Orbital Meningoencephalocele and Pulsatile Proptosis: A Rare Entity

Orbital roof defects are rare congenital osseous abnormalities that cause protrusion of intracranial contents into the orbit, resulting in a condition known as the orbital meningoencephalocele, a rare cause of pulsatile proptosis. We present a case of a 25-year-old lady, presented to us with complaints of left frontal headache, left eye protrusion and double vision from the left eye for the past three months. Her higher mental functions were intact. Local examination revealed non-axial pulsatile proptosis and an outward depression of the left eyeball along with diplopia. Extraocular movements and pupillary light response were normal with no bruit or orbital tenderness. Computed tomography (CT) scan of the brain and orbit with contrast showed deficient bone on the left orbital roof and floor with left frontal gliotic brain compressing the eyeball. Magnetic resonance imaging (MRI) of the brain and orbit with contrast showed an asymmetrical deformity of the skull and left cerebral hemisphere which was bulging towards the left orbit. We planned a two-staged surgical reconstruction. The orbital roof was first reconstructed using a titanium mesh. Within two weeks of surgery her pulsatile proptosis, diplopia, and headache had considerably improved and the proptosis had resolved with no visible pulsations. She is scheduled for second stage surgery after three months for reconstruction of the orbital floor.