Ocular Coloboma

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Eyes with ocular colobomas are at a significantly increased risk of detachment and account for approximately 0.5% of paediatric retinal detachments [25]. Giant retinal tears are seen in association with lens colobomas [19] and rheg-matogenous detachment may develop in eyes with choroidal coloboma, when small retinal breaks may be found in the hypoplastic retina overlying the coloboma. Assessment of vision can be difficult and the diagnosis of detachment can be further impaired by nystagmus, microphthalmos and cataract. Retinal breaks occurring away from the colobomatous area may be managed by conventional buckling techniques, provided the sclera is of sufficient quality for suturing and the break can be adequately closed. More usually, the retinal break overlies the colobomatous area. Breaks are often small and may be multiple, and their localization can be aided peroperatively by the identification of "schlieren" during internal drainage. Argon laser photocoagulation may be applied around the border of the colobomatous area and, where this includes the papillomacular bundle, this may be applied prior to retinal reattachment to minimize associated thermal damage to the nerve fibre layer. Both retinal pigment epithelium and Müller cells are vestigial or absent within the coloboma so that to be effective, retinopexy needs to be applied outside the margin. Recurrent detachment is common [15], so permanent internal tamponade is often required.

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