Pseudotumor cerebri in children receiving recombinant human growth hormone.

PURPOSE: This article represents the first report in the ophthalmology literature of an association between pseudotumor cerebri (PTC) and recombinant human growth hormone (rhGH). DESIGN: Noncomparative case series. PARTICIPANTS: Three children receiving rhGH for short stature with Turner syndrome, Jeune syndrome, or Down syndrome. METHODS: Children underwent full ocular examination. After papilledema was identified, patients underwent lumbar puncture and imaging with either magnetic resonance imaging or computerized tomography. Treatment was under the guidance of the primary physician or neurosurgeon. The rhGH was discontinued in all children. MAIN OUTCOME MEASURES: Visual acuity and evaluation of the optic nerve for resolution of papilledema were followed at each examination. RESULTS: In all three cases, papilledema resolved with the cessation of rhGH, and treatment with acetazolamide or prednisone. Visual acuity was unchanged in case 1, decreased by two to three lines in case 2, and was inconsistent in case 3. One child (case 2) required a ventriculoperitoneal shunt for persistent elevation of intracranial pressure. CONCLUSION: There appears to be a causal relationship between the initiation of rhGH with the development of PTC. Children should have a complete ophthalmic evaluation if they report headache or visual disturbances. Baseline examination with routine follow-up should be instituted when children cannot adequately communicate.

Strabismus in premature infants in the first year of life. Cryotherapy for Retinopathy of Prematurity Cooperative Group.

OBJECTIVES: To present the 3- and 12-month strabismus data from 3030 premature infants with birth weights less than 1251 g enrolled in the Multicenter Trial of Cryotherapy for Retinopathy of Prematurity. DESIGN: Data from the 3- and 12-month examinations conducted at 23 regional study centers were tabulated for all infants. The main outcome measure, ocular motility, was compared with baseline demographic variables and retinopathy of prematurity severity for the worse eye. Findings at 3 months were compared with the incidence of strabismus at 12 months. RESULTS: At 3 months, 200 (6.6%) of the 3030 infants were strabismic. In the 2449 infants examined at both time points, 289 (11.8%) were found to have strabismus at 12 months. Retinopathy of prematurity was significant for strabismus at both 3 and 12 months (P<.001). The presence of strabismus at 3 months was found to be a highly significant predictor of strabismus at 12 months. Anisometropia, abnormal fixation, and unfavorable retinal structure also were significant predictors of strabismus at 1 year. The total prevalence of strabismus in the first year of life was 14.7%. CONCLUSION: The presence of acute-phase retinopathy of prematurity places the premature infant at increased risk for strabismus.

Occlusion and levodopa-carbidopa treatment for childhood amblyopia.

PURPOSE: The purpose of the current study was to compare the effects of levodopa-carbidopa with and without part-time occlusion on visual function in older amblyopic children. METHODS: Thirteen older amblyopic children were randomly assigned to receive or not receive part-time occlusion (3 h/day) combined with 7 weeks of oral dosing with levodopa-carbidopa (1.02 mg/0.25 mg/kg body weight three times daily). Visual acuity, contrast sensitivity, and fusion were measured at baseline; 1, 3, 5, and 7 weeks during the treatment regimen; and 4 weeks after termination of all treatment. At these same times health status was assessed with standard laboratory blood tests, physical examination, and subjective questionnaire. RESULTS: From baseline to the follow-up test trial, both groups improved in visual acuity in the amblyopic eyes (occlusion group 20/116 to 20/76, P < .001; no occlusion group 20/90 to 20/73, P < .01) and dominant eyes (occlusion group 20/18 to 20/15, P > .05; no occlusion group 20/20 to 20/16, P < .01). The occlusion group exhibited a significant decrease in the difference in acuity between the dominant and amblyopic eyes of 1.3 lines (P < .02), whereas the no occlusion group revealed no significant effect. A comparison between groups revealed a significantly greater improvement in visual acuity in the amblyopic eye in the occlusion group compared with the no occlusion group (P = .01). In contrast, there was no significant difference between groups in terms of the change in visual acuity in the dominant eye (P = .15). Mean log contrast sensitivity in the amblyopic eye significantly improved in the occlusion group and did not significantly change in the no occlusion group. Fusion changed