Abstract
PURPOSE: To report a rare case of Aicardi syndrome presenting with concurrent peripheral retina nonperfusion with 360-degree neovascularization in the right eye and stalk tissue with a peripapillary fibrovascular membrane and tractional retinal detachment (TRD) in the left eye. OBSERVATIONS: A one-month-old girl was referred for an ophthalmic evaluation to confirm the diagnosis of Aicardi syndrome due to abnormal brain magnetic resonance imaging. A 360-degree circumferential peripheral avascular retina with extensive neovascularization was present in the right eye. Stalk tissue with fibrovascular proliferation causing TRD was found in the left fundus. The retina of the right eye became quiescent after completing peripheral laser photocoagulation. The detached retina in the left eye was flattened, and the peripapillary chorioretinal lacunae became visible one year after surgical removal of the traction. In addition, the axial length growth of the left eye regained. CONCLUSION AND IMPORTANCE: This is a rare case of Aicardi syndrome with concurrent peripapillary fibrovascular traction in one eye and peripheral retina nonperfusion in the other eye. Surgical intervention is vital not only for removing the traction and flattening the retina but also for promoting continual growth of the eyeball.