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April 2014 to corneal scarring and infection. The most important consideration after diagnosis is ensuring that aggressive systemic immunosuppres- sion has been used to achieve disease control prior to considering any surgical intervention. Corticos- teroids, dapsone, azathioprine, methotrexate, cyclosporine, mycophenolate mofetil, cyclophos- phamide, and intravenous im- munoglobulin therapy have all demonstrated efficacy in arresting progression of OCP. 1 Given this patient's age, it is important to use non-corticosteroid immunosuppres- sants to limit steroid-related side ef- fects. Visual rehabilitation for these patients requires consideration of the multiple challenges caused by OCP. Dry eye, cicatricial entropion, trichiasis, limbal stem cell defi- ciency, and deficiency of mucin-pro- ducing goblet cells all threaten the survival of any corneal graft or the type I Boston keratoprosthesis. After the inflammation is con- trolled, surgical planning of the eye- lid, anterior segment and cornea can be done based on the visual needs of the patient and prognosis after sur- gery. Initial steps involve the repair of the cicatricial entropion of the left upper eyelid followed by fornix reconstruction with amniotic membrane grafting, as described by Barabino. 2 After fornix reconstruc- tion, the stability of the ocular surface and limbal stem cells should be assessed prior to consideration of corneal grafting. Limbal stem cell deficiency, especially in a severe case as our patient, may present a signifi- cant challenge to graft survival. Persistent epithelial defects or poor wound healing may occur, which could result in graft failure. Limbal stem cells from living relatives, ex- panded ex vivo in culture, may offer hope for such patients in the future, but this therapy remains investiga- tional. 3 We commonly wait a minimum of three months between anterior segment reconstruction and any type of corneal transplantation or keratoprosthesis surgery. This will allow time for healing, resolution of inflammation from the surgery, and time to monitor for recurrence of symblepharon, which may recur in one-third of cases. Given that this patient has limited functional vision due to the significant eyelid and anterior segment involvement, we have recommended a type I or type II Boston keratoprosthesis, depending on the quality of the ocular surface after anterior segment reconstruction. Outcome This patient is scheduled to undergo combined repair of the left upper eyelid cicatricial entropion with lysis of symblepharon, ankyloblepharon and fornix reconstruction with am- niotic membrane grafting. She will be further considered for placement of a keratoprosthesis. EW References 1. Foster CS, Sainz De La Maza M. Ocular cicatricial pemphigoid review. Curr Opin Allergy Clin Immunol. 2004; 5:435-9. 2. Barabino S, Rolando M, Bentivoglio G, Mingari C, Zanardi S, Bellomo R, Calabria G. Role of amniotic membrane transplantation for conjunctival reconstruction in ocular-cicatricial pemphigoid. Ophthalmology. 2003; 110: 474- 80. 3. Burman S, Sangwan V. Cultivated limbal stem cell transplantation for ocular surface reconstruction. Clin Ophthalmol. 2008; 2: 489- 502. Editors' note: Drs. Elia and Huang are affiliated with Yale University School of Medicine, Department of Ophthalmology and Visual Science, New Haven, Conn. They have no financial interests related to this article. Contact information Huang: john.huang@yale.edu EyeWorld factoid Cicatricial pemphigoid usually begins in late adulthood (e.g., 50s or 60s), affects more women than men, and has a variable prognosis. Source: National Institutes of Health digital.eyeworld.org Digital.eyeworld.org is EyeWorld's web-based mobile optimized site. Users may view the current edition, past editions, and all other publications available from ASCRSMedia including supplements and digital media. Viewers can download an issue, send articles via email or a variety of social media outlets, and bookmark items for later viewing. Included on this site are links to: • EyeWorld's monthly digital edition and archives • Ophthalmology Business and archives • EyeWorld Asia-Pacific edition • EyeWorld's new & improved Video Reporter • EyeWorld's Twitter & Facebook pages • EyeWorld's symposia registration and supplements EWrePlay.org Live interviews from major meetings now archived and searchable by meeting, doctor and content across all meetings. Watch the experts discuss clinical trends and pearls to help you in your daily practice.

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