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Results for “Persistent Hyperplastic Primary Vitreous”

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PubMed2002

Angle closure in younger patients.

PURPOSE: Angle-closure glaucoma is rare in children and young adults. Only scattered cases associated with specific clinical entities have been reported. We evaluated the findings in patients in our database aged 40 or younger with angle closure. METHODS: Our database was searched for patients with angle closure who were 40 years old or younger. Data recorded included age at initial consultation; age at the time of diagnosis; gender; results of slit-lamp examination, gonioscopy, and ultrasound biomicroscopy (from 1993 onward); clinical diagnosis; and therapy. Patients with previous incisional surgery were excluded, as were patients with anterior chamber proliferative mechanisms leading to angle closure. RESULTS: Sixty-seven patients (49 females, 18 males) met entry criteria. Mean age (+/- SD) at the time of consultation was 34.4 +/- 9.4 years (range, 3-68 years). Diagnoses included plateau iris syndrome (35 patients), iridociliary cysts (8 patients), retinopathy of prematurity (7 patients), uveitis (5 patients), isolated nanophthalmos (3 patients), relative pupillary block (2 patients), Weill-Marchesani syndrome (3 patients), and 1 patient each with Marfan syndrome, miotic-induced angle closure, persistent hyperplastic primary vitreous, and idiopathic lens subluxation. CONCLUSION: The etiology of angle closure in young persons is different from that in the older population and is typically associated with structural or developmental ocular anomalies rather than relative pupillary block. Following laser iridotomy, these eyes should be monitored for recurrent angle closure and the need for additional laser or incisional surgical intervention.

Adolescent↗

PubMed2003

Identification and characterization of TRIP8 gene in silico.

TRIP1-TRIP15 genes encode thyroid hormone receptor beta (TR beta)-binding proteins. TRIP10 gene encodes FNBP1 family protein with FCH, FBH, and SH3 domains. Among 15 TRIP genes, TRIP8 gene remained uncharacterized except TRIP8 partial cDNA (L40411). Here, we determined the complete coding sequence of TRIP8 gene by using bio-informatics. Nucleotide sequence of full-length TRIP8 cDNA was determined in silico by assembling nucleotide sequences of FLJ14374 and DKFZp761F0118 cDNAs. TRIP8 protein (2540 aa) was found to consist of two bipartite nuclear localization signals (codon 352-368 and 2365-2381), TRI8H1 domain (codon 1697-1873), TRI8H2 domain (codon 2057-2351), and JMJC domain (codon 2387-2486). TRI8H1, TRI8H2 and JMJC domains were conserved among TRIP8, 5qNCA (C5orf7) and TSGA proteins. TR beta-binding domain was overlapped with N-terminal part of TRI8H2 domain, and C2HC4-type zinc finger-like motif was located within C-terminal part of TRI8H1 domain. Because JMJC domain proteins are implicated in chromatin remodeling, TRIP8 was predicted to be a transcriptional regulator associated with nuclear hormone receptors. Human TRIP8 gene, consisting of 26 exons, was about 300 kb in size. Intra-species comparative genomics revealed that TRIP8-EGR2 locus at human chromosome 10q21.3 and 5qNCA-EGR1 locus at human chromosome 5q31 are paralogous regions within human genome. Microsatellite marker D10S1225, associated with Alzheimer's disease, non-syndromic congenital retinal non-attachment (NCRNA) and non-syndromic autosomal recessive persistent hyperplastic primary vitreous (arPHPV), was located within the TRIP8-EGR2 locus. This is the first report on comprehensive characterization of the TRIP8 gene.

ATPases Associated with Diverse Cellular Activitie↗

PubMed2003

[Leukokoria. Diagnosis and treatment].

