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Results for “Persistent Hyperplastic Primary Vitreous”

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PubMed2003

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 aged 40 or younger with angle closure in our database. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Our database was searched for patients aged 40 years or younger with angle closure. Data recorded included age at initial consultation, age at the time of diagnosis, gender, slit-lamp examination, gonioscopy, ultrasound biomicroscopy (from 1993 onward), clinical diagnosis, and therapy. Patients with prior 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 at the time of consultation was 34.4 +/- 9.4 (standard deviation) 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. CONCLUSIONS: The etiology of angle closure in young individuals differs from the older population and is typically associated with structural/developmental ocular anomalies rather than relative pupillary block. After laser iridotomy, these eyes should be monitored for recurrent angle closure and the need for additional laser or incisional surgical intervention.

Adolescent↗

PubMed1979

Management of congenital cataracts.

The management of congenital cataracts at the Wilmer Institute is presented, with emphasis on the need for careful preoperative assessment. The indications and techniques for surgery in uncomplicated cataracts are discussed, and the advantages of using vitrectomy instruments in complicated congenital cataracts, such as those associated with persistent hyperplastic primary vitreous, are described.

Cataract↗

PubMed1989

Magnetic resonance imaging versus computed tomography of leukocoric eyes and use of in vitro proton magnetic resonance spectroscopy of retinoblastoma.

To evaluate the usefulness of magnetic resonance imaging (MRI) in the evaluation of leukocoric eyes, the authors studied 28 patients with either leukocoria or intraocular mass with a 1.5-tesla (T) MRI imager. Retinoblastomas were reliably distinguished from Coats' disease, toxocariasis, and persistent hyperplastic primary vitreous on the basis of MRI findings. Calcification cannot be reliably detected on MRI scans. Lesions elevated less than 4 mm may not be detected reliably by MRI at this time. Computed tomography (CT) can detect calcification with a high degree of accuracy. Retinoblastomas appeared as moderately hyperintense masses on T1- and proton-weighted MRIs. They became hypointense in T2-weighted MRIs. This MRI characteristic is similar to that of uveal melanoma. Intraocular calcification in children especially younger than 3 years of age is highly suggestive of retinoblastoma. In the diagnosis of retinoblastoma, MRI is not as specific as CT because of its lack of sensitivity in detecting calcification. However, MRI, because of its superior contrast resolution, offers more information in the differentiation of pathologic intraocular conditions responsible for leukocoria. The authors also describe their preliminary work of in vitro proton magnetic resonance spectroscopy of eyes with retinoblastoma and an eye with uveal melanoma in an 18-year-old black woman.

Adolescent↗

PubMed1995

The optokinetic response differences between congenital profound and nonprofound unilateral visual deprivation.

BACKGROUND: The occurrence of monocular naso-to-temporal optokinetic nystagmus (OKN) asymmetry, as a reflection of the immature oculomotor system in infants, and its persistence with early onset monocular visual deprivation, is well known. This asymmetry has been linked with poor binocular function and attributed to disruption of the development of binocular cortical projections to the pretectum. Optokinetic nystagmus symmetry in patients with congenital uniocular total pattern vision deprivation has not been fully investigated. METHODS: The authors compared the optokinetic responses in six children with "profound" uniocular visual deprivation, who were born with untreated conditions (microphthalmos and persistent hyperplastic primary vitreous), with ten aphakic children treated early for congenital unilateral cataracts (nonprofound unilateral visual deprivation). Eye movements were recorded using dc-electro-oculography, and OKN was elicited using a full-field patterned curtain. Flash and pattern visual-evoked responses were also measured in each subject. RESULTS: Latent nystagmus was present in six children in the nonprofound group, whereas none was detected in the profound group. All children in the nonprofound group showed statistically significant monocular naso-to-temporal asymmetry for either eye. Subjects in the profound group had symmetric OKN. CONCLUSIONS: The authors conclude that unequal input from the two eyes and interocular rivalry lead to OKN asymmetry. Their results suggest that if vision from one eye is so negligible that it does not compete with the neuroanatomic connections of the fellow eye, then the input from this eye remains undisturbed, and OKN remains symmetric.

Adolescent↗

PubMed1995

Efficacy of unanesthetized spiral computed tomography scanning in initial evaluation of childhood leukocoria.

