Bilateral Gelatinous Drop-like Corneal Dystrophy: Outcomes of Sequential Keratoplasty

Purpose: Gelatinous drop-like corneal dystrophy (GDLD) is a rare autosomal recessive condition characterized by amyloid deposition in the corneal epithelium and stroma, leading to progressive vision loss. This report aims to present a case of GDLD in a young female, highlighting the clinical course, histopathological findings, and surgical outcomes over the course of 2 years. Case Summary: A 21-year-old female presented with reduced visual acuity in the left eye and a positive family history of vision related issues. She had undergone penetrating keratoplasty (PKP) in the right eye one year earlier at another institution. Examination revealed a clear graft in the right eye with no rejection and diffuse, dense, white opacification with stromal haze in the left eye. Visual acuity (VA) was 20/100 in the right eye and counting fingers near face in the left eye. Given the advanced disease in the left eye, the patient underwent PKP, with successful surgical outcomes. Histopathological analysis of the excised corneal tissue revealed subepithelial amyloid deposits staining positively with Congo red and birefringent under polarized light, confirming GDLD. Postoperatively, the patient demonstrated stable vision and clear grafts in both eyes. Conclusion: GDLD is a challenging condition due to its progressive nature and recurrence post-surgery. This case highlights the importance of histopathological confirmation and tailored surgical management. The successful surgical and postoperative outcomes in this patient highlight the role of keratoplasty in restoring corneal clarity and importance of early interventions to prevent amblyopia. Keywords: Gelatinous drop-like corneal dystrophy, penetrating keratoplasty, corneal dystrophy, subepithelial amyloid deposits, TACSTD2. Declaration of conflict of interest: The authors declare no conflict of interest. Funding sources: None