Corneal nerve and nerve conduction abnormalities in children with type 1 diabetes.

OBJECTIVE: In vivo corneal confocal microscopy (CCM) is a novel, rapid, and non-invasive technique that identifies early small fiber damage and can predict the progression and development of clinical neuropathy in adults with type 1 diabetes. However, its usefulness in children is not well established. This study compared corneal confocal microscopy with neuropathic symptoms, signs, and objective measures of neuropathy for the diagnosis of diabetic neuropathy in children with type 1 diabetes. RESEARCH DESIGN AND METHODS: A total of 83 children with type 1 diabetes and 83 healthy participants of similar age underwent assessment of neuropathy symptoms, signs, nerve conduction studies, quantitative sensory and autonomic function testing, and in vivo CCM. RESULTS: Only of 3/83 (4%) children with type 1 diabetes had subclinical neuropathy. However, corneal nerve fiber density (p = 0.001), branch density (p = 0.006), fiber length (p = 0.002), tibial motor nerve amplitude and conduction velocity, and sural sensory nerve amplitude and conduction velocity (all p < 0.004) were lower in participants with type 1 diabetes than in the controls. Vibration, cooling, and warm perception thresholds and deep breathing heart rate variability were not found to be different (all p > 0.05) between children with type 1 diabetes and healthy controls. Multivariate regression analysis identified a possible association between body mass index and decreased corneal nerves. CONCLUSIONS: Decreased corneal nerves and abnormal nerve conduction were found in children with type 1 diabetes. CCM may allow rapid objective detection of subclinical diabetic neuropathy in children and adolescents with type 1 diabetes.

Evidence for small fiber neuropathy in early Parkinson's disease.

INTRODUCTION: Parkinson's disease (PD) is neurodegenerative movement disorder affecting primarily the central nervous system with several recognized non-motor symptoms that can occur at various stages of the disease. Recently it has been shown that patients with PD may be prone to peripheral nervous system pathology in the form of a peripheral neuropathy (PN). It is unclear if PN is an inherent feature of PD or if it is an iatrogenic effect of the mainstay PD treatment Levodopa. METHODS: To determine if peripheral neuropathy occurs in early untreated PD we employed a case-control study design using gold standard tests for PN, including neurological examination according to the Utah Early Neuropathy Scale (UENS) and nerve conduction studies, as well as new, more sensitive and informative tests for PN including the skin biopsy and corneal confocal microscopy (CCM). RESULTS: We studied 26 patients with PD and 22 controls using the neurological examination and nerve conduction studies (NCS) and found no significant difference between groups except for some reduced vibration sense in the PD group. Epidermal nerve densities in the skin biopsies were similar between our cohorts. However, using CCM - a more sensitive test and a surrogate marker of small fiber damage in PN, we found that patients with PD had significantly reduced corneal nerve fiber densities and lengths as compared to controls. CONCLUSIONS: We conclude that our positive CCM results provide evidence of preclinical PN in newly diagnosed PD patients.

The Reliability and Reproducibility of Corneal Confocal Microscopy in Children.

PURPOSE: To assess the image and patient level interrater agreement and repeatability within 1 month for corneal nerve fiber length (CNFL) measured using in vivo corneal confocal microscopy (IVCCM) in children. METHODS: Seventy-one subjects (mean [SD] age 14.3 [2.6] years, range 8-18 years; 44 with type 1 diabetes and 27 controls; 36 males and 35 females) were included. 547 images (∼6 images per subject) were analyzed manually by two independent and masked observers. One-month repeat visit images were analyzed by a single masked observer in 21 patients. Automated image analysis was then performed using a specialized computerized software (ACCMetrics). RESULTS: For CNFL, the ICC (95% CI) were 0.94 (0.93-0.95) for image-level, 0.86 (0.78-0.91) for patient-level, and 0.88 (0.72-0.95) for the 1-month repeat assessment, and the Bland-Altman plots showed minimal bias between observers. Although there was excellent agreement between manual and automated analysis according to an ICC 0.89 (0.82-0.93), the Bland-Altman plot showed a consistent bias with manual measurements providing higher readings. CONCLUSIONS: In vivo corneal confocal microscopy image analysis shows good reproducibility with excellent intraindividual and interindividual variability in pediatric subjects. Since the image-level reproducibility is stronger than the patient-level reproducibility, refinement of the method for image selection will likely further increase the robustness of this novel, rapid, and noninvasive approach to detect early neuropathy in children with diabetes. Further studies on the use of IVCCM to identify early subclinical neuropathy in children are indicated.

