal leading to dislocation of the
lens/cataract. Some patients also have nystagmus. The Cornea Research Foundation and Price
Vision Group have long had an interest in helping aniridia patients and provide treatments for
lack of iris tissue, corneal scarring, glaucoma and complicated cataracts.

Artificial Iris Lenses

Congenital and traumatic aniridia are relatively rare, so there has been little incentive for major
ophthalmic companies to seek approval of artificial iris devices in the United States due to the
high costs associated with the approval process. Artificial iris implants have been available in
Europe for over 10 years and are available in the U.S. on a compassionate use basis.

Several artificial iris devices are available. Dr. Price is the Medical Monitor for the USA clinical
study of the Ophtec Model 311, which is available in three colors: blue, brown or green. Morcher
makes a black artificial iris that has been available for a number of years, but Morcher has not
initiated a study to get this lens approved for use in the USA. A new artificial iris from
Humanoptics provides the most attractive cosmetic appearance because it can be custom
matched to the patient’s natural iris color. Humanoptics plans to initiate a USA clinical study in
which the Cornea Research Foundation will participate.
We reported the initial results from the artificial iris study in the following publication:
Price MO, Price FW, Chang DF, Kelley KA, Olsan MD, Miller KM. Ophtec iris reconstruction
lens United States clinical trial phase I. Ophthalmology, 2004, III; 1874-1852.
Ophtec Artificial Iris

One normal eye, one with an

artificial iris

Artificial Iris Before

After
An artificial iris may help reduce glare and light sensitivity in people who are missing
some, or all of the iris, which is the colored part of the eye.
Dr. Francis W. Price, Jr. is medical monitor for the first clinical trial evaluating use of the Ophtec
artificial iris* in patients. He implanted an Ophtec artificial iris in first study patient in 2002 and
has been the surgeon who has consistently performed the most implants in the study. * Artificial
Iris Implant is manufactured by Ophtec USA.

The Ophtec Artificial Iris Implant is made of PMMA, the most common type of plastic used to
make hard contact lenses and artificial intraocular lenses to replace the natural lens. Until now,
this plastic has been primarily used in cataract surgeries. The artificial iris comes in blue, green
and brown. This new iris can block excess light from entering the eye so patients are not as
easily incapacitated by glare and light sensitivity as they were before when they did not have a
complete iris.
People who are most likely to benefit from this type of procedure are those with a missing iris, or
an iris defect, or people whose iris has endured trauma. To date, most patients have been over the
age of 21. However, as Dr. Price recently pointed out, “The artificial iris can also benefit
children who are born without an iris or who lose the iris in an accident. It can help improve
their vision and restore a more normal appearance. Enrollment in the study has been completed,
but patients may still receive an artificial iris through a compassionate use exemption from the
FDA.

*Artificial Iris Implant is manufactured byOphtec USA.

Amazing new development with Trischa Hudson!
Learn more about Trischa’s new life and new vision.
Boy Gets Artificial Iris
by: Mary McDermott
(Indianapolis-February 1, 2005) - A five-year-old New York boy has come to Indianapolis for a
chance to dramatically improve his vision.

Michael Cruz was born with troubled eyes. He does not have any irises - the part that would
usually be blue, brown, green or hazel. The iris controls the amount of light that enters the eye. It
is a ring-shaped tissue with a central opening, which is called the pupil.

In a normal eye, the pupil will constrict in bright light and dilate in dimmer light. For Michael,
light in an ordinary room is too bright to see clearly because his eyes can’t filter it.

“Kind of like coming out of a movie matinee in the bright sun—you know how just devastating
it is—you can’t keep your eyes open, you can’t lift your head up. And that’s the way these people
are all the time from the time they’re born. So Michael doesn’t even realize what it’s like not to
be that way,” said Dr. Francis Price, eye specialist.

Soon, though, Michael should see the world with new eyes. An operation he had at St. Vincent
Hospital Tuesday morning gave him an artificial iris. He’s only the third child in the country to
get such an implant.

“The artificial iris has a clear central area that’s visual, and an outer area that’s nine millimeters
in diameter that’s opaque. It comes in blue, green, or brown, and his family chose brown for
him,” said Dr. Price.
It was all part of a clinical trial involving 112 people who received artificial irises. Dr. Price has
performed nearly half of those procedures. He expects Michael to heal quickly and he hopes the
implants eventually get federal approval for wider use.

“I think it’s a tremendous asset for these people who have these conditions. It’s just phenomenal.
Now, not everybody has a dramatic response but most of the people have a really dramatic
improvement,” said Dr. Price.
Michael received one artificial iris Tuesday. He is scheduled to get the other one in a few
months.

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To See and Be Seen: An Artificial Iris Update

New options are providing these patients with better cosmetics and smaller-incision surgery.
Christopher Kent, Senior Editor
5/8/2010
Perhaps because the number of patients needing an iris prosthesis is so small, the technology in
this arena has progressed slowly. Recently, however, several new options for replacing or
supplementing a missing or damaged iris have appeared. One in particular that's not widely
known in the United States—the CustomFlex iris prosthesis from HumanOptics in Germany —
offers some features not previously available. Here is a profile of the CustomFlex lens, along
with the other current options.

Better Appearance, Smaller Incision

Until recently, most iris prostheses only addressed the optical problems caused by iris damage:
photophobia; aberration disorders; glare effects; and loss in depth of focus. They were not
designed to mimic the patient's original iris. In fact, many iris prostheses are still only available
in black, and most are rigid, requiring a large incision for implantation.

