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Results for “Persistent Hyperplastic Primary Vitreous”

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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↗

PubMed1989

[Computer tomography in the diagnosis of persistent hyperplastic primary vitreous body].

The authors described and evaluated clinical and CT pictures of five children with persistence of hyperplastic primary vitreous body originating due to regression of embryonal hyaloid vascular system. It becomes clinically manifest especially in leucocoria, reduced globe of the eye, prolonged ciliary processi and the formation of fibrovascular changes behind the lens. CT picture is characterized by a) increased density of vitreous body, b) dense stripes going in retrolental direction and in the course of the Cloquet canal, c) microphthalmus, d) absence of calcifications and e) facultative changes on the lens and anterior chamber. These signs represent an important criterium for differentiating persistence of hyperplastic primary vitreous body from retinoblastoma.

Child↗

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↗

PubMed1981

Surgical management of persistent hyperplastic primary vitreous.

Instruments designed for pars plana vitrectomy can be used to manage complicated congenital cataracts such as those with persistent hyperplastic primary vitreous (PHPV). We have applied closed-eye vitrectomy techniques through a limbal approach in seven eyes with PHPV. A clear pupillary space was achieved in all cases. The management of children with complicated congenital cataracts such as PHPV is discussed.

Cataract↗

PubMedDate not supplied

Inherited retinal dysplasia and persistent hyperplastic primary vitreous in Miniature Schnauzer dogs.

The objectives of this study were to define the clinical syndrome of retinal dysplasia and persistent primary vitreous in Miniature Schnauzer dogs and determine the etiology. We examined 106 Miniature Schnauzers using a biomicroscope and indirect ophthalmoscope. The anterior and posterior segments of affected dogs were photographed. Four enucleated eyes were examined using routine light microscopy and scanning electron microscopy. A pedigree was constructed and related dogs were test-bred to define the mode of inheritance of this syndrome. Congenital retinal dysplasia was confirmed in 24 of 106 related Miniature Schnauzer dogs. Physical and postmortem examinations revealed that congenital abnormalities were limited to the eyes. Biomicroscopic, indirect ophthalmoscopic, and neuro-ophthalmic examinations confirmed that some of these dogs were blind secondary to bilateral retinal dysplasia and detachment (nonattachment) (n = 13), and the remainder had generalized retinal dysplasia (n = 11). Fifteen of these dogs were also diagnosed with unilateral (n = 9) or bilateral (n = 6) persistent hyperplastic primary vitreous. Nutritional, infectious, or toxic etiologies were not evident on physical, postmortem, light microscopic, or transmitting and scanning electron microscopic examination of four affected Miniature Schnauzers. We examined the pedigree and determined that an autosomal recessive mode of inheritance was most likely. Three test-bred litters including those from affected parents, carrier and affected parents, and carrier parents confirmed this mode of inheritance. This study confirms that retinal dysplasia and persistent hyperplastic primary vitreous is a congenital abnormality that is inherited as an autosomal recessive condition in Miniature Schnauzers.

Animals↗

PubMed1983

Management of persistent hyperplastic primary vitreous.

Instruments designed for pars plana vitrectomy can be used to manage selected complicated congenital cataracts such as those with persistent hyperplastic primary vitreous (PHPV). We have applied closed-eye vitrectomy techniques through a limbal approach in seven eyes with PHPV. A clear pupillary space was achieved in all cases and two of the seven eyes achieved useful vision. The management of children with complicated congenital cataracts such as those with PHPV is discussed.

Cataract↗

PubMedDate not supplied

Incising the thick retrolental fibrovascular tissue with a hooked sclerotome in persistent hyperplastic primary vitreous.

A technique for incising thick retrolental fibrovascular tissue and extensive cyclitic membrane is reported in a case of anterior persistent hyperplastic primary vitreous. A membranectomy was performed in a 1-month-old post-lensectomy baby via a limbal approach. A sclerotome tip was hooked to cut through an extremely thick fibrovascular tissue by rotating the sclerotome by its grip. Sutherland microscissors (Grieshaber, Switzerland) and a vitrectomy cutter were used for further membranectomy. The baby was followed-up until age 18 months. A total of 3 membranectomy sessions were required because of rapid cyclitic membrane formation, severe centripetal retraction of the membrane on the ciliary processes, and posterior synechia. Thorough membranectomy and cutting the iris edge maintained a clear pupillary area during the 13-month postoperative period. Extremely thick retrolental fibrovascular tissue is a challenging condition that can be dealt with by delicate instrumentation.

Anterior Eye Segment↗

PubMed2001

Locus for autosomal recessive nonsyndromic persistent hyperplastic primary vitreous.

