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Color Doppler imaging diagnosis of intra-ocular tumor.

OBJECTIVE: To demonstrate the color Doppler imaging (CDI) of intra-ocular tumors and its importance in the diagnosis of these tumors. METHODS: Ninety-two patients with intra-ocular tumors (105 eyes) were examined by using CDI to observe the vasculature of these tumors, and using SAS soft ware to analyze the results. RESULTS: Blood flow signal was found in retinoblastoma, melanoma of choroid, angioma of choroid, angioma of optic disc, Coats' disease, persistent hyperplastic primary vitreous (PHPV) and metastatic tumors of choroid, but not found in choroid osteoma and melanocytoma of optic disc. The blood flow decreases in central retinal artery in choroidal melanoma and also in ophthalmic artery, central retinal artery and posterior ciliary artery in angioma of choroid (P < 0.01). It was found that blood velocity is much faster in choroidal melanoma than in choroidal angioma (P < 0.05). CONCLUSIONS: CDI is very important and helpful in the diagnosis of intra-ocular tumors.

Adolescent↗

Early morphogenesis of persistent hyperplastic tunica vasculosa lentis and primary vitreous. A transmission electron microscopic study.

This report provides transmission electron microscopic observations on the early pathogenesis of persistent hyperplastic tunica vasculosa lentis/persistent hyperplastic primary vitreous (PHTVL/PHPV) in affected canine fetuses at days 28-44 postcoitum. The retrolental tissue by which this anomaly is characterized consists of loosely arranged fibroblasts in a randomly oriented meshwork of collagenous fibrils. Some of these cells contain melanosomes at day 44. In one day-44 eye, cells of neuroectodermal origin (Müller cells; fibrous astrocytes) were observed. From day 37 onward, the posterior subcapsular part of the lens contains rounded, increased intercellular spaces, resembling vacuoles, which deform the shape of the lens fibers. The posterior lens capsule develops normally until day 30. From day 35 onward the capsule has an amorphous ultrastructure, as opposed to the clearly laminated ultrastructure in reference eyes at day 35. In addition, the capsule's thickness increases until day 35, and, instead of growing thicker, decreases thereafter. Based on these results, it is hypothesized that a primary metabolic disorder in the lens fibers, subsequently leading to the formation of an abnormal posterior lens capsule, constitutes the primary defect in the sequence of events leading to PHTVL/PHPV.

Animals↗

Ultrasonographic diagnosis of persistent hyperplastic tunica vasculosa lentis/persistent hyperplastic primary vitreous in two dogs.

Ultrasonography was performed on a Basset hound and a Doberman clinically suspected of persistent hyperplastic tunica vasculosa lentis/persistent hyperplastic primary vitreous. In both dogs, hyperechoic lenses with a triangular-shaped echodense structure retrolentally were visible, and a very thin hyperechoic strand was seen penetrating the anechoic vitreous from this retrolental tissue to the area of the optic nerve. Using color Doppler imaging, blood flow was evident in parts of the retina in both dogs. With power Doppler imaging there was blood flow in the lens and hyperechoic strand of the Basset hound on the first examination; whereas, on re-examination 5 months later, this was not found. Other abnormalities, such as retinal detachment, endophthalmitis, vitreous hemorrhage, microphthalmia, and posterior neoplasia could be excluded.

Animals↗

Surgical management and histologic and immunohistochemical features of a cataract and retrolental plaque secondary to persistent hyperplastic tunica vasculosa lentis/persistent hyperplastic primary vitreous (PHTVL/PHPV) in a Bloodhound puppy.

The objective of this study was to describe the clinical, histologic and immunohistochemical features, the surgical treatment, and outcome of a cataract secondary to persistent hyperplastic tunica vasculosa lentis/persistent hyperplastic primary vitreous (PHTVL/PHPV) in a dog. A 4-month-old male Bloodhound dog presented for evaluation of a cataract. A complete ophthalmic examination and ocular ultrasonography were performed. A resorbing cataract with intralenticular hemorrhage, lens induced uveitis, and PHTVL/PHPV were diagnosed. Extracapsular cataract extraction using phacoemulsification was performed. A primary posterior capsulectomy was performed to remove a retrolental plaque with the posterior capsule; the excised plaque was submitted for histopathology and immunohistochemical staining. A 41-Diopter intraocular lens (IOL) was implanted. Functional vision was maintained postoperatively during the 21-month follow-up period. Histologically, the posterior capsule was coiled and exhibited duplication. The retrolental plaque was comprised of dense fibrous connective tissue, blood vessels, free red blood cells, hemosiderin-laden macrophages, a pocket of neural tissue and numerous perivascular mast cells. With immunohistochemical staining, the neural elements were determined to be glial cells compatible with astrocytes. Cataract secondary to PHTVL/PHPV can be successfully treated using phacoemulsification and planned posterior capsulectomy. Posterior lens capsule duplication, mast cells and astrocytic glial cells may be normal components of the fibrovascular retrolental plaque associated with PHTVL/PHPV.