PURPOSE: Is to evaluate the importance of the imaging tests when the posterior segments of the eye cannot be visualized due the opaque ocular media. MATERIAL AND METHODS: The paper presents 50 patients with unilateral or bilateral leukokoria treated în the University Eye Hospital from Cluj-Napoca. The parameters who had been watched were: the visual acuity, ultrasonography of the eye, and the management of the lesions associated with leukokoria. The ultrasonography was indicated for the diagnosis of the conditions associated with leukokoria, using a transducer of 7.5 MHz. RESULTS: The conditions associated with leukokoria were: intraocular tumors în 8% of cases; congenital cataract în 24% of cases; persistent hyperplastic primary vitreous în 2% of cases; complicate or pathologic cataract în 27% of cases; retinopathy of prematurity în 6% of cases; vitreous opacities în 33% of cases. In 30% of cases ultrasonography of the eye was normal. Echography is also important for the differential diagnosis of secondary retinal detachment. We found exudative retinal detachment în 20% of cases; în 28% of cases there was a tumor retinal detachment, în 44% a tractional retinal detachment, and în 8% a posterior vitreous detachment. The performed management was represented by: enucleation în 12% of cases; EEC în 21% of cases; EEC + sinechiolisis în 11% of cases; sinechiolisis în 2% of cases; vitrectomy în 11% of cases; evisceration în 6% of cases; medical treatment în 20% of cases. CONCLUSIONS: Ultrasonography is an important imaging test for the diagnosis of the conditions associated with leukokoria. Taking into account echographic results the adequate treatment of the eye lesions can be established.

Adult↗

PubMed1992

[A case of invasion of the hyaloid artery into the lens].

A 6-year-old boy with hyaloid artery invasion into the crystalline lens is reported. Both lenses had localized opacities and some brown membranous tissue attached to the posterior lens capsule. He had bilateral persistent hyaloid arteries, and in the right eye this vessel penetrated the lens capsule and invaded the lens. The lens opacity had not increased for 6 years. In persistent hyperplastic primary vitreous we can often see the invasion of fibrovascular tissue into the lens, and this process usually leads to destruction and absorption of the lens. This is an unusual case because the lens showed no significant changes for long period of time in spite of vascular invasion into the lens.

Arteries↗

PubMed1992

Inflammation after cataract surgery in children.

We reviewed the records of 32 consecutive children (43 eyes) who underwent lensectomy/vitrectomy between January 1988 and August 1990 at the Medical Center of the University of California at San Francisco, to study the incidence and characteristics of clinically significant postoperative inflammation (CSPI). No eyes of patients 18 months old or younger (22 eyes) developed CSPI; nine of 21 eyes of older patients did develop CSPI. Other ocular abnormalities (microphthalmos, persistent hyperplastic primary vitreous, retinopathy of prematurity, and Axenfeld's syndrome) and systemic syndromes did not influence the incidence of CSPI. Prolonged inflammation delayed refractive and amblyopia therapy but did not affect final visual acuity. The absence of CSPI in younger patients may be related to the general immaturity of their immune system and a resultant weak inflammatory response, or to their specific immunologic tolerance to lens crystallins.

Betamethasone↗

PubMed2004

Surgical results of persistent fetal vasculature.

BACKGROUND: To evaluate the surgical results of patients with persistent fetal vasculature (PFV), also known as persistent hyperplastic primary vitreous (PHPV). METHODS: From 1991 to 2001, a retrospective, noncomparative study of 7 eyes of 7 patients diagnosed with PFV was conducted. In each case, type of anterior and/or posterior PHPV findings, preoperative testing, surgical procedures, and visual outcomes were obtained from the records. Follow-up ranged from 12 to 43 months, with a mean of 15 months. RESULTS: Of the 7 eyes, one (14%) had strictly anterior PFV, 1 (14%) had strictly posterior PFV, and 5 (71%) had components of both anterior and posterior disease. Initial lens aspiration only was performed in 3 (43%) eyes. Initial vitrectomy only was performed in 1 eye (14%). Initial lensectomy and vitrectomy was performed in 3 (42.8%) eyes. The reoperation rate was 43% for membrane reproliferation, glaucoma, vitreous hemorrhage, and retinal detachment. Final best-corrected visual acuity ranged from light perception only to 20/70 on the Snellen chart. CONCLUSIONS: Functional vision is possible in selected patients. However, poor final visual outcome despite adequate anatomic success were noted in this study. The poor outcomes might have been due to patients delaying vitrectomy until retinal detachment developed or that the patients had poor compliance with postoperative ocular rehabilitation.

