Anomalous relation between axial length and retinal thickness in amblyopic children.

PURPOSE: To investigate the relationship between retinal thickness and axial length in amblyopic eyes compared to healthy eyes. METHODS: In this observational, transversal study, 36 amblyopic children and 30 healthy controls underwent full ophthalmological and orthoptic examinations, volume scanning of the macula with spectral domain optical coherence tomography (3D OCT-1000; Topcon Corporation, Tokyo, Japan), and measuring of axial length using the IOLMaster (Carl Zeiss Meditec AG, Jena, Germany). The average pericentral retinal thickness was calculated. RESULTS: A strong correlation was observed between the axial lengths of both eyes in the control group (R = 0.98, P < 0.01) and between the axial lengths of the amblyopic and fellow eye in the amblyopic group (R = 0.77, P < 0.01); the amblyopic and their fellow eyes were significantly shorter than the nonamblyopic control eyes. The pericentral retinal thickness of both eyes of an individual is highly correlated in nonamblyopic controls (R = 0.92, P < 0.01) and in amblyopic children (R = 0.82, P < 0.01). There is no significant difference in mean pericentral retinal thickness between healthy, amblyopic, and fellow eyes. In healthy eyes a moderate inverse correlation exists between axial length and pericentral retinal thickness (R = -0.41, P = 0.02); this relationship was not found in the amblyopic eyes or the normal fellow eye. CONCLUSIONS: In this patient cohort, there was an anomalous relation between the axial length and the pericentral retinal thickness in both amblyopic and their fellow eyes.

Long-term follow-up after bilateral Artisan aphakia intraocular lens implantation in two children with Marfan syndrome.

We present two patients with Marfan syndrome and bilateral crystalline lens dislocation who underwent bilateral lens extraction and Artisan aphakia intraocular lens (IOL) implantation. With a follow-up of more than 12 years, we found a good visual outcome, no serious IOL-related complications, and endothelial cell densities within the expected range for eyes without cataract surgery.

Ocular straylight in albinism.

PURPOSE: Albinism is an inherited disorder that affects the melanin biosynthesis pathway, which results in reduced or absent pigment formation. This may lead to increased light transmission through the iris and more reflected light from the fundus. Both these effects contribute to the occurrence of ocular straylight. One aim of this study is to determine whether and how increased iris transmission and fundus reflection in subjects with albinism contributes to the occurrence of ocular straylight. The other aim is to determine the effect that an iris print-contact lens (CL) could have in terms of reducing the occurrence of ocular straylight. METHODS: Ocular straylight was quantified by means of the straylight parameter s and measured as a function of angle and wavelength in 17 subjects with different types of albinism, none of whom wore an iris print-CL. The measurements were then repeated with the subjects wearing an iris print-CL to reduce the iris transmission component and thus the occurrence of ocular straylight. The contributions of transmission and reflectance components were estimated for each individual. RESULT: Straylight level increase varied from normal (s ≈9) to severe (8x). In 15 cases, the reflectance component contributed s >3 to up to s = 17. In eight cases, the transmission component contributed s >3 to up to s = 101. A significant reduction in straylight was observed using an iris print-CL in six subjects with elevated straylight values. In the other 11 subjects with albinism, the iris print-CL had no significant effect on straylight because of the low values of the transmission component. CONCLUSIONS: This study gives insight into the effects of transmission and reflectance on the total measured straylight occurrence in subjects with albinism. Subjects experiencing increased ocular straylight values may benefit significantly from wearing iris print-CLs because transmission of light through the natural iris may cause a significant increase in straylight.

Long-term follow-up of the corneal endothelium after aphakic iris-fixated IOL implantation for bilateral cataract in children.

PURPOSE: To evaluate the long-term corneal endothelial cell density (ECD) and outcomes after iris-fixated aphakic intraocular lens (IOL) implantation in children after bilateral congenital or juvenile cataract extraction and to compare the outcomes with data in the literature. SETTING: Academic Medical Centre, Amsterdam, The Netherlands. DESIGN: Case series. METHODS: This retrospective study evaluated the charts and endothelial photographs of children who had Artisan aphakic IOL implantation. The main outcome measure was the ECD at the last follow-up visit. RESULTS: The mean age of the 10 patients (20 eyes) at IOL implantation was 7.4 years (range 4.3 to 11.1 years) and at the last follow-up, 19.6 years (range 14.3 to 26.6 years). After a mean follow-up of 12.3 years (range 10.0 to 15.6 years), the mean ECD was 2702 cells/mm(2) (range 1382 to 3974 cells/mm(2)). Although this is comparable to the mean normal endothelial cell counts in this age group reported in the literature, a wider range of ECD was found in the current study. CONCLUSIONS: The mean corneal ECD after more then 10 years of follow-up was comparable to the mean normal ECD for this age group reported in the literature. The high standard deviation of the mean ECD in the current study highlights the importance of prospective studies on the ECD after iris-fixated aphakic IOL implantation in young patients.

Implantation of the Artisan iris reconstruction intraocular lens in 5 children with aphakia and partial aniridia caused by perforating ocular trauma.

