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S. Rofagha, T. N. Hwang, W. F. Hoyt, M. Hafez, T. J. McCulley; Progressive Bilateral Enophthalmos Following Cerebral Spinal Fluid Shunting. Invest. Ophthalmol. Vis. Sci. 2008;49(13):6022.
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Enophthalmos following CSF shunting is a relatively unknown entity, with only one published case series in patients with congenital hydrocephalus. In this report we describe a series of adult patients with acquired hydrocephalus.
Interventional case series with orbital imaging (n=4) and biopsy (n=1).
Four patients were evaluated for severe progressive enophthalmos following venticuloperitoneal shunting. Subjects included a 40 year old female with elevated intracranial pressure related to a ruptured aneurysm, a 34 year old female with idiopathic intracranial hypertension and two males, ages 19 and 21 years, both shunted for hydrocephalus related to a traumatic intracranial bleeds. Enophthalmos onset was difficult to estimate in 2 patients and within one year of shunting in the other 2 patients. Duration at time of presentation ranged from one to fifteen years, with relentless progression in all. On examination severe symmetric enophthalmos (mean exophthalmomety measurement of 6.7 mm) to the degree that the globes lost partial contact with the eyelids was evident. Other findings included ocular surface disease related to the loss eyelid apposition and limited extraocular motility in all directions. All subjects had orbital imaging. Despite posterior displacement of the globes, redundancy of the optic nerves was not present; rather the images revealed short, taut, thickened optic nerves. Orbital and sinus volumes also appeared increased with a relative lack of orbital fat. One patient underwent orbital exploration with biopsies of multiple tissues (fat, muscle, and fibrous septum) all of which were entirely normal. Although suggested, no shunts were removed.
Progressive enophthalmos to a degree that the globes lose contact with the eyelids can occur following CSF shunting for acquired hydrocephalus. Based on imaging findings possible mechanisms include tethering of the globes via tension on the optic nerves secondary to a posterior and downward shift in brain position, expansion of bony orbital volume and fat atrophy.
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