The purpose of this report is to describe a pediatric case of total retinal detachment (RD) with secondary glaucoma in the setting of posterior coloboma with the metaplastic retinal pigment epithelium showing abrupt transition to choroid plexus tissue.Methods:
Retrospective case report.Results:
A 3-month-old patient presented with leukocoria and enlarged right eye. She was found to have a funnel RD with anterior displacement of lens–iris diaphragm and secondary glaucoma. Orbital imaging ruled out retinoblastoma, and posterior coloboma was identified. Intraocular pressures remained significantly elevated despite maximal medical therapy on glaucoma drops and transscleral cycloablation, and the eye was enucleated for comfort. Histologic analysis confirmed neovascularization of the iris, total RD, and posterior coloboma with the associated metaplastic retinal pigment epithelium showing abrupt transition to choroid plexus tissue.Conclusion:
This is the first reported case of choroid plexus in the human eye. A close association of choroid plexus with coloboma and RD raises possibility that this tissue may have functionally contributed to pathogenesis of RD by secreting cerebrospinal fluid within subretinal space.