William V. Good

The incidence and course of retinopathy of prematurity: findings from the early treatment for retinopathy of prematurity study.

Objectives. To estimate the incidence of retinopathy of prematurity (ROP) in the Early Treatment for Retinopathy of Prematurity (ETROP) Study and compare these results with those reported in the Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) Study. Methods. The ETROP Study, as part of its protocol, screened 6998 infants at 26 centers throughout the United States. Serial eye examinations were conducted for infants born weighing <1251 g, making it possible to estimate the frequency of ROP in different birth weight and gestational age categories. ROP was categorized according to the International Classification for ROP. Results. The incidence of any ROP was 68% among infants of <1251 g. The findings were compared with those for infants born in 1986 and 1987 in the CRYO-ROP Study. The overall incidences of ROP were similar in the 2 studies, but there was more zone I ROP in the ETROP Study. Among infants with ROP, more-severe ROP (prethreshold) occurred for 36.9% of infants in the ETROP Study and 27.1% of infants in the CRYO-ROP Study. The gestational age of onset of ROP of different severities has changed very little since the CRYO-ROP Study was conducted. Conclusions. ROP remains a common important problem among infants with birth weights of <1251 g. The incidence of ROP, time of onset, rate of progression, and time of onset of prethreshold disease have changed little since the CRYO-ROP natural-history study.

Screening Examination of Premature Infants for Retinopathy of Prematurity

This statement revises a previous statement on screening of preterm infants for retinopathy of prematurity (ROP) that was published in 2006. ROP is a pathologic process that occurs only in immature retinal tissue and can progress to a tractional retinal detachment, which can result in functional or complete blindness. Use of peripheral retinal ablative therapy by using laser photocoagulation for nearly 2 decades has resulted in a high probability of markedly decreasing the incidence of this poor visual outcome, but the sequential nature of ROP creates a requirement that at-risk preterm infants be examined at proper times and intervals to detect the changes of ROP before they become permanently destructive. This statement presents the attributes on which an effective program for detecting and treating ROP could be based, including the timing of initial examination and subsequent reexamination intervals.

Cortical visual impairment in children

Cortical visual impairment (CVI) in children is most commonly caused by peri- or post-natal hypoxia-ischemia, but may also occur following other insults, e.g., trauma, epilepsy, infections, drugs or poisons, and certain neurologic diseases. The disorder differs considerably in etiology, physical findings, and, perhaps, prognosis, from the cortical blindness seen in adults. The same event that causes CVI by damaging the geniculate and/or extrageniculate visual pathways may also damage other areas of the brain, or the retina, optic nerves, or chiasm. Thus, children with CVI often have other neurological problems. Diagnosis may require the participation of a multidisciplinary team and the use of special visual testing techniques. Due to the uncertainty concerning the prognosis in CVI, clinicians should remain optimistic about the childs potential for some vision recovery.

Chronic cortical visual impairment in children: aetiology, prognosis, and associated neurological deficits

BACKGROUND/AIMS To evaluate prevalence, aetiology, prognosis, and associated neurological and ophthalmological problems in children with cortical visual impairment (CVI). METHODS The records of 7200 outpatients seen in the paediatric ophthalmology practice over the past 15 years were reviewed in order to compile data concerning CVI. In addition, the authors devised and applied a system for grading visual recovery in order to assess prognosis. RESULTS CVI occurred in 2.4% of all patients examined. The four most common causes of CVI were perinatal hypoxia (22%), cerebral vascular accident (14%), meningitis (12%), and acquired hypoxia (10%). Most children with CVI had associated neurological abnormalities. The most common were seizures (53%), cerebral palsy (26%) hemiparesis (12%), and hypotonia (5%). Associated ophthalmological problems were esotropia (19%), exotropia (18%), optic nerve atrophy (16%), ocular motor apraxia (15%), nystagmus (11%), and retinal disease (3%). On average, CVI patients improved by two levels as measured by the authors’ scale. CONCLUSION The majority of children with CVI showed at least some recovery. In this group of children, CVI is often accompanied by additional ophthalmological problems and is nearly always associated with other, serious neurological abnormalities.

