Surgical Management Of Advanced Schnyder’S Crystalline Dystrophy And Intumescent Cataract: An Open-Sky Approach In A Complex Anterior Segment

To report the surgical management of an advanced case of Schnyder’s Crystalline Dystrophy (SCD) associated with an intumescent cataract and complex anterior segment anatomy. The case highlights the successful use of a combined penetrating keratoplasty and open-sky cataract extraction to address severe corneal and lenticular opacities.

Hospital de Clínicas “José de San Martín”, University of Buenos Aires, Buenos Aires, Argentina.

A 59-year-old male presented with severe bilateral corneal opacity and visual loss. Ocular history included bilateral trabeculectomies and Ahmed glaucoma valve (AGV) implantation in the right eye (OD). Visual acuity was hand motion in the OD and poor light projection in the left eye (OS).

Slit-lamp examination revealed bilateral prominent arcus lipoides and dense, disc-shaped, polychromatic crystalline deposits involving the central corneal stroma. The OD showed an intumescent white cataract, athalamia, a flat trabeculectomy bleb, and an AGV tube (Figure 1). In the OS, a cataract and a functioning trabeculectomy bleb were observed (Figure 2). Fundus examination was not possible due to media opacity; B-scan ultrasonography confirmed retinal attachment in both eyes. (Figure 3).

Ultrasound biomicroscopy  of the OD demonstrated occlusion of the AGV tube by iris tissue, extensive anterior and posterior synechiae, athalamia, and an enlarged intumescent lens with increased anteroposterior diameter (Figure 4a-4d).

Given the severe corneal opacity, distorted anterior segment anatomy, and high surgical risk, a combined penetrating keratoplasty and open-sky extracapsular cataract extraction was performed. Open-sky ECCE allowed safe lens removal and restoration of anterior segment anatomy. The eye was left aphakic, with secondary intraocular lens implantation planned after stabilization.

Advanced Schnyder’s Crystalline Dystrophy may present with severe corneal opacity and coexist with complex anterior segment anatomy secondary to prior glaucoma surgery. In selected cases, an open-sky approach combining penetrating keratoplasty and cataract extraction represents a safe and effective surgical strategy. 

Early recognition of SCD is essential, and lipid profile assessment should be considered. Multidisciplinary management, including cardiovascular evaluation, is recommended due to the systemic association with dyslipidemia.