Eyeworld

90 | EYEWORLD | SPRING 2024 C ORNEA Relevant disclosures Deng: None Farid: None Jeng: None Contact Deng: deng@jsei.ucla.edu Farid: mfarid@hs.uci.edu Jeng: Bennie.Jeng@Pennmedicine. upenn.edu Marjan Farid, MD Dr. Farid has found the best approach for severe cases of LSCD to be ocular surface stem cell transplantation with systemic immunosuppres- sion, and her practice at UC Irvine is a site that's set up to perform these procedures and manage them postoperatively. Limbal stem cell deficiency occurs when the limbal stem cells of the ocular surface are dis- eased, Dr. Farid said. Patients might have mild early disease, she said, which can be caused by chronic contact lens wear resulting in chronic hypoxic damage to the limbal stem cells. "Those early stem cell deficiency patients, where part of their limbus is affected, some of these can be medically managed," she said. This could in- clude things like removing the offending agents from ocular surface, using preservative free eye drops, and decreasing the inflammation of the ocular surface. Early disease can also be caused by chronic exposure to preservatives in drops, such as long-term use of glaucoma medications, Dr. Farid said. Then, she said, you have patients who have very severe ocular surface disease and severe limbal stem cell deficiency, and it may be combined with significant conjunctival scarring. These are patients who may have had chemical or thermal burns, patients with severe auto- immune-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 pa- tients will often need surgical intervention, Dr. Farid said. "These patients respond very poorly to primary keratoprosthesis, for example. They do much better when ocular surface is trans- planted, 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 re- ally 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 treat- ment 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 coordi- nator 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 a higher rejection risk." In terms of learning how to use this tech- nique of transplantation with systemic immu- nosuppression, 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 ed- ucate 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. continued from page 88

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