However, new options that address the need for a better cosmetic result—and a smaller incision
—have recently begun to appear. One such device is a foldable prosthesis individually
manufactured to match the appearance of a patient's original iris tissue: the CustomFlex (also
known as the ArtificialIris) from HumanOptics. The CustomFlex is made of a pigmented,
biocompatible, foldable silicone elastomer that's been used for many years in intraocular lenses.
It's available in two versions—one containing a polymer fiber meshwork that allows the device
to be sutured without tearing, and one without the meshwork. Both versions can be folded and
implanted through a small incision. (The company recommends a 3.2-mm incision for the fiber-
meshwork version, and a 2.5-mm incision for the fiber-free version.) The CustomFlex does not
incorporate a refractive lens.

The prescribing surgeon provides the manufacturer with a high-quality photograph of the desired
iris pattern; the color patterns in the final product are handmade and fully customized to match
the photograph supplied, and the surface structure is designed to closely match the appearance of
the natural iris. The surgeon also receives two standby implants with a slightly different color
composition. The final match can be chosen based on viewing the prosthesis while still
immersed in its sterile saline solution, compared to the remaining iris tissue. (One of the two
standbys can also be used if the first choice becomes contaminated or damaged.)

The device comes as a complete 12.8-mm diameter iris with a 3.35-mm central aperture, a
central circumference thickness of 0.4 mm and a peripheral thickness of 0.25 mm. It can be
trimmed with a trephine or sharp scissors to custom fit the device as a segment sutured to an iris
remnant, for transscleral support, or to reduce the diameter for a small eye.

The company recommends that if the eye is phakic, the crystalline lens should be replaced with
an IOL, even if no cataract is apparent. The recommended placement of the CustomFlex is the
ciliary sulcus, with a peripheral iridectomy. Contraindications to use of the CustomFlex include
severe chronic uveitis, endothelial corneal dystrophy, microphthalmus, retinal detachment,
untreated chronic glaucoma, rubella cataract, rubeosis of the iris and proliferative diabetic
retinopathy.
Working With the CustomFlex
Michael E. Snyder, MD, who practices at the Cincinnati Eye Institute and is a volunteer assistant
professor at the University of Cincinnati School of Medicine, has implanted 30 of the
CustomFlex devices so far and has also worked with most of the other available alternatives. Dr.
Snyder says the CustomFlex has three significant advantages. "First, its realistic appearance is a
big plus," he says. "The cosmetic results have been outstanding. One patient told me that two
weeks after implantation his mother couldn't tell which iris had been damaged.

"Second, this device only requires a 2.75-mm incision," he continues. "That's important because
most of the eyes we've implanted so far have congenital aniridia syndrome; so the epithelial cells
on the surface of the cornea—where we make our incision—are particularly vulnerable.
Furthermore, for individuals who have suffered trauma, a large incision increases the likelihood
of intraoperative complications and could compromise future glaucoma surgery.

"A third advantage of this product is that it's made of a flexible material, so a stitch can be passed
through it," he adds. "In some patients, fixation of the iris element to the wall of the eye may be
necessary, either at the time of implantation or later. That's something you can't do with any other
device." Dr. Snyder adds that so far he hasn't seen any downside to the prosthesis.

To order a CustomFlex Dr. Snyder takes a picture of the remaining uninjured iris, if possible. "If
both irises are gone, we ask the patient to take a picture of someone else's iris that would be a
desired appearance match for them," he says. "One 7-year-old aniridic child that we worked on
chose his mother's iris."

Dr. Snyder says that the Europeans have been implanting this device primarily into the ciliary
sulcus. "When there's an intact capsular bag, I place the device in the bag while performing the
cataract surgery," he says. "If there's no intact capsular bag, I place the device in the sulcus and
usually use suture fixation to guarantee a lack of movement.

"When the patient has a partial iris defect," he continues, "many European surgeons cut a small
segment of the CustomFlex and stitch it to the remaining iris with very nice results. In my
experience, the cosmetic appearance is better when I place the entire diaphragm behind the
remaining iris. Either way is acceptable."

A Variety of Options
Dr. Snyder has also worked with most of the other iris prostheses that are available.
"Most ophthalmologists are aware of the Morcher products, owing to their longer history in
the United States," he notes. "They have several different models made of solid black PMMA
that come with or without a central refractive lens. Some require a large incision, while others
designed on a capsular tension ring backbone can be passed through a smaller incision and
placed in the capsular bag. These intracapsular devices (the 50 and 96 series) have no cosmetic
benefit and can't be sutured directly, but they do have a functional benefit and they're very cost-
competitive. More recently, Morcher has offered a one-piece PMMA option that comes in 45
different colors and uses a color match system; it requires a 9- to 9.5-mm incision.

"Ophtec offers several options as well," he continues. "One is a 9-mm-diameter rigid device with
a central opening of 4 mm; it can be ordered with an optic filling the central space and requires a
9-mm incision. That model has a smooth surface and comes in three colors: light blue; light
brown and light green. Ophtec also has a device that comes in five different pieces that can go
through an approximately 5-mm opening. I've implanted four or five of them, but I've found that
getting all of those pieces oriented inside the eye is fairly challenging. The incision size is a little
smaller than the incision needed for the rigid diaphragm devices."

In terms of a timetable for receiving FDA approval of the CustomFlex prosthesis, Dr. Snyder
says that HumanOptics plans to begin clinical trials soon. For more information, visit artificial-
iris.com.

Dr. Snyder has no shareholder stake in the CustomFlex, but does consult for HumanOptics and
will be principal investigator if the CustomFlex goes into clinical trials for U.S. Food and Drug
Administration approval. He is an investigator in the ongoing Ophtec 311 iris prosthesis study.