PURPOSE: To map the disease locus in a six-generation, consanguineous Pakistani family affected by nonsyndromic autosomal recessive persistent hyperplastic primary vitreous (arPHPV). All affected individuals had peripheral anterior synechiae and corneal opacities with variable degrees of cataract and a retrolenticular white mass behind the lens. METHODS: Genomic DNA from family members was typed for alleles at more than 400 known polymorphic genetic markers, by polymerase chain reaction. Alleles were assigned to individuals, which allowed calculation of lod scores. RESULTS: A maximum two-point lod score of 4.07 was obtained with marker D10S1225 with no recombination. Two recombinations with marker D10S208 and D10S537 localized the disease within a region of approximately 30 centimorgans (cM). However, homozygosity across the region refined the arPHPV locus to 13 cM. CONCLUSIONS: Linkage analysis shows localization of nonsyndromic arPHPV to chromosome10q11-q21.

Adolescent↗

PubMedDate not supplied

Persistent hyperplastic primary vitreous. A clinicopathologic study of 62 cases and review of the literature.

This is a clinicopathologic study of 62 cases of persistent hyperplastic primary vitreous (PHPV). The cases were divided into two main groups. Group 1 consisted of 55 unilateral cases not associated with any systemic abnormalities, including 36 eyes (58%) which were considered "pure cases" (Group 1A) and 19 (31%) which disclosed other ocular abnormalities in addition to PHPV (Group 1B). Group 2 consisted of 7 (11%) bilateral cases of PHPV accompanied by other ocular and systemic malformations. The most common presenting clinical signs are leukocoria, microphthalmia and cataract. The main histopathologic features of this condition are outlined, including those responsible for the disastrous results to the eye (retinal detachment, glaucoma, phthisis bulbi). Several clinical entities, usually mistaken for or associated with PHPV, such as retinoblastoma, congenital cataract, retinal dysplasia, trisomy 13 syndrome, and falciform retinal folds are discussed briefly.

Adolescent↗

PubMed1992

[Persistent hyperplastic primary vitreous. Middle-term results of vitrectomy].

In this study, the authors present a homogeneous series of seven children suffering from persistent hyperplastic primary vitreous, in its complete anterior and posterior form. These children were operated by pars plana lensectomy and vitrectomy. The surgical operation was beneficial in every case: not only were there no complications, but the operation also prevented progression towards neovascular glaucoma, vitreous hemorrhages and phtisis bulbi. In addition, one of the benefits of this technique was esthetic, with the disappearance of leucocoria, the occasional correction of strabismus and the reduction in microphthalmos. Visual recovery can be surprisingly good, in the absence of any associated retinal malformation.

Eye Abnormalities↗

PubMed1983

Persistent hyperplastic primary vitreous. Surgical treatment.

Instruments designed for pars plana vitrectomy can be used to manage selected complicated congenital cataracts such as those with persistent hyperplastic primary vitreous (PHPV). We have applied closed-eye vitrectomy techniques through a limbal approach in seven eyes with PHPV. A clear pupillary space was achieved in all cases and two eyes achieved useful postoperative vision. The management of children with complicated congenital cataracts, such as with PHPV, is discussed.

Cataract↗

PubMed1989

Rieger's eye anomaly and persistent hyperplastic primary vitreous.

The authors present a Turkish family (two generations, five affected persons) with symptoms of Rieger's eye anomaly as well as Persistent Hyperplastic Primary Vitreous (PHPV). Although Rieger's anomaly has been described in combination with other conditions, according to their knowledge a familial combination of Rieger's anomaly and PHPV has never been described before. Only once an isolated case with the combination of an anterior chamber cleavage malformation and bilateral PHPV has been described. The authors suggest two possible explanations for the coexistence of PHPV and Rieger's eye anomaly. It is feasible that we are dealing with symptoms until yet not diagnosed within the anomaly of Rieger. Another possibility is that there is a linkage between the genes for PHPV and Rieger's anomaly and as a consequence they are inherited together in this family.

Adolescent↗

PubMedDate not supplied

Sympathetic ophthalmia in vater association combined with persisting hyperplastic primary vitreous after cyclodestructive procedure.