Intermittent exotropia: facts, opinions, and unknowns.

INTRODUCTION: Intermittent exotropia (IXT) can be a controversial topic, often eliciting lively discussion. This lecture will discuss its definition, incidence, age of onset, presentation, natural variation, criteria for deterioration, goals of treatment, effectiveness of surgical treatment, types of surgical treatment, and unwanted effects of surgical treatment. METHOD: Results from the scientific literature, opinions of respected colleagues, the opinion of the author, and the results of live polling of the audience during the John Pratt-Johnson lecture are presented. RESULTS: IXT is defined as an exotropia that is present intermittently predominantly for distance. Its incidence is about 1% and it usually has an onset before age 5. Patients often present because of concern regarding the appearance of the eye misalignment. There is natural variation in the control of IXT, the angle of IXT, and the amount of stereopsis. Criteria that denote deterioration are increasing frequency of IXT, progressively and consistently increasing angle of IXT, loss of binocular vision, and increasing concern regarding the patient's appearance and its effect on social interaction. Goals of treatment are to retain equal or nearly equal vision, to obtain acceptable cosmesis, and to retain binocular vision. The long-term success of surgical treatment is not well proven. Persistent postoperative overcorrection is an unwanted effect of surgical treatment. CONCLUSION: The inherent biologic variation that occurs when measuring the components of IXT makes it difficult to be dogmatic about IXT, particularly when trying to decide when deterioration is occurring.

Indoor soccer-related eye injuries: should eye protection be mandatory?

BACKGROUND: Our objectives were to present the spectrum of eye injuries caused by indoor soccer, as seen at our institution, and to initiate discussion as to whether eye protection should become mandatory for this indoor sport. METHODS: Chart review of patients presenting to our institution with eye injuries from indoor soccer. RESULTS: Five cases were identified from 2001-2005, all occurring during the winter or late fall. Each injury was due to contact with the soccer ball itself. Initially, all 5 patients presented with commotio retinae (1 with a prominent retinal and vitreous hemorrhage and 2 with smaller retinal hemorrhages), 2 with hyphema and traumatic mydriasis, 1 with subconjunctival hemorrhage, and 1 with upper lid edema and ecchymosis. Three resolved uneventfully with 20/20 or better vision; however, in 2 there were findings of choroidal rupture with chorioretinal scarring. One of these had 20/20 vision and a discontinuous choroidal rupture peripherally, and the other had 20/40 vision and extensive chorioretinal scarring. One patient also showed a peculiar persistent iris scar. INTERPRETATION: Soccer-related eye injuries have been recognized as an important ophthalmologic problem in Europe and now increasingly so in North America. With the increasing popularity of indoor soccer in Canada, serious eye injuries have become more prevalent. On the basis of the prevalence and the nature and mechanism of the ocular trauma, we believe there may be a need to make eye protection mandatory for all forms of soccer.

Ophthalmic findings in Setleis syndrome: two new cases in a mother and son.

CASE REPORT: Setleis syndrome is a rare ectodermal dysplasia with characteristic ophthalmic findings. We describe the first 2 reported cases in Canadian individuals of Aboriginal descent. COMMENTS: Although most ophthalmic findings are benign, it is important to recognize the clinical significance for management and genetic counselling. We postulate an autosomal dominant inheritance in our cases.

Consecutive exotropia following surgically corrected cyclic esotropia.

