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Letter to the Editor
Ophthalmic features of primary oxalosis after combined liver/kidney transplantation
  1. D M ALCORN
  1. Stanford University School of Medicine, Department of Ophthalmology and Pediatrics, Stanford, CA 94305, USA
  2. University of California-San Francisco, Department of Ophthalmology, San Francisco, CA 94120, USA
  1. D R FREDRICK
  1. Stanford University School of Medicine, Department of Ophthalmology and Pediatrics, Stanford, CA 94305, USA
  2. University of California-San Francisco, Department of Ophthalmology, San Francisco, CA 94120, USA
  1. Dr Deborah Alcorn, Stanford University School of Medicine, Department of Ophthalmology, 300 Pasteur Drive, Suite No A157, Stanford, CA 94305–5308, USA dalcorn{at}leland.Stanford.edu

https://doi.org/10.1136/bjo.84.11.1318i

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    Editor,—Primary oxalosis is a rare autosomal recessive inborn error of glyoxylate metabolism in which two different enzyme defects lead to increased serum oxalate levels resulting in calcium oxalate crystal deposition in various tissues including the eyes, kidney, myocardium, brain, synovia, skin, and peripheral vessels.1 2 This contributes to urolithiasis and end stage renal failure.

    Ocular features of oxalosis have characteristically included the crystalline retinopathy (flecked retina), black geographic maculopathy, and optic atrophy.1 3-8 We report two additional cases of primary oxalosis who underwent combined liver/renal transplantation at 1 year of age, but who initially did not manifest crystalline retinopathy or optic atrophy but developed poor vision despite successful transplantation.

    CASE REPORTS

    Case 1

    An 18 month old female was initially evaluated before her simultaneous kidney/liver transplant. She was undergoing peritoneal dialysis for her end stage renal disease secondary to biopsy confirmed primary oxalosis, diagnosed at age 6 months.

    Visual acuity was CSM in each eye. She had no pupillary abnormalities. Extraocular motility was normal and she was orthophoric. Her anterior segment was entirely unremarkable without any evidence of conjunctival, corneal, or lenticular opacities (Figs 1, 2, 3).

    Funduscopically she demonstrated striking pigmentary changes symmetrically throughout both eyes. Her optic nerves appeared healthy without optic atrophy. Her vessels were not attenuated. Her foveal area had increased pigment clumping but without exudate or discrete crystals. She had diffuse RPE changes throughout her periphery.

    She underwent combined renal/liver transplantation at 18 months of age and subsequently had annual ophthalmological examinations. On her most recent evaluation at age 6, visual acuity was 5/140 in …

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