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Pediatric Corneal Transplantation
Every
year, hundreds of infants are born throughout the world with genetic diseases that cause
opacities or cloudiness of the cornea (the window of the eye). Opacified corneas prevent
light impulses from reaching the brain, which results in permanent blindness. But
blindness may be prevented in these infants through the use of cornea transplant surgery.
In this procedure, the opacified cornea is replaced with a donor cornea. This provides a
new clear window that allows light impulses to be transmitted to the brain so that visual
development may occur.
Cornea transplant surgery in children is very complex and technically far more
challenging than adult corneal transplantation because of the fragile nature of infant
tissue and the difficulty in visual rehabilitation in infants. Indeed, only a handful of
surgeons in the world feel comfortable performing corneal transplants on infants because
of the possibility of poor results, technical difficulties, and the enormous commitment
needed to visually rehabilitate these patients. In many parts of the world, surgery is not
even offered as an option, so that these children remain blind.
The Cornea-Genetic Eye Medical Clinic is one of the few facilities in the western
United States dedicated to pediatric corneal transplantation and research to improve the
ultimate visual outcomes in these extremely complex cases. This unique facility benefits
from having access to excellent pediatric anesthesia, state-of-the-art technology, and one
of the most sophisticated genetic divisions in the worldthe Medical Genetics-Birth
Defects Center at Cedars-Sinai Medical Center, Mark Goodson Building. Thus, patients receive appropriate genetic
work-up and genetic counseling prior to embarking on any surgical procedure.
Currently, more than forty pediatric corneal procedures and over one hundred ocular and
genetic evaluations are performed annually. There is an active research program committed
to improving visual outcomes following surgery and investigating the underlying causes of
genetic eye diseases. Every other year, a "Genetic Disease and the Eye"
conference is held at Cedars-Sinai Medical Center to provide healthcare advances in this
area, including how to provide appropriate medical care to patients with genetic eye
diseases.
Milestones
In Pediatric Corneal Transplantation at Cedars-Sinai Medical Center |
| 1992 |
First "Genetic Disease and the Eye"
conference. |
| 1992 |
First novel corneal procedure is performed on
baby with Fraser Cryptophthalmos syndrome. |
| 1993 |
Second "Genetic Disease and the Eye"
conference is held. |
| 1994 |
A dozen pediatric corneal procedures are
performed annually. |
| 1994 |
Recommendations for preventing blindness in
children with congenital corneal disease are presented to the World Health Organization at
the United Nations Conference in New York. |
| 1995 |
The Cornea-Genetic Eye Medical Clinic becomes
an international center with referrals from Mexico, Italy and Saudi Arabia. |
| 1996 |
More than 200 healthcare professionals attend
the third "Genetic Disease and the Eye" conference. |
| 1996 |
Favorable outcomes are achieved with pediatric
corneal transplantation and the results presented at the European Congress of
Ophthalmology in Milan, Italy. |
| 1997 |
Good visual outcome in an infant with
Frasers Syndrome, who received a cornea transplant in 1992 and is now five years
old, is reported and published in the American Journal of Ophthalmology. |
| 1997 |
To date, approximately 40 pediatric corneal
procedures and 100 genetic consultations are being performed, annually, with referrals
from more than half-a-dozen countries. |
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