Maurice F. Rabb » Congenital Retinal Folds

A 13 year old white male was referred for evaluation of "congenital retinal folds." The patient was the product of an uncomplicated full term delivery. There was no oxygen administered. "Irritation" was described for both eyes at 11 months made at that time. There has been little change over the inervening 12+ years.

The patient has never had useful vision in the left eye. Best corrected vision was counting fingers at 5 feet OD and hand motions at 1 foot OS. The spectacle correction was -13/75 +2.00X160 OS. Constant, horizontal, jerky nystagmus was present. The intraocular pressures were normal. Slit lamp exam showed early band keratopathy OU. There was a complicated cataract, 2+ flare, and early posterior synechiae OS.

Fundus examination showed a razor falciform retinal fold extending from the disc OD through the macula to the ora serrata in the 9:00 meridian (Slide of patient "A" OD). The retina was attached, but most of the retinal vessels were seemingly absent, because they were pulled into the retinal fold. In the left eye, there was an enormous amount of subretinal yellow material suggesting a Coats-like syndrome. (Slide of patient "A" OS). In addition, there was a large falciform fold extending from the disc to the ora serrata OS in the 5:30 meridian. Elsewhere, the retina was attached except in the far periphery where the falciform fold extended around the ora serrata region from 2:00 to 5:00 O'clock.