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To document clinical features and subsequent management of pyramidal anterior polar cataracts in children.Retrospective, noncomparative case series and clinicopathologic correlation.Fifteen patients who presented to the pediatric ophthalmology clinic.All patients underwent measurement of visual acuity, assessment of ocular motility, examination of the anterior and posterior segments, and cycloplegic refraction. Amblyopia treatment was instituted when appropriate. When visual impairment occurred from cataract progression or amblyopia or both, cataract removal with or without lens implantation was performed. After surgery, correction of refractive error and treatment of amblyopia were instituted. Several pyramidal opacities were retrieved during cataract extraction and examined by light and electron microscopy.Visual acuity at initial presentation, size of lens opacity before surgery, amblyopia status, most recent visual acuity after cataract extraction, and histologic examination of lens opacity.Nine children had bilateral and six had unilateral pyramidal cataracts (24 eyes). There was no discernible inheritance pattern. Patients were followed for 27 months on average. Twenty of 24 eyes developed cortical opacification that extended significantly beyond the base of the pyramidal lesion. Nineteen eyes required cataract surgery: 10 eyes underwent lensectomy with anterior vitrectomy and 9 had extracapsular cataract extraction, 8 of which had insertion of a posterior chamber intraocular lens. Amblyopia was present or developed in all six patients with unilateral cataract and in eight of nine patients with bilateral cataract. Visual acuity in many eyes remained poor despite amblyopia therapy. The pyramidal opacities consisted of hyperplastic lens epithelium, which exhibited a loss of polarity and was surrounded by a collagenous matrix.Pyramidal anterior polar cataracts are present at birth and may represent a variant of anterior polar lens opacities. They may be unilateral or, if bilateral, they may be either symmetric or asymmetric. They consist of hyperplastic lens epithelium in a collagenous matrix. Patients with pyramidal cataracts are likely to develop amblyopia. This can result from either unilateral occurrence or asymmetry of bilateral opacities and is often worsened by surrounding cortical opacification. Many patients require cataract surgery. All infants and young children with anterior polar opacities showing this configuration should be followed for cataract progression and amblyopia.

The purpose of the study was to assess integration of porous polyethylene allografts in 37 anophthalmic sockets of retinoblastoma enucleations.A clinical review.Thirty-four patients (19 female, 15 male) whose age when diagnosed with retinoblastoma ranged from 1 to 72 months participated. Sixteen patients had unilateral retinoblastoma and 18 had bilateral retinoblastoma.Enucleation (3 bilateral, 31 unilateral) with implantation of a porous polyethylene (PP) sphere (16-20 mm in diameter) was performed. Of the 37 spheres, 34 were primary implants and 3 were secondary implants. In the eight patients with postimplantation exposure, the PP implant had to be removed.Data on eight sockets with exposed PP implant were measured.There were no implant extrusions, but conjunctival dehiscence-exposure occurred in eight patients (21.6%). In 3 of 37 sockets, the volume replacement was not good, and in 5 cases, fornices were too shallow to accommodate a well-fitting prosthesis. In all but one socket, the overall cosmesis was graded subjectively from acceptable (grade 2) to excellent (grade 3).Although the PP implant is a useful and a less-expensive alternative to hydroxyapatite in the porous matrix implant category, if conjunctival exposure takes place, the implant presents a serious management problem because of its nonresponsiveness to medical and surgical treatments.

This study aimed to examine and describe three siblings with alacrima, the eldest of whom had associated achalasia and adrenocortical insufficiency.Three affected siblings and four age-matched control subjects participated. INTERVENTION/MAIN OUTCOME MEASURES: The three children underwent complete ophthalmologic examinations; computed tomographic scanning of brain, orbit, chest, and abdomen; and measurement of serum cortisol. All three were subjected to a short synacthen challenge. Lacrimal gland biopsies were performed on the two younger subjects, and specimens were studied by light and electron microscopy.All three children showed virtually absent tear secretion as tested by the Schirmer test. The resulting keratopathy was most severe in the oldest child, who developed bilateral corneal melting. The two younger children showed interpalpebral corneal staining with rose bengal. All three children improved after punctal occlusion. Addison's disease was present in the oldest child. Computed tomographic scanning showed absent lacrimal and shrunken adrenal glands in association with achalasia of the cardia in the oldest child. The lacrimal glands were found to be reduced in size in the next eldest child. When evaluated by electron microscopy, the lacrimal gland biopsy specimens from the two younger children showed neuronal degeneration associated with depletion of secretory granules in the acinar cells.In this disease, radiologic evidence of reducing lacrimal gland size with increasing age could represent a degenerative process. This may be paralleled by other signs and the possibility of adrenocortical insufficiency and achalasia of the cardia should be investigated in all children presenting with dry eyes. These children appear to have a progressive neuronal disease.

