Corneal perforation in patients with a diagnosis of ocular graft-vs-host disease (GVHD) occurred an average of 3 years after receiving a hematopoietic stem cell transplant (HSCT), highlighting the need for extended follow up in these patients, according to a study published in the American Journal of Ophthalmology Case Reports.

The researchers used a retrospective case series of 14 patients from 4 academic centers in the United States to investigate the clinical disease course and histopathologic correlation in patients with ocular GVHD who progressed to corneal perforation, a rare but vision-threatening complication. 

All patients had received an HSCT before developing ocular GVHD. The investigators evaluated patient demographics, time interval between HSCT and ocular events, visual acuity throughout clinical course, corticosteroid and infection prophylaxis regimens at time of corneal perforation, medical/surgical interventions, and histopathology.


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Among the 14 patients, 14 eyes developed corneal perforation following HSCT. The average age at the time of HSCT was 47±13 years. The majority of patients were men (86%) and White (86%). The indication for HSCT was acute myeloid leukemia in 50% of the cohort; other underlying hematologic malignancies included Waldenstrom’s macroglobulinemia, chronic leukemias, acute promyelocyte leukemia, and Burkitt lymphoma.

The mean interval between HSCT and diagnosis of ocular GVHD was 9.5 months and between HSCT and corneal perforation was 37 months. At first presentation to ophthalmology clinics, patients’ initial best-corrected visual acuity (BCVA) in the affected eye was 20/40 or better in 9 eyes. At the time of corneal perforation, best-corrected visual acuity was poor among all eyes, with the best being 20/150. All eyes underwent corneal gluing, and 8 patients also had subsequent keratoplasty.

The mean follow-up duration after perforation was 34 months (range, 2-140). Despite aggressive management, all eyes had moderate or poor visual outcomes. At the last visit, 36% of eyes had a final BCVA of 20/100 or better, while 50% of eyes perceived only hand motion or worse. Prior to perforation, 79% of patients were taking oral prednisone.

Histopathologic analysis of corneal tissue from 2 patients who had corneal transplant or evisceration after corneal perforation demonstrated ulcerative keratitis with perforation, minimal inflammatory cells, and no microorganisms in the acute phase and filling of the perforation site with a fibrous scar in the healed phase. According to the study authors, these findings were consistent with sterile corneal melt in the setting of immunosuppression, but the role of steroids in the pathogenesis remains unclear.

The primary limitations of the study included its retrospective nature and incomplete medical records.

“In these patients, an extended time interval was identified between the diagnosis of ocular graft-versus-host disease and corneal perforation. This represents a critical window to potentially prevent this devastating outcome,” investigators report. “Further study is required to identify those patients at greatest risk as well as to optimize prevention strategies.”

Reference

Zhang CY, Farooq AV, Harocopos GJ, et al. Corneal perforation in ocular graft-versus-host disease. Am J Ophthalmol Case Reports. 2021;24:101224. doi:10.1016/J.AJOC.2021.101224