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Results for “Persistent Hyperplastic Primary Vitreous”

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PubMed2003

Complications in infants undergoing surgery for congenital cataract in the first 12 weeks of life: is early surgery better?

PURPOSE: To determine if there is an association between the timing of surgical intervention for congenital cataract within the first 12 weeks of life and the prevalence of postoperative complications. METHODS: We performed a retrospective review of records from 1990 to 2000 of infants who underwent surgery for congenital cataract within the first 12 weeks of life. Eighty eyes in 55 children were involved with a minimum follow up of 6 months. Bilateral cataracts were present in 25 and monocular cataracts in 30 infants. A limbal approach lensectomy-vitrectomy was performed in all infants. Children with aphakia were rehabilitated with contact lens or glasses. Operative and postoperative complications-including glaucoma, nystagmus, strabismus, retinal detachment, and posterior capsule opacification/secondary membranes-were recorded. Ocular and systemic associations were noted. Statistical analysis was carried out with classification and regression trees (CART). RESULTS: The mean age at the time of surgery was 31.5 +/- 23.3 days (median, 26.5; range, 2 to 84). Mean follow up from the time of surgery was 2.85 +/-1.9 years (median, 2; range, 0.5 to 8). Persistent fetal vasculature (persistent hyperplastic primary vitreous) was present in 14 eyes. One infant with bilateral persistent fetal vasculature had bilateral retinal dysplasia and was excluded from the analysis. Glaucoma developed in 12 infants (22%); nystagmus was present in 18 infants (33%); strabismus developed in 28 infants (52%); and secondary membranes developed in 7 eyes (13%). CART analysis suggests that glaucoma is more prevalent in infants when the surgery was performed between 13.5 and 43 days of life (CART = 0.370); nystagmus when surgery is performed between 48 and 84 days of life (CART = 0.500); strabismus when surgery is performed between 55.5 and 84 days of life (CART = 0.600); and secondary membranes when surgery is performed between 26.5 and 40 days of life (CART = 0.4). CONCLUSIONS: Our data suggest that the first 2 weeks of life comprise the most favorable time for decreasing postoperative complications resulting from surgical intervention for infants presenting with cataracts within the first 12 weeks of life.

Cataract↗

PubMed1994

[Vitreous hemorrhage associated with persistent hyaloid artery. Apropos of a case].

We report a case of vitreous haemorrhage from a persistent hyaloid artery that occurred to a 50 year-old patient. The case report is followed by a review of the embryologic development and a physiopathologic discussion about the origin of vitreous haemorrhage. The authors emphasize the distinction between persistent hyaloid artery and persistent hyperplastic primary vitreous.

Humans↗

PubMed1994

Differential diagnosis of leukokoria: radiologic-pathologic correlation.

Leukokoria is an abnormal pupillary light reflection that usually results from an intraocular abnormality and is seen most often in children. One-half of the cases of childhood leukokoria are caused by retinoblastoma, a malignant tumor of immature retinoblasts that manifests in a normal-sized eye as a calcified mass, is often partially necrotic, and grows into the vitreous and through the choroid. Retinoblastoma enhances with contrast material and, unlike most tumors, may be darker than vitreous on T2-weighted images. When leukokoria is associated with microphthalmia, persistent hyperplastic primary vitreous (PHPV) (28% of cases) or retinopathy of prematurity (ROP) (5% of cases) should be considered. PHPV is a congenital, usually unilateral lesion that appears as a dense tubular mass extending from the lens to the retina along the course of the hyaloid canal. Hemorrhage from PHPV produces a subhyaloid (or subretinal) fluid collection, often with characteristic blood-fluid levels. ROP is bilateral and usually manifests in premature infants who received supplemental oxygen therapy. Coats disease (16% of leukokoria) is a sporadic unilateral idiopathic retinal telangiectasia that produces a lipoproteinaceous subretinal exudate leading to complete retinal detachment. The globe has normal size but increased attenuation and signal intensity from hemorrhage without calcification or enhancement. Toxocaral endophthalmitis (16% of leukokoria) is a granulomatous reaction to the parasite in the vitreous and uveoretinal coat. Retinal astrocytoma (3% of leukokoria), which manifests in a normal-sized globe, is an indolent benign neoplasm commonly associated with phakomatoses (usually tuberous sclerosis).

