In contrast to most other paediatric blinding retinal disorders, blindness through retinal detachment is in most cases potentially avoidable if a rationale for the prediction and prevention of retinal detachment could be developed. This goal has been frustrated by a lack of understanding of the factors influencing retinal detachment even in high-risk groups, which are only now beginning to be unravelled.
Factors traditionally associated with retinal detachment include refractive error, a positive family history, visible lattice retinopathy and fellow eye involvement, but the nature of these associations is poorly understood. The prevalence of myopia varies enormously and even in Stickler syndrome up to 20 % patients may have no significant refractive error. Many patients with retinal detachment have none of the accepted risk features such as lattice retinopathy and, in those that do, retinal tear formation frequently occurs in areas remote from such pathology, so that the associations with accepted risk factors requires refinement.
In Stickler syndrome there is a high risk of giant retinal tear (GRT) which is often bilateral and a frequent cause of blindness. The rationale for offering prophylaxis in such high-risk cases is to prevent progression of GRT to detachment by applying treatment to the post-oral retina at the site of giant tear development.
A study investigating the role of prophylactic 360° cryotherapy in type 1 Stickler syndrome has recently been completed. Although there were significant differences between the ages of the control and study groups, the risk of retinal detachment in 204 patients with type 1 Stickler syndrome reduced from 62% to between 3% and 10% .
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