Hyperostosis cranii ex vacuo from ventricular shunting

Abstract

A legally blind 24-year-old young man with a history of extensive traumatic brain injury due to a motor vehicle accident at age 5.5, status post childhood ventriculoperitoneal shunt placement for hydrocephalus and longstanding refractory epilepsy, was admitted for worsening of his seizures. Neurological examination showed a non-verbal patient, unable to follow commands and unable to track. Pupils were asymmetric (left fixed and dilated), eyes were dysconjugate with the right displaced superiorly and laterally, and no reaction to visual stimuli. Upper extremity was hypotonic and atrophic, and lower extremities were spastic with sustained clonus on the left. Plantar response was upgoing bilaterally. During the admission, the patient had multiple clinical seizures consistent with tonic seizures characterized by arm flexion and stiffening with episode of tonus and neck extension. Cranial computed tomography and magnetic resonance imaging of the brain were notable for extensive dural calcifications (white arrows, Fig. 1A) as well as calvarial thickening (Fig. 1B and white arrows, 1C on magnetic resonance imaging) in the relative absence of thickening of the facial bones and jaw (Fig. 1C, black arrows). A right frontal-approach ventricular catheter with its tip