Child↗

PubMed1997

[Retinal and corneal dysplasias in the Yorkshire terrier and other dog breeds in Austria].

Mother and son of a Yorkshire Terrier family showed excessive multifocal retinal dysplasia (RD1) and geographical retinal dysplasia (RD2), intra- and preretinal hemorrhages partial and total retinal detachment (total RD/RD3), residues of the pupillary membrane and as a newness in this combination a geographical, subepithelial corneal opacity, composed of small dots. One, not examined son of the same litter was congenitally blind. Similar corneal opacities were also found in two Rough Collies affected with CEA, RD, and microphthalmia, a young German Wirehair Pointer with primary absolute glaucoma in conjunction with goniodysplasia and RD in the healthy seeming fellow eye as well as in a poodle puppy affected with bilateral posterior suture-line cataract in connection with persistent primary hyperplastic vitreous (PHPV) in one and a globe-shaped deformed retina in connection with a persistent, blood conducting hyaloid artery in the other eye. The latter finally resulted in intraocular hemorrhage and secondary glaucoma. A pathogenetic connection between ocular malformations and subepithelial corneal opacity seems to be likely.

Animals↗

PubMed1979

Pars plana surgical approach for various anterior segment problems.

Various anterior segment problems can be approached through the pars plana and treated with instrumentation that has been developed for vitreous surgery. Two cases of aphakic bullous keratopathy due to vitreous touch and three cases of subluxated lenses were successfully treated. Of two cases of advanced, persistent hyperplastic primary vitreous, one showed a successful result.

Aged↗

PubMedDate not supplied

Persistence of the primary vitreous in association with the morning glory disc anomaly.

Two patients with the morning glory disc anomaly are reported. In one, fibrovascular stalk passed forward into the vitreous cavity, while in the second a persistent hyaloid artery extended anteriorly from a tuft of fibrous tissue to the posterior lens capsule. These cases suggest that in some eyes the morning glory disc anomaly occurs as a variant of persistent hyperplastic primary vitreous (PHPV).

Adult↗

PubMed1982

Morning glory syndrome. A functional analysis including fluorescein angiography, ultrasonography, and computerized tomography.

The Morning Glory syndrome (MGS) is a congenital optic nerve anomaly that has received scant attention since first described by Handmann in 1929. We studied ten eyes and eight patients displaying variations of the syndrome. We found that the syndrome may be bilateral, hereditary, and associated with other congenital anomalies. When bilateral, visual function was only moderately disturbed. The anomaly is usually limited to the eye, and the retrobulbar nerve and brain appear normal, as documented by ultrasound and computed tomography. Surprisingly, increasing fibrovascular tissue produces traction on the disc substance and vasculature, vitreous, macula, and peripapillary retina. These findings may place these patients within a spectrum of congenital optic nerve anomalies ranging from staphylomata to posterior persistent hyperplastic primary vitreous.

Adolescent↗

PubMedDate not supplied

Bilateral retinal vascular hypoplasia associated with persistence of the primary vitreous: a new clinical entity?

Three infant boys with a bilateral congenital retinopathy characterized by retinal vascular hypoplasia and persistence of the primary vitreous are described. The infants were healthy and had no non-ocular physical abnormalities. None had histories of prematurity or low birth weight. Family histories were negative to similar retinopathy, and the fundi of all examined blood relatives were normal. The described congenital retinopathy is compared and contrasted with persistent hyperplastic primary vitreous, Norrie's disease, idiopathic retinal dysplasia, familial exudative vitreoretinopathy, retinopathy of prematurity, and incontinentia pigmenti.

Electroretinography↗

PubMed1984

Diagnostic cytopathology of ocular fluid specimens.

The brief history of the development of cytopathologic study of ocular fluids has been reviewed. The utilization of cytopathology techniques has become increasingly important with the introduction and widespread use of vitreous surgery. The cytopathologic study of ocular fluids have diagnostic value in infectious and noninfectious ocular inflammatory diseases, neoplastic lesions, some forms of secondary glaucoma, epithelial ingrowth, proliferative vitreoretinopathy, persistent hyperplastic primary vitreous and amyloidosis.

