Management of Retinal Detachment Due to Giant Retinal Tear in Osteogenesis Imperfecta

Abstract

Purpose: To report the management of a giant retinal tear (GRT)-related subtotal retinal detachmentcomplicated with choroidal detachment in a patient of osteogenesis imperfecta (OI).Method: Interventional Case Report.Result: A 29-year-old female of OI visited our clinic due to decreased vision (od) for 3 weeks. Ocular examination showed a GRT-related (tear from 7 to 10 o'clock) subtotal retinal detachment, complicated with choroidal detachment of the right eye. She was then scheduled for surgery with pars plana vitrectomy, fluid-gas exchange, endolaser photocoagulation, and silicone oil tamponade under local anesthesia. No perfluorocarbon (PFC) liquid was used during surgery. However, 12 days postoperatively, recurrent retinal detachment was noted due to small inferior retinal breaks, as well as macular pucker formation. Another surgery by 25-gauge sutureless pars plana vitrectomy with noncontact wide-field viewing system was performed to remove macular pucker and re-attach the retina but this was barely completed due to poor cooperation of the patient under local anesthesia. Final logarithm of minimum angle of resolution (logMAR) best-corrected visual acuity (BCVA) by Snellen chart was 2.0, with residual macular pucker and localized retinal detachment of the right eye.Conclusion: In light of thin sclera in patients of OI, as well as GRT and choroidal detachment in our patient, management of retinal detachment is extremely challenging. Our patient underwent the first surgery with retina fully reattached, but recurrent retinal detachment developed. The second surgery was performed but was barely completed due to patient instability under local anesthesia. This is the first report of surgical management of GRT-related retinal detachment in an OI patient. Further study is needed for better surgical outcome.