PURPOSE: To evaluate the use of spiral/helical computed tomography (CT) in the preoperative evaluation of pediatric leukocoria. METHODS: A total of 34 patient charts and imaging studies were reviewed from a consecutive series of children referred with the diagnosis of presumed retinoblastoma. Of these patients, 31 had a confirmed diagnosis of retinoblastoma. Three patients had simulating lesions, including one patient with persistent hyperplastic primary vitreous and two with Coats disease. RESULTS: In all patients, a diagnosis of intraocular retinoblastoma could be established or excluded at the same clinical level by spiral CT as by conventional CT. Spiral CT of the eye, orbit, and midline structures was performed without a requirement for patient anesthesia. This technique resulted in a reduced volume of contrast material required for evaluation of the central nervous system and a small decrease in total radiation exposure. There was an attendant reduction in the amount of monitoring equipment required and the necessity for attending anesthesia staff. CONCLUSIONS: The primary advantage of spiral CT is reduced anesthesia risk in small children. In addition, this technique provides necessary clinical information with a reduction in image acquisition time, monitoring equipment, and monitoring staff.

Anesthesia↗

PubMed2001

Visual outcomes and complications of posterior chamber intraocular lens implantation in the first year of life.

PURPOSE: To document the visual outcome and postoperative complications in infants who had congenital cataract surgery with posterior chamber intraocular lens (PC IOL) implantation in the first year of life. SETTING: The Children's Hospital, Dublin, Ireland. METHODS: Twenty-seven eyes of 20 infants were reviewed. Seven infants (14 eyes) had bilateral congenital cataract and 13 (13 eyes), uniocular cataract. The mean age at surgery was 4 months (range 3 weeks to 11 months). A standard surgical technique involved anterior capsulorhexis, phacoemulsification with or without posterior capsulorhexis with in-the-bag PC IOL implantation, and no anterior vitrectomy. Surgery was performed by 1 surgeon. The mean follow-up was 41 months (range 6 to 88 months). RESULTS: The main complication was lens reproliferation into the visual axis. Of the 11 eyes that did not have a primary posterior capsulorhexis, 10 had 1 or more capsulotomies. Seven required a neodymium:YAG (Nd:YAG) laser capsulotomy a mean of 6 months postoperatively, and 2 had 2 Nd:YAG capsulotomies. Six eyes also had a surgical capsulotomy when the membrane was deemed too thick for further laser treatment. Fourteen of 25 eyes had a primary posterior capsulorhexis; 8 had no further intervention. Four eyes had persistent hyperplastic primary vitreous (PHPV), 3 required a surgical capsulotomy, 2 had an Nd:YAG laser capsulotomy, 2 had an anterior vitrectomy, and 1 developed open-angle glaucoma. There was a mean refractive shift of 6.0 diopters after a mean follow-up of 41 months, with most of the myopic shift occurring in the first 24 months. CONCLUSIONS: Visual axis reopacification was the main complication of IOL implantation in infants, with PHPV leading to more complications and repeat procedures. Anterior vitrectomy appeared to reduce the reoperation rate. Results indicate that primary posterior capsulorhexis is important and Nd:YAG capsulotomy is not satisfactory in infants. In addition, the reduction in glaucoma with IOL implantation, if borne out over the long term, is a significant advantage in cases of congenital cataract.

Capsulorhexis↗

PubMed1991

Pediatric cataract surgery and intraocular lens implantation: a new technique for preventing or excising postoperative secondary membranes.

This report describes a series of six children who had a retropseudophakic vitrectomy performed via a limbal approach (limbal approach retropseudophakic vitrectomy, LARV). This technique ensures a permanently clear visual axis when performed at the time of cataract/implant surgery and can also be used to excise secondary membranes in patients who have had prior cataract extraction and intraocular lens implantation. A modification of this method permits the retrolenticular opacity in children with persistent hyperplastic primary vitreous (PHPV) to be relocated or excised at the time of cataract or cataract/implant surgery.

Cataract Extraction↗

PubMed2003

Hydroxyapatite orbital implant exposure in children.

PURPOSE: Hydroxyapatite orbital implants are buried implants that may be integrated into the overlying prosthesis after enucleation. We report problems encountered using these implants during a 14-year period in a pediatric population. METHODS: Retrospective analysis of a pediatric population from 1987 through 2001. RESULTS: Indications for enucleation (N = 19) included retinoblastoma (n =17), persistent hyperplastic primary vitreous (n =1), and painful blind eye (n =1). Conjunctival erosion (36.84%) and consequent implant exposure (15.70%) were the main problems identified in this study. There were no cases of orbital infection. Management included resuturing and scleral patching. CONCLUSION: Conjunctival erosion of hydroxyapatite implants contributed to significant morbidity in 19 children who underwent enucleation and hydroxyapatite orbital implant.

