Long-term results of anterior chamber iris claw intraocular lens implantation in children with ectopia lentis in Marfan syndrome.

PURPOSE: To report the long-term clinical and endothelial cell count (ECC) results of lensectomy with primary anterior chamber iris claw lens implantation in the eyes of patients ≤18-year-old with ectopia lentis due to Marfan syndrome. METHODS: The medical records of Marfan patients operated on at a single institution from September 2007 to August 2020, with minimum follow-up of 2 years, were reviewed retrospectively. The following data were analyzed: sex, age at surgery, indication for surgery, the position of the lens in relation to the undilated and dilated pupil, corneal endothelial cell counts (ECC), peri- and postoperative complications, pre- and postoperative best-corrected visual acuity. RESULTS: A total of forty-two eyes of 23 patients (12 girls and 11 boys) were included. At least two or more postoperative ECCs were collected from 33 eyes (17 patients). Median age at IOL implantation was 6.1 years (range, 1.8-18). Median overall follow-up time was 6.2 years (range, 2-13.5). Median ECC follow-up time was 6.2 years (range, 2-10). Mean best-corrected visual acuity was 0.71 ± 0.38 logMAR before surgery and 0.02 ± 0.25 logMAR at final follow-up. The mean annual ECC decline was 0.71% ± 2.24. Total cell loss from first to last postoperative measurement was 150 cells ± 394 cells/mm2 (4.81%). Pre- and first postoperative data were available for 17 eyes of 10 patients, with a mean cell loss before and directly after surgery of 269 ± 268 cells (7.94%). Surgery related complications were iris bombé due to blockage of peripheral iridectomy in 3 eyes and claw dislocation due to direct impact trauma in 3 eyes. CONCLUSIONS: In our large, pediatric study cohort, anterior chamber iris claw IOL implantation resulted in an excellent visual outcome and normal endothelial cell loss compared with normative data. Safety measures are recommended to avoid traumatic dislocation of IOLs.

Cornea verticillata supports a diagnosis of Fabry disease in non-classical phenotypes: results from the Dutch cohort and a systematic review.

BACKGROUND: Screening for Fabry disease (FD) increasingly reveals individuals without characteristic features and with a variant of unknown significance in the α-galactosidase A (GLA) gene. Cornea verticillata (CV) assessment, as a characteristic sign of FD, may be a valuable diagnostic tool to assess whether these individuals have a non-classical phenotype or no FD at all. METHODS: We performed a systematic review to estimate the prevalence of CV in FD. Additionally, CV prevalence was assessed in the Dutch FD cohort. Data were stratified by gender and phenotype (classical, non-classical, uncertain, no-FD) using predefined criteria. RESULTS: CV was assessed in 21 cohorts (n=753, 330 men, age 0-85 years). Pooled prevalence was 69% (74% men, 66% women). In six studies, 77 (19 men) individuals with a non-classical or uncertain diagnosis were identified. Individual data were available in 4/6 studies (n=66, 16 men). CV was present in 24% (n=16, 2 men). 101 (35 men) subjects from the Dutch cohort were grouped as classical, of whom 86% (94% men, 82% women including five women who used amiodarone) had CV. Of the 25 (11 men) non-classical patients, 4 (three men) had CV. Subjects in the uncertain and no-FD groups did not have CV. CONCLUSIONS: CV is related to classical or biopsy-proven non-classical FD, with a very high sensitivity in classical men. Thus, presence of CV in an individual with an uncertain diagnosis of FD indicates a pathogenic GLA variant, in the absence of medication that may induce CV; if CV is absent, FD cannot be excluded.

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.

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.

Axial eye length growth and final refractive outcome after unilateral paediatric cataract surgery.

AIM To compare the axial eye length growth of the two fellow eyes within one patient after unilateral paediatric cataract surgery and to assess changes in refraction and keratometry. PATIENTS/METHODS A retrospective study in 90 eyes of 45 patients was performed. The 45 patients were divided into group 1 (patients younger than 18 months at the time of surgery, n=25) and group 2 (patients 18 months or older at the time of surgery, n=20). The axial eye length, spherical equivalent refraction and keratometry were measured during surgery and at follow-up. All outcome data in the operated eyes were compared with the outcome data of the fellow non-operated eyes. The Student t test was used for statistical analysis. Values of p<0.05 were considered statistically significant. RESULTS In group 1 the absolute growth (mm) of the operated eyes was borderline statistically significantly less than in the fellow non-operated eyes (p=0.049). No statistically significant difference in the rate of axial growth between the two eyes was found (p=0.25). A larger myopic shift (p=3.85x10(-5)) and a larger change in keratometry (p=0.02) were found in the operated eyes. In group 2 no statistically significant differences were found between the two eyes. CONCLUSION We did not find a statistically significant difference in axial length growth between the operated eyes and fellow non-operated eyes in our unilateral paediatric cataract 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.