PURPOSE: To describe the long-term clinical results of the implantation of the Artisan iris reconstruction IOL in 5 eyes of 5 children for aphakia and partial aniridia attributable to penetrating ocular trauma. METHODS: The charts of 5 children were retrospectively reviewed. The nature and the extent of injury; age at IOL implantation; visual, refractive, and cosmetic outcome; endothelial cell density; and complications and subsequent surgical interventions were evaluated. RESULTS: Mean follow-up period was 8.9 years (range, 4.9-12.4). Mean age at implantation of the Artisan iris reconstruction IOL was 9.5 years (range, 7.7-12.7). Visual acuity improved in 2 of 5 eyes, remained stable in 2 of 5 eyes, and decreased in 1 of 5 eyes. Complaints of photophobia were reduced, and a satisfactory cosmetic outcome was achieved in 3 of 5 patients. The mean spherical equivalent refraction error at last follow-up was -4.0 D. Mean endothelial cell loss when compared with the healthy fellow eye was 42%. Two cases were complicated by partial luxation of the IOL, one case by persistent anterior uveitis and secondary glaucoma. One eye developed a retinal detachment. CONCLUSIONS: The Artisan iris reconstruction IOL is a treatment option for the treatment of aniridia and aphakia due to penetrating ocular trauma in children. We emphasize the high-risk characteristics of the eyes treated and the importance of careful patient selection in the outcome of the implantation of the Artisan iris reconstruction IOL.

Traumatic pediatric cataract: a decade of follow-up after Artisan aphakia intraocular lens implantation.

PURPOSE: To describe the long-term clinical outcome of Artisan((R)) aphakia intraocular lens (IOL; Ophtec, Groningen, The Netherlands) implantation in five aphakic eyes of five children, without capsular support, after cataract extraction following penetrating ocular trauma. METHODS: The charts of the five children were retrospectively reviewed. The data collected included follow-up time, nature of injury, age at cataract extraction and IOL implantation, visual outcome, endothelial cell counts, complications, and subsequent surgical interventions. RESULTS: Average follow-up was 11.0 years (range, 8.0-14.6 years). All eyes had a corneal perforation with various degrees of anterior segment injury. Mean patient age at lens extraction was 7.8 years (range, 5.6-10.2 years). Mean age at Artisan aphakia IOL implantation was 7.9 years (range, 5.7-10.2 years). The best spectacle-corrected visual acuity at last follow-up was 20/40 or better in four eyes. Mean endothelial cell loss compared with the healthy fellow eye was 40%. No patients experienced IOL dislocation, corneal decompensation, chronic anterior uveitis, cystoid macular edema, or iris atrophy. One eye had a retinal detachment 19 months after primary injury and needed vitreoretinal surgery. CONCLUSIONS: The Artisan aphakia IOL offers a useful alternative for correction of traumatic childhood aphakia. Although we only have results of a small number of patients, taking into account our long follow-up period, we feel that implantation of the Artisan aphakia IOL can be considered a treatment option in aphakic eyes of children that lack capsular support due to trauma.

Long-term follow-up of the corneal endothelium after artisan lens implantation for unilateral traumatic and unilateral congenital cataract in children: two case series.

PURPOSE: To retrospectively estimate the long-term corneal endothelial cell loss in children after perforating corneal trauma and implantation of an iris-fixated anterior-chamber intraocular lens (IOL), either the Artisan aphakia lens or the Artificial Iris Implant, and to compare this corneal endothelial cell loss to that in children who received an Artisan aphakia lens to correct aphakia after cataract extraction for unilateral congenital cataract. METHODS: A retrospective study was performed, evaluating the charts and endothelial photographs of 6 patients with unilateral traumatic cataract, with a mean age at IOL implantation of 9.5 years (range: 5.8-12.8 years) and a mean follow-up after IOL implantation of 10.5 years (range: 8.0-14.7 years), and of 3 children who were operated on for unilateral congenital cataract at a mean age of 2.7 years and who received an Artisan aphakia IOL, with a mean follow-up after IOL implantation of 9.5 years (range: 4.7-14.5 years). Parameters that were studied were central endothelial cell density (CECD) in both the operated and the normal eye at the last follow-up visit, percentage of cell loss in the operated eye compared with the normal eye, and length and location of the corneal scar in the injured eye. RESULTS: : In the traumatic cataract group, CECD was, on average, 41% (range: 22%-58%) lower in the operated eye (1.647 +/- 322 [SD] cells/mm) than the normal eye (2.799 +/- 133 cells/mm). A significant negative linear correlation was found between the length of the corneal perforation scar and CECD. In the congenital cataract group, no statistical difference in CECD was found between the operated (3.323 +/- 410 cells/mm) and the unoperated (3.165 +/- 205 cells/mm) eye. CONCLUSION: Endothelial cell loss 10.5 years after iris-fixated IOL implantation for traumatic cataract was substantial and related to the length of the corneal scar of the original trauma. In children operated on for congenital cataract, no difference was found in CECD in the operated and unoperated eyes 9.5 years after Artisan aphakia IOL implantation.

New mutations in the NHS gene in Nance-Horan Syndrome families from the Netherlands.

Mutations in the NHS gene cause Nance-Horan Syndrome (NHS), a rare X-chromosomal recessive disorder with variable features, including congenital cataract, microphthalmia, a peculiar form of the ear and dental anomalies. We investigated the NHS gene in four additional families with NHS from the Netherlands, by dHPLC and direct sequencing. We identified an unique mutation in each family. Three out of these four mutations were not reported before. We report here the first splice site sequence alteration mutation and three protein truncating mutations. Our results suggest that X-linked cataract and NHS are allelic disorders.

The Peters' plus syndrome: a review.

Peters' plus syndrome is an infrequently described entity that combines anomalies in the anterior chamber of the eye with other multiple congenital anomalies, and a developmental delay. Major symptoms are extremely variable anterior chamber anomalies, cupid bow of the upper lip, cleft lip and palate, short stature, broad hands and feet, and variable mental delay. The syndrome follows an autosomal recessive pattern of inheritance. The etiology is unknown, but may involve abnormal neural crest development. A review of the pertinent literature is provided.