Nystagmus In Children

8/7/14

Nystagmus is a condition of uncontrolled eye movements. Patients with nystagmus are unable to maintain their eyes in a fixed position of focus. The movements can be pendular, swaying evenly side to side, or, jerk into one direction and drift toward the opposite direction. It can be present early in life or acquired as an adult. It can occur in eyes with poor vision from other anomalous development, or eyes that appear perfectly normal. In almost all patients the vision is compromised to some degree. In some patients, the eye movement is less, and the vision better, in an eccentric position that causes the patient to adopt a face turn, tilt or head posture so they can use this quieter position (“null point”) to navigate during their daily activities. To date there have been no consistently effective treatments for this condition.

Lingua and Grace - nystagmus
Dr. Lingua and Grace Nassar

Treatment efforts have been either medical (drugs to reduce the amplitude of the nytagmus movement) or surgical (to move the “null point” into straight ahead gaze to eliminate a head turn, or, directed at reducing the effective contracture of all the eye muscles to reduce the amount of movement). In general, surgical treatment of nystagmus has been disappointing.

In 2002, Dr. Robert Sinskey, noted cataract surgeon and phacoemulsification pioneer, proposed a revolutionary concept, that nystagmus could only be truly effectively controlled by removing the forward portion of the eye muscle and detach it completely from the eye. Since the twitching eye muscles were controlled by nerves sending that pulsatile information, any operation that allowed the muscles to remain attached to the eye would never quiet the movement. He performed this novel surgery in 2000 and published the results in 2002. It did not receive attention in the nystagmus surgery community, as most experts worried that the surgery would limit normal eye movements excessively. The operation does remove the forward portion of the eye muscle but, surprisingly, the eyes are still able to move to allow reading, computing, and driving.. In 2012, I had the opportunity to view a patient he operated 10 years prior and was impressed with how successful the results were even after 10 years. Coincidentaly, I was caring for a 17 year-old patient with nystagmus who had already undergone the 2 currently accepted eye muscle procedures for nystagmus without success. His movements remained uncontrolled, he could not maintain eye contact with anyone, and is his vision was less than that needed for a drivers license. In 2013, I offered him the Sinskey procedure and the results were remarkable. His nystagmus was quieted, his vision improved (20/25) enough to qualify for a drivers license and to return to school.

Since 2012, we have adapted, augmented and perfected the procedure and performed the surgery on over 12 patients with similar remarkable results. All patients experience a marked reduction in the amplitude of the nystagmus (60-100%), and all patients demonstrate improved vision (1-8 lines of the acuity chart), especially at the reading position.

Visit the YouTube posting “Meet Grace for an example of how this surgery can impact a child’s life and the hopes of their parents. Visit www.eye.uci.edu for further information, contact information and scientific data on the procedure.

Robert Lingua, MDRobert W. Lingua, MD
Director, Pediatric Ophthalmology and Strabismus
Gavin Herbert Eye Institute, UC Irvine

What You Need to Know About Cataracts

6/5/14

Do you feel like your vision is getting worse? Do you feel like colors are not as vibrant as they used to be? Are you having more trouble with glare? If you have any of these symptoms, you may be experiencing the effects of cataracts.

Cataracts are a normal aging process of the crystalline lens in the eye. Well you may ask — what is the crystalline lens? It is easiest to think of the eye as a camera. The eye has a lens (actually two) – the cornea (the front window of the eye) and the crystalline lens (inside the eye). It also has an aperture (the colored iris), and film (the retina). All these structures work together to focus light and allow us to see – just like a camera. When we are young (less than 40), the crystalline lens is flexible. This is why we are able to see distance and then near without the need for reading glasses. The crystalline lens is able to change its shape depending on where one is looking.

Figure 1 – Slit-lamp photo of a visually significant cataract.
Figure 1 – Slit-lamp photo of a visually significant cataract.

As we age, the crystalline lens becomes less flexible, thereby causing one’s near vision to be more blurry. This necessitates the need for reading glasses. As the crystalline lens become less flexible with age, the lens also starts to become more yellow and can also become cloudy. When the yellowing and/or clouding become visually significant, we refer to this as a cataract (figure 1).

Are cataracts dangerous? The simple answer is no. In the vast majority of cases, a cataract can be monitored until it becomes visually significant (drop in vision, glare, decreased contrast, vision related difficulties with day to day activities, etc). However, there are a few instances in which cataract removal is a medical necessity. Routine examinations by your eyecare provider can help you determine if you are at risk for these less common instances.

