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Priyanka Kumar, Jonathan E Sears; Coloboma associated retinal detachment: characteristics, surgical management, and outcomes. Invest. Ophthalmol. Vis. Sci. 2014;55(13):1111.
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Colobomas are congenital anomalies caused by incomplete fusion of the optic fissure. The purpose of this study was to evaluate the characteristics, management, and outcomes of coloboma associated retinal detachment.
The study design was a retrospective chart review of 9 eyes from 8 patients diagnosed with colobomatous retinal detachments who underwent retinal detachment repair. Each patient received complete medical and ophthalmic evaluation.
Patient age ranged from 3 to 32 years. One patient had LENZ syndrome and one patient had CHARGE syndrome. All patients had bilateral colobomas and an associated iris coloboma (9/9). Over half the patients had microphthalmos or microcornea (5/9). Two patients had optic nerve colobomas alone. All detachments were rhegmatogenous. Eight of nine eyes underwent scleral buckling and vitrectomy, while one eye received scleral buckle alone. Silicone oil (SO) was used in 3/9 eyes, SF6 in 5/9, and 1 eye received no tamponade. Locations of retinal breaks were varied and included holes in the intercalary membrane (3/9), peripheral horseshoe tears directly inferior to the coloboma (3/9), giant retinal tear (1/9), or open retinal hole on the optic nerve head (2/9). Six of nine eyes remained attached after initial intervention. Three of nine eyes had recurrent detachment requiring repeat vitrectomy. Of the eyes that received SO, 2/3 detached after SO removal. Eight of nine eyes had colobomas that affected the fovea and never achieved acuity better than 20/200 postoperatively, whereas the sole extrafoveal coloboma-associated detachment improved to 20/25 after surgery.
Patients with large choroidal colobomas may develop retinal detachments from a variety of holes, breaks or tears. Holes in the intercalary membrane require endolaser encircling the coloboma whereas peripheral tears may benefit from scleral buckling. We postulate that inferior horsehoe tears might be related to the location of inferior iris/zonular defects.
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