Neuro-ophthalmic manifestations of Ehlers-Danlos syndrome.

PURPOSE OF REVIEW: To review the neuro-ophthalmic manifestations of Ehlers-Danlos syndrome (EDS). RECENT FINDINGS: Ehlers-Danlos syndrome (EDS) is a rare genetic disorder with an estimated prevalence of 1 in 5000 individuals, but its true prevalence may be underestimated because of variable clinical presentations and limited awareness among healthcare professionals. The neuro-ophthalmic features of EDS may be difficult to recognize in context but new molecular genetic testing is now available for identification of specific subtypes of EDS. SUMMARY: Ophthalmic manifestations of EDS include loss of vision and double vision (strabismus), high myopia, retinal detachment, and blue sclera. The vascular subtype of EDS can present as a carotid-cavernous fistula, intracranial aneurysm, or arterial dissection.

Self-inflicted transorbital pontine stab injury with a retained pen.

Self-inflicted transorbital stab injuries are not commonly seen in clinical practice. These lesions usually lead to major disability depending on the intracranial structures transected. We present a case of a 34-year-old man with a self-inflicted stab injury in his right orbit from a pen. Computed tomography revealed that the pen crossed intracranially from the orbit to the posterior fossa and pierced the pons. Such injuries are usually seen in the context of major psychiatric or drug abuse disorders. A multidisciplinary approach between ophthalmology and neurosurgery is essential in the management of these patients.