Anterior Chamber↗

PubMed1999

A novel hereditary developmental vitreoretinopathy with multiple ocular abnormalities localizing to a 5-cM region of chromosome 5q13-q14.

BACKGROUND: To undertake a clinical and molecular analysis of a previously unpublished kindred with a phenotypically distinct vitreoretinopathy characterized by associated ocular developmental abnormalities. DESIGN: Family genetic study. PARTICIPANTS: A total of 23 members, both affected and unaffected, of 1 kindred with vitreoretinopathy. METHOD: Individuals within the kindred were examined clinically and blood samples taken for DNA analysis. Genetic analysis was performed for the proximal region of chromosome 5q by means of polymerase chain reaction (PCR). MAIN OUTCOME MEASURES: Detection of vitreoretinopathy and associated abnormalities. RESULTS: This novel, hereditary vitreoretinopathy, showing the classic features of vitreous pathology and early-onset retinal detachments, was associated with a variety of ocular developmental abnormalities, including posterior embryotoxon, congenital glaucoma, iris hypoplasia, congenital cataract, ectopia lentis, microphthalmia, and persistent hyperplastic primary vitreous. There were no associated systemic features. Genetic mapping with markers from the proximal region of 5q13-q14 showed linkage to a 5-cM region between the markers D5S626 and D5S2103. CONCLUSIONS: The 5-cM region is within that implicated in the etiology of both Wagner and erosive vitreoretinopathies. This suggests that this novel condition may be allelic, refines the genetic mapping for vitreoretinopathies that map to 5q13-q14, and implicates a gene important not only in vitreous production but also in early ocular development.

Abnormalities, Multiple↗

PubMed2004

[Criswick-Schepens syndrome -- familial exudative vitreoretinopathy. Report of six cases in two consanguineous families].

BACKGROUND: Familial exudative vitreoretinopathy is a hereditary, bilaterally progressive formation of a vitreoretinal membrane. It usually occurs in full-term newborns without previous treatment with hyperbaric oxygen. In this report, we present six cases of this disease with various abnormalities of the posterior segment diagnosed in two Syrian families related by first degree of consanguinity. DESIGN: A retrospective family analysis and presentation of cases. CASE PRESENTATION: Six members of the two consanguineous families, aged between 3.5 and 13 years, who were systemically healthy, presented with a visual acuity ranging between light perception and 0.4 with bilateral fundus changes. The findings included: papillary, macular, and retinal temporal traction in 11 eyes, a retinal fold in 7 eyes, a fibrovascular mass in 11 eyes, vitreoretinal traction in 5 eyes, subretinal exudation in 2 eyes, pigmentary abnormalities in 2 eyes, temporal or total tractional retinal detachment in 2 eyes, and vitreous hemorrhage in 1 eye. SUMMARY: Familial exudative vitreoretinopathy is characterized by fundus changes that resemble retinopathy of prematurity and must be differentiated from other diseases (e.g., Coats' disease, incontinentia pigmenti, persistent hyperplastic primary vitreous, and Norrie's disease).

Adolescent↗

PubMed1980

Prepapillary hemorrhage and persistent hyaloid artery.

A patient with bilateral persistent hyaloid vessels suffered a prepapillary and intravitreal hemorrhage. The clot hemolyzed and lines in the retina extended in all directions, involving the fovea. There was no vitreous separation and visual acuity was 6/6-3 (20/20-3). The patient was lost to follow-up shortly thereafter. Although persistent hyaloid vessels rarely cause intravitreal hemorrhage, they are often found in cases of persistent hyperplastic primary vitreous and must be considered possible complicating factors at surgery.

Adult↗

PubMed1996

Fetal alcohol syndrome--an ophthalmological and socioeducational prospective study.