Recent advances in cortical visual impairment

Cortical visual impairment (CVI) is the leading cause of bilateral visual impairment in children in Western countries1–3. This finding reflects better methods for identifying visual impairment due to CNS injury and also advances in perinatal care, which have increased the survival rate of children with neurological morbidity. This review will describe advances in the diagnosis and management of CVI. Central to our discussion is a definition of CVI that includes a decrease in visual acuity. New treatment and rehabilitative measures are badly needed for this disorder. We hope to stimulate interest in CVI, a disease that has become a significant public health problem. The incidence of CVI is increasing3. In a study of five Nordic countries, Rosenberg and coworkers5 noted that brain damage accounts for a growing number of cases of childhood visual impairment. They suggested that better medical care has lowered the mortality rate of children with severe medical problems. Although CVI alone is not life threatening, its associated neurological disorders may have been fatal in the past. In one study from Chile, 2.1% of children enrolled in schools for the blind, who are either visually impaired or blind, had visual diagnoses involving CVI, although this may be an underestimate6. In another study from Liverpool, Rogers7 found that CVI was the most common cause of visual impairment in children with associated neurological disorders (49% of the study population). The Oxford Register of Early Childhood Impairments8 reports the overall incidence of bilateral vision impairment at 0.14%, with 29.5% of cases due to CVI and 14.1% due to nystagmus: the second major cause of impairment in this study population. In Northern California, CVI was also found to be the leading cause of visual impairment in children under the age of 5 years9.

Outcome of Eyes Developing Retinal Detachment During the Early Treatment for Retinopathy of Prematurity Study (ETROP)

OBJECTIVE To describe the structural and visual outcomes at age 6 years of retinal detachment (RD) from retinopathy of prematurity (ROP) in the Early Treatment for Retinopathy of Prematurity (ETROP) study. METHODS Prospective multicenter nonrandomized series of infants with high-risk prethreshold ROP who developed an RD by 6 months corrected age treated with observation or vitreoretinal surgery. RESULTS Of 401 patients, 63 (89 eyes) experienced RD. Follow-up at age 6 years was available for 70 eyes (79%) of 49 surviving patients. The RDs were stage 4A in 28 eyes (40%), stage 4B in 14 (20%), stage 5 in 13 (19%), and not classified in 15 (21%). The macula was attached in 17 of 50 eyes (34%) after vitrectomy with or without scleral buckle, in 6 of 9 (67%) after scleral buckle only, and in 2 of 11 eyes (18%) observed. An attached macula at age 6 years after vitreoretinal surgery was present in 5 of 16 eyes (31%) with stage 4A, 6 of 10 (60%) with stage 4B, and 0 of 10 with stage 5. Favorable visual acuity (>20/200) was found in 6 of 70 eyes (9%); 5 had stage 4A, and 1 was not classified. CONCLUSIONS Macular attachment was achieved in approximately one-third of eyes with RD and favorable visual acuity in some eyes with stage 4A.

Grating visual acuity results in the early treatment for retinopathy of prematurity study

OBJECTIVE To compare grating (resolution) visual acuity at 6 years of age in eyes that received early treatment (ET) for high-risk prethreshold retinopathy of prematurity (ROP) with that in eyes that underwent conventional management (CM). METHODS In a randomized clinical trial, infants with bilateral, high-risk prethreshold ROP (n = 317) had one eye undergo ET and the other eye undergo CM, with treatment only if ROP progressed to threshold severity. For asymmetric cases (n = 84), the high-risk prethreshold eye was randomized to ET or CM. MAIN OUTCOME MEASURE Grating visual acuity measured at 6 years of age by masked testers using Teller acuity cards. RESULTS Monocular grating acuity results were obtained from 317 of 370 surviving children (85.6%). Analysis of grating acuity results for all study participants with high-risk prethreshold ROP showed no statistically significant overall benefit of ET (18.1% vs 22.8% unfavorable outcomes; P = .08). When the 6-year grating acuity results were analyzed according to a clinical algorithm (high-risk types 1 and 2 prethreshold ROP), a benefit was seen in type 1 eyes (16.4% vs 25.2%; P = .004) undergoing ET, but not in type 2 eyes (21.3% vs 15.9%; P = .29). CONCLUSION Early treatment of eyes with type 1 ROP improves grating acuity outcomes, but ET for eyes with type 2 ROP does not. APPLICATION TO CLINICAL MEDICINE: Type 1 eyes should be treated early; however, based on acuity results at 6 years of age, type 2 eyes should be cautiously monitored for progression to type 1 ROP. Trial Registration clinicaltrials.gov Identifier: NCT00027222.

