A Natural History of Silent Brain Syndrome over 36 Years: A case report

A 34-year-old Caucasian male who underwent a ventricular shunt at age 21 presented with bilateral enophthalmos, poor eyelid-globe apposition and exposure keratopathy characteristic of silent brain syndrome. Progressive enophthalmos and corneal decompensation were documented in serial photographs and radiographic studies over 36 years. Over this period, no sequelae of shunt over-drainage were observed. A lumbar puncture at the last follow-up measured CSF opening pressure to be within the normal range. Additional systemic findings included pneumosinus dilatans, loss of adipose tissue in the temporalis fossa and atrophy of the dorsal interossei of the hand. Surgical interventions to preserve ocular function included insertion of orbital floor wedge and sheet implants, sheet orbital roof implants, and retroplacement of canthal tendons. This report chronicles the long-term clinical course of a patient with silent brain syndrome. The systemic changes suggest factors beyond low intracranial pressure may contribute to the pathogenesis of the condition in our patient.