RETINAL-DETACHMENT IN IDENTICAL-TWINS WITH STICKLER-SYNDROME TYPE-1

Background - The high incidence of retinal detachment and its poor sur gical prognosis in patients with Stickler syndrome are well known. How ever, the vitreoretinal relation to retinal detachment in this syndrom e is uncertain. Methods - Vitreoretinal examination with a binocular i ndirect ophthalmoscope and a Goldmann three mirror contact lens was pe rformed on identical twin boys with Stickler syndrome. Each had retina l detachment in the left eye, and many aspects of their fundus finding s were similar. The vitreous showed distinct abnormalities consistent with congenital vitreous anomaly of type 1 Stickler syndrome. The twin s were followed up for 4 years after undergoing a successful operation to reattach their left retinas. Results - Rhegmatogenous detachment w ith multiple tears occurred in the right eye of only one twin during t he follow up period, despite the similar condition of their fundi. Alt hough vitreous body was not present in most parts, slightly opaque vit reous cortex was attached to the retina near the ora serrata, and neit her twin had posterior vitreous detachment during the follow up period . Conclusion - Multiple retinal tears appeared simultaneously in the r ight eye of one twin, indicating some tractional force had acted on th e retina. It is believed that this force was caused by very thin vesti gial vitreous cortex attached to the retina. Although these observatio ns have been limited, vitreoretinal findings of the twins and their fa ther were consistent and suggested presence of thin vitreous cortex at tached to the retina without posterior vitreous detachment.