BACKGROUND: The eye is a sensitive indicator of adverse effects of prenatal alcohol exposure. Anomalies of the eyes and their adnexa are known to be associated with the fetal alcohol syndrome (FAS), although long-term effects of these malformations are unknown. DESIGN: A prospective ophthalmologic follow-up (median, 11 years; range, 4 to 19 years) was performed in 25 children with FAS. Their social situation and educational status were also investigated. RESULTS: All but one of the children had ophthalmologic abnormalities. Fundus anomalies were observed in 23 children, of whom 19 had optic nerve hypoplasia. Thirteen children had concomitant strabismus. Microphthalmos, buphthalmos, phthisis, microcornea, coloboma of the iris and uvea, blepharoptosis, cataract, persistent hyperplastic primary vitreous, and nystagmus were observed in single cases. The dysmorphology of the eyes remained unchanged during the follow-up period. In 2 children with severe mental retardation and, initially, very poor vision, the severe visual handicap persisted. Seventeen children had an initial visual activity > or = 20/70, which remained unchanged in 10 children and improved in 7 children, despite the presence of optic nerve hypoplasia in 14 of the children. Ten mothers died, 8 of them because of alcohol-related diseases, and only 4 of the mothers were able to take care of their children. Sixteen children went to schools for the mentally retarded, and only 3 children had a normal school education without extra teaching assistance. CONCLUSIONS: In children with FAS, the major sequela, ie, brain, damage, remains despite extensive medical, educational, and social support. The presence of ophthalmic signs, which persisted but did not deteriorate during the follow-up period, strengthens the diagnosis of FAS, and the high frequency of ocular involvement indicates the importance of a complete ophthalmologic evaluation in children with FAS.

Adolescent↗

PubMed1992

Results of late surgery for presumed congenital cataracts.

We reviewed the results of cataract extraction and visual rehabilitation in 76 eyes of 47 infants and children with presumed congenital cataracts who were first seen after they were 10 months old. Eighteen patients underwent surgery for unilateral cataracts, including five patients with persistent hyperplastic vitreous, five with posterior lenticonus, one with a nuclear cataract, six with posterior subcapsular cataracts, and one with a lamellar cataract. Of these 18 patients, seven (39%) attained a visual acuity of 20/60 or better, one (6%) had a visual acuity of 20/100, and ten (60%) had a visual acuity of 20/200 or worse. Twenty-nine patients (62 eyes) underwent bilateral cataract extraction. The visual acuity could be measured in 22 patients (44 eyes). Visual acuity improved to 20/60 or better in 32 eyes (73%), was between 20/70 and 20/150 in 11 eyes (25%), and became worse than 20/200 in one eye (2%). Results were good in patients with persistent hyperplastic primary vitreous, posterior lenticonus, and bilateral cataracts.

Adolescent↗

PubMed1997

[Color Doppler ultrasonography in evaluation of intraocular lesions].

OBJECTIVE: To evaluate the value of color Doppler ultrasonography (CDU) in the diagnosis of intraocular lesions. METHODS: 82 cases of intraocular diseases were examined by high-resolution CDU (7.5 mHz), and the blood flow pattern was evaluated at the base of two-dimensional image. RESULTS: CDU might show the blood flow of retinal vessels in all 21 cases of retinal detachment, no blood flow could be detected in 21 of 22 vitreous hemorrhages and/or membranes, and only in one case with diabetic proliferative retinopathy did fine blood flow be seen. Intratumor blood flow with branchy pattern could be detected in 17 of 18 cases with choroidal melanomas and 3 of 4 cases with choroidal metastatic carcinoma, and spectral analysis revealed high-resistance, simulating arterial blood flow waves. In 5 cases with choroidal hemangioma, intratumor blood flow with filled or sparkled pattern was detected, and the spectral analysis revealed both arterial and venous blood flow waves. No blood flow in the lesion was detected in simulating benign lesions i.e. 5 choroidal hemorrhages and 2 subretinal hemorrhages. In 3 cases (4 eyes) with persistent hyperplastic primary vitreous (PHPV), CDU showed band-shaped arterial blood flow coursing from optic nerve disc to the posterior surface of the lens in the vitreous cavity. CONCLUSION: CDU is helpful in the differential diagnosis among retinal detachment, vitreous hemorrhage, vitreous membrane, malignant choroidal melanoma, choroidal metastatic tumor, choroidal benign lesion or hemangioma, PHPV and lesions with white pupil.

Adolescent↗

PubMed1996

The role of magnetic resonance imaging in children with intraocular tumors and simulating lesions.

