Silent Brain Syndrome

A 34-year-old male presents for corneal evaluation with progressive decline in vision in both eyes associated with pain, redness and discharge. The patient’s past medical history is notable for a cerebellar cyst that was removed at age 21 and placement of a ventricular-atrial shunt for associated hydrocephalus. Visual acuity is 20/50 in the right and 20/20 in the left. On exam the patient is noted to have a cachectic appearance and is found to have severe bilateral enophthalmos with poor lid-globe apposition and exposure keratopathy. A CT scan of the orbit reveals upward bowing of the orbital roof. Bloodwork is notable for a low serum leptin (0.6) correlating with low body fat mass. The patient is followed for thirty years with progressive upward expansion of the orbital roof. He is managed with successive surgeries to restore lid-globe apposition including orbital floor implant, retroplacement of medial and lateral canthal tendons, and orbital roof implant. 

Presentation Date: 01/30/2020

Issue Date: 08/01/2020