Child↗

PubMed1992

Norrie's disease vs. PHPV: one family's dilemma.

This paper presents the numerous similarities between Norrie's disease and bilateral persistent hyperplastic primary vitreous. A description of each disease is given, as well as a case report which demonstrates the difficulty in distinguishing the two disease processes when there is a negative family history of blindness. A discussion section focuses on the need for a better differential protocol for Norrie's disease, which would aid the clinician when a family history of blindness is absent.

Blindness↗

PubMed2006

[LAMB2 gene mutation as a cause of congenital nephrotic syndrome with distinct eye abnormalities and hypotonia].

UNLABELLED: Mutations in the LAMB2 gene encoding laminin beta2, a component of the glomerular basement membrane and the neuro-muscular junction are responsible for the characteristic renal and eye abnormalities of Pierson syndrome. We report a girl with confirmed LAMB2 mutation who presented with early onset Congenital Nephrotic Syndrome (CNS) with renal failure and ocular findings of bilateral microcoria, persistent hyperplastic primary vitreous, right microphtalmia and left eye cataract. Automated peritoneal dialysis was started from the 3rd month of life. Severe muscle hypotonia with motor and mental delay were observed during the first year of life. She experienced numerous serious infections from birth and died at the age of 15 months due to a fulminant infection. Genetic studies revealed two novel mutations in LAMB2 gene (compound heterozygosity). CONCLUSIONS: 1. Mutations in LAMB2 gene should be included in the work-up of patients with CNS in the presence of eye anomalies. 2. Severe phenotypes of Pierson syndrome are associated with marked handicaps and a poor outcome.

Eye Abnormalities↗

PubMed1991

Adipose tissue in the lens.

A case of persistent hyperplastic primary vitreous with replacement of the lens by adipose tissue was observed in a 31-year-old man. A review of the literature revealed only two other cases in which this particular finding was documented.

Adipose Tissue↗

PubMedDate not supplied

Keratometry in children.

Corneal curvature was evaluated in 70 children by central keratometric measurements. The mean keratometric reading in each age group was seen to decline systematically from birth to about 54 months of age. The mean in the newborn-to-6-month-old group was 47.59 D; in the 12-18-month-old group it had decreased to 45.56 D. The cornea appears to stabilize at about 54 months, with an average reading of 42.69 D. Evaluation of 11 eyes diagnosed as having persistent hyperplastic primary vitreous revealed that eyes with this diagnosis generally have steeper corneas than normal eyes at any given age.

Analysis of Variance↗

PubMed1986

Unusual ophthalmological findings in a case of partial trisomy 15.

The clinical and pathological findings in a two-month old girl having partial trisomy 15, are presented. The patient showed bilateral microphthalmia accompanied by a retrolental mass in her left eye. She underwent vitrectomy, lensectomy and accidental retinectomy. During the course of the operation, persistent hyperplastic primary vitreous (PHPV) was found with findings of retinal dysplasia on the pathologic examination.

Chromosomes, Human, Pair 15↗

PubMed1986

Revlens: a new, soft acrylic rubber extended-wear contact lens.

Revlens is a new, soft acrylic rubber extended-wear contact lens. We fitted 21 eyes in 15 patients. The indications included 15 eyes in 10 patients with aphakia due to congenital cataracts. The developmental cataracts were associated with persistent hyperplastic primary vitreous, Down's syndrome, microphthalmos and Peter's anomaly. The remaining five patients included one Tyrosinase negative oculocutaneous albino and four anisometropic amblyopes. Three of the aphakic patients were unable to wear this lens. One preferred her previous rigid lens, another refused any contact lens and went back to glasses, and a third did not adapt to the lens because of social circumstances. Thus 12 of the 15 patients (80%) who were monitored for at least 6 months (average: 9 months) readily accepted this new lens.

Acrylates↗

PubMed1987

Latent/manifest latent nystagmus reversal using an ocular prosthesis. Implications for vision and ocular dominance.

