Other measures, such as surgical correction of eyelid deformities or eyelash ablation for trichiasis, are occasionally required to achieve ocular surface quiescence. Intraocular surgery is best delayed until disease activity has been under control for an extended period. Hard palate and buccal mucosal grafting can be useful techniques in fornix reconstruction in severe cases. Punctal occlusion, which may already have resulted from cicatrization, can be useful in managing any associated dryeye condition. In general, patients with cicatrizing conjunctivitis have a higher rate of spontaneous extrusion of silicone punctal plugs; thus, permanent punctal occlusion with cautery is often required. Standard PK is generally associated with a very guarded prognosis in patients who develop severe corneal disease in MMP. The Boston keratoprosthesis has been used with some success in these patients.

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Ocular Graft-vs-Host Disease

PATHOGENESIS The pathogenesis of ocular surface disease in graft-vs-host disease (GVHD) is multifactorial but has 2 main components: (1) conjunctival inflammation with or without subepithelial fibrosis and (2) severe keratoconjunctivitis sicca (KCS) from lacrimal gland infiltration by GVHDeffecting T lymphocytes. KCS occurs in 40%–60% of patients with chronic GVHD (cGVHD).

GVHD is a relatively common complication of allogeneic bone-marrow transplantation, which is performed most commonly for hematopoietic malignancies. In this condition, the grafted cells can attack the patient’s tissues, including the skin, gut, lungs, liver, gastrointestinal system, and eyes. Although GVHD can be acute or chronic (developing more than 3 months after bone-marrow transplantation), most ocular complications occur as a manifestation of cGVHD. Clinical features of ocular GVHD (KCS, cicatricial conjunctivitis, scleritis, and others) mirror other inflammatory ocular conditions associated with autoimmune/collagen-vascular diseases.

Conjunctival inflammation in GVHD can be severe and even associated with limbal stem cell deficiency and secondary corneal scarring. Amniotic membrane transplantation and even autologous or allogeneic stem cell transplantation may be performed for more severe cases. Fortunately, the stem cell deficiency is rare.

MANAGEMENT Aggressive lubrication and punctal occlusion are the mainstays of local therapy. Punctal fibrosis is common and must be monitored closely because it can lead to plug extrusion. Severe filamentary keratitis can be treated further with mucolytic agents (10% acetylcysteine) or bandage soft contact lenses. Severe ocular surface disease in GVHD may be associated with active nonocular (often skin) GVHD and may require increased systemic immunosuppression by cyclosporine or tacrolimus (FK506). Topical cyclosporine may also be useful in controlling the disease. Visual disturbances are more commonly due to surface irregularity, but it is important to remember that these patients have a high rate of posterior subcapsular cataracts, which can cause decreased vision. Gas-permeable scleral contact lenses or even soft therapeutic contact lenses can be an important management tool for patients with severe ocular surface disease (Fig 7-14). A keratoprosthesis may be tried as a last resort.