Persistent hyperplastic primary vitreous. A clinicopathologic study of 62 cases and review of the literature (original) (raw)
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Unilateral persistent hyperplastic primary vitreous: Course and outcome
Journal of American Association for Pediatric Ophthalmology and Strabismus, 2002
Purpose: Improved surgical techniques enable more favorable results in the management of persistent hyperplastic primary vitreous (PHPV). The purpose of our study was to evaluate the outcome of PHPV eyes managed conservatively or after surgery (with or without intraocular lens implantation). Methods: A total of 89 children (37 boys, 52 girls) with PHPV in one eye (mean follow-up of 6.3 Ϯ 5.7 years) were included. The children were subgrouped according to treatment modality. Twenty-eight (31.5%) children were managed conservatively (nonoperated). Cataract extraction combined with vitrectomy and removal of embryonic remnants was carried out in 61 eyes (68.5%). Intraocular lenses were implanted in 30 of the operated eyes (pseudophakic) and 31 eyes remained without lens (aphakic). Results: Final evaluated visual acuity in the entire group was 6/15 or better in 12.6% (11 of 87) of the eyes. A total of 11.5% (10 of 87) had a visual acuity of 6/21 to 6/60, 46.0% (40 of 87) obtained 6/90 to light perception and 26 of 87 (29.9%) had no light perception in the involved eye. The rate of no light perception was significantly lower in patients with pseudophakia (10.0%) compared to those with aphakia (43.3%) or nonoperated (37.0%) eyes (P ϭ .009). Intraocular pressure was adequately assessed repeatedly in 72 eyes. High intraocular pressure and glaucomatous changes were observed in 7 of 31 (22.6%) patients with aphakia, 2 of 24 (8.3%) patients with pseudophakia and in 2 of 17 (11.8%) nonoperated eyes (P ϭ .34). Poor cosmetic outcome was seen in 12 of 31 (38.7%) children with aphakic eyes and 5 of 30 (16.7%) children with pseudophakic eyes (P ϭ .08). Prosthesis or cosmetic shells were needed for 8 of 31 patients with aphakia, for none of the patients with pseudophakia and for 2 of 28 of the nonoperated children (P ϭ .003). Conclusion: PHPV eyes have a potential for developing useful vision with favorable cosmetic outcome after surgery. Intraocular lens implantation may be a favorable and beneficial option for the management of children with unilateral PHPV. (J AAPOS 2002;6:92-9) Journal of AAPOS FIG 1. Age distribution at surgery of children with unilateral PHPV. The mean age for lensectomy was 0.9 Ϯ 1.5 years in the group with aphakia and 1.4 Ϯ 2.2 years in children with intraocular lens implantation (P ϭ .28).
Bilateral persistent hyperplastic primary vitreous
Indian Journal of Ophthalmology, 2009
Persistent hyperplastic primary vitreous (PHPV) is an uncommon condition, presenting clinically as leukokoria (white pupillary reß ex), micro-ophthalmia, and cataract. Bilateral PHPV is rare. Most important diff erential diagnosis is retinoblastoma, which can be diff erentiated by imaging features. Case Report A 3-month-old male infant presented with bilateral leukokoria. Birth history of the child was unremarkable. On initial examination, the size of the cornea looked smaller. The child was referred for ocular ultrasound examination, with a clinical suspicion of retinoblastoma. An ultrasound study was performed with a high-frequency transducer operating at 10 MHz (L5, GE Medical Systems, USA). Gray scale evaluation revealed an echogenic band in the posterior segment of both globes extending from the posterior surface of the lens capsule to the optic disc. The axial length of the globes was 16 mm, which was suggestive of micro-ophthalmia. Color doppler examination revealed the presence of blood flow in the band [Fig. 1A]. Spectral analysis of this blood vessel showed arterial waveforms [Fig. 1B]. Hyperechoic foci were seen in both the posterior segments adjacent to the artery, suggestive of associated vitreous hemorrhage [Fig. 1B]. No calciÞ cation was seen.
International Ophthalmology Clinics, 2008
Persistent hyperplastic primary vitreous (PHPV) has been called by various names, such as persistent tunica vasculosa lentis, persistent posterior fetal fibrovascular sheath of the lens, congenital retinal septum, and ablation falciformis congential. 1 It was first reported by Jules Cloquet in 1818 in Mémoire sur la membrane pupillae, et sur la formation du petit cercle artériel de l'iris. 2 In 1949, Reese defined this entity histopathologically. In 1955, Reese coined the term persistent hyperplastic primary vitreous (PHPV) 1,3,4 a congenital anomaly in which the embryonic hyaloid vasculature fails to regress normally. 4,5 In 1997, Goldberg 1 renamed this entity as persistent fetal vasculature (PFV) 3 as to encompass the complete spectrum of the disease. PHPV has been classified into 3 types 3,6-8 : Anterior: presence of retrolental opacity, elongated ciliary processes, or cataract. Posterior: presence of one or more of the following features: elevated vitreous membrane or stalk from the optic nerve, a retinal fold or retinal dysplasia, retinal detachment, or optic nerve hypoplasia. Combined (both anterior and posterior) depending on which ocular structures are involved.
Case Report Unusual findings in persistent hyperplastic primary vitreous: two cases
Persistent hyperplastic primary vitreous is a result of failure of regression of the embryonic hyaloid artery and presents commonly as leucocoria in the first few weeks of life. It leads to abnormal lenticular development and secondary changes in the retina and orbit. We discuss two cases of persistent hyperplastic primary vitreous with unusual findings. The first case is an example of unilateral involvement, and on high frequency ultrasound, the commonly associated retrolental mass was absent. The hyaloid artery was well visualized on colour and spectral Doppler. The second case had bilateral retrolenticular masses. The hyaloid artery could be demonstrated in one side on colour Doppler. Thickening of the choroido-retinal layer was noted in both sides. Retinal detachment or vitreous haemorrhages, which are commonly associated findings with persistent hyperplastic primary vitreous, were not seen in either case.
Surgical results of persistent hyperplastic primary vitreous
Ophthalmology, 1999
To evaluate the surgical success of patients with persistent hyperplastic primary vitreous (PHPV) and to identify preoperative indicators of visual outcome. Design: Noncomparative case series. Methods: The diagnosis of PHPV was made in 35 eyes of 27 patients from 1982 to 1994. In each case, anterior and/or posterior PHPV findings, preoperative testing, surgical procedures, and visual outcomes were documented. Twenty-nine of 35 eyes were managed surgically. Follow-up ranged from 2 months to 12 years. Results: Of the 35 eyes, 2 (5.7%) had strictly anterior PHPV, 8 (22.9%) had strictly posterior PHPV, and 25 (71.4%) had components of both anterior and posterior disease. Initial lensectomy and vitrectomy was performed in 24 eyes (68.6%). Surgery was withheld in four eyes secondary to severity of disease with an unrecordable visual-evoked potential (VEP). Reoperation rate was 32.3% for membrane reproliferation, glaucoma, vitreous hemorrhage, retinal detachment, or strabismus. Best-corrected final visual acuity ranged from 20/60 to no light perception. Six eyes (17%) maintained Snellen visual acuity despite posterior PHPV with some degree of retinal dysplasia. Conclusions: Surgical treatment of PHPV can result in functional visual outcome despite posterior segment involvement. The degree of ocular malformation, however, will ultimately limit the amount of visual improvement.
BMJ case reports, 2015
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...
Ophthalmic Doppler in persistent hyperplastic primary vitreous atypical presentation: case report
Arquivos brasileiros de oftalmologia, 2015
The purpose is to document a case of persistent hiperplastic primary vitreous (PHPV) with atypical Bergmeister's papilla and patent hialoid artery that had its conclusive diagnosis done by doppler ultrasound. We report a case of child, who had unilateral leucokoria. This child's fundoscopic examination showed a white mass on the optic nerve that extended over to adjacent retina. Performed ultrasound that remained a doubt with the following diagnostic hypotheses: persistent hiperplastic primary vitreous, granuloma (toxocara), astrocytic hamartoma and retinoblastoma. The diagnosis was only established when the doppler ultrasound showed a blood flow inside of the membrane, thus confirming the diagnosis of persistent hiperplastic primary vitreous associated with the Bergmeister'spapilla.
Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine, 2016
Persistent hyperplastic primary vitreous is a spectrum of congenital ocular abnormalities characterized by leukocoria, microphthalmia, cataracts, extensive intravitreal hemorrhage, persistence of the hyaloid artery, glaucoma, and retinal detachment. It might be isolated or associated with congenital syndromes such as trisomy 13, Walker-Warburg syndrome, and Norrie disease. We present 2 cases of persistent hyperplastic primary vitreous diagnosed by prenatal sonography in the early third trimester. Bilateral hyperechoic lenses and retinal nonattachment were detected in the sonographic examination of the first case, whereas irregular echogenic bands between the lenses and posterior walls of the eyes were prominent in the second case. In both of the cases, ocular findings were accompanied by intracranial findings, including severe hydrocephalus, an abnormal gyral pattern, and cerebellar hypoplasia, suggesting the diagnosis of Walker-Warburg syndrome. We also present a review of the lite...