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.