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<p>Purtscher-like retinopathy is a rare occlusive microvasculopathy that causes acute, painless visual loss and characteristic retinal lesions. Although typically trauma-related, it can occur in systemic conditions such as nephrotic syndrome (NS), driven by hypercoagulability, complement activation, and microembolization. We report a 3-year-old girl with steroid-resistant NS presenting with sudden vision loss, including inability to fixate and intermittent exotropia, without trauma or hypertension. Fundoscopy revealed bilateral peripapillary retinal whitening with periarteriolar sparing, cotton wool spots, and intraretinal hemorrhages. Laboratory tests showed hypoalbuminemia (1.7&#xa0;g/dL) and nephrotic-range proteinuria; kidney biopsy suggested podocytopathy. She was receiving prednisolone and cyclosporine (trough 84 ng/mL), which was discontinued due to concern for drug-induced vasculopathy. Treatment included intravenous methylprednisolone pulses followed by oral tapering. Whole-exome sequencing identified a de novo pathogenic WT1 variant (c.1447 + 5G &gt; A), establishing a genetic etiology (NPHS4) for steroid-resistant nephrotic syndrome in this patient. At 6 months, visual acuity improved from light perception to counting fingers at a distance of 2&#xa0;m. This case highlights the need to consider Purtscher-like retinopathy in NS patients with acute vision loss, particularly in the context of steroid resistance or calcineurin inhibitor therapy. Prompt ophthalmologic evaluation and careful medication review are critical to prevent permanent visual impairment and to optimize recovery.</p>

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Purtscher-like retinopathy presenting as acute vision loss in childhood nephrotic syndrome: case report with review of literature

  • 期刊论文

  • 发表日期：

    2026年6月29日

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  • Deepthika Sadasivam,
  • Simar Rajan Singh,
  • Sushmitha Poojary,
  • Divya Chauhan,
  • Pujitha Vallabhaneni,
  • Karalanglin Tiewsoh,
  • Lesa Dawman

摘要

Purtscher-like retinopathy is a rare occlusive microvasculopathy that causes acute, painless visual loss and characteristic retinal lesions. Although typically trauma-related, it can occur in systemic conditions such as nephrotic syndrome (NS), driven by hypercoagulability, complement activation, and microembolization. We report a 3-year-old girl with steroid-resistant NS presenting with sudden vision loss, including inability to fixate and intermittent exotropia, without trauma or hypertension. Fundoscopy revealed bilateral peripapillary retinal whitening with periarteriolar sparing, cotton wool spots, and intraretinal hemorrhages. Laboratory tests showed hypoalbuminemia (1.7 g/dL) and nephrotic-range proteinuria; kidney biopsy suggested podocytopathy. She was receiving prednisolone and cyclosporine (trough 84 ng/mL), which was discontinued due to concern for drug-induced vasculopathy. Treatment included intravenous methylprednisolone pulses followed by oral tapering. Whole-exome sequencing identified a de novo pathogenic WT1 variant (c.1447 + 5G > A), establishing a genetic etiology (NPHS4) for steroid-resistant nephrotic syndrome in this patient. At 6 months, visual acuity improved from light perception to counting fingers at a distance of 2 m. This case highlights the need to consider Purtscher-like retinopathy in NS patients with acute vision loss, particularly in the context of steroid resistance or calcineurin inhibitor therapy. Prompt ophthalmologic evaluation and careful medication review are critical to prevent permanent visual impairment and to optimize recovery.