The study aimed to review pediatric intraocular lens (IOL) implantations performed in the authors' institution for the past 7 years.The study design was a retrospective chart review.All children who underwent IOL placement between January 1, 1989, and January 31, 1996, at ages 2 to 16 years were studied. Follow-up was a minimum of 6 months up to 4 years with an average of 13 months.Placement of a primary or secondary posterior chamber IOL was performed.Visual acuity and possible complications were tabulated.Fifty-seven percent of the cataracts were caused by trauma, and 37% were infantile cataracts. Visual acuities of 20/40 or better were attained in 44% of eyes and visual acuities of 20/50 to 20/80 were attained in 27% of eyes. Visual acuities were better in children older than 4 years of age (P = 0.001). Compliance with amblyopia therapy improved vision (P = 0.004). Fibrinous membranes occurred in 41 eyes (13%), and posterior capsule opacification occurred in 120 eyes (39%). There were no significant differences in visual outcome and complications between primary and secondary implants (17%) or between capsular bag and sulcus fixation (27%). The first stable postoperative refraction was predicted accurately in most patients, based on the Sanders-Retzlaff-Kraff II (SRK II) formula and intraoperative corneal curvature and axial length determinations. The mean refraction differed from the goal by only +0.28 diopter and 84% were within +/- 2 diopters. A few patients showed large overcorrections or undercorrections. On average, no myopic shift occurred.Short-term results of IOLs implanted in children older than 2 years of age were favorable. Visual acuities obtained were better than 20/80 in 71% despite a high prevalence (28%) of penetrating trauma. Major complications were rare.

The means available to screen for retinoblastoma metastases, including bone marrow aspiration, lumbar puncture, and radionuclide scans, offer variable usefulness at different stages of the disease. In this study, the authors attempted to assess the value of these tests as part of the initial workup for metastases.Medical files of 261 patients with retinoblastoma were reviewed, and the results of bone marrow, lumbar puncture, and bone and liver radionuclide scans were correlated with the laterality, clinical staging, and histopathologic findings. The presence or absence of tumor in the choroid and the optic nerve also were correlated with the results of diagnostic tests.Of 261 patients with retinoblastoma, 147 (56.3%) and 114 (43.7%) had unilateral and bilateral disease, respectively; 11.6% of unilateral cases and 14.9% of bilateral cases had distant metastasis (P = 0.2). Bone marrow aspirations were performed on 101 patients (38.7%), and 10 (9.9%), of these specimens showed the presence of retinoblastoma cells in the aspirate. Ninety-four (36%) of the patients were examined with spinal tap cytology, and the results of four (4.3%) of these were positive for tumor cells. Radionuclide bone and liver scans were performed on 49 (18.8%) and 48 (18.4%) patients, respectively; 5 (10.2%) had abnormal bone scans and 3 (6.2%) had abnormal liver scans. The average age in the metastatic group was 3.1 years, whereas the average age in the nonmetastatic group was 2.3 years. All of our abnormal test results were found in patients with stages III and IV disease, with the exception of one abnormal lumbar puncture in a patient with stage II disease. The correlation of diagnostic tests with the histopathologic findings showed that with no choroidal involvement, no positive diagnostic tests were encountered. Correlation between positive diagnostic test results and the level of optic nerve involvement failed to indicate any trends.Our study indicated a good interrelation between the positivity of diagnostic tests (bone marrow and bone scan) and higher stages of the disease (stages III and IV) and choroidal involvement in enucleated eyes, with statistically significant correlations; correlations with lumbar puncture and liver scan were not significant.

Large choristomas that straddle the cornea and limbus can be removed and a lamellar graft sutured to the excision site. A central penetrating keratoplasty performed later can restore a clear visual axis.