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Purpose To describe ocular findings in a patient with Type VI Ehlers-Danlos syndrome (EDS) and make ophthalmologists aware of the potential ophthalmic complications of this particular type of EDS. To briefly report the surgical technique utilized for the repair of spontaneous scleral rupture that may be associated with Type VI Ehlers-Danlos syndrome. Observations A 36-year-old female visited the Emergency Room due to sudden vision loss, edema, and redness of the right eye consistent with spontaneous scleral rupture secondary to scleral thinning due to Type VI EDS. Repair with scleral patch graft resulted in improvement in visual acuity, a decrease in hyphema, and discomfort resolution. Conclusions and Importance Spontaneous scleral perforation may occur in patients with Type VI EDS. A scleral patch graft may serve as a viable surgical repair alternative for such patients.

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