Use of Cross-linked Donor Corneas as Carriers for the Boston Keratoprosthesis

• Lack of sterile ulcers via slit lamp [ Time Frame: post op day 1, week 1, 4, 8, 12, 16, 24, 36, 52. ] [ Designated as safety issue: No ]
• Ocular safety [ Time Frame: measured at day 1, and week 1, 4, 8, 12, 16, 24, 36, 52 ] [ Designated as safety issue: Yes ]
  incidence and severity of ocular adverse events during the study (ophthalmic examination, adverse events spontaneously reported)
• Systemic safety [ Time Frame: measured at day 1, and week 1, 4, 8, 12, 16, 24, 36, 52 ] [ Designated as safety issue: Yes ]
  incidence and severity of systemic adverse events during the study (clinical laboratory, adverse events spontaneously reported).

The purpose of the pilot study is to test the efficacy and safety of riboflavin/Ultraviolet A (UVA) cross-linked human donor corneas as carriers for the Boston Keratoprosthesis (Boston KPro) in patients with higher risk for corneal melting (keratolysis). The corneal cross-linking technique has been shown to strengthen the cornea and resist degradation by the body's own enzymes. The use of highly-resistant donor corneas is meant to eliminate the serious post-operative complication of corneal melting (keratolysis) that repeatedly occurs in Boston KPro patients with severe ocular burns, autoimmune and cicatrizing diseases.

• Chemical Injuries
• Unspecified Complication of Corneal Transplant
• Autoimmune Diseases
• Ocular Cicatricial Pemphigoid
• Stevens Johnson Syndrome
• Lupus Erythematosus, Systemic
• Rheumatoid Arthritis
• Other Autoimmune Diseases

Inclusion Criteria:

1. Ability to provide written informed consent and comply with study assessments for the full duration of the study.
2. Age > 18 years.
3. A negative urine pregnancy test.
4. Candidate for a Boston Keratoprosthesis/Corneal transplant.
5. Generally good stable overall health.

6. Patients with an eye at risk for a cornea sterile ulcer which includes:

   • Chemical injuries.
   • Autoimmune diseases (ocular cicatricial pemphigoid, Stevens Johnson Syndrome, systemic lupus erythematosus, rheumatoid arthritis, or other autoimmune diseases).
   • History of previous sterile corneal ulceration requiring a cornea transplant.

Exclusion Criteria:

1. Age < 18 years.
2. Inability to provide written informed consent and comply with study assessments for the full duration of the study.
3. Pregnant or lactating women.
4. No or minimal tear production.
5. Ocular or periocular malignancy.
6. Inability to wear a contact lens due to lid abnormalities or shortened fornix.
7. Signs of current infection, including fever and current treatment with antibiotics.
8. Participation in another simultaneous medical investigation or trial