Child, Preschool↗

PubMed1997

Baerveldt implant surgery in the treatment of advanced childhood glaucoma.

BACKGROUND: The efficacy of Baerveldt implant (Pharmacia & Upjohn, Inc., Kalamazoo, Mich.) surgery in the treatment of advanced childhood glaucoma is unknown. METHODS: We reviewed the results of 23 consecutive 350 mm Baerveldt implants in 20 eyes of 17 children. Results were classified as follows: (1) success; no further reoperation, no decrease in vision, and intraocular pressure at last follow-up less than 21 mm Hg with no medications; (2) qualified success; medication necessary to bring intraocular pressure to less than 21 mm Hg or complication not associated with tube failure; and (3) failure; intraocular pressure >20 mm Hg, tube failure complication or reoperation causing tube removal, phthisis, or enucleation. RESULTS: Original glaucoma types were bilateral aphakic (five), unilateral aphakic or persistent hyperplastic primary vitreous (four), primary infantile (four), juvenile (three), secondary(two), Peter syndrome (one), and Lowe syndrome (one). Patients had undergone a mean of 2.8 previous intraocular procedures. Mean preoperative intraocular pressure was 33.6 mm Hg; average number of preoperative glaucoma medications was 3.0. Mean follow-up was 19 months (range, 6 to 32 months). Eight procedures were considered successful (mean intraocular pressure 15.5 mm Hg), six were qualified successes (mean number of medications 0.8; mean intraocular pressure 16 mm Hg), and nine failed. Two eyes in the qualified success group do not have useful vision as a result of complications. Complications included retinal detachment (five), corneal decompensation (five), corneal graft rejection in five of six grafts; dislocated tubes (three), and recurrent uveitis (two). One of these eyes is phthisic and one has been enucleated. Only two of nine procedures in eyes with a history of one or no previous intraocular operations failed,whereas seven of 13 procedures in eyes with a history of three or more previous procedures failed. Only seven of 13 procedures in aphakic eyes were successes or qualified successes, whereas seven of 10 procedures in phakic eyes had good results. CONCLUSION: Baerveldt implants can produce good short-term results, especially in phakic eyes. Aphakic eyes and eyes that have undergone multiple procedures are at a much higher risk for devastating complications.

Adolescent↗

PubMed1999

The epidemiology of pediatric glaucoma: the Toronto experience.

BACKGROUND: This study was conceived to provide an insight into the spectrum of glaucoma in the pediatric population. We also set out to compare the success of disease control and the prognosis for vision within the different diagnostic subgroups. This is the largest single population of children with glaucoma that has been so described and compared. METHODS: The charts of children who were first seen between birth and age 16 years and who attended the Hospital for Sick Children with any form of glaucoma between January 1974 and January 1995 were reviewed and entered into the study. RESULTS: Data are presented for 306 children. Congenital glaucoma was the most common subtype, accounting for 38%. Patients with congenital glaucoma were young, had surgery, and had more operations than any other group except those with aniridia. Goniotomy offered a cure in 47.8% of the patients. A bimodal distribution reflected their visual performance. Patients with aphakic glaucoma, the next most prevalent group (20%), presented at an older age (4.5 years). Surgical intervention was performed in 50% of these children. Nearly all patients with Sturge-Weber syndrome (80%) had surgery. The following glaucoma groups were associated with a poor visual outcome: aniridia, anterior segment developmental anomalies involving the cornea, uveitis with glaucoma other than steroid induced, retinopathy of prematurity, and persistent hyperplastic primary vitreous. Steroid-induced glaucoma and anterior segment dysgenesis, excluding Peters anomaly, had uniformly good outcomes. CONCLUSION: The ability to control glaucoma in childhood and visual prognosis is highly variable. Particular diagnostic categories do consistently well and some do poorly.

Adolescent↗

PubMed1999

Stereopsis and binocular vision after surgery for unilateral infantile cataract.