Latent/manifest latent nystagmus (LMLN) is a jerk nystagmus with a decreasing-velocity or linear slow phase whose fast phase is in the direction of the fixating eye. Change of the fixating eye by alternating tropias or cover will cause reversal of the LMLN to preserve this relationship. In the dark, where no fixation is possible, the fast phases of LMLN are in the direction of the intended fixating eye; actual visual input will override this effect of intention. A patient with persistent hyperplastic primary vitreous of the right eye, which was enucleated at age 9 due to glaucoma, had LMLN with fast phases to the left. His congenitally blind right eye was replaced with an ocular prosthesis whose movements were conjugate with his seeing eye. In the dark, we found that his LMLN spontaneously reversed as the normally fixating left eye became esotropic. Furthermore, as is the case with LMLN patients who have sight in both eyes, he was able to willfully reverse his LMLN in the dark by alternating his "fixating" eye. We conclude from these observations that the direction of LMLN is determined at a cortical level, is intimately related to the intended fixating eye and that eye "dominance" is predetermined and not altered by visual abnormalities, including blindness.

Adolescent↗

PubMed1984

Incontinentia pigmenti associated with nasolacrimal duct obstruction.

A case is reported of a five-month-old female with incontinentia pigmenti associated with nasolacrimal duct obstruction. Ocular manifestations of incontinentia pigmenti have previously been described to include persistent hyperplastic primary vitreous, retinal dysplasia, retrolental fibroplasia, corneal opacities, cataract, optic atrophy, and strabismus. This case is believed to be the first reported instance of incontinentia pigmenti associated with nasolacrimal duct obstruction. The management of this patient is also discussed.

Dacryocystorhinostomy↗

PubMed1994

[Late complications and functional results at least 5 years following pars plana lentectomy for congenital cataract].

Pars plana or pars plicata lentectomy was introduced for congenital cataract at the University Eye Clinic of Freiburg 12 years ago. Records of 32 patients (48 eyes) who were operated on during a period of 7 years were retrospectively analyzed to determine the anatomical and functional results in comparison to previous methods of treatment for congenital cataract. The documented follow-up was at least 5 years long (maximum 11 years, mean 7.3 years). Seventeen patients had bilateral, 15 patients monolateral cataract operations. Thirteen eyes were operated on during the first 2 months of life, and 31 of the 48 eyes during the first year. Sixteen of the 48 eyes had additional malformations, like microphthalmus, different stages of persistent hyperplastic primary vitreous, or anterior chamber malformation. Visual acuity was at least 20/50 at the last visit in 3 of 15 eyes after monolateral lentectomy and in 19/33 eyes after bilateral lentectomy. Twenty-four children are now able to attend a regular school; only one child is attending a school for the visual handicapped; another child attends a school for deaf children. Postoperatively, secondary cataract or a retropupillary membrane was found in two eyes. Glaucoma developed in two eyes, requiring additional surgery. Postoperative retinal detachment caused by a giant tear complicated the postoperative course in one case. The anatomic results turned out to be better than after discission and aspiration with fewer postoperative complications like secondary cataract, glaucoma, or retinal detachment. The functional outcome is mainly dependent on good postoperative orthoptic and pleoptic management of the children.

Adolescent↗

PubMed1994

Ectopic expression of gamma interferon in the eyes of transgenic mice induces ocular pathology and MHC class II gene expression.

