EyeWorld Asia-Pacific June 2024 Issue

CORNEA

deficiency, and it may be combined with significant conjunctival scarring. These are patients who may have had chemical or thermal burns, patients with severe autoimmune-related disease (i.e., Stevens Johnson syndrome), or patients with congenital aniridia who develop full LSCD because they genetically have abnormalities with the limbus. These patients will often need surgical intervention, Dr. Farid said. “These patients respond very poorly to primary keratoprosthesis, for example. They do much better when the ocular surface is transplanted, restored with healthy limbal stem cells first, but it does require a whole process.” Dr. Farid said that the process is similar to solid organ transplantation. These patients need systemic immunosuppression just like kidney transplant patients and they need monitoring for blood levels while on those medications, she said. “We have a nurse coordinator who helps manage immunosuppression, manage for toxicity, and so on. Unlike kidney transplant patients who are often very sick to begin with, our patients are relatively healthy. They do really well on these systemic immunosuppressive medications, and then we maintain those for several years, and they can be tapered off slowly over several years,” Dr. Farid said. She called this treatment option “the only really successful way of managing these severe ocular surface disease patients.” Other treatment options, like primary corneal transplants or primary keratoprosthesis transplants on these eyes lead to corneal melts, infection, and failure at a very high rate, she said. Dr. Farid added that management of these patients takes a whole team, and you really need a center that will support having that team, which includes a nurse transplant

coordinator and a nephrology team/kidney transplant doctors who are available to consult with when their help is needed in managing the systemic immunosuppression. Because of this, Dr. Farid said there are only a handful of centers that have the ability, the training, the expertise, and the multidisciplinary approach to manage these patients. The ophthalmology team includes not only the corneal surgeon but often requires oculoplastics and glaucoma surgeons to manage other ocular co-morbidities in these patients. Dr. Farid hopes that more large centers that have access to transplant services will begin to offer this option to patients. “It’s really the right way of doing it and decreases the risk of long-term failures,” she said. “If patients get treated the wrong way or have multiple transplants that they fail, it also primes their body to be at a higher risk of rejection. So even down the road, if we want to do things the right way, their body has been primed to be at a higher rejection risk.” Dr. Farid said there are a lot of resources in the literature on the procedure, techniques, and management. “If someone is at a center where they want to incorporate this, there’s a channel now, and several experts who are committed to helping their peers start this program,” she said. “We don’t want to be the only ones doing it. There’s a commitment to educate our corneal colleagues on this procedure.” One of the big fears from ophthalmologists and cornea specialists is when treatment goes beyond the eye and patients have to be put on systemic therapies. She said it’s important to approach transplant colleagues to help with this and to glean from the knowledge from those doing these in massive numbers and who have had success. In terms of learning how to use this technique of transplantation with systemic immunosuppression,

About the Physicians Sophie Deng, MD, PhD | Professor, Walton Li Endowed Chair in Cornea and Uveitis, Co-Chief, Cornea Division, Stein Eye Institute, University of California, Los Angeles, Los Angeles, California | deng@jsei.ucla.edu Marjan Farid, MD | Director of Cornea, Cataract, and Refractive Surgery, Gavin Herbert Eye Institute, University of California, Irvine, Irvine, California | mfarid@hs.uci.edu Bennie H. Jeng, MD | Harold G. Scheie Chair and Professor, Chair, Department of Ophthalmology, Director, Scheie Eye Institute, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania | bennie.jeng@pennmedicine.upenn.edu

Relevant Disclosures Deng : None Farid : None Jeng : None

This article originally appeared in the March 2024 issue of EyeWorld. It has been slightly modified and appears here with permission from the ASCRS Ophthalmic Services Corp.

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EyeWorld Asia Pacific | June 2024