[Penetrating keratoplasty in ocular cicatricial pemphigoid]. / La kératoplastie transfixiante dans la pemphigoïde oculaire cicatricielle.

ABSTRACT

PURPOSE: Ocular cicatricial pemphigoid (OCP) is a chronic progressive disease characterised by exacerbations of immunologically driven conjunctival inflammation. In the advanced stages of the disease, severe xerosis with mechanical factors may contribute to the development of blinding keratopathy. The authors report two cases of penetrating keratoplasty (PK) in patients with OCP and discuss the modalities of the surgical procedure for this particular disease. METHODS: Two patients with stage III OCP according to Foster's classification, underwent PK. Initial and final visual acuity, indications of PK, surgical procedure, postoperative therapy, and complications were recorded. RESULTS: For the first patient, after 3 months of follow-up, the graft is still clear, with a remarkable improvement in vision (3/10). For the second patient, however, graft rejection developed 15 days after the operation, complicated later by endophthalmitis, which was controlled with an antibiotic. DISCUSSION: The progression of ocular pemphigoid involves the filling of the conjunctival fornices, formation of symblepharon with lagophthalmos, and dry eye, from which blinding keratopathy can originate. A corticosteroid-based or immunosuppressant treatment blocks the progression of the fibrosis but does not get rid of palpebrale or corneal anomalies, making surgery necessary. This surgery generally gives disappointing results and should be planned when the disease is perfectly under control, during a lull in the disease so as to avoid reactivation of the fibrosis process. Reconstruction of the conjunctival fornices should also precede any corneal transplantation. CONCLUSION: These results indicate that PK may be performed to restore of sight in patients with advanced OCP after controlling the primary immunological process and aggressive treatment of the mechanical factors damaging the ocular surface.