Subtle Combined Hamartoma of the Retina and Retinal Pigment Epithelium Causing Recurrent Exodeviation

A 3-year-old girl presented with recurrent exotropia following primary strabismus surgery. Careful fundus examination of the left eye revealed loss of the foveal reflex and presence of a subtle grayish mass with overlying white fluff. Optical coherence tomography through the lesion revealed disorganization of inner and outer retinal layers with accompanying epiretinal gliosis. Together, these findings were suggestive of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). No syndromic association was found. CHRRPE is a rare retinal tumor that usually presents with visual loss, strabismus, or follows an asymptomatic course. Retinal tumors must be kept in mind whenever loss of foveal reflex occurs concurrently with strabismus.