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Results for “Persistent Hyperplastic Primary Vitreous”

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PubMed1982

Computed tomography in the evaluation of patients with persistent hyperplastic primary vitreous (PHPV).

The computed tomographic (CT) findings in 5 patients with proven persistent hyperplastic primary vitreous (PHPV) are described. PHPV arises when the embryonic hyaloid vascular system fails to regress normally. Clinically, this condition usually manifests as unilateral or bilateral leukokoria and is one of the more important and frequent conditions mimicking retinoblastoma. The spectrum of CT findings include: (a) soft-tissue replacement (infiltration) of the vitreous body; (b) retrolental soft tissue along the Cloquet canal; (c) absence of abnormal calcification within the orbit; (d) microphthalmus; (e) retrohyaloid layered blood; and (f) hypervascularity of the vitreous humor.

Diagnosis, Differential↗

PubMed1977

Persistent hyperplastic primary vitreous: roto-extraction and other surgical experience.

We discuss nine cases of persistent hyperplastic primary vitreous seen recently, including mode of presentation, management and postsurgical follow-up. Emphasis is placed on early operative intervention in order to preserve the globe, as well as to prevent irremedial stimulus deprivation amblyopia. The advantages of roto-extraction in the surgical approach to this entity are considered, and the difficulties of postoperative visual rehabilitation are also discussed.

Contact Lenses↗

PubMedDate not supplied

Persistent hyperplastic primary vitreous in association with neurofibromatosis 2.

We describe a father and son with persistent hyperplastic primary vitreous occurring in association with neurofibromatosis 2. This report demonstrates rare vertical transmission compatible with autosomal dominant inheritance and the uncommon association of the autosomal dominant systemic disorder neurofibromatosis 2.

Adult↗

PubMed1993

[Value of ultrasonography in persistent hyperplastic primary vitreous body. Apropos of 4 cases].

The authors report 4 patients with complicated persistent hyperplastic primary vitreous. The complications observed are cataract (2 eyes) and retinal detachment (2 eyes). The affection is bilateral in two cases and unilateral in the two other cases. After a short embryologic vitreous recall, the authors present clinical and paraclinical aspects of the condition. The authors focus on the importance of echographic data.

Child, Preschool↗

PubMed2004

[Persistent hyperplastic primary vitreous and Aicardi syndrome].

INTRODUCTION: Aicardi syndrome is characterized by infantile spasms, agenesis of the corpus callosum and chorioretinal lacunae. This disorder affects mostly females, with early embryonic lethality in males. Numerous general and ocular disorders may be associated with this affection. We present here a case of persistent hyperplastic primary vitreous (PHPV) in association with Aicardi syndrome in a 30-year-old woman. CASE REPORT: The authors report a case of a 30-year-old woman with Aicardi syndrome associated with persistent hyperplastic primary vitreous. DISCUSSION: Aicardi syndrome is a polymalformative disease occurring at an early period of embryogenesis. It can affect many ocular structures. This syndrome is essentially described in female children, who rarely reach an adult age. The observation we report is particular because of the patient's age (30-years-old) and the association with a persistent hyperplastic primary vitreous, exceptional in this context. CONCLUSION: With a review of the literature, the Authors discuss the clinical neuroradiological and prognostic aspects of this polymalformative syndrome and different associated general and ocular abnormalities, emphasizing particularly those of persistent hyperplastic primary vitreous in this affection.

Adult↗

PubMedDate not supplied

[Persistent hyperplastic primary vitreous--diagnosis and differentiation].

PURPOSE: Presentation of the cases with a rare form of persistent hyperplastic primary vitreous. MATERIAL AND METHODS: 5 children, aged 3-14, were referred to the clinic with suspicion of neoplasm changes or congenital anomalies of the eyeball. Unilateral, pathological lesions, in the form of opaque membranes, partially or totally covered the optic disc, were found. In 2 cases persistent fragments of arteria hyaloidea were also observed. Diagnosis was mode basing on three mirror glass examination, ultrasonography and fluorescein angiography. In differential diagnosis, retinoblastoma and other entities determined in the literature as pseudo-retinoblastoma were taken into account. The children remained under clinical follow-up.

Adolescent↗

PubMed2006

[Persistent hyperplastic primary vitreous--developmental anomaly of the eye in children].

Persistent hyperplastic primary vitreus in children continues to be a diagnostic and therapeutic challenge for ophthalmologists. It can occur in isolation, in association with other ocular disorders and rarely as a part of systemic disorder. Characteristic features include microphthalmic eye, white vascularized retrolental tissue with or without a persistent hyaloid artery, centrally dragged ciliary processes, an anteriorly shifted and (or) swollen lens, and varing degrees of lenticular opacification. PHPF is the most common associaton with unilateral cataracts. Differential diagnosis and functional effect of treatment are discussed.