INTRODUCTION: Cyclic esotropia is a rare form of strabismus consisting of regular intervals of esotropia alternating with periods of orthophoria in a rhythmic/cyclic manner. In the vast majority of cases, surgery appears to permanently correct the esotropia, with no sequelae after years of follow-up. We report a case of consecutive exotropia in a patient five years after bilateral medial rectus recessions for cyclic esotropia. METHODS: A case report involving review of a clinical chart. RESULTS: A two-year-old male presented with right esotropia and mild amblyopia. He was treated with patching and following resolution of the amblyopia he developed a cyclic esotropia. Surgical correction was performed for the full amount measured on a "manifest" day. Following the surgery, he was orthophoric and demonstrated binocular vision. He remained stable for five years, and then returned with occasional diplopia and an intermittent exotropia. CONCLUSION: Cyclic esotropia is a rare disorder of ocular motility that spontaneously appears and disappears at regular intervals. After surgical correction, the deviation disappears and recurrence of esotropia is very infrequent. We present the first reported case of consecutive exotropia following surgical correction of a cyclic esotropia.

Long-term refractive change after intraocular lens implantation in childhood.

BACKGROUND: To determine refractive change occurring with age in children who had cataract removal with intraocular lens implantation and in whom the immediate postoperative refraction was targeted either to match the refractive error of the opposite eye in unilateral cases, or for only a small refractive error when surgery was bilateral. METHODS: Retrospective review of the refractive error over time in 36 eyes of 25 children who underwent cataract removal (11 bilateral) with insertion of an intraocular lens from 1987 to 1998 and who had at least 4 years follow-up, but no glaucoma. RESULTS: Mean age at surgery was 5.5 years (median 5.7 y, range 1.3-12 y), with a mean follow-up of 8 years (median 6 y, range 4-16 y). The average refraction followed a logarithmic decline with age. Although eyes with unilateral surgery had a slightly faster rate of change and lower final refraction than did eyes with bilateral surgery, this difference was not statistically significant. Variation from this trend was also observed in 3 patients. When the hyperopic refractive error created immediately after surgery was small, children usually became significantly myopic when older, often creating anisometropic myopia in unilateral cases. INTERPRETATION: When implanting intraocular lenses bilaterally one should aim for a significant but balanced hyperopic correction immediately postoperatively in young patients, anticipating that there will be emmetropization with aging. Parents should be warned that variations can occur.

The natural history of surgically untreated intermittent exotropia-looking into the distant future.

PURPOSE: We studied patients with distance intermittent exotropia with at least 5 years follow-up before any surgical therapy, to determine how often amblyopia developed, whether there was progressive worsening in the angle or control of the exodeviation, whether there was deterioration in binocular vision, and other parameters. METHODS: Retrospective review of charts of patients with exodeviations examined consecutively over 17 years. RESULTS: There were 109 patients followed for a mean of 9 years (range 5 to 25). Amblyopia was mild (20/25 in 33, 20/30 in 15, and 20/40 in 2). There was no significant change in the mean exoangle from initial to final visit (20.6 to 20.9 PD), but the initial to final exoangle decreased by more than 10 PD in 19%, remained stable in 58%, and increased by more than 10 PD in 23%. There was no statistically significant trend for worsening or improving when serial measurements were plotted and the aggregate slopes were analyzed. Change in control in the distance from initial to final visit was favorable in 26%, showed no change in 51%, and was unfavorable in 23%, with 17% deteriorating to a constant distance exotropia. Mean near stereoacuity at the final visit was 88 seconds of arc. Strabismus surgery was performed in 14% of patients from 5 to 18 years (mean 9) after their initial visit. CONCLUSIONS: Visual acuity and near stereoacuity were generally good. There was little change in the mean angle of deviation from initial to final visit for the group, with a nearly chance occurrence for the distance deviation to progressively worsen or improve, perhaps explaining why strabismologists have had difficulty definitively prognosticating about the long-term stability of intermittent exotropia.

Enhanced models for teaching slit-lamp skills.

This article describes enhancements to models previously devised for demonstrating slit-lamp findings to students and physicians. The models can simulate the optical appearance of the anterior segment, "flare" and "cells" in the anterior chamber, hypopyon, gross and microscopic hyphema, the red reflex, cataract, corneal epithelial defects (including fluorescein staining) and superficial corneal foreign bodies, whose removal can be practised.