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.

Validation of tissue quality parameters for donor corneas, designated for emergency cases: corneal graft survival.

PURPOSE: To validate tissue quality parameters for donor corneas designated for emergency grafting for corneal graft survival. METHODS: In a longitudinal cohort follow-up study, 131 emergency penetrating grafts were studied. Grafts were performed with a pool of organ-cultured donor corneas designated for emergency grafting and prepared for immediate use with all safety tests performed. Assignation criteria were: corneas with a small superficial stromal opacity but meeting all selection criteria for PKP tissue and corneas without stromal opacity, but an endothelial cell density from 1800 to 2300 cells/mm(2) or mild polymegathism or pleomorphism. Cox multivariate regression analysis, Kaplan-Meier survival and log rank test were applied. RESULTS: Of the 131 keratoplasties, 115 could be followed. One eye was lost during surgery because of an expulsive bleeding. In 15 cases, a conjunctival transplantation finished off the penetrating graft. Corneal graft survival was not significantly related to the presence of PKP quality of the donor endothelium, neither with a cloudy graft nor with endothelial decompensation as the cause of failure. Main risk factors for a failed graft were vascularization of the host cornea (p=0.0001), the presence of a systemic auto immune disease in the recipient (p=0.003) and the disease leading to the (imminent) perforation and emergency graft (p=0.021). CONCLUSION: A selected pool of donor corneas designated for emergency grafting that does not interfere with the scheduled procedures allows more efficient and safe use of donor tissue in case of a(n) (imminent) perforation. Corneal graft survival rates justify the criteria for selection.

Validation of tissue quality parameters for donor corneas designated for emergency use in preservation of the globe.

PURPOSE: To validate tissue quality parameters for donor corneas designated for emergency grafting to preserve the globe. METHODS: In a longitudinal cohort follow-up study, 151 emergency grafts in the Netherlands were studied. Grafts were performed with a pool of organ-cultured donor corneas designated for emergency grafting and prepared for immediate use with all safety tests performed. Assignation criteria were corneas with a small superficial stromal opacity but meeting all selection criteria for penetrating keratoplasty tissue and corneas without stromal opacity but an endothelial cell density from 1800 to 2300 cells per square millimeter or mild polymegethism or pleomorphism. Cox multivariate regression analysis, Kaplan-Meier survival, and log rank test were applied. RESULTS: All requests for corneal tissue were honored within 24 hours. Ninety-one patients showed a complete and 60 an imminent perforation. One hundred thirty-one penetrating grafts and 20 lamellar grafts have been used. The globes were saved in 140 of the 151 patients (92.7%). Globe preservation was not significantly related to the absence of penetrating keratoplasty quality of the donor endothelium, the type of grafting, the degree of vascularization in the host cornea, or diabetic disease in the recipient. The main risk factor for globe preservation was the presence of a systemic autoimmune disease in the recipient (P = 0.0021). CONCLUSIONS: A selected pool of donor corneas designated for emergency grafting, that does not interfere with the scheduled procedures, allows more efficient and safe use of donor tissue in case of an imminent perforation. Globe preservation rates justify the quality criteria for designation of this tissue.

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.

Outcomes of low-vision services using optometric and multidisciplinary approaches: a non-randomized comparison.

Consecutive patients (n = 215) who were referred to optometric (55%) or multidisciplinary (45%) low-vision services and above 50 years of age were recruited from four hospitals in the Netherlands. They completed two vision-related quality of life questionnaires, the Vision Quality of Life Core Measure (VCM1) and the Low Vision Quality of Life Questionnaire (LVQOL), before their first visit with low-vision services and 1 year later. At follow-up, patients referred to multidisciplinary low-vision services had lower scores on the mobility subscale of the LVQOL than patients referred to optometric low-vision services [5.3 points; 95% confidence interval (CI): 0.2-10.5]. Paired sample t-tests for the two groups of patients taken together show improvement for the VCM1 (3.1 points; 95% CI: 0.6-5.6) and deterioration for the basic aspects of vision (3.5 points; 95% CI: 1.1-5.9) and the mobility (6.6 points; 95% CI: 3.7-9.5) subscales of the LVQOL. In conclusion, people referred to optometric services showed less deterioration in mobility than those referred to multidisciplinary services. No differences were observed for any of the other subscales of the LVQOL and the VCM1. Future research in this field should include randomized controlled designs comparing low-vision services with no treatment or placebo.

Reduced corneal swelling and deswelling response in the iridocorneal endothelial syndrome.