Animals↗

Early morphogenesis of persistent hyperplastic tunica vasculosa lentis and primary vitreous (PHTVL/PHPV). Scanning electron microscopic observations.

This study provides scanning electron microscopic observations on the early morphogenesis of persistent hyperplastic tunica vasculosa lentis and primary vitreous (PHTVL/PHPV) in canine fetuses at days 28 35 postcoitum (D28 and D35). From previous studies regarding PHTVL/PHPV it is known that a retrolental plaque of fibrovascular tissue is present in eyes of affected canine fetuses from the D33 stage. The contribution of vitreous cells to the formation of the plaque is supported by the results of this study. The lens capsules at the stages described were not found to contain abnormalities such as transparent (thinner) parts or rents, as have been described for postnatal cases of PHTVL/PHPV. These findings support the hypothesis that the capsular anomalies observed in postnatal patients are secondary entities.

Animals↗

Outcomes of vitreoretinal surgery in patients with X-linked retinoschisis.

BACKGROUND AND OBJECTIVE: To assess the outcomes of vitreoretinal surgery in the treatment of vision-threatening posterior segment complications of X-linked retinoschisis. PATIENTS AND METHODS: The authors performed a retrospective analysis of 16 eyes from 11 patients who underwent vitreoretinal surgery. All the patients had a documented positive family history of X-linked retinoschisis, and all patients had bilateral macular disease. RESULTS: The ages of the patients ranged from 14 months to 37 years (mean age 15.1 years; median age 11.5 years), and postoperative follow-up ranged from 3 months to 10 years (mean 2.8 years; median 1 year). The indications for surgical intervention included rhegmatogenous retinal detachment (12 eyes), vitreous hemorrhage (2 eyes), progression of the schisis cavity through the fovea (2 eyes), cataract associated with a persistent hyperplastic primary vitreous-like condition (2 eyes), and exudative maculopathy (1 eye). The primary surgical intervention included pars plana vitrectomy alone (7 eyes), pars plana vitrectomy and pars plana lensectomy (4 eyes), and a scleral buckle procedure alone (5 eyes). Surgical success (defined as reattachment of the retina, removal of media opacities, or arrest of schisis progression) was achieved in 14 of 16 eyes, after an average of 1.2 procedures per eye. The major reason for reoperations was recurrent retinal detachment due to proliferative vitreoretinopathy. Two eyes were eventually enucleated due to pain associated with neovascular glaucoma resulting from recurrent retinal detachment. Of the remaining 14 eyes, visual acuity improved in 8 eyes and remained unchanged in 6 eyes. CONCLUSION: Vitreoretinal surgery is often helpful in stabilizing or improving visual function in patients with posterior segment complications from X-linked retinoschisis.

Adolescent↗

Ocular findings in cerebro-ocular-myopathy syndrome (COMS). A possible role of growth factors?

The clinical and histopathological findings are described in a case of cerebral and ocular abnormalities associated with (congenital) muscular dystrophy. Histopathological examination of the eyes revealed a fetal configuration of the anterior chamber angle, elongated ciliary processes, cataract, persistence of primary hyperplastic vitreous and total retinal detachment with retinal dysplasia. The similarity of ocular findings in Walker-Warburg syndrome, muscle-eye-brain disease and Fukuyama's congenital muscular dystrophy, and the role of growth factors as a possible unifying (foetal) cause are discussed. This case has been presented at the meeting of the EOPS Verhoeff Society, Nürnberg 1991.

Abnormalities, Multiple↗

Visual impairment and ocular abnormalities in children with fetal alcohol syndrome.

Children with fetal alcohol syndrome (FAS) may have impaired vision and various ocular abnormalities. All parts of the eye may be affected and anomalies such as microphthalmus, microcornea, Peters' anomaly, cataract, persistent hyperplastic primary hyaloid vitreous body, coloboma of the iris and choroid, retinal dysplasia and, most commonly, optic nerve hypoplasia and tortuosity of the retinal vessels, have been reported. The periocular facial features consist of short palpebral fissures (included in the criteria for diagnosis of FAS), telecanthus, epicanthus and blepharoptosis. Visual function may be reduced to a moderate or severe degree. Refractive errors and strabismus are common. Considering the high frequency of ocular pathology in FAS, an ophthalmological examination is helpful in making the diagnosis. The early detection of impaired vision and ocular abnormalities in affected children is important in the management of the disorder. In this article, the ophthalmological signs and symptoms in children with FAS are presented.