PURPOSE: To evaluate the role of pre- and post-contrast magnetic resonance (MR) studies in children with intraocular tumors and simulating lesions. METHODS: Patients younger than 12 years of age with the diagnosis of an intraocular tumor or simulating lesion with a thickness greater than 2.0 mm underwent pre- and post-contrast MR studies with surface coil. All post-contrast-enhanced images were performed with fat suppression techniques. RESULTS: There were 40 children with the clinical diagnosis of an intraocular lesion as follows: retinoblastoma (n = 22), Coats disease (n = 5), ciliary body medulloepithelioma (n = 2), primary hyperplastic persistent vitreous (PHPV) (n = 2), retinal capillary hemangioma (n = 3), massive retinal gliosis (phthisis bulbi) (n = 2), uveal melanoma (n = 1), ciliary body leiomyoma (n = 1), retinopathy of prematurity (ROP) with total retinal detachment (n = 1), and post-traumatic retinal gliosis (n = 1). In the authors' series, solid intraocular tumors greater than 2.0 mm in thickness such as retinoblastoma, ciliary body medulloepithelioma, leiomyoma, choroidal melanoma, and retinal capillary hemangioma appeared hyperintense on T1-weighted images and hypointense on T2-weighted images. Secondary serous or exudative retinal detachment, regardless of the underlying etiology (neoplasm, Coats disease, PHPV, phthisis bulbi, ROP), showed hyperintensity of the subretinal space on both T1- and T2-weighted images. Retinal gliosis was hypointense on both T1- and T2-weighted images. On contrast-enhanced T1-weighted images with fat suppression techniques, minimal to marked enhancement was observed in retinoblastoma, medulloepithelioma, retinal capillary hemangioma, leiomyoma, and choroidal melanoma. No enhancement was demonstrated in the subretinal space of Coats disease, PHPV, ROP, and in retinal gliosis. Calcification was identified in 54% of retinoblastoma tumors on MR sequences. Calcification showed low signal intensity on both T1- and T2-weighted images, with lack of enhancement on contrast-enhanced sequences, thus differentiating it from surrounding tumor or tissue necrosis. The associated serous or exudative subretinal fluid secondary to intraocular tumors or simulating lesions did not demonstrate enhancement after contrast administration, therefore differentiating it from the causative lesion. CONCLUSION: Pre- and post-contrast MR studies allowed differentiation of solid intraocular tumors such as retinoblastoma, medulloepithelioma, retinal capillary hemangioma, leiomyoma, and choroidal melanoma from intraocular lesions with primary retinal detachment such as Coats disease, PHPV, massive retinal gliosis (phthisis bulbi), ROP, and associated subretinal fluid or hemorrhage. Heterogeneity within retinoblastoma suggests foci of tumor necrosis and/or calcification. The various solid intraocular tumors were not reliably differentiated from one another based on MR features.

Child↗

PubMed1999

Retinal hamartoma in oral-facial-digital syndrome.

Only recently have intraocular findings been described in oral-facial-digital syndrome (OFDS), including 5 cases of chorioretinal colobomas and 1 case of optic nerve coloboma. We report a case of a new ocular anomaly associated with this syndrome: a retinal hamartoma in a male infant with OFDS. The patient had bilateral retinal masses that were suspicious for retinoblastoma because of a family history of retinoblastoma. Physical examination and imaging studies of the retinal masses could not differentiate between retinoblastoma, hamartoma, or persistent hyperplastic primary vitreous. Subsequent pathologic study of an enucleated globe was diagnostic of a retinal hamartoma. This case further illustrates the heterogeneity of ocular anomalies in OFDS and underscores the importance of a complete ophthalmologic evaluation in patients with this syndrome.

Eye Abnormalities↗

PubMed1978

Multiple ocular anomalies associated with maternal LSD ingestion.

Severe ocular malformations, including microphthalmos, intraocular cartilage, cataract, persistent hyperplastic primary vitreous, and retinal dysplasia, occurred in a premature baby girl. The mother had ingested LSD during the first trimester of pregnancy. To our knowledge, this is the third case reported of ocular teratogenesis associated with maternal LSD ingestion. Further cases must be documented to establish an actual cause and effect relationship between the drug and the induced malformations.

Abnormalities, Drug-Induced↗