PURPOSE: Despite severe abnormalities of the corneal endothelium in the iridocorneal endothelial (ICE) syndrome, the cornea can remain clear and maintain its normal thickness for years before corneal decompensation occurs. The aim of this study is to analyze this discrepancy by studying corneal hydration control in the ICE syndrome. METHODS: In four subjects with unilateral ICE syndrome, without signs of corneal decompensation, a "corneal stress test" was performed in both the affected and the unaffected eye. The stress test measures the recovery of corneal thickness after swelling induced by wearing a soft contact lens with the eyes closed. Corneal thickness was measured by noncontact pachymetry. RESULTS: Mean baseline corneal thickness of affected eyes (535+/-56 microm) was not different from unaffected eyes (526+/-39 microm). On the other hand, the corneal stress test showed a smaller induced swelling in affected eyes (33+/-15 microm) compared with unaffected eyes (67+/-11 microm) and a slower recovery of corneal thickness in affected eyes. In one of four subjects, the cornea of the affected eye was calculated not yet to have recovered its original thickness before midnight. After this finding, although the cornea of this subject did not show any signs of clinical decompensation, the study was discontinued. CONCLUSIONS: Corneal hydration control is altered in the ICE syndrome. The ICE cornea can maintain its normal thickness despite severe morphologic abnormalities of the endothelium. Because we found that recovery of corneal thickness can be extremely slow, it cannot be guaranteed that such corneal "stress tests" are completely safe for subjects with the ICE syndrome, and we therefore feel that they should no longer be performed in these subjects.

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 patients.

Validity of endothelial cell analysis methods and recommendations for calibration in Topcon SP-2000P specular microscopy.

PURPOSE: To report on the calibration of the Topcon SP-2000P specular microscope and the Endothelial Cell Analysis Module of the IMAGEnet 2000 software, and to establish the validity of the different endothelial cell density (ECD) assessment methods available in these instruments. METHODS: Using an external microgrid, we calibrated the magnification of the SP-2000P and the IMAGEnet software. In both eyes of 36 volunteers, we validated 4 ECD assessment methods by comparing these methods to the gold standard manual ECD, manual counting of cells on a video print. These methods were: the estimated ECD, estimation of ECD with a reference grid on the camera screen; the SP-2000P ECD, pointing out whole contiguous cells on the camera screen; the uncorrected IMAGEnet ECD, using automatically drawn cell borders, and the corrected IMAGEnet ECD, with manual correction of incorrectly drawn cell borders in the automated analysis. Validity of each method was evaluated by calculating both the mean difference with the manual ECD and the limits of agreement as described by Bland and Altman. RESULTS: Preset factory values of magnification were incorrect, resulting in errors in ECD of up to 9%. All assessments except 1 of the estimated ECDs differed significantly from manual ECDs, with most differences being similar (< or =6.5%), except for uncorrected IMAGEnet ECD (30.2%). Corrected IMAGEnet ECD showed the narrowest limits of agreement (-4.9 to +19.3%). CONCLUSIONS: We advise checking the calibration of magnification in any specular microscope or endothelial analysis software as it may be erroneous. Corrected IMAGEnet ECD is the most valid of the investigated methods in the Topcon SP-2000P/IMAGEnet 2000 combination.

Different aspects of visual impairment as risk factors for falls and fractures in older men and women.

UNLABELLED: Visual impairment has been implicated as a risk factor for falling and fractures, but results of previous studies have been inconsistent. The relationship between several aspects of vision and falling/fractures were examined in a prospective cohort study in 1,509 older men and women. The analyses showed that impaired vision is an independent risk factor for both recurrent falling and fractures. INTRODUCTION: Falls and fractures are a major health problem among the elderly. Visual impairment has been implicated as a risk factor for both falls and fractures. However, results from studies are inconsistent. The inconsistency between findings can primarily be attributed to differences in the designs of these studies. Most studies have been cross-sectional or case-control studies, and many have not correctly adjusted for potential confounders. Furthermore, until now, the potential mediating effects of functional limitation, physical performance, and physical activity have not been examined. MATERIALS AND METHODS: A total of 1,509 people was examined in 1995-1996. Contrast sensitivity was assessed with the VCTS_6000-1 chart for near vision. In addition, self-reported visual impairment was assessed by questions on recognizing faces from a distance of 4 m, reading the small print in the newspaper, and problems with glare. Furthermore, many potential confounders and mediators were assessed. Falls and fractures were assessed prospectively during a 3-year follow-up period. The associations between the vision variables and falls and fractures were examined using Cox proportional hazards analyses. RESULTS: After adjustment for potential confounders, contrast sensitivity was shown to be associated with recurrent falling (hazard ratio [HR] = 1.5), and the question on recognizing faces was shown to be associated with fractures (HR = 3.1). Furthermore, functional limitations and physical performance were shown to be mediators in the relationship between vision variables and recurrent falling/fractures. CONCLUSIONS: The results indicate that impaired vision is an independent risk factor for falling and fractures, but different aspects of visual functioning may have different relationships to falling and fractures.

