title: Unilateral persistent hyperplastic primary vitreous: intensive management approach with excellent outcome beyond visual maturation.
abstract: Persistent hyperplastic primary vitreous (PHPV) is an ocular developmental disorder resulting from incomplete apoptosis of the embryonic hyaloid vasculature. Unilateral PHPV is traditionally associated with a poor prognosis because of the challenges associated with managing progressive anisometropic amblyopia. We report a child with unilateral PHPV who underwent cataract extraction, primary posterior capsulotomy with anterior vitrectomy and intraocular lens implantation followed by combined trabeculectomy/trabeculotomy within the first 8 weeks of life. Intensive optometric and orthoptic input was required for many years to manage the increasing anisometropic amblyopia with final visual acuity of 20/40 unaided in the affected eye and without evidence of glaucomatous optic neuropathy. This case illustrates the excellent visual outcome possible in a child with complex, unilateral PHPV using an intensive management approach comprising: early surgical intervention for congenital cataract and secondary glaucoma, meticulous monitoring of refraction, visual acuity and intraocular pressure and motivated parents who engaged in the management.
authors.affiliation: Oxford Eye Hospital, Oxford, UK.
authors.forename: Imran H
authors.initials: IH
authors.lastname: Yusuf
authors.affiliation: Oxford Eye Hospital, Oxford, UK.
authors.forename: Chetan Kantibhai
authors.initials: CK
authors.lastname: Patel
authors.affiliation: Oxford Eye Hospital, Oxford, UK.
authors.forename: John F
authors.initials: JF
authors.lastname: Salmon
mesh_terms: D002387:Cataract Extraction; D005124:Eye Abnormalities / Q000473:pathology / Q000601:surgery*; D005260:Female; D006801:Humans; D006965:Hyperplasia; D007231:Infant, Newborn; D019654:Lens Implantation, Intraocular; D016896:Treatment Outcome; D014792:Visual Acuity; D014821:Vitrectomy; D014822:Vitreous Body / Q000002:abnormalities* / Q000473:pathology*