PURPOSE: To report on the occurrence of histology-proven sympathetic ophthalmia in a patient with VATER association and persisting hyperplastic primary vitreous (PHPV) after a cyclodestructive procedure was performed to treat secondary angle-closure glaucoma. METHODS: The left eye of a 13-year-old boy with VATER association was microphthalmic from birth and had been diagnosed with PHPV at age 1 year. It developed iris neovascularization and secondary angle-closure glaucoma, which was treated by combined cyclocryocoagulation and cyclophotocoagulation. Six weeks later, a bilateral fibrinous iritis developed. Despite intensive topical and systemic steroid treatment, the iritis persisted so that the left blind eye was enucleated. RESULTS: Histology of the enucleated eye showed a marked intraocular inflammation with lymphocytes, epithelioid cells, and multinuclear giant cells grouped around remnants of melanin-bearing cells. CONCLUSIONS: Sympathetic ophthalmia may occur in patients with VATER association and PHPV after a secondary angle-closure glaucoma is treated by a combined cyclocryocoagulation and cyclophotocoagulation.

Abnormalities, Multiple↗

PubMed1985

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.

Aged↗

PubMed1990

Persistent hyperplastic primary vitreous with vertical transmission.

The authors report a 15-month-old white male and his 30-year-old mother who both have persistent hyperplastic primary vitreous (PHPV) unassociated with other congenital anomalies. Although there are two previous reports of PHPV in siblings suggesting autosomal recessive inheritance, this is the first report compatible with autosomal dominant inheritance.

Adult↗

PubMed2003

Persistent hyperplastic primary vitreous: magnetic resonance imaging and clinical findings.

BACKGROUND: This study presents magnetic resonance imaging (MRI) findings of both the anterior and posterior types of persistent hyperplastic primary vitreous (PHPV) to facilitate the differential diagnosis from other intraocular abnormalities. METHODS: Seventeen patients with PHPV who were evaluated using non-contrast and contrast-enhanced T1- and T2-weighted images were retrospectively reviewed. RESULTS: Among the 17 patients with PHPV (6 males, 11 females), 13 had unilateral PHPV (11 left eyes, 2 right eyes), and 4 had bilateral PHPV. The MRI findings of the anterior type of PHPV included a shallow or collapsed anterior chamber, an anterior segment anomaly, and a retrolental vascular membrane which demonstrated hyperintensity after contrast enhancement. The MRI findings of the posterior type consisted of microphthalmos; a tubular image, representing the hyaloid vessel; a funnel-shaped retinal detachment, with the subretinal fluid hyperintense on both T1- and T2-weighted images; the fluid-fluid level, which was hypointense on both T1- and T2-weighted images and probably corresponded to the presence of hemorrhage in the subretinal space; a retrolental mass; and vitreous hemorrhage. The most common clinical presentations of patients with PHPV in our study were microphthalmos, a shallow or collapsed anterior chamber, and leukocoria. CONCLUSIONS: The presentation of PHPV at different stages was variable; the MRI features of PHPV along with clinical findings were able to facilitate the differential diagnosis from other intraocular abnormalities such as retinoblastoma and Coats' disease.

Adolescent↗

PubMed1999

Persistent hyperplastic primary vitreous with retinal tumor in tuberous sclerosis: report of a case including tumoral immunohistochemistry and cytogenetic analyses.

OBJECTIVE: The authors describe an ocular lesion combining the characteristics of persistent hyperplastic primary vitreous (PHPV) and a retinal tumor in an infant with tuberous sclerosis complex (TSC). STUDY DESIGN: Case report. METHODS: Immunohistochemistry and cytogenetic studies were performed on TSC cells from an intraocular tumor in a 6-week-old infant. RESULTS: Histopathologic examination showed a thick fibrovascular membrane between the aspect of the lens and the astrocytic component of the mass. Glial fibrillary acidic protein (GFAP) showed a variable intracytoplasmic reaction in the astrocytic proliferation, involving approximately 50% of the cells. Tissue culture studies showed a fairly rapid proliferation of fusiform cells, consistent with bipolar astrocytic cells. Cytogenetic studies showed one abnormal clone consisting of three hyperdiploid cells with a loss of chromosome 9 and a gain of chromosomes 6 and 12. CONCLUSION: The atypical localization of the retinal tumor could be explained by the fact that it was trapped during its proliferation by the retinal detachment associated with the PHPV.

Astrocytoma↗

PubMed2001

Persistent hyperplastic tunica vasculosa lentis and persistent hyperplastic primary vitreous (PHTVL/PHPV) in two cats.

Two domestic shorthair cats (6 and 9 months old) were presented for examination of ocular opacities. One cat had bilateral persistent pupillary membranes, unilaterally accompanied by persistent hyperplastic tunica vasculosa lentis, persistent hyperplastic primary vitreous (PHTVL/PHPV), and cataract. The second cat had bilateral PHTVL/PHPV with dense white plaques in the posterior lens capsule and subcapsular cortex.

Animals↗