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El siguiente reportaje esta copiado
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Center for the Treatment of Ocular Disease
A Center for the Treatment of Ocular Surface Disease (CTOSD) has been established under the directorship of Dr. Edward J. Holland. Dr. Holland and his partner Dr. Michael L. Nordlund have developed the CTOSD in collaboration with the Cincinnati Eye Institute, the Department of Ophthalmology at the University of Cincinnati and the St. Elizabeth Hospital in Edgewood, KY. The mission of the CTOSD is to provide expert clinical care for patients with complicated ocular surface stem cell disorders, to further investigate the pathogenesis of stem cell disease and to develop ever improving therapies. Currently, the CTOSD is focusing on stem cell disease resulting from aniridia, thermal and chemical injuries and inflammatory conditions such as Stevens Johnson Syndrome, ocular cicatricial pemphigoid and atopic keratoconjunctivitis. Patients interested in scheduling an evaluation with Dr. Holland or Dr. Nordlund can contact the CTOSD at the phone number and address listed below.
The CTOSD is conducting several scientific studies. Current basic science research projects are directed at improving our understanding of the pathogenesis of limbal abnormalities in aniridia and the response of stem cells to chemical and mechanical injuries of the cornea. Several clinical studies are also in progress. One study is aimed at more clearly defining the natural history of aniridia and its manifestations. This study primarily involves reviewing charts of aniridic patients to chronical the development of the manifestations of aniridia. Patients with aniridia can participate by providing copies of their ophthalmic medical records. A second study that will be starting in the near future involves evaluating aniridic limbal tissue with varying stages of keratopathy. Patients undergoing stem cell transplantation will be eligible to participate in this study. Patients interested in participating in these clinical investigations can contact the CTOSD using the contact information below.
Dr. Edward Holland received his MD degree from Loyola-Stitch School of Medicine, Chicago, IL. Following a one year internship at Henry Ford Hospital in Detroit, Dr. Holland received his ophthalmology residency training at the University of Minnesota. Dr. Holland then completed fellowships in both Cornea and External Disease at the University of Iowa and Ocular Immunology at the National Eye Institute, National Institutes of Health. He has been a member of the Cornea Editorial Board since 1996. He is co-editor of the textbook Cornea and has over 100 publications in peer-reviewed journals. Dr. Holland is a national and international guest lecturer and has been invited to speak at over 125 lectures.
Dr. Holland is the Director of Cornea Services at the Cincinnati Eye Institute and Medical Director at the Northern Kentucky Eye Laser Center. Additionally, he is a Professor of Ophthalmology at the University of Cincinnati, College of Medicine. He is Chairman of the Medical Advisory Board of the Eye Bank Association of America and of the Skill Transfer Committee for the American Academy of Ophthalmology. He is the recipient of the 1993 Academy of Ophthalmology Honor Award and President-elect for the Castroviejo Cornea Society (International Society of Corneal and Refractive Surgeons).
Michael L. Nordlund, M.D./Ph.D. joined the Cincinnati Eye Institute one year ago in the Division of Cornea and External Disease and Refractive Surgery. After completing his undergraduate degree in biology at Bucknell University, Dr. Nordlund entered the Medical Scholars Training Program at University of Cincinnati, College of Medicine. He graduated with doctorate degrees in both medicine and neuroscience in 1996. During his graduate training, Dr. Nordlund published many important scientific papers and developed his first interests in ophthalmology. He subsequently completed an internship in internal medicine at University Hospital in Cincinnati and a residency at the prestigious Kellogg Eye Center at the University of Michigan. Recently he returned to Cincinnati to complete a fellowship in Cornea, External Disease and Refractive Surgery under the direction of Dr. Edward J. Holland at the Cincinnati Eye Institute and the University of Cincinnati.
