Diffuse lipid exudative retinopathy secondary to lipoprotein X- induced hyperlipidemia in the setting of cholestatic liver dysfunction

Purpose: To describe a case of bilateral lipid exudative retinopathy secondary to lipoprotein-X – induced hyperlipidemia in a patient who recently underwent liver transplantation. Methods: A retrospective, longitudinal, chart review was conducted. The patient was seen several times over an eight-month period by a multidisciplinary team. Imaging modalities included ocular coherence tomography (OCT), fundus photography, fluoresceine angiography (FA), and fundus autofluorescence (FAF). Results: Ophthalmic imaging reveled rapidly progressive bilateral lipid exudation, macular edema, and retinal hemorrhages in association with decreased visual function. Laboratory data revealed dangerously elevated levels of cholesterol, low density lipoprotein, and non-HDL lipoprotein levels in association with normal Apolipoprotein-B levels – diagnostic for lipoprotein-x hyperlipidemia in the setting of cholestasis. Therapeutic plasma exchange was conducted which resulted in markedly reduced lipid levels, decrease in the retinal exudation, and improvement in the visual acuity. Conclusion: This case highlights an exceedingly rare ocular complication of lipoprotein-x hyperlipidemia and the limited therapeutic treatment options available for improvements in ocular symptoms.