Persistent Hyperplastic Primary Vitreous Found at the Time of Cataract Surgery in an Adult

Purpose: To report a rare case of PHPV found at the time of cataract surgery in an adult. Methods: Observational case report. Results: A 37-year-old woman presented complaining of longstanding left leukocoria and poor vision. She was found to have a mature, white cataract in the setting of a microphthalmic left eye, and a left afferent pupillary defect. During cataract extraction, she was found to have a dense, opaque, fibrotic retrolental membrane, adherent to the ciliary body. Intraocular scissors and anterior vitrectomy were used to clear away the membrane. Follow up funduscopic exam revealed the presence of fibrovascular tissue extending anteriorly from the inferonasal optic disc, confirming a diagnosis of adult persistent hyperplastic primary vitreous (PHPV). Postoperatively, the patient had normal intraocular pressure, regained some peripheral vision and had a good cosmetic outcome. Conclusion: PHPV is a congenital anomaly resulting from failed regression of embryonic structures. We report an unusual case of PHPV found in an adult eye, involving both anterior and posterior segments. This is the first reported case of adult PHPV in Hawaii, and the tenth reported case found by our literature search.