PURPOSE: To assess the prevalence and level of binocular function in children with unilateral congenital or very early infantile cataract. METHODS: We retrospectively reviewed the charts of all patients with unilateral congenital or very early infantile cataract who underwent operation before 4 months of age, at the W. K. Kellogg Eye Center/University of Michigan Hospitals, from 1985 to 1995. Amblyopia was treated with a reduced patching schedule consisting of 1 hour per day per month of age for the first 6 months of life, in an attempt to improve binocular function by allowing more hours of binocular interaction during the presumed critical period for development of binocular cortical pathways. RESULTS: Thirteen patients met the inclusion criteria. Seven patients had persistent hyperplastic primary vitreous (PHPV) cataract and 6 had non-PHPV cataract. Overall, visual acuity of 20/80 or better developed in 69% of patients; 100% of eyes with non-PHPV cataract achieved visual acuity of 20/60 or better. Stereopsis of 400 arc seconds or better was detectable in 62% of patients, including 3 with PHPV cataract and 3 who required strabismus surgery in the first year of life. Three children had better than 150 arc seconds of stereopsis. The incidence of large-angle strabismus was 54%. CONCLUSIONS: Binocular cooperation, including gross and fine stereopsis, can develop in children with unilateral aphakia as a result of early removal of infantile cataracts. A less-strenuous patching schedule than has been historically advocated may foster this process, while restoring and maintaining good central visual acuity in patients with excellent compliance with contact lens and occlusion regimens.

Amblyopia↗

PubMed2006

[Leucocoria in childhood].

BACKGROUND: There are a number of conditions in infants and young children which produce a white reflex in the pupillary area, termed leucocoria. In cases of leucocoria, retinoblastoma has to be excluded without delay. METHODS: We analysed data from consecutive children who were presented to our hospital because of leucocoria between January 1999 and June 2005. The analysis was performed by methods of descriptive statistics. RESULTS: Leucocoria was found in 83 eyes of 58 children. Leucocoria was caused in most cases by congenital cataract (35 %), followed by malformations (18 %) (persistent hyperplastic primary vitreous, coloboma, disc anomaly, combined developmental abnormalities), hereditary vitreoretinal disorders (13 %), retinopathy of prematurity (12 %), trauma-associated diseases (8 %). Five children suffered from retinoblastoma (6 %). Following in frequency were inflammatory disorders (5 %) and Coats' disease (2 %). DISCUSSION: Several ocular conditions of childhood can clinically cause leucocoria. Knowledge of the clinical features that serve to differentiate retinoblastoma from simulating lesions may assist the clinician in arriving at the correct diagnosis.

Cataract↗

PubMed1999

[Leukokoria in a child: emergency and challenge].

PURPOSE: To heighten the awareness of the medical world to the importance of correct and rapid diagnosis in the presence of leukocoria in the child. METHODS: Starting with the presenting symptom, the authors present the guide lines to follow in a practical manner in order to reach a diagnosis in the principal retinal diseases causing leukocoria. RESULTS: A white pupil is due to retinoblastoma in almost half of all cases. Other possible causes, in order of frequency, are: persistent hyperplastic primary vitreous, Coats' disease, ocular toxocariasis, retinopathy of prematurity, retinal hamartomas. Diagnosis can usually readily be made by ophthalmoscopy, but may be problematic when the clinical presentation is atypical or in the presence of late complications. Age, sex, laterality, heredity, and in particular the presence or absence of calcifications and the size of the globe, are the main criteria for diagnosis. Ultrasonography plays a major role in this essential quest for correct diagnosis, quest which may ultimately lead to enucleation. CONCLUSION: Leukocoria in the child is a danger signal demanding certain diagnosis within the shortest possible time.

Child↗

PubMed1994

[Surgical results of uni- and bilateral congenital and traumatic cataract in infancy to adolescence].