PURPOSE: To direct the expression of gamma IFN to the eyes of transgenic mice as a means of investigating the possible role of this lymphokine in ocular pathogenesis. METHODS: Transgenic mouse strains were generated by injection of a DNA fragment containing the murine alpha A-crystallin promoter fused to the coding sequence of murine gamma IFN gene. PCR and RT-PCR were used to screen for the presence of the transgene and mRNA analyses, respectively. Methacrylate-embedded eye sections were analyzed for morphology and cryosections for immunoperoxidase antibody staining. RESULTS: The most notable effects of gamma IFN in these transgenic mice include cataract, microphthalmia, blepharophimosis, microphakia, impairment of lens fiber formation, arrest of retinal differentiation, serous retinal detachment with presence of macrophages in the subretinal space, persistent hyperplastic primary vitreous, and corneal vascularization. MHC class II mRNA levels were significantly increased in the transgenic eyes and MHC class II proteins were expressed in their cornea, iris, ciliary body, choroid, lens and RPE. CONCLUSIONS: Ectopic expression of gamma IFN in the lens affected the growth of the whole eye, resulting in microphthalmia and microphakia. The author's data suggest that alpha ACry-gamma IFN transgenic mouse ocular cells express functional gamma IFN receptors and that interaction of gamma IFN with its receptor induced biochemical and morphologic changes in the transgenic eyes. These mice provide an animal model for the study of the linkage between aberrant MHC expression and predisposition to autoimmune diseases.

Animals↗

PubMedDate not supplied

Cytodiagnosis of Coats' disease from an ocular aspirate. A case report.

This paper describes the cytologic findings in a rare condition, Coats' disease, which was diagnosed from a sample of ocular aspirate in a 19-month-old boy. The cytologic findings were characterized by the presence of numerous cholesterol crystals, rare foamy macrophages, several pigment-laden macrophages, pigment-bearing epithelial cells and free pigment, which stained positive for melanin by Schmorl's stain. The case was unusual in that the clinical diagnosis was persistent hyperplastic primary vitreous, and other diagnoses, such as Coats' disease, were unsuspected. The cytologic findings suggested Coats' disease and seemed to exclude retinoblastoma or another malignancy, although the negative findings with the presence of cholesterol crystals and macrophages were regarded as somewhat nonspecific for unequivocally excluding any of the above.

Chronic Disease↗

PubMed1976

[Etiology and final clinical cause for 1000 enucleations. (A clinico-pathologic study) (author's transl)].

From 1969-1974 1000 unselected enucleated globes have been examined histopathologically. 277 derive from the University Eye Hospital in Hamburg, 723 from various Eye Hospitals in northern and southern Germany. They originate from 589 men and 408 women, three times the sex was unknown. 86 globes had to be removed from children less than 15 years old. 6 groups of etiologies have been distinguished: trauma (308), histologically confirmed neoplastic disease (281), ocular manifestations of systemic diseases (diabetes mellitus, occlusions of central retinal vessels presumably following generalized vascular disease etc.: 128), "operative ocular disease" (164), primary inflammatory disease (71), miscellaneous (malformations, high myopia, pseudo-glioma and pseudo-melanoma: 48). The etiology "operative ocular disease" consists of 67 primary glaucomas (57 adults, 10 buphthalmus), 41 idiopathic cataracts (7 of these congenital) and 3 primary corneal dystrophies, as well as 53 cases of primary retinal detachment. Among the 281 neoplastic diseases, there are 238 primary intraocular malignant melanomas of the uvea, 18 retinoblastomas, 4 primary reticulumcellsarcomas of the retina, 2 choroidal nevi, 10 intraocular metastases and 9 orbital tumors. 16 enucleations among the 1000 enucleations have been performed for pseudo-gliomas (5 x Coats disease, 5 x persistent primary hyperplastic vitreous, 2 x retrolental fibroplasia, others 4 x). The manifestations of systemic disease are consisting of 68 central retinal vein-occlusions, 30 complications of diabetes mellitus and 10 central retinal artery occlusions as well as 20 other generalized diseases. A primary inflammatory disease led to enucleation 50 times due to an intraocular process, 5 times due to scleritis and 18 times as a consequence of keratitis (including 13 times herpes simplex). As the final clinical cause for enucleation the following categories have been elaborated: secondary glaucomas (416), clinical diagnosis of "tumor" (275), atrophy and phthisis bulbi (118), inflammation (112), acute trauma to 4 weeks after the accident (72), others (7). In conclusion the central role of rubeosis iridis leading to secondary angle closure glaucoma is emphasized. This process presents a challenge to ophthalmologic research. Finally the significance of early surgery for primary angle closure glaucomas and for complete restoration of the anterior chamber after trauma and any intraocular procedure is stressed.

Cataract Extraction↗