Edward G. Buckley

A Randomized, Controlled Trial of Corticosteroids in the Treatment of Acute Optic Neuritis

Background and Methods. The use of corticosteroids to treat optic neuritis is controversial. At 15 clinical centers, we randomly assigned 457 patients with acute optic neuritis to receive oral prednisone (1 mg per kilogram of body weight per day) for 14 days; intravenous methylprednisolone (1 g per day) for 3 days, followed by oral prednisone (1 mg per kilogram per day) for 11 days; or oral placebo for 14 days. Visual function was assessed over a six-month follow-up period. Results. Visual function recovered faster in the group receiving intravenous methylprednisolone than in the placebo group; this was particularly true for the reversal of visual-field defects (P = 0.0001). Although the differences between the groups decreased with time, at six months the group that received intravenous methylprednisolone still had slightly better visual fields (P = 0.054), contrast sensitivity (P = 0.026), and color vision (P = 0.033) but not better visual acuity (P = 0.66). The outcome in the oral-prednisone group did ...

Multiple sclerosis risk after optic neuritis: Final optic neuritis treatment trial follow-up

OBJECTIVE To assess the risk of developing multiple sclerosis (MS) after optic neuritis and the factors predictive of high and low risk. DESIGN Subjects in the Optic Neuritis Treatment Trial, who were enrolled between July 1, 1988, and June 30, 1991, were followed up prospectively for 15 years, with the final examination in 2006. SETTING Neurologic and ophthalmologic examinations at 13 clinical sites. PARTICIPANTS Three hundred eighty-nine subjects with acute optic neuritis. MAIN OUTCOME MEASURES Development of MS and neurologic disability assessment. RESULTS The cumulative probability of developing MS by 15 years after onset of optic neuritis was 50% (95% confidence interval, 44%-56%) and strongly related to presence of lesions on a baseline non-contrast-enhanced magnetic resonance imaging (MRI) of the brain. Twenty-five percent of patients with no lesions on baseline brain MRI developed MS during follow-up compared with 72% of patients with 1 or more lesions. After 10 years, the risk of developing MS was very low for patients without baseline lesions but remained substantial for those with lesions. Among patients without lesions on MRI, baseline factors associated with a substantially lower risk for MS included male sex, optic disc swelling, and certain atypical features of optic neuritis. CONCLUSIONS The presence of brain MRI abnormalities at the time of an optic neuritis attack is a strong predictor of the 15-year risk of MS. In the absence of MRI-detected lesions, male sex, optic disc swelling, and atypical clinical features of optic neuritis are associated with a low likelihood of developing MS. This natural history information is important when considering prophylactic treatment for MS at the time of a first acute onset of optic neuritis.

A cohort study of transcutaneous oxygen tension and the incidence and severity of retinopathy of prematurity.

Abstract Background. Retinopathy of prematurity is a disease affecting the blood vessels of the retina in premature infants that may result in scarring, retinal detachment, and loss of vision. An association between this condition and the exposure of premature infants to supplemental oxygen has been postulated, but the relation between retinopathy of prematurity and blood oxygen levels has not been defined. The purpose of this study of a cohort of preterm infants was to correlate the incidence and severity of retinopathy of prematurity with the duration of exposure to different ranges of oxygen tension as measured by transcutaneous monitoring (tcPO2). Methods. One hundred one premature infants (birth weight, 500 to 1300 g) requiring supplemental oxygen had continuous monitoring of tcPO2. The number of hours during which the tcPO2 was 80 mm Hg or higher was tabulated for each infant during the first four weeks of life. Results. There was a significant association between the amount of time that the tcPO2 w...

Oncogenic Point Mutations in the Human Retinoblastoma Gene: Their Application to Genetic Counseling

Mutations of the retinoblastoma gene, most of which cannot be detected by conventional Southern blotting, are known to cause both the nonhereditary and hereditary forms of retinoblastoma and have been implicated in the development of other cancers. Nonhereditary retinoblastoma is caused by a somatic mutation. Hereditary retinoblastoma is caused by a germ-cell mutation, most often a new one, and thus there is usually no family history of the disease. Unlike patients with the nonhereditary disease, those with the hereditary form are at risk for additional retinoblastomas, and their progeny are at risk for the tumors. We used a sensitive technique of primer-directed enzymatic amplification, followed by DNA sequence analysis, to identify mutations as small as a single nucleotide change in tumors from seven patients with simplex retinoblastoma (with no family history of the disease). In four patients the mutation involved only the tumor cells, and in three it involved normal somatic cells as well as tumor cells but was not found in either parent; thus, these mutations appeared to be new, germ-cell mutations. In addition, we found point mutations in cells from a bladder carcinoma, a small-cell carcinoma of the lung, and another retinoblastoma. We conclude that the technique that we have described can distinguish hereditary from nonhereditary retinoblastoma and that it is useful in risk estimation and genetic counseling.

Axenfeld-Rieger syndrome. A spectrum of developmental disorders

The clinical and histopathologic features of Axenfelds anomaly and Riegers anomaly and syndrome are reviewed, and recent findings regarding the pathogenesis of this spectrum of developmental disorders are discussed. Based on these observations, it has been suggested that a developmental arrest, in the third trimester of gestation, of tissues derived from the neural crest cells accounts for the ocular and most of the nonocular abnormalities in this group of disorders. Since previous collective terms, such as mesodermal dysgenesis and anterior chamber cleavage syndrome, are not consistent with these new observations, the alternative name, Axenfeld-Rieger syndrome, has been proposed. The differential diagnosis of the syndrome includes two additional spectra of disorders: the iridocorneal endothelial syndrome and the posterior polymorphous dystrophies. The most serious ocular problem in Axenfeld-Rieger syndrome is the associated glaucoma, which occurs in a high percentage of patients and is typically difficult to control. Recent observations regarding the mechanism of the glaucoma, as reviewed in this paper, provide guidance in the management of this aspect of Axenfeld-Rieger syndrome.

Management of the Posterior Capsule During Pediatric Intraocular Lens Implantation

One of the major obstacles in pediatric intraocular lens implantation has been the subsequent dense opacification of the posterior capsule. We used a modification of the standard pediatric cataract surgical procedure, which involved endocapsular cataract extraction, posterior chamber intraocular lens implantation, pars plana posterior capsulotomy, and pars plana anterior vitrectomy in 20 consecutive patients with unilateral traumatic, radiation-induced, and developmental cataracts. Visual axes were rapidly restored in all patients without further intervention for posterior capsule opacification. Visual acuity returned to 20/40 or better in all patients and 75% of all patients (15 patients) reached maximum improvement by five weeks. No complications attributed to intraoperative removal of the posterior capsule occurred.

A framework for developing excellence as a clinical educator

The current emphasis on providing quality undergraduate and postgraduate medical education has focused attention on the educational responsibilities of all doctors. There is a greater awareness of the need to train doctors as educators and courses have been set up to satisfy this need. Some courses, such as those on how to conduct appraisal, are specific to one task facing a medical educator. Other courses take a broader view and relate educational theory to practice. In this paper we describe an outcome‐based approach in which competence in teaching is defined in terms of 12 learning outcomes. The framework provides a holistic approach to the roles of the teacher and supports the professionalism of teaching. Such a framework provides the basis for the development of a curriculum for teaching excellence. It helps to define important competences for different categories of teachers, communicate the areas to be addressed in a course, identify gaps in course provision, evaluate courses, assist in staff planning and allow individuals to assess their personal learning needs. The framework is presented to encourage wider debate.

Development of Myopia in Infants with Birth Weights Less than 1251 Grams

The authors report on the incidence of myopia in a large group of premature infants with birth weights of less than 1251 g followed as part of the multicenter study of Cryotherapy for Retinopathy of Prematurity. None of the eyes reported here underwent cryotherapy. Eyes were refracted using cycloplegic retinoscopy at 3 months (n = 2916), 12 months (n = 2626), and 24 months (n = 961 at 5 of the 23 centers) after term. Myopia was observed in approximately 20% of the children at each test age. The percentage of high myopia (greater than or equal to 5 diopters) doubled from 2% to 4.6% between 3 and 12 months and remained stable thereafter. Lower birth weight and increasing severity of retinopathy of prematurity (ROP) were strong predictors of myopia and high myopia. In addition, anisometropia, astigmatism, and the presence of posterior pole residua from ROP also were associated with a higher incidence of myopia and high myopia.

Long-term Results and Complications of Botulinum A Toxin in the Treatment of Blepharospasm

The authors review their long-term results and complications with the use of botulinum A toxin in the treatment of facial dystonias. Two hundred thirty-two patients in three diagnostic groups--essential blepharospasm, hemifacial spasm, and Meiges syndrome--were treated with botulinum A toxin. A total of 1044 treatments were given over a 4-year period. A reduction in orbicularis spasm intensity was noted in 1012 (96.9%) treatments (mean duration, 13.3 weeks). There was no clear relationship between toxin dose and the amount of spasm reduction or duration of response, and average duration of beneficial effect remained constant from the first through the twelfth injections. Complications occurred in 236 (22.6%) treatments. In most cases, these were local and transient. Symptomatic dry eye was the most common side effect, noted in 7.5% of cases. Ptosis was reported in 7.3% of treatments and photophobia in 2.5%. Diplopia involving the inferior oblique or lateral rectus muscles was seen in less than 1% of cases. There were no differences in degree of response or in complications among the three diagnostic groups, although there was a slight difference in duration of effect. Patients who had undergone previous eyelid surgery for blepharospasm did not respond differently from those without prior surgery.

A randomized clinical trial comparing contact lens with intraocular lens correction of monocular aphakia during infancy: grating acuity and adverse events at age 1 year.

OBJECTIVE To compare the visual outcomes and adverse events of contact lens with primary intraocular lens (IOL) correction of monocular aphakia during infancy. METHODS In a randomized, multicenter (12 sites) clinical trial, 114 infants with a unilateral congenital cataract were assigned to undergo cataract surgery between 1 to 6 months of age either with or without primary IOL implantation. Contact lenses were used to correct aphakia in patients who did not receive IOLs. Grating visual acuity was tested at 1 year of age by a masked traveling examiner. MAIN OUTCOME MEASURE Grating visual acuity at 1 year of age. RESULTS The median logMAR visual acuity was not significantly different between the treated eyes in the 2 groups (contact lens group, 0.80; IOL group, 0.97; P = .19). More patients in the IOL group underwent 1 or more additional intraocular operations than patients in the contact lens group (63% vs 12%; P < .001). Most of these additional operations were performed to clear lens reproliferation and pupillary membranes from the visual axis. CONCLUSIONS There was no statistically significant difference in grating visual acuity at age 1 year between the IOL and contact lens groups; however, additional intraocular operations were performed more frequently in the IOL group. APPLICATION TO CLINICAL PRACTICE Until longer-term follow-up data are available, caution should be exercised when performing IOL implantation in children aged 6 months or younger given the higher incidence of adverse events and the absence of an improved short-term visual outcome compared with contact lens use.