10 research outputs found
Two families with dyshidrotic ectodermal dysplasia with limbal hair growth and Bitot-like conjunctival anomalies
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/mm2). 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 patient
Implantation of the Artisan (R) 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 IO
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 surger
The Effect of Therapy Refusal Against Medical Advice in Retinoblastoma Patients in a Setting Where Treatment Delays are Common
Changes to the subscales of two vision-related quality of life questionnaires are proposed
BACKGROUND AND OBJECTIVE: Psychometrically sound questionnaires for the assessment of vision-related quality of life (QOL) are scarce. Therefore, the objective was to further validate two vision-related QOL questionnaires in a Dutch population of visually impaired elderly. METHODS: A total of 329 visually impaired older persons referred to low vision services completed the low vision QOL (LVQOL) and Vision-Related Quality of Life Core Measure (VCM1) questionnaires at baseline, after 1-4 weeks (retest), and after 5 months. Confirmatory factor analyses were performed on baseline data. The smallest detectable change (SDC) was assessed, based on the standard error of measurement (SEM). Change scores between the baseline and 5 months follow-up data were related to a general transition question to assess the minimal important change (MIC). Furthermore, the MIC was related to the SDC, to examine whether the MICs were detectable beyond measurement error. RESULTS: The original factor structures could not be confirmed. After omitting items and remodeling, adequate fits were obtained. SDCs comprised at least one quarter of the scale for all scales and subscales on the individual level and exceeded the MICs on every occasion. CONCLUSION: We propose MICs of 5-10 points for the scales and subscales of the LVQOL and VCM1. The questionnaires are not useful in the follow-up of individual patient
De betekenis van betrouwbaarheid van meetinstrumenten voor het vaststellen van verandering : een voorbeeld bij slechtziende ouderen.
Orofacial granulomatosis in a patient with Crohn's disease.
Orofacial granulomatosis encompasses the previously recognized clinical entities Melkersson-Rosenthal syndrome and cheilitis granulomatosa. We report the case of a 39-year-old patient with cheilitis granulomatosa, intestinal Crohn's disease, and optic neuropathy. Cheilitis granulomatosa and optic neuropathy represent 2 rare manifestations of orofacial granulomatosis in Crohn's disease