PURPOSE: In a retrospective study the question to be answered is if and at what time unilateral and/or bilateral congenital cataracts have to undergo surgical treatment and from what age of life lens implantation, also after traumatic cataract, is effective. MATERIAL AND METHODS: The inquiry includes 75 patients who were treated by lensectomy at the Department of Ophthalmology, University of Zürich, before the end of the 20th year of their life in the period of 1985-1990. Of the test group, 26 patients had congenital cataracts (14 unilateral and 12 bilateral), 30 patients had traumatic cataracts and 19 patients had developmental cataracts of various etiology. RESULTS: Congenital cataract. Patients with bilateral cataracts had better visual results than those with unilateral cataracts. The amblyopic risk is relatively small. For infants, being children under the age of one year, the test group until 1990 is not large enough to declare a valid test group. Depending on various indications of surgery, in cases with monocular lens opacity a higher visual acuity is reached if surgery is performed between the 5th and the 15th year (acuity 0.4-1.0) versus before the 5th year (acuity 0.4). Cases with a posterior chamber lens implant have better visual results than those without. Cataracta varia (cataract after birth). Unilateral and bilateral surgical procedures are always justified, where possible with an intraocular lens (IOL). Traumatic cataract. Successful results occur, as soon as an IOL can be implanted. CONCLUSION: Unilateral congenital cataracts have to be surgically treated. In individual cases, astonishing results are possible, particularly in cases with persistent hyperplastic primary vitreous (PHPV). The extent and type of the lenticular opacity determines the indication and the timeframe of the surgical procedures. Knowledge about the growth of the eye-ball, experiences to date and the easing of rehabilitation by implantation of a posterior chamber lens, justify its application after the age of 18 months.

Cataract↗

PubMed1993

[Excentric lentiglobus posterior].

BACKGROUND: The posterior lentiglobe is a rare anomaly of the lens shape. Two cases of excentric protuberance of the posterior capsule are presented. To our knowledge, only one description of a similar case exists so far. PATIENTS: An eight-year-old girl (diagnosis by routine examination, V.A. 20/20) and a six-year-old boy (V. A. 20/200, esotropia) are presented. Apart from a persistent hyperplastic primary vitreous and a posterior pole cataract a vitreous cyst adherent to the posterior surface of the lens has to be considered as differential diagnosis. The etiology remains unclear. CONCLUSION: In excentric posterior lengtiglobe a good vision is possible. However, if visual acuity is impaired by posterior pole opacity or distortion of the spherical surface, removal of the lens followed by optical correction (contact lens, intraocular lens) of aphakia should be performed.

Cataract Extraction↗

PubMed2003

Mutations of the PAX6 gene detected in patients with a variety of optic-nerve malformations.

The PAX6 gene is involved in ocular morphogenesis and is expressed in the developing central nervous system and numerous ocular tissues during development. PAX6 mutations have been detected in various ocular anomalies, including aniridia, Peters anomaly, corneal dystrophy, congenital cataracts, and foveal hypoplasia. However, it has not been identified in patients with optic-nerve malformations. Here, we identified novel mutations in eight pedigrees with optic-nerve malformations, including coloboma, morning glory disc anomaly, optic-nerve hypoplasia/aplasia, and persistent hyperplastic primary vitreous. A functional assay demonstrated that each mutation decreased the transcriptional activation potential of PAX6 through the paired DNA-binding domain. PAX6 and PAX2 are each thought to downregulate the expression of the other. Four of the detected mutations affected PAX6-mediated transcriptional repression of the PAX2 promoter in a reporter assay. Because PAX2 gene mutations were detected in papillorenal syndrome, alternation of PAX2 function by PAX6 mutations may affect phenotypic manifestations of optic-nerve malformations.

Adult↗

PubMed1981

Diagnostic vitrectomy.

The histopathologic and cytopathologic study of ocular fluids obtained for diagnostic purposes is presented. The analysis of fluid specimens with millipore filter, celloidin bag-cell block, and transmission electron microscopy techniques has proved to be effective for establishing and confirming clinical diagnoses. Diagnostic vitrectomy can be readily used to evaluate such conditions as intraocular tumors, lens and blood-induced glaucoma, inflammatory conditions, amyloidosis, persistent hyperplastic primary vitreous (PHPV), epithelial ingrowth, and tractional vitreoretinal membranes.

Adult↗

PubMed2002

A male with polysyndactyly, linear skin defects and sclerocornea. Goltz syndrome versus MIDAS.

Focal dermal hypoplasia (FDH) or Goltz syndrome is a rare clinical syndrome presenting with cutaneous, skeletal, dental, ocular, central nervous system and soft-tissue defects. We report on a male infant with characteristic skin defects of the face, trunk and extremities, polysyndactyly and unusual ocular and brain findings. He had sclerocornea of the right eye, anterior persistent hyperplastic primary vitreous of the left eye and hydrocephalus. Clinical findings support the diagnosis of Goltz syndrome. The clinical picture of Goltz syndrome is compared with that of MIDAS syndrome.

Abnormalities, Multiple↗