As a fellow, Dr. Nordlund has pursued his interest in ocular surface stem cell disease. He authored a chapter for a new textbook on stem cell diseases. He was recently nominated for a young investigator achievement award from the Research to Prevent Blindness. Dr. Nordlund continues his research in stem cell disease as a faculty member of the Department of Ophthalmology at the University of Cincinnati. Dr. Nordlund has been elected into several honor societies including Alpha Omega Alpha and the Society for Heed Fellows. He is also a member of many professional societies including the American Academy of Ophthalmology, the Patton Society, the Castroviejo Cornea Society and the Association of Cataract and Refractive Surgeons.
We have attached an update on new developments in therapies for patients with congenital aniridia. Additionally, the update describes the establishment of the CTOSD and important research programs that we are currently conducting. We hope you will post this information on your web site. It is important that patients with aniridia know that corneal transplantation by itself has a poor prognosis and that there is an alternative. Patients who have unsuccessful corneal transplants have increased risks of rejection for future therapies including ocular surface stem cell transplantation. We have briefly described Dr. Holland’s and Dr. Nordlund’s credentials. If you feel you need additional information please contact us.
Sincerely,
Edward J. Holland, MDMichael L. Nordlund, MD,PhDCenter for the Treatment of Ocular Surface DiseaseCincinnati Eye Institiute10494 Montgomery RoadCincinnati, OH 45242Phone (800) 984-5133Fax (513) 984-4870
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New Developments in Aniridia
Congenital aniridia is an autosomal dominantly inherited congenital disorder of the eye characterized by maldevelopment of multiple ocular structures. The disease occurs in 1/64,000 to 1/96,000 live births. Affected individuals express a spectrum of iris abnormalities with the most severe being clinical absence of the iris and the least severe being mild stromal hypoplasia with a normal appearing pupil. In addition to iris abnormalities, aniridic patients suffer from foveal hypoplasia, optic nerve hypoplasia, glaucoma, and cataracts. In youth, visual function is dependent upon foveal hypoplasia and nystagmus. As patients age, cataract and glaucoma may progress to further limit vision.
An additional and under-recognized manifestation contributing to progressive visual loss in aniridic patients is corneal disease. Aniridic keratopathy manifests in the first decade of life as thickened irregular epithelium of the peripheral cornea. The epitheliopathy is typically accompanied by fine superficial neovascularization, advances centripetally with age and eventually involves the entire corneal surface. Dysfunction of the abnormal epithelium can be confirmed clinically by the presence of late staining with fluorescein. Overtime subepithelial fibrosis and stromal scarring result and predispose aniridic patients to recurrent erosions, corneal ulceration, chronic pain and blindness.
Traditional treatment of visually significant aniridic keratopathy has been limited to lamellar and penetrating keratoplasty with dismal results. Kremer et al. reported recurrence of ocular surface abnormalities in 91% of treated eyes and rejection of corneal grafts in 64% of patients. Gomes documented graft failure in 100% of patients requiring repeat penetrating keratoplasty for recurrent aniridic epithelial disease. Despite these findings, many ophthalmologists continue to treat aniridic keratopathy with corneal transplantation alone.
The pathogenesis of aniridic keratopathy remains unknown. The most widely accepted theory is that the epitheliopathy results from a deficiency of progenitor cells responsible for renewing the corneal epithelium. These corneal epithelial stem cells are located exclusively at the limbus and thus have been termed limbal stem cells. The suggestion that aniridic keratopathy results from dysfunction of limbal stem cells originates from several observations. First, the clinical morphology of the limbus in aniridic patients has been shown to be abnormal. Specifically, aniridic patients lack the palisades of Vogt, which are radial infoldings of epithelium at the corneoscleral limbus thought to demarcate the location of limbal stem cells. Additionally, work by Kinoshita suggests that the palisades serve as a marker for corneal stem cell differentiation. The absence of this important limbal structure suggests localization of the epithelial abnormalities in aniridic patients to the limbus. Additional studies of aniridic tissue have indeed documented aberrant differentiation of the corneal epithelium. Healthy corneal epithelium is a stratified squamous epithelium that contains no goblet cells or other conjunctival specific elements. In aniridic patients, goblet cells are present in significant numbers in the peripheral corneal epithelium.
