K T R Jayasanka¹, W D V Thilakarathna¹, D N Nelson¹, A M Nawfer¹, R Ranawaka¹
1- Lady Ridgeway Hospital, Sri Lanka
Abstract Body
Background: Nephrotic syndrome is a common childhood glomerular disorder characterized by nephrotic-range proteinuria, hypoalbuminemia, hyperlipidemia, and edema. While most children are steroid-sensitive, up to 40–60% develop steroiddependent nephrotic syndrome (SDNS), requiring steroid-sparing agents. Cyclosporine, a calcineurin inhibitor, is widely used to maintain remission; however, it is associated with nephrotoxicity and rare neurotoxic complications. Papilloedema secondary to cyclosporine is uncommon but potentially reversible if identified early .
Case presentation: We report a 4-year-old girl with SDNS diagnosed at 2 years of age,managed with prolonged corticosteroids and subsequently commenced on cyclosporine for relapse prevention. She remained in remission for four years with therapeutic trough levels and normal renal function. She later presented with persistent headache, visual blurring, andintermittent vomiting. Neurological examination was unremarkable except for bilateral optic disc oedema on fundoscopy . Neuroimaging and lumbar puncture demonstrated no intracranial mass lesion or raised intracranial pressure. Renal function and cyclosporine trough level (58.8 ng/mL) were within therapeutic range. Cyclosporine-induced papilloedema wassuspected. The drug was discontinued, and acetazolamide initiated. Her symptoms resolved completely within weeks, with full resolution of papilloedema on follow-up. Renal biopsy showed no evidence of chronic cyclosporine nephrotoxicity . She was transitioned to mycophenolate mofetil without recurrence.
Conclusion: Cyclosporine-induced papilloedema is a rare but reversible complication thatmay occur despite therapeutic drug levels and normal renal function. Clinicians should maintain vigilance in children with SDNS presenting with headaches or visual symptoms. Early recognition and prompt withdrawal of cyclosporine are crucial to prevent permanent visual impairment.