Keratoprosthesis in dry eye disease

Abstract

Bilateral corneal blindness with severe dry eye disease (DED), total limbal stem cell deficiency with underlying corneal stromal scarring and vascularization, combined with adnexal complications secondary to chronic cicatrizing conjunctivitis is a highly complex situation to treat. In such eyes, procedures such as penetrating keratoplasty alone or combined with limbal stem cell transplantation are doomed to fail. In these eyes, keratoprosthesis (Kpro) or an artificial cornea is the most viable option, eliminating corneal blindness even in eyes with autoimmune disorders such as Stevens-Johnson syndrome, ocular mucous membrane pemphigoid, Sjogren's syndrome, and nonautoimmune disorders such as chemical/thermal ocular burns, all of which are complex pathologies. Performing a Kpro in these eyes also eliminates the need for systemic immunosuppression and may provide relatively early visual recovery. In such eyes, the donor cornea around the central cylinder of the Kpro needs to be covered with a second layer of protection to avoid desiccation and progressive stromal melt of the underlying cornea, which is a common complication in eyes with severe DED. In this review, we will focus on Kpro designs that have been developed to survive in eyes with the hostile environment of severe DED. Their outcomes in such eyes will be discussed.

Keywords: Chronic cicatrizing conjunctivitis; Stevens–Johnson syndrome; dry eye disease; keratoprosthesis; limbal stem cell deficiency; mucous membrane pemphigoid; ocular chemical burns.

Figure 1

Preoperative images before Kpro surgery in dry eye disease. (a) Post–toxic epidermal necrolysis, corneal neovascularization with total leukomatous corneal scar is found in eye. A Boston type II Kpro was performed in this eye; the postoperative image is shown in Fig. 3b. (b) Post–acid injury, an eye showing superior symblepharon with total leukomatous corneal scar with dermalization of the corneal surface. A tibial Kpro was performed in this eye; the postoperative image is shown in Fig. 3c. (c) Post-Stevens–Johnson syndrome sequelae. Superior symblepharon with dermalization of the entire ocular surface with underlying total leukomatous corneal scar and severe dry eye disease; an LVP Kpro was performed in this eye. Kpro = keratoprosthesis

Figure 2

Different stages of the same eye, from acute stage of cicatrizing conjunctivitis to keratoprosthesis. (a) Right eye of a patient immediately after TEN. (b) Complete ankyloblepharon with dermalization of the ocular surface 2 years after TEN. (c) The postoperative image of the same eye, 3months after the ocular surface mucous membrane grafting was performed. (d) Six months after Lux keratoprosthesis. TEN = toxic epidermal necrolysis

Figure 3

Different types of keratoprosthesis in dry eye disease secondary to chronic cicatrizing conjunctivitis. (a) Left eye of a patient 5 years after MOOKP was performed for ocular sequelae secondary to SJS. (b) Left eye of a patient 14 years after Boston type II keratoprosthesis was performed for SJS sequelae. (c) Right eye of a patient 5 years after tibial keratoprosthesis was performed for ocular chemical burn secondary to acid injury. (d) Right eye of a patient 4.5 years after LVP keratoprosthesis was performed for SJS sequelae. (e) Right eye of a patient 3 months after Lux keratoprosthesis was performed for SJS sequelae. MOOKP = modified osteo-odonto keratoprosthesis, SJS = Stevens–Johnson syndrome