A Rare Cause of Leukocoria in Children: Persistent Hyperplastic Primary Vitreous

Introduction: Leukocoria, a whitish pupillary reflection, is a common warning sign of various pediatric ocular pathologies, including Persistent hyperplastic primary vitreous (PHVP). This case report describes the observation of a 3-year-old child with melanoderma, presenting with a white spot in the left eye since birth. Observation: Uncorrected distance visual acuity was 2/10 in the right eye and hand movements were perceived at 2 meters in the left eye. Examination of the anterior segment in the left eye revealed leukocoria and microphthalmia. The fundus examination was normal in the right eye but revealed a white mass extending from the center of the optic disc towards the temporal region in the left eye. Ocular imaging, including ocular ultrasound and optical coherence tomography, confirmed the diagnosis of PHVP, illustrated by a residual white mass at the center of the optic disc in the left eye. Conclusion: PHVP should be considered in the presence of leukocoria in a child, with urgent exclusion of retinoblastoma. This observation underscores the importance of early diagnosis for appropriate management.