Psychometric properties of vision-related quality of life questionnaires: a systematic review.

The increased attention for quality of life (QOL) as an outcome measure has led to the development of numerous questionnaires to assess this construct in the field of ophthalmology. This article presents a systematic review on QOL questionnaires for people with vision impairments. We systematically searched and selected the literature. Furthermore, a rating list with descriptive aspects (e.g. target population) and psychometric aspects (e.g. reproducibility) was developed. The 31 questionnaires that were included in the review were rated on the basis of criteria stated in this rating list. The questionnaires were mostly targeted at cataract patients or visually impaired persons in general. Only a few of the questionnaires demonstrated sufficient psychometric quality. A recent development is the (re-)evaluation of questionnaires with Rasch analysis. This is one of the most important issues to which future research in this field should be directed.

Prognostic value of Hutchinson's sign in acute herpes zoster ophthalmicus.

PURPOSE: To determine the prognostic value of nasociliary skin lesions (Hutchinson's sign) for ocular inflammation and corneal sensory denervation in acute herpes zoster ophthalmicus. METHODS: A longitudinal observational study with a 2-month follow-up was performed involving 83 non-immunocompromised adults with acute herpes zoster ophthalmicus, with a skin rash duration of less than 7 days, referred by their general practitioner. All skin lesions at the tip, the side and the root of the nose, representing the dermatomes of the external nasal and infratrochlear branches of the nasociliary nerve, were documented by taking photographs and marking anatomical drawings. Ocular inflammatory signs were observed by slit-lamp biomicroscopy, and corneal sensitivity was measured with the Cochet-Bonnet esthesiometer at 2-month follow-up. RESULTS: Hutchinson's sign was a powerful predictor of ocular inflammation and corneal denervation in herpes zoster ophthalmicus [relative risks: 3.35 (CI 95%: 1.82-6.15) and 4.02 (CI 95%:1.55-10.42), respectively]. The manifestation of herpes zoster skin lesions at the dermatomes of both nasociliary branches was invariably associated with the development of ocular inflammation. CONCLUSION: Clinicians should be alert for early skin lesions within the complete nasociliary dermatome, because they are a reliable prognostic sign of sight-threatening ocular complications in acute herpes zoster ophthalmicus.

Radius and asphericity of the posterior corneal surface determined by corrected Scheimpflug photography.

PURPOSE: To obtain the shape of the posterior corneal surface in a healthy population, using Scheimpflug photography corrected for distortion due to the geometry of the Scheimpflug imaging system and the refraction of the anterior corneal surface. METHODS: The posterior corneas of 83 subjects, ranging in age from 16 to 62 years, were measured in the vertical meridian using corrected Scheimpflug photography. The aspherical shape of the anterior Corneal surface was also determined in conjunction with the correction of Scheimpflug images. RESULTS: The average radius of the anterior corneal surface was 7.87 +/- 0.27 mm (SD), while the average radius of the posterior corneal surface was 6.40 +/- 0.28 mm (SD). The ratio between the posterior and the anterior radius of curvature was 0.81 +/- 0.02. The asphericity of the anterior and the posterior corneal surfaces was 0.82 +/- 0.18 and 0.62 +/- 0.27, respectively. The asphericity of the posterior corneal surface decreased significantly with age. The posterior/anterior asphericity ratio is also dependent on age and was 0.98 +/- 0.17 at 16 years of age and 0.53 +/- 0.30 at 62 years of age. CONCLUSION: Corrected Scheimpflug photography is an appropriate technique for measuring the radius and asphericity of the posterior corneal surface. The asphericity of the posterior corneal surface changes with age.

Cheilitis granulomatosa and optic neuropathy as rare extraintestinal manifestations of Crohn's disease.

Crohn's disease can be accompanied by extraintestinal manifestations. The authors report on a 39-year-old patient who presented with cheilitis granulomatosa as the first manifestation of Crohn's disease. Four years later, intestinal Crohn's disease was diagnosed. One year afterward, acute loss of visual acuity from optic neuropathy developed as another rare extraintestinal manifestation of Crohn's disease.