PROSE device being filled with sterile saline prior to application. Information on PROSE network sites can be found at www.bostonsight.org
Case Study—Therapeutic Lens Options in Pediatric Ocular Surface Disease
Stevens–Johnson syndrome (SJS) and its most severe form, Toxic Epidermal Necrolysis (TEN), are acute blistering disorders of the skin, and mucous membranes that commonly result in significant scarring of the ocular surface and eyelids. The result can be painful, blinding, and debilitating with children often affected. Repairing the damage caused to the eyelids and ocular surface can be challenging and fraught with complications.
This 7-year-old Male was referred for consideration of PROSE treatment for support of the ocular surface and stabilization of pannus 2 years after hospitalization for TEN. At the time of referral, there was forniceal shortening, cicatricial lagophthalmos, exposure keratitis, trichiasis, distichiasis, and inferior pannus with keratinization OU. Multiple lid procedures had been undertaken OU, including mucous membrane grafting to lid margins OS only. Topical regimen was loteprednol drops BID and vitamin A ointment at night. Daily disposable hydrogel lenses were worn on an extended wear basis for comfort with weekly replacement. The patient was correctable to 20/20 in each eye. Figure 9.2a is image of OD at presentation with hydrogel soft contact lens in place. Theessels appeared inactive, so a recommendation was made to switch to daytime only wear of a higher Dk SiHy lens, with substitution of ointment for lens wear at night, to reduce any hypoxic challenge that might trigger progression of vessels. Daily disposable SiHy lenses were substituted when that option became available. Eventually, the topical steroid was eliminated from the regimen. Figure 9.2b shows the OD with a soft contact lens in place 18 months after initial referral, when patient was referred again for consideration of alternative treatment options for OS. Improved clarity of inferior pannus OD with extended wear of higher Dk soft lens is notable. This repeat evaluation was sought after a tuft of corneal neovascularization was noted inferiorly OS, Fig. 9.3a, and introduction of topical anti-VEGF therapy was being considered. It was decided to optimize surface support prior to treating the vessels themselves. Topical steroid was resumed and PROSE treatment was undertaken with the objective of eliminating any contact with cornea that might induce breakdown and activate neovascularization. Figure 9.3b reveals stabilization of fine pannus with inactive vessels after 3.5 years of daily wear of PROSE device, with no anti-VEGF treatment undertaken. Five years after presentation vision is 20/20 in each eye with daily wear of PROSE device OU and regimen of loteprednol gel and vitamin A ointment QHS.
a Right eye of 7 year old male at time of referral, 2 years after hospitalization for TEN. There is daily disposable soft contact lens in place which was being worn on a weekly replacement extended wear basis. b Right eye 18 months later after switch to daytime only wear of higher Dk silicone hydrogel lens. Note improved clarity of inferior pannus. Topical steroid was eliminated at some time during the prior year
a Left eye at this same 18 month visit referred for new development of inferior neovascular pannus despite switch to daily wear only of higher Dk silicone hydrogel lens. Topical steroid was resumed and PROSE treatment was undertaken to eliminate any mechanical contact between contact lens and cornea that might contribute to inflammation and neovascularization. b Left eye in PROSE device 3.5 years later, age 12, with continuation of very low dose soft steroid. There has been no progression of vessels or pannus. Vision remains 20/20 in each eye in PROSE devices
Contact lenses play an important role in the management of eye disease in children. Contact lenses are useful when there is high refractive error or irregular cornea during the critical period for amblyopia, especially in unilateral cases. Contact lenses can be considered as an alternative to spectacle correction, if patients or their caregivers can manage insertion and removal, and if they are able to comply with instruction in lens care and hygiene. Contact lenses can also be very useful in the management of ocular surface disease in the pediatric age group. Ophthalmologists caring for children with corneal disease should be aware of advances in the field of contact lens and be familiar with various contact lens options for the pediatric population.
Compliance with Ethical Requirements
Deborah S. Jacobs and Aaron C. Barrett declare that they have no conflict of interest. Deborah S. Jacobs, M.D. is a full-time salaried employee of Boston Foundation for Sight, 501(c)3. She has no proprietary or financial interest in any contact lens or prosthetic device.
No human or animal studies were carried out by the authors for this article.
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