Surgical And Medical Management Of Ocular And Cutaneous Gvhd Presenting With Severe Ocular Involvement And Ichthyosis

Ocular graft-versus-host disease (oGVHD) typically involves both cell-mediated and humoral immune mechanisms, leading to inflammation of the lacrimal gland, conjunctiva, and ocular surface. While the pathophysiology remains unclear, it is believed that the disease process results in conjunctival and corneal abnormalities through mechanisms. These changes can lead to both evaporative and aqueous-deficient dry eye, conjunctival scarring, corneal neovascularization, ulcers, scarring, and, in severe cases, corneal perforation.

In this study, we aim to highlight the appropriate management of a patient who developed skin symptoms one year after undergoing bone marrow transplantation for ALL and was initially followed with a different diagnosis.

This case was managed in the Cornea Department of Çam and Sakura City Hospital. We evaluated a patient with severe skin and eye involvement who was initially treated under a different diagnosis. The study showed that GVHD's underlying mechanism cannot be controlled with topical treatments alone. Without regular monitoring and systemic treatment, the disease may lead to severe ocular complications, including corneal perforation.

We present the case of a 50-year-old male who reported vision loss, pain, and a stinging sensation in the right eye. The patient had undergone allogeneic bone marrow transplantation 3 years prior for acute lymphocytic leukemia.The patient had no prior history of ocular surgery or trauma in the right eye.

On examination, best corrected visual acuity (BCVA) was hand motion in both eyes. The right eye was hypotonic. Slit-lamp biomicroscopy of the right eye revealed meibomian gland dysfunction, corneal edema, and desmefold. A 1.5x1.5 mm full-thickness corneal defect with a clear border adjacent to the limbus was noted at the 4 o'clock position, along with iris prolapse through the perforation. The anterior chamber was shallow, and a posterior subcapsular cataract was present. 

In the left eye the anterior chamber was shallow, and the patient had widespread posterior synechiae with mature cataract. The left eye was evaluated as inoperable due to chronic detachment.               

A corneal patch graft was planned to preserve the ocular integrity of the right eye following spontaneous corneal perforation.A corneal graft derived from the donor rim was implanted to the patient. The donor rim was punched to equal the corneal defect size and sutured to the recipient with interrupted 10-0 nylon sutures.  An amniotic membrane was also placed over the graft to promote healing and provide additional protection to the ocular surface. No perioperative complications developed.

Management of oGVHD typically includes topical treatments to alleviate symptoms and control inflammation, as well as systemic treatments in more severe cases. In our case, a corneal patch was planned to preserve ocular integrity following corneal perforation. Temporary stabilization can be achieved with SMILE derived lenticule, corneal rim patch grafts, or full-thickness corneal transplantation.Without proper topical and systemic treatment, these interventions' success is limited

The case underscores the importance of diagnosing oGVHD through a multidisciplinary approach based on systemic findings, as well as the need for comprehensive systemic and surgical treatments to suppress inflammation and improve ocular surface survival.