Figure 2 – Intraoperative photo during cataract surgery (prior to cataract removal).
Figure 2 – Intraoperative photo during cataract surgery (prior to cataract removal).

What can I expect during cataract surgery? Do you have to replace the crystalline lens with anything? Cataract surgery involves removing the clouded crystalline lens (figure 2) and replacing it with an artificial lens known as an intraocular lens (IOL) (figure 3). Surgery generally takes 10-15 minutes under a mild sedative, and you don’t have to stop any of your current medications. Anesthesia is achieved with drops and you will only feel mild pressure during the surgery. IOLs come in different styles – Standard IOLs grossly correct your vision and you can fine tune your vision (distance and near) with glasses post-operatively;

Figure 3 – Intraoperative photo during cataract surgery (after implantation of an IOL).
Figure 3 – Intraoperative photo during cataract surgery (after implantation of an IOL).

Toric (astigmatism correcting) IOLs allow for increased spectacle independence, but glasses will still be needed for distance or near; Accommodating IOLs “flex” within the eye to decrease your dependence on distance and near glasses; Multifocal IOLs allow spectacle independence for distance and near. I always counsel patients that there is no perfect IOL and you have to determine which IOL is best for your particular situation. Your doctor can help you decide which IOL is best for you. Generally, cataract surgery is extremely safe. Your doctor will discuss particular risks specific to your eye.

How do I know if cataract surgery is right for me? The best way to know if you have a cataract and if it time to consider surgery is to consult with your local ophthalmologist. If you have experienced a recent drop in vision, that is not correctable with glasses, cataract surgery may be able to restore your vision!

Garg feb 2014 thumbSumit “Sam“ Garg, MD
Medical Director
Vice Chair of Clinical Ophthalmology
Assistant Professor of Ophthalmology
Gavin Herbert Eye Institute – UC, Irvine

New Hope for Corneal Scarring

5/22/14

There are several etiologies for limbal stem cell deficiency of the front of the eye. These include chemical and thermal burns, Steven-Johnson syndrome (which is an autoimmune severe allergic reaction that causes a burn from within), congenital aniridia, and a few other insults such as contact lens over-wear. All of these cause severe ocular surface scarring and problems with the cornea. Many eyes with these diseases have problems with corneal healing. They do not have the stem cells to support ocular surface health. The scarring can be so severe in many cases that severe corneal blindness can result.

Limbal stem cells from the human cornea, with a protein known as p63 stained yellow. Cell nuclei (which hold the DNA) are stained red.  From eurostemcell.org
Limbal stem cells from the human cornea, with a protein known as p63 stained yellow. Cell nuclei (which hold the DNA) are stained red. From eurostemcell.org

In these cases, a simple corneal transplant will quickly fail and not result in any visual improvement. The reason for this is that the stem cells of the ocular surface have been damaged or burned out.

Visual rehabilitation for these eyes usually requires a limbal-corneal stem cell transplantation. The stem cells can be taken from the other healthy eye of the same patient, a living related donor, and or cadaveric tissue. In most cases systemic immunosuppression medications need to be taken for 1 to 3 years following surgery in order to minimize risk of rejection. Management of these patients is done in conjunction with an immunologist or a transplant specialist who can co-manage and monitor for systemic toxicity while the patient is on the these immunosuppressive medications. As most of these eyes also have concomitant glaucoma and scarring of the eyelids to the globe, co-management with a glaucoma specialist and an oculoplastic specialist is also required.

For patients who cannot be on systemic immunosuppression for other health reasons such as diabetes or cancer, they may require an artificial corneal transplantation. The artificial corneal transplantation is reserved as a last step for visual rehabilitation in these eyes. The only artificial cornea that has shown potential, is the Boston keratoprosthesis. Even this artificial cornea carries a high risk for infection and glaucoma. Very close monitoring of eyes that have an artificial cornea is required to monitor for infection and glaucoma progression. However these eyes do not require systemic immunosuppression.

Eye with Boston keratoprosthesis
Eye with Boston keratoprosthesis


The management of eyes with severe ocular surface disease is a difficult one for the cornea specialist. A subspecialist in severe ocular surface disease and limbal stem cell transplantation is required to manage these very sick eyes. At the Gavin Herbert Eye Institute, we have developed a team approach for the management of severe ocular surface disease patients and have successfully treated and are managing many patients who have otherwise no place to go.

Farid 3.6.14Marjan Farid, MD
Director of Cornea, Cataract, and Refractive Surgery
Vice-Chair of Ophthalmic Faculty
Director of the Cornea Fellowship Program
Associate Professor of Ophthalmology
Gavin Herbert Eye Institute, University of California, Irvine