Abnormalities, Multiple↗

PubMed2001

Intraocular lens implantation in a child with monocular cataract and anterior persistent hyperplastic primary vitreous.

A 3-year-old girl had phacoemulsification during which the presence of anterior persistent hyperplastic primary vitreous (PHPV) was discovered. Visual rehabilitation comprised contact lens use for 1 year. However, visual acuity deteriorated gradually because of secondary cataract formation. In a second surgery 1 year after the first, the posterior capsule was incised, followed by an anterior vitrectomy and intraocular lens implantation. At the last follow-up 6 months after the second surgery, there was no evidence of ocular complications and best corrected visual acuity was 0.6.

Cataract↗

PubMed1983

[Surgery of posterior polar cataract in persistent hyperplastic primary vitreous].

Between 1975 and 1982 surgery was performed on 13 eyes with posterior polar cataract and persistent hyperplastic primary vitreous (PHPV), in order to prevent loss of the eye due to the usual complications. Depending on the extent of the retrolental fibrovascular tissue, one of two possible surgical procedures was employed: (1) scissor excision through a large corneal incision in the wide-open bulb (8 eyes) in cases with a very firm, coarse and extensive retrolental membrane; (2) excision with a vitrectomy instrument, from a corneal or corneoscleral approach (5 eyes) in the "closed eye" in cases where the membrane was more circumscribed. A pars plana approach is generally contraindicated, as sensory retina often extends as far as the pars plicata. There were no serious intraoperative or postoperative complications. Prolonged local corticosteroid therapy is advisable postoperatively. In one patient, suffering from the anomaly in both eyes, there was an encouraging improvement in function to 0.6 and 0.1. The authors recommend early surgery in cases of posterior polar cataract with PHPV.

Adrenal Cortex Hormones↗

PubMed2002

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.

Aphakia, Postcataract↗

PubMed1988

Megalocornea and persistent hyperplastic primary vitreous masquerading as congenital glaucoma.

A 7-month-old infant referred with suspected bilateral congenital glaucoma was noted under general anaesthesia to have simple megalocornea in association with contralateral glaucoma in an eye suspected of harbouring an intraocular tumour. Following enucleation, histopathology demonstrated persistent hyperplastic primary vitreous. The presence of simple megalocornea of autosomal recessive inheritance in association with contralateral persistent hyperplastic primary vitreous in a buphthalmic eye is of ophthalmic interest, and appears to be unique.

Cornea↗

PubMed1999

Intrauterine exposure to clomiphene and neonatal persistent hyperplastic primary vitreous.

A 4-year-and-10-month-old girl was diagnosed shortly after birth with persistent hyperplastic primary vitreous (PHPV). Her mother took clomiphene 100 mg daily for approximately 4 weeks and discontinued the drug once she had a positive pregnancy test. The exact time of gestation was not clear. Clomiphene is an estrogen antagonist effective in the treatment of anovulation. Various ocular side effects have been described in women taking the drug, including decreased vision, mydriasis, flashing lights, central scotoma, photophobia, diplopia, allergic reactions, retinal vasospasms, detachment posterior vitreous, and possibly posterior subcapsular cataracts. These occur in 1.5-10% of patients taking clomiphene. The potential effects of clomiphene on the fetus have been investigated in five animal studies. Cataracts were observed in fetal mice and rats, but not in monkeys. In humans, a case of congenital retinal aplasia was described. The possibility of clomiphene-induced congenital PHPV should be considered, especially in pregnant women who are taking a high and prolonged dose.

Animals↗

PubMed2002

Congenital cataracts and persistent hyaloid vasculature in a llama (Lama glama).

A 9-month-old llama was evaluated for apparent blindness. Bilateral cataracts were diagnosed and cataract surgery was performed on the right eye. At the time of surgery persistent hyperplastic primary vitreous, persistent hyperplastic tunica vasculosa lentis, and a persistent hyaloid artery were observed. Prior to surgery Power Doppler ultrasound revealed a patent tunica vasculosa lentis OS. Despite reports of a poor success rate for llama cataract surgery, through use of careful tissue handling, phacoemulsification, viscoelastic endothelial protectants, anti-inflammatories, and BSS-Plus irrigating solution, vision was successfully restored in both eyes of the llama.

Animals↗

PubMed2001

Histopathological examination of two cases of anterior staphyloma associated with Peters' anomaly and persistent hyperplastic primary vitreous.

