FIGURE 54.1 These images are from the 1986 Transaction of the American Ophthalmological Society paper showing the total retinal detachment (A) and coloboma/morning glory disc (B). Although recognized since 1970, the treatment is still challenging. (Reprinted from Irvine AR, Crawford JB, Sullivan JH. Pathogenesis of retinal detachment with morning glory disc and optic pit. Trans Am Ophthalmol Soc 1986;84:280–292, with permission.)
Peripheral retinal vascular nonperfusion in children has been recognized in several congenital conditions, such as retinopathy of prematurity, Coats disease, familial exudative vitreoretinopathy, facioscapulohumeral muscular dystrophy, and incontinentia pigmenti (2). Recently peripheral areas of nonperfusion have been reported in four patients with morning glory disc anomaly (3).
One of the most difficult problems accompanying coloboma or morning glory–type discs is rhegmatogenous retinal detachment. It is seen in 14% to 37% of eyes (1–5). This type of retinal detachment can be extremely difficult to repair and often, when seen in youngsters, is associated with late discovery of proliferative vitreoretinopathy. Coloboma/morning glory disc anomaly can be bilateral but often is unilateral, and when unilateral colobomas are accompanied with rhegmatogenous retinal detachment, it may be important to discuss very carefully with the child’s family and the child the visual potential of the eye given what often is a very difficult operative procedure often requiring multiple operations. In several large series, colobomatous retinal detachment repair has required as many as five to seven operations to achieve successful retinal reattachment. Again the visual potential of the coloboma is determined by the damage to the foveal area secondary to retinal detachment or the colobomatous distribution itself in the back of the eye. Spontaneous retinal reattachment can occur in up to 36% of cases over a slow course, averaging 7.5 years (4–7).
RHEGMATOGENOUS RETINAL DETACHMENT IN COLOBOMA AND MORNING GLORY DISC ANOMALY
When a rhegmatogenous retinal detachment is associated with a coloboma, a careful peripheral retinal examination is necessary to determine if a peripheral retinal break is present. The peripheral retinal break is often treated first with a buckling procedure. This can result in retinal reattachment. However, often due to the limited initial visual potential, the child may not be aware that the retina has detached and may not present until proliferative vitreoretinopathy is present. In addition, following chronic detachment, there may be retinal atrophy or even retinal precipitates present suggesting a chronic retinal detachment. These features again must be discussed with the family to determine if a surgical intervention is warranted.
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