Validity of the convergence insufficiency symptom survey: a confirmatory study.

Purpose. The objectives of the present study were to evaluate whether investigator bias influenced the Convergence Insufficiency Symptom Survey (CISS) scores of children with normal binocular vision (NBV) in our original validation study, reevaluate the usefulness of the cutoff score of 16, and reexamine the validity of the CISS. Methods. Six clinical sites participating in the Convergence Insufficiency Treatment Trial (CITT) enrolled 46 children 9 to <18 years with NBV. Examiners masked to the child’s binocular vision status administered the CISS. The mean CISS score was compared with that from the children with NBV in the original, unmasked CISS study and also to that of the 221 symptomatic convergence insufficiency (CI) children enrolled in the CITT. Results. The mean (±standard deviation) CISS score for 46 subjects with NBV was 10.4 (±8.1). This was comparable with our prior unmasked NBV study (mean = 8.1 (±6.2); p = 0.11) but was significantly different from that of the CITT CI group (mean = 29.8 ± 9.0; p < 0.001). Eighty-three percent of these NBV subjects scored <16 on the CISS, which is not statistically different from the 87.5% found in the original unmasked study (p = 0.49). Conclusions. Examiner bias did not affect the CISS scores for subjects with NBV in our prior study. The CISS continues to be a valid instrument for quantifying symptoms in 9 to <18-year-old children. These results also confirm the validity of a cut-point of ≥16 in distinguishing children with symptomatic CI from those with NBV.

Abnormalities of the Visual System in Infants Exposed to Cocaine

The authors describe 13 cocaine-exposed infants with optic nerve abnormalities, delayed visual maturation, and prolonged eyelid edema. Prolonged and potentially vision-threatening eyelid edema is a new clinical entity. The pharmacology of cocaine, its easy access to fetal circulation, and its neurotropic characteristics can be used to explain optic nerve abnormalities and delayed visual maturation. In infants with any of these eye abnormalities, a careful investigation for cocaine abuse is advisable.

Progression of myopia and high myopia in the Early Treatment for Retinopathy of Prematurity study: findings at 4 to 6 years of age.

PURPOSE To report the prevalence of myopia and high myopia in children <6 years of age born preterm with birth weights <1251 g who developed high-risk prethreshold retinopathy of prematurity and who participated in the Early Treatment for Retinopathy of Prematurity trial. METHODS Surviving children from the cohort of 401 participants who had developed high-risk prethreshold ROP in one or both eyes underwent cycloplegic retinoscopy at 6 and 9 months corrected age and yearly between 2 and 6 years postnatal age. Eyes were randomized to receive treatment at high-risk prethreshold ROP or conventional management with treatment only if threshold ROP developed. Myopia (spherical equivalent ≥0.25 D) or high myopia (≥5.00 D) in eyes at 4-, 5-, and 6-year examinations was reported. RESULTS At ages 4, 5, and 6 years, there was no difference in the percentage of eyes with myopia (range, 64.8%-69.9%) and eyes with high myopia (range, 35.3%-39.4%) between earlier treated and conventionally managed eyes. CONCLUSIONS Approximately two-thirds of eyes with high-risk prethreshold ROP during the neonatal period are likely to be myopic into the preschool and early school years. In addition, the increase in the proportion of eyes with high myopia that had been observed in both earlier-treated and conventionally managed eyes between ages 6 months and 3 years does not continue between ages 3 and 6 years.