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HS Bains, LM Jampol, R Equi, MF Goldberg; Retinal Tears from Vitreo-retinal Traction in Patients with Incontinentia Pigmenti . Invest. Ophthalmol. Vis. Sci. 2002;43(13):617.
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Purpose: Incontinentia Pigmenti (IP) is an X-linked disorder characterized by abnormalities of the skin, teeth, central nervous system and eyes. The retinal manifestations clinically resemble retinopathy of prematurity, with peripheral retinal avascularity complicated by neovascular proliferation and vitreoretinal traction. In this study we report two cases of patients with localized rhegmatogenous retinal detachments due to retinal tears associated with vitreo-retinal traction at the transition from normal to avascular peripheral retina. Methods: A retrospective review of the patient history, complete ophthalmic exam including retinal examination with scleral depression, fundus photographs and fluorescein angiograms was undertaken. Results: The first case was a 46 year-old woman with IP who underwent panretinal photocoagulation in the right eye for preretinal neovascularization, and over a course of four months, presented with horseshoe shaped tears during three sequential follow up visits. The tears and localized detachments were walled off using laser photocoagulation. The second case was a 41 year-old Caucasian female with IP who presented with a horse-shoe shaped tear and a small localized retinal detachment in the superior nasal quadrant of the left eye at the transition between normal and avascular retina. Prophylactic laser photocoagulation was used to wall off the tear. Conclusion: In patients with IP, in addition to commonly seen complications such as retinal neovascularization and retinal traction, retinal tears and rhegmatogenous detachments can occur at the transition from normal peripheral retina to avascular retina, even in older patients.
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