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including microphthalmia, anophthalmia, anisometropia, retinal detachment (RD), cataract, choroidal neovascularization, and hypoplasia of the optic nerve.
Intravitreal bevacizumab in choroidal neovascularization associated with congenital choroidal and optic nerve coloboma in children: long-term improvement in visual acuity.
We present a unique case of bilateral colobomas of the choroid, iris, and lens associated with bilateral avascular peripheral retina.
A full-term, 12-year-old female patient with a history of bilateral colobomas was referred to our practice by her pediatric ophthalmologist, who had been following her for some time, for suspicion of retinal detachment in her left eye. On office presentation, her visual acuities were 20/80 OD and 20/400 OS. Anteriorly, her ocular examination revealed colobomatous irides inferiorly and clear lenses. Posterior examination revealed an inferior coloboma at the disc with attached retina OD (Fig. 1) and a hemorrhagic exudative retinal detachment with possible subretinal hemorrhage OS (Fig. 2), with the question of a choroidal neovascular membrane as the source. A fluorescein angiogram obtained at the time of the examination under anesthesia revealed no such membrane but, interesting, demonstrated avascular peripheral retina bilaterally with late leakage OS (Fig. 3, Fig. 4). Subsequently, laser photocoagulation was applied OD to prevent a similar scenario in this eye.
Fig. 1Color fundus photo of the right eye demonstrating a large inferior coloboma.
As far as we are aware, this is the first published case of bilateral chorioretinal colobomas seen with an avascular peripheral retina. This case grants new insight at the complex and interwoven nature by which ocular structures develop, hinting at a possible common genetic pathway between retinal vasculogenesis and closure of the embryonic fissure. Avascularization of the peripheral retina can lead to exudative and tractional retinal detachments in conditions such as Coat's disease, familial exudative vitreoretinopathy, and, most notably, retinopathy of prematurity. Although it is unclear how this may have contributed to this patient's RD, the benefits of laser ablation of the avascular retina in these diseases are well established, and it is on this basis that the right eye of this patient was treated. Further research will be required to establish the rate and significance of avascular retinal periphery in ocular coloboma.
References
Nakamura K.M.
Diehl N.N.
Mohney B.G.
Incidence, ocular findings, and systemic associations of ocular coloboma: a population-based study.
Intravitreal bevacizumab in choroidal neovascularization associated with congenital choroidal and optic nerve coloboma in children: long-term improvement in visual acuity.
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