Abstract

A 12 year-old male,with gradual diminution of vision since 1year(OD) associated with headache,seizures and hyperpigmented patches all over body,no history of trauma,redness,pain.Positive family history noted in father.On examination- he had café-au lait spots all over body; higher mental function tests are within normal limits.Visual acuity OD- perception of light negative,OS-6/9.Refraction no improvement(OD) -1 DS(OS).Anterior segment(OU)-1-2mm dome shaped,tan dark brown pigmented lesion on iris(Lisch nodules) OD:APD. Fundus(OD)- pale optic disc and (OS)-temporal pallor of the disc noted. MRI brain-gross communicating hydrocephalous with periventricular CSF seepage causing pressure mass effect on supratentorial neuroparenchyma