Child↗

Early morphogenesis of the canine lens capsule, tunica vasculosa lentis posterior, and anterior vitreous body. A transmission electron microscopic study.

This study provides transmission electron microscopic observations on the early morphogenesis (from days 25-35 post coitum) of the canine posterior lens capsule, the tunica vasculosa lentis (TVL) posterior and the anterior part of the vitreous body. The presence of an anlage of the posterior lens capsule as early as day 25, recently described histologically, was confirmed by this study. In the period from day 25 to day 35, the polar part of the posterior lens capsule develops 2-29 continuous and parallel lamellae, matching 50 nm and 1.74 microns, respectively. At these early stages, the TVL consists of capillaries that are simple endothelial tubes. From day 28 onward, these can be classified as A-1-alpha capillaries according to the classification of Bennett et al. [3]. In direct proximity to the lens capsule, the vitreous body contains fibrillar material with a morphological appearance similar to that of the lens capsule. This material probably derives from both the capillary endothelial cells' basal lamina and the lens capsule. Only few cellular components were observed in the anterior vitreous body. The development of the described structures is grossly in accordance with that observed in other mammalian species. The observations presented serve as a reference for studies on the pathogenesis of persistent hyperplastic tunica vasculosa lentis/persistent hyperplastic primary vitreous (PHTVL/PHPV), which is an important cause of leucocoria in children and in some dog breeds.

Animals↗

Use of the ocutome in anterior segment surgery.

9 patients are presented on whom anterior vitrectomy was performed. The indications included congenital rubella cataract with microphthalmos, traumatic cataract, persistent anterior and posterior hyperplastic primary vitreous, postcataract pupillary membranes, and postcataract vitreous prolapse with cystoid mascular edema. All patients to date have responded satisfactorily to the procedure. There has been no incidence of complicating retinal detachment.

Adult↗

Persistent hyperplastic tunica vasculosa lentis and persistent hyaloid artery in a 2-year-old basset hound.

A 2-year-old Basset hound with unilateral persistent hyperplastic tunica vasculosa lentis and primary vitreous is described. There was leukokoria in the left eye, caused by bluish-white polar densities and haemorrhagic discoloration in the nucleus of the lens. Ultrasonographic examination of the eye revealed a small hyperechoic lens and a hyperechoic linear soft-tissue strand extending from the posterior pole of the lens through the optic disc. Power Doppler imaging revealed blood flow in this persistent hyaloid artery, but 5 months later, immediately prior to surgery, there was no flow in this artery. Ultrasonography, including Power Doppler imaging, was useful to confirm the diagnosis and to estimate the complications of surgery, especially the risk of vitreal haemorrhage after cutting of the persistent hyaloid artery. Comparison of the ultrasonographic images with those obtained by computed tomography suggested that the resolution of computed tomography is inadequate for identifying structures as fine as a persistent hyaloid artery.

Animals↗

The incidence of PHTVL/PHPV in Doberman and the results of breeding rules.

Between 1967 and 1987, in the Netherlands 3,775 Dobermans were examined for hereditary eye diseases. In 238 of these dogs severe persistent hyperplastic tunica vasculosa lentis and primary vitreous (PHTVL/PHPV) was diagnosed. After a brief description of the clinical features the results of breeding over the recent years are discussed. The incidence (1978-1987) of severely affected dogs in the litter controls decreased from 5% to 1% in the individual controls from 19% to 8%. This is an encouraging downward tendency. Better results are to be expected once test mating and progeny-testing methods are accepted as breeding methods in dog breeding.

Animals↗

[Posterior form of persistent hyperplasia of the primary vitreous body associated with central serous chorioretinopathy].

The authors report a case of posterior hyperplastic primary vitreous which was associated by chance with a central serous choroidoretinopathy. Examination of the left eye revealed a dense white vitreous condensation attached to the optic disc associated with retinal folds. Fluorescein Angiography showed a typical central serous choroidoretinopathy. No treatment was required as retinal complications due to the posterior hyperplastic primary vitreous were not found; central serous choroidoretinopathy had a satisfactory course. Posterior hyperplastic primary vitreous is a rare disease, different from Reese's anterior form and may mimic congenital abnormalities of the optic disc. Clinical appearance, clinical course and treatment are discussed.

Adult↗

Diffuse infiltrating retinoblastoma associated with persistent primary vitreous.

A two-and-a-half-year-old male child presented with unilateral leukocoria. Clinical examination disclosed what was believed to be a mild form of persistent hyperplastic vitreous associated with total retinal detachment and exophytic retinoblastoma. The enulceated eye contained diffuse infiltrating retinoblastoma with tumor cells involving a stalk of persistent primary vitreous without hyperplasia.

Child, Preschool↗