Diagnosis and management of primary congenital lens cyst.

We report the case of a 2-year-old girl with an unusual unilateral congenital cystic lesion of the crystalline lens in the right eye. We describe the clinical features, ultrasound biomicroscopy, hypothesis for the possible origin of the cyst, and management of this rare and previously undescribed developmental anomaly.

Pupillary-iris-lens membrane with goniodysgenesis: a case report.

We describe a rare case of pupillary-iris-lens membrane with goniodysgenesis, a unilateral neurocristopathy. The membrane represents ectopic iris on the lens with abnormal iris stroma and anterior chamber angle from aberrant induction, migration or regression of neural crest cells. The membrane can be progressive. Catastrophic vision loss from angle closure can occur and may be controlled with surgery. This subject needed treatment for amblyopia.

Congenital pupillary-iris-lens membrane with goniodysgenesis.

BACKGROUND: A unilateral congenital pupil-iris-lens membrane with goniodysgenesis syndrome, not benign tunica vasculosa lentis, was first described by Cibis et al. One of three cases developed angle closure. Robb described catastrophic vision loss from angle closure in one of his seven cases. METHODS: We did a retrospective review of previously unreported cases of pupil-iris-lens membrane with goniodysgenesis seen in our practices. RESULTS: We report the clinical spectrum of a further nine cases, three of which needed surgery for angle closure, two of which needed surgery for clearing the visual axis. CONCLUSION: Congenital pupil-iris-lens membrane with goniodysgenesis is a unilateral membrane clearly differentiated from benign persistent tunica vasculosa lentis tissue. The membrane represents ectopic iris on the lens with abnormal iris stroma and chamber angle from aberrant induction, migration, or regression of neural crest cells. The membrane can be progressive. Catastrophic vision loss from angle closure can occur and may be controlled with surgery. Surgery may be needed to open the visual axis even when glaucoma is not present and may prevent angle closure.

Goniosynechialysis with lens aspiration and posterior chamber intraocular lens implantation for glaucoma in spherophakia.

A 17-year-old girl presented with bilateral angle-closure glaucoma associated with spherophakia. A previous bilateral laser iridotomy failed to control intraocular pressure (IOP). Goniosynechialysis with lens aspiration and posterior chamber intraocular lens implantation were performed in both eyes. Peripheral iridoplasty was performed 3 days later. The postoperative IOP was controlled without medication for 12 months in the right eye and 24 months in the left eye. By restructuring the physiologic aqueous outflow route, goniosynechialysis safely and effectively treated secondary glaucoma from spherophakia.

Cornea with Peters' anomaly: perturbed differentiation of corneal cells and abnormal extracellular matrix in the corneal stroma.

PURPOSE: We examined histopathologically the anterior ocular segment including the cornea and lens of an eye which had been enucleated in a patient with Peters' anomaly because of untreatable corneal perforation. Special effort was made to differentiate the corneal stromal and endothelial cells, and the stromal extracellular matrix. METHODS: Light microscopy, with hematoxylin and eosin staining, and transmission electron microscopy were employed. RESULTS: Corneal endothelial cells and Descemet's membrane were not detected in the central cornea, where there were immature cells with a fibroblastic configuration. The inner surface of the peripheral cornea was covered with cells containing pigment granules in the cytoplasm. Cell density in the central corneal stroma was relatively high. The diameter of the stromal collagen fibrils was not uniform. A mature collagen fibril-free area was also seen in the central corneal stroma. CONCLUSIONS: Differentiation of neural crest-derived cells in corneal stroma and endothelium might have been perturbed in the cornea of this patient with Peters' anomaly, inducing the defect in the corneal endothelium and the qualitative and quantitative abnormalities of the extracellular matrix.

Congenital double crystalline lenses.

We report an unusual case of an eye with 2 congenital crystalline lenses; ie, a duplicated lens. The horizontal axis of the eye was obliquely placed. One lens was situated in the upper temporal quadrant and the other, in the lower nasal quadrant. One lens was larger than the other.

High levels of binocular function are achievable after removal of monocular cataracts in children before 8 years of age.

OBJECTIVE: To investigate the visual acuity and binocular function results achieved in children who had monocular cataracts removed before 8 years of age. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Clinical records of 171 patients who underwent a unilateral cataract removal between January 1986 and 1996 were reviewed retrospectively. Seventy-four eyes were included in the study: 19 congenital, 11 developmental, 19 posterior lenticonus, 19 traumatic, and 6 complicated cataracts. Patients with less than 2 years of follow-up; eyes with cataracts resulting from retinoblastoma; prematurity; and those associated with dense corneal scars, lens dislocation, and persistent hyperplastic primary vitreous were excluded. INTERVENTION: Visual acuity was measured by means of age-appropriate tests such as the fixation pattern, Allen object recognition cards, isolated optotypes with the Sheridan Gardiner test, and Snellen letters. Sensory fusion was assessed with the Worth 4-dot test, and stereo acuity was assessed with the Titmus stereo test. MAIN OUTCOME MEASURES: Cataracts were classified regarding type, extent, age at onset, duration of the opacity, age at surgery, method of removal, development of secondary membrane, form of optical rehabilitation, and presence of strabismus. Visual acuity levels between 6/6 and 6/12 were considered "good." Fusion of the Worth 4-dot test at distance and near, and presence of stereo acuity of 100 seconds of arc or better were considered "good" binocular function. Multiple logistic regression analysis was used to define factors that correlated with achieving good visual outcome. RESULTS: Visual acuity was 6/12 or better in 27 (36.5%) eyes. However, good binocular function was achieved in only 11 of these 27 patients. Results of univariate analysis showed that later age at onset of cataract and absence of strabismus were significant for good visual acuity and binocular function. The presence of strabismus increases the risk of not achieving good visual acuity by 5.45-fold. CONCLUSIONS: Good visual acuity and binocular function can be achieved after removal of monocular cataracts in visually immature children. Patients with strabismus at presentation or during the follow-up period have the least chance of achieving a good sensory result.

Surgical removal of congenital pupillary-iris-lens membrane.

A pupillary membrane in a case of congenital pupillary-iris-lens membrane with goniodysgenesis was surgically peeled from the lens without causing cataract formation. Histopathology revealed ectopic iris. The ectopic iris found in this condition differentiates congenital pupillary-iris-lens membrane with goniodysgenesis as an entity from persistent pupillary membrane, hereditary goniodysgenesis, and Rieger's anomaly. We suggest that congenital pupillary-iris-lens membrane with goniodysgenesis is a neurocristopathy. The finding of ectopic iris muscle is consistent with avian chimera experiments that have suggested that iris sphincter muscle is derived from the neural crest, not neural ectoderm. Membranes in this condition can be successfully removed when they cause vision loss and amblyopia.

Microspherophakia in association with the rhizomelic form of chondrodysplasia punctata.

Chondrodysplasia punctata is a multisystem disorder, primarily involving the musculoskeletal system, skin, and eyes. Children with the rhizomelic form of this syndrome have characteristic facies with a saddle-nose deformity, hypertelorism, and frontal bossing. The musculoskeletal changes include proximal limb shortening, flexion contractures, congenital hip dislocations, and the characteristic radiographic finding of epiphyseal stippling of the axial skeleton and long bones. The most common ocular findings are cataracts and optic atrophy. We describe herein the first reported case of microspherophakia in association with the rhizomelic form of chondrodysplasia punctata.