Pseudo-duplication of the optic disc with maculo-schisis in a 9-year-old patient

PURPOSE: To report a unique case of pseudo-duplication of the optic disc with maculo-schisis. OBSERVATIONS: A 9-year-old girl presented with decreased vision in the left eye. Her measured visual acuity was 20/50. Dilated fundus examination revealed an optic disc-like lesion with a large crater-like depression, pale color and aberrant retinal vasculature arising from its lower region. Optical coherence tomography (OCT) demonstrated maculo-schisis emerging from the optic disc complex and involving the foveal region. Further pathological findings of cellophane maculopathy with retinal striae was observed at the papillo-macular bundle. On MRI scan there were neither signs of doubling of the optic nerve nor any other optic nerve malformations. The patient underwent 25 Gauge Pars Plana vitrectomy (PPV) with posterior hyaloid peel, ILM peel and gas tamponade with SF6. Eight month post-operatively, a significant reduction in intra-retinal fluids and an improvement in the maculo-schisis magnitude were seen. CONCLUSIONS AND IMPORTANCE: To the best of our knowledge, this is the first reported case of a pseudo-duplication of the optic disc with maculo-schisis treated with PPV.