AIMS: To clarify the developmental mechanism and critical period for the uncommon complex of Peters' anomaly and persistent hyperplastic primary vitreous (PHPV). METHODS: Two eyes with Peters' anomaly and PHPV were histologically examined by serial section. One eye was enucleated at age 7 months (case 1) and the other at age 4 months (case 2) owing to severe anterior staphyloma. RESULTS: In both eyes, defects in the endothelium, Descemet's membrane, and posterior stroma were observed in the central cornea, and the degenerative lens adhered to the posterior surface of the defective corneal stroma. Also, in both eyes, the anterior chamber space was not formed and the undifferentiated iris stroma adhered to the posterior surface of the peripheral cornea. Mesenchymal tissue containing melanocytes was observed behind the degenerative lens, and the pigment epithelium was absent at the lower nasal side of the ciliary body in case 1. In case 2, mesenchymal tissue containing scattered melanocytes in the vitreous cavity was seen on the posterior retina. Based on the histological findings, both cases were diagnosed as Peters' anomaly caused by the faulty separation of the lens vesicle, PHPV, maldevelopment of the iris and ciliary body, and goniodysgenesis. CONCLUSION: Migratory disorders of neural crest cells from 4 to 7 weeks of gestation may be responsible for various ocular anomalies including Peters' anomaly and PHPV, as observed in these cases.

Abnormalities, Multiple↗

PubMed1998

Persistent hyperplastic primary vitreous (PHPV) in two Siberian husky dogs.

Three eyes in two Siberian husky dogs were clinically diagnosed as persistent hyperplastic primary vitreous (PHPV) by means of ophthalmoscopy and ultrasonography (USG). Examination of mildly affected PHPV eyes with an ophthalmoscope showed the axial part of the posterior capsule to be opaque. The central lesion of the posterior capsule in severely affected eyes had been opaque with many blood vessels. Echographic changes in mild cases of PHPV were outside of the lens, linearly hyperechoic, parallel to the posterior lens capsule. In a severely affected eyeball, funnel-shaped hyperechoic change was noted in the retrolental space. Two months later, phacoemulsification was performed for diagnostic treatment of PHPV since progressive cataract was observed in this eye.

Animals↗

PubMed2001

[Persistent hyperplastic primary vitreous--a case report of adult onset acute angle-closure glaucoma].

BACKGROUND: We report a patient with persistent hyperplastic primary vitreous(PHPV) who presented with acute angle-closure glaucoma in his adult life. CASE: A 30-year-old man had an attack of acute angle-closure glaucoma associated with retrolenticular fibrous tissue, atrophic retina, and elongated cilliary process in his right eye. RESULT: Ultrasound biomicroscopy(UBM) study showed iris bowing, shallow anterior chamber, and elongated cilliary body which were being pulled by the retrolenticular mass. The posterior chamber was normal. CONCLUSION: Although the mechanisms of secondary angle-closure glaucoma in PHPV are complicated, we suspected pupillary block resulting from constriction by the retrolenticular mass in this case.

Acute Disease↗

PubMed2004

[Persistent hyperplastic primary vitreous associated with retinal folds].

The paper presents the case of a 18 years old male suffering from persistent hyperplastic primary vitreous (PHPV) associated with congenital retinal folds. The clinical features and the pathogenic correlations of the two affections are discussed. Comparing to the PHPV, consequence of a embryogenesis flow appeared in the development of the primary hyaloid-vitreous complex, the congenital retinal folds are considered to be the expression of secondary changes, generated by the background of a varied vitreo-retinal pathology.

Adult↗

PubMed1999

Familial exudative vitreoretinopathy mimicking persistent hyperplastic primary vitreous.

PURPOSE: To report an unusual case of familial exudative vitreoretinopathy in an infant. METHODS: Case report. A 6-day-old girl had unilateral microphthalmia in the right eye, with a retrolental plaque initially diagnosed as persistent hyperplastic primary vitreous. Three months later, peripheral retinal vascular changes and a fibrovascular ridge were noted in the left eye, suggesting familial exudative vitreoretinopathy as the cause in both eyes. RESULTS: The microphthalmic right eye was unsalvageable. The left eye developed an exudative retinal detachment despite photocoagulation of the peripheral avascular retina. Additional cryotherapy resulted in resolution of the detachment and regression of the vascular changes. CONCLUSIONS: With highly asymmetric involvement, neonatal familial exudative vitreoretinopathy can mimic persistent hyperplastic primary vitreous. Fellow eye involvement can progress rapidly.

Cryotherapy↗