Ocular surface stem cell transplantation is a new therapy aimed at correcting stem cell deficiency and has been highly successful in treating the corneal disease associated with aniridia. The goal of the surgery is to transplant normal stem cells from donor eyes to the stem cell deficient aniridic eye. The grafted tissue is place circumferentially around the recipient cornea and secured with sutures. With time, the donor tissue resurfaces the recipient cornea with normal epithelium. A recent analysis of patients treated at our center demonstrates the establishment of a stable ocular surface in 90.5% of aniridic patients treated with appropriate immunosuppression. Of these patients, approximately one third were spared the need for subsequent corneal transplantation. In those patient in which significant scarring had already occurred prior to stem cell transplantation, corneal transplantation was performed and was successful in 85% of patients on appropriate immunosuppression. Ocular surface stem cell transplantation has significantly improved the prognosis for aniridic patients to recover and maintain their best possible vision.
A second exciting development in the care of aniridic patients has been the development of the Morcher artificial iris diaphragm. Morcher makes two styles of the artificial iris. One version consists of an artificial black iris attached to a standard polymethylmethacrylate intraocular lens. This type of iris is best used when suturing of the lens is required to secure the lens in place. An advantage of this implant is that it is very stable. Unfortunately, large incisions are required to place the implant in the eye because it is not foldable. The second type of iris implant is a flexible ring which can be placed through a small incision. It is best used in patients with an intact capsule (the structure which holds the natural lens of the eye in place). Typically, a foldable acrylic intraocular lens is inserted through a small incision and the iris rings are then placed in front of the lens. Both versions have the potential to greatly reduce glare and photosensitivity and to improve visual acuity significantly in aniridic patients.
A Center for the Treatment of Ocular Surface Disease (CTOSD) has been established under the directorship of Dr. Edward J. Holland. Dr. Holland and his partner Dr. Michael L. Nordlund have developed the CTOSD in collaboration with the Cincinnati Eye Institute, the Department of Ophthalmology at the University of Cincinnati and the St. Elizabeth Hospital in Edgewood, KY. The mission of the CTOSD is to provide expert clinical care for patients with complicated ocular surface stem cell disorders, to further investigate the pathogenesis of stem cell disease and to develop ever improving therapies. Currently, the CTOSD is focusing on stem cell disease resulting from aniridia, thermal and chemical injuries and inflammatory conditions such as Stevens Johnson Syndrome, ocular cicatricial pemphigoid and atopic keratoconjunctivitis. Patients interested in scheduling an evaluation with Dr. Holland or Dr. Nordlund can contact the CTOSD at the phone number and address listed below.
The CTOSD is conducting several scientific studies. Current basic science research projects are directed at improving our understanding of the pathogenesis of limbal abnormalities in aniridia and the response of stem cells to chemical and mechanical injuries of the cornea. Several clinical studies are also in progress. One study is aimed at more clearly defining the natural history of aniridia and its manifestations. This study primarily involves reviewing charts of aniridic patients to chronical the development of the manifestations of aniridia. Patients with aniridia can participate by providing copies of their ophthalmic medical records. A second study that will be starting in the near future involves evaluating aniridic limbal tissue with varying stages of keratopathy. Patients undergoing stem cell transplantation will be eligible to participate in this study. Patients interested in participating in these clinical investigations can contact the CTOSD using the contact information below.
Center for the Treatment of Ocular Surface Disease
Edward J. Holland, MD
Michael L. Nordlund, MD,PHD
Cincinnati Eye Institiute
10494 Montgomery Road
Cincinnati, OH 45242
Phone (800) 984-5133
Fax (513) 984-4870
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