Severe retinopathy in a child with hypoplastic left heart syndrome.

Purpose: We present the first reported case of a condition similar to retinopathy of prematurity in a full-term patient with hypoplastic left heart syndrome.

Design: Interventional case report.

Methods: We describe the clinical presentation and surgical treatment of a male baby with severe retinopathy and retinal detachments.

Results: The clinical course of this baby was similar to retinopathy of prematurity. The patient had areas of avascular retina, significant retinal neovascularization, fibrous proliferation, and tractional detachment. One eye had a totally detached retina; the other had functional vision after the retina was reattached by several vitreoretinal surgeries, including the use of intraocular silicone oil.

Conclusions: This cyanotic condition can cause a retinopathy with chronic retinal neovascularization and retinal detachments. Permanent surgical retinal reattachment is difficult in the presence of neovascular activity that leads to intravitreous cicatrization.