George S. Ellis

Low-dose Methotrexate in the Treatment of Severe Juvenile Rheumatoid Arthritis and Sarcoid Iritis

OBJECTIVE To assess the efficacy of low-dose oral methotrexate (MTX) therapy for children with severe iritis. METHODS MTX in a weekly dose of 7.25 to 12.5 mg/m2 was administered orally to four patients (two with juvenile rheumatoid arthritis [JRA] and two with sarcoidosis) with severe iritis not adequately controlled by topical and systemic corticosteroid therapy. The treatment was initiated with half of the total dose and increased every 2 weeks until the final dose was reached. Iritis was graded from 0 to +4 according to the density of cells in the anterior chamber of the eye. RESULTS There were three girls and one boy with a mean age of 10.5 years. Two patients were African American and two were Caucasian. The mean age at onset of iritis was 6 years. The mean duration of MTX therapy was 28.8 months. Significant improvement was noted in two of the four patients in ocular inflammation, demonstrated by reduction of cell density from +4 to +1. Two patients had a mild improvement of the iritis. However, corticosteroids were significantly reduced in all patients. One patient was completely off steroids within 30 months of MTX therapy. In the remaining three cases, the steroid dose was successfully tapered from 0.82 mg/kg/d to 0.15 mg/kg/d (mean doses) within a mean duration of 20 months. No side effects were observed with MTX therapy. CONCLUSION Low-dose MTX therapy was effective and safe, and displayed steroid-sparing properties in four children with severe iritis.

Teller Acuity Cards Versus Clinical Judgment in the Diagnosis of Amblyopia with Strabismus

Teller acuity card testing, which is a form of the preferential-looking procedure, is a popular way of assessing visual acuity in preverbal patients. The authors suspected that the clinical judgment of a pediatric ophthalmologist is superior to the Teller acuity cards in diagnosing amblyopia when strabismus is present. Acuity card and fixation preference measurements on each eye were compared at the same clinical visit in a group of 108 strabismic patients. The authors found that the acuity cards could be used to detect amblyopia. However, the pediatric ophthalmologist was more sensitive in diagnosing amblyopia than the Teller acuity cards in the presence of strabismus.

Myelinated Nerve Fibers, Axial Myopia, and Refractory Amblyopia: An Organic Disease

Six patients with the syndrome of myelinated nerve fibers, high axial myopia, and refractory amblyopia were studied with particular emphasis on their sensory status. Ocular examination included keratometry, B-scan ultrasonography, measurement of axial lengths, and fundus photography. Sensory testing included visual fields, photostress testing, and neutral density filter visual acuities. Electrophysiological testing was also performed on some patients. Our findings suggest an organic etiology for the poor vision, although all patients had superimposed functional amblyopia as well. We suggest that myelination about the macula is the most likely cause of this poor vision and that the myelination, in some way, may have stimulated the development of the high myopia.

Epikeratophakia in children with traumatic cataracts.

Epikeratophakia provides a permanent optical correction for aphakia in children with congenital or traumatic cataracts; suturing the epikeratophakia graft onto the cornea eliminates the problems of contact lens or spectacle non-compliance in these young and generally uncooperative patients and provides tectonic support to scarred and irregular corneas. Eighteen children under the age of six years underwent epikeratophakia for the correction of aphakia after the removal of trauma-induced cataracts. Graft success rate was 88%; the average change in keratometry in the patients with successful grafts was 14.82 +/- 2.0 diopters. In the 13 patients eligible for visual acuity tabulation, preoperative acuities ranged from light perception to 20/200, and postoperative acuities ranged from hand motions to 20/30. Ten (77%) had acuities of 20/80 or better. Poor results in three patients with less than 20/200 acuities were likely the results of non-compliance with amblyopia therapy. Present work indicates that in cases of traumatic cataract, the epikeratophakia procedure facilitates amblyopia therapy and decreases the astigmatism in scarred and irregular corneas.

Bilateral idiopathic Brown's syndrome with delayed onset in the second eye.

PURPOSE We describe 6 cases of a previously unreported variation of bilateral Browns syndrome that presented in congenital form in one eye and developed later in the fellow eye with no underlying cause. METHODS We reviewed the clinical records of 6 patients from 6 separate practices to determine whether there were any common clinical features on presentation or in their clinical courses. RESULTS All 6 patients were diagnosed with unilateral congenital Browns syndrome at the first ophthalmologic assessment but showed no evidence of the syndrome in the fellow eye. In 5 cases the contralateral syndrome developed in the second eye after surgery was performed on the first eye, and in 1 case it developed before any surgery was done. The ages at onset of the syndrome in the second eye ranged from 2 to 8 years. None of the children had any evidence of systemic illness or local orbital disease to explain an acquired Browns syndrome. CONCLUSION To our knowledge, this is the first reported series of cases of bilateral Browns syndrome that manifested sequentially in the eyes with no known causes for an acquired syndrome in the second eye. This finding supports the premise that congenital and acquired Browns syndrome are on a continuum with a common pathophysiology of restriction of free movement of the superior oblique tendon in the trochlea.

Manifest Strabismus Following Pseudostrabismus Diagnosis

Introduction Pseudostrabismus is a common diagnosis in a busy pediatric ophthalmology practice. This purpose of this study was to determine the prevalence of true strabismus in patients previously diagnosed with pseudostrabismus, to determine risk factors, and to examine the sensory outcome. Methods This is a retrospective review of patients diagnosed with pseudostrabismus and with at least one follow-up visit. Age at first visit, gender, family history, birth weight, developmental history, who initially suspected strabismus and its direction, refractive error, and quality of examination were recorded. Diagnosis and sensory outcome were recorded for those patients who went on to develop a manifest deviation. Results Eighty-three patients met the inclusion criteria. Twelve percent (10/83) were later diagnosed with manifest strabismus. There was no statistically significant difference between those who went on to develop strabismus and those who did not with regard to any of the factors evaluated; however, there was a strong trend for higher risk in developmentally delayed subjects. Additionally, there was a trend for higher risk when poor patient cooperation or an inexperienced clinician precludes a good quality exam. Two subjects developed amblyopia before diagnosis. At the final exam, there was evidence of bifoveal fusion in five subjects and of peripheral fusion in three subjects. Conclusions The prevalence of strabismus was higher in patients diagnosed with pseudostrabismus than was reported for the general pediatric population. Therefore, patients diagnosed with pseudostrabismus might be considered “at risk”. With pseudostrabismus, management should reflect this increased risk of true strabismus. Confirmatory studies are needed.

The Acuity Card Procedure: Longitudinal Assessments

Traditional methods of visual assessment in preverbal pediatric patients rely on refined but subjective measurement techniques. A standard ophthalmologic examination includes evaluation of a childs fixation patterns, with performance ranked on the basis of ability to fix and follow an object (F & F) or maintain central, steady fixation (CSM). In the hands of a skilled clinician, these evaluations are important for diagnosis and treatment. Documentation of quantitative changes in visual abilities of preverbal patients, however, has only recently become feasible. We began using the acuity card procedure in our pediatric clinical practice more than 3 years ago. This assessment, a modified version of the standard Forced-Choice Preferential Looking paradigm (FPL), provides quantitative evaluation of visual functioning in preverbal patients. The total number of patients assessed on one or more occasions exceeds 900. Of this group, we followed 83 patients with at least four acuity card evaluations on separate visits. Thirty of these patients, all with different diseases, have been evaluated with acuity cards on six or more visits. We found the information provided by the acuity card assessments extremely helpful in quantifying the developmental and therapeutic changes in vision, previously monitored only qualitatively.

Management of Central Sensory Deficits Following Trauma

Trauma can disrupt single binocular vision by interfering with the sensory requirements for fusion. Injuries to the eye that can interfere with sensory fusion by disrupting the fusion stimulus (image) are discussed. Central disruptions of fusion that can occur with head injury are described along with diagnostic techniques and management strategies for the associated diplopia.

Approach to Visual Acuity Assessment and Strabismus Evaluation of the Pediatric Patient

Children present unique ophthalmologic considerations and require an examination approach that corresponds to his or her level of visual and psychosocial development. Employment of age-related, patient-specific strategies may be utilized to maximize the information obtained from the clinical examination as well as make the examination both enjoyable and rewarding for the patient, family, and practitioner. This chapter will discuss the approach to the ophthalmologic examination in pediatric patients including visual assessment, motility, strabismus, and motor fusion.