Unilateral Persistent Hyperplastic Primary Vitreous Research Articles

Persistent Hyperplastic Primary Vitreous: A Case Report and Literature Review

Persistent hyperplastic primary vitreous (PHPV) is a rare ocular disorder caused by the incomplete regression of primary vitreous with the abnormal persistence of hyaloid vasculature. It is the second most common cause of leukocoria after retinoblastoma. Aim of this work is to report the case of unilateral PHPV in a 6 months old child. We will describe the main ocular, echographic and magnetic resonance findings, that are essential to conduct a right differential diagnosis.

Persistent hyperplastic primary vitreous: case report and literature review

Persistent hyperplastic primary vitreous (PHPV) is a congenital developmental anomaly of the eye caused by the incomplete regression of primary vitreous with the abnormal persistence of hyaloid vasculature. Aim of this work is to report the case of unilateral PHPV in a 4-week-old child presented to our Department with microphtalmia and leukocoria in the right eye. We will describe the main ocular, echographic and magnetic resonance findings that are essential to conduct a right differential diagnosis.

Buphthalmos in the setting of persistent hyperplastic primary vitreous cataract

Purpose To report a case of unilateral persistent hyperplastic primary vitreous cataract presenting with ipsilateral buphthalmos. Design Retrospective case report. Methods The patient chart was reviewed, and a relevant literature search was performed. Results A four-month-old child presented with persistent hyperplastic primary vitreous cataract in the right eye and ipsilateral buphthalmos with no discernable angle abnormalities. He underwent lensectomy, anterior vitrectomy, and limited transscleral diode cyclodestruction in the right eye. Conclusions This appears to be the first report of buphthalmos presenting with unilateral persistent hyperplastic primary vitreous. Despite two comorbidities, the patient has thus far responded well to initial treatment and visual rehabilitation.

Unilateral persistent hyperplastic primary vitreous: Course and outcome

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 ± 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 = .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 = .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 = .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 = .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)

High-frequency ultrasonography findings in persistent hyperplastic primary vitreous

Purpose: To identify anatomic correlates in eyes with persistent hyperplastic primary vitreous (PHPV) by using high-frequency ultrasonography. Method: Three main groups of patients were studied by means of high-frequency ultrasonography over a 19-month period. Group I included 9 eyes of 9 patients with newly diagnosed unilateral PHPV. Group II included 4 eyes of 4 patients with unilateral PHPV that had been previously surgically treated. Group III included 22 eyes and was a control group of patients without PHPV. This group consisted of the 5 contralateral normal eyes of 5 patients with unilateral PHPV in the fellow eye, both eyes of one patient with uncomplicated unilateral cataracts, the affected eye of one patient with unilateral uncomplicated cataract, the affected eye of one patient with isolated retinal coloboma, and 13 normal eyes of 7 young adults. Group I and II patients also had B-scan ultrasonography performed and had any intraoperative findings noted. Results: Characteristic features of PHPV, such as centrally dragged ciliary processes and swollen anteriorly displaced lens, were observed only in those eyes with PHPV. A new echographic finding of a double linear echo was observed in the region of the pars plana or plicata only in eyes with PHPV. This finding was confirmed intraoperatively to be consistent with a thickened adherent anterior hyaloid face and not to be an anteriorly inserted peripheral retina. Conclusion: High-frequency ultrasound can be reliably used to distinguish characteristic features of PHPV. To our knowledge this is the first such description of the use of high-frequency ultrasonography in PHPV eyes. Furthermore, the presence of a thickened adherent anterior hyaloid face may help explain the well-recognized complications of peripheral retinal tears and retinal detachments during and after surgical intervention. (J AAPOS 2000;4:217–23)

Changes in the Contralateral Eye in Uncomplicated Persistent Hyperplastic Primary Vitreous in Adults

In two adults (a 62-year-old man and a 71-year-old woman) uncomplicated full-blown unilateral persistent hyperplastic primary vitreous was diagnosed on the basis of characteristic clinical features and ultrasonography. In the contralateral uninvolved eyes, we found open-angle glaucoma, anomalous blood vessels along the entire circumference of the anterior chamber angle, band keratopathy, and heterochromia iridis. The axial length of one involved eye was about 0.85 mm larger than that of the uninvolved eye.

Retinoblastoma and Persistent Hyperplastic Vitreous Occurring in the Same Patient

A patient with inherited retinoblastoma in the left eye and persistent hyperplastic primary vitreous (PHPV) in the right eye is presented. These two conditions are often confused in differential diagnosis; however, they are typically quite different in clinical appearance. We suspect that the association of retinoblastoma and PHPV in one patient represents rare chance occurrence, because the karyotype was normal, the conditions were present in opposite eyes, and unilateral PHPV has not been reported to be inherited.