Abstract

To describe a severe case of crystalline retinopathy secondary to hyperoxaluria from short gut syndrome. Case report. A 62-year-old Caucasian woman with short gut syndrome and end-stage renal disease from renal oxalosis presented with chronic bilateral vision loss. She had previously been treated for presumed occlusive vasculitis. Visual acuity on initial examination was 20/400 right eye and 20/100 left eye with an afferent pupillary defect of the right eye. Examination revealed attenuated retinal vasculature and diffuse crystalline infiltration of retinal arterial lumina and throughout the retinas bilaterally. Optical coherence tomography revealed inner retinal atrophy with crystalline deposition in the inner retinal layers. Fluorescein angiography demonstrated delayed vascular filling and dropout consistent with severe ischemic vasculopathy. It was concluded that the short-gut syndrome led to overabsorption of oxalate with subsequent hyperoxaluria leading to retinal atherosclerotic oxalosis. Retinal calcium oxalate deposits due to hyperoxaluria have been previously noted; however, this degree of severe retinal vascular infiltration has not been described. Our patient was receiving hemodialysis, which is associated with high rebound increases in systemic oxalate concentrations. It is important to keep hyperoxaluria in mind as a potential cause of retinopathy in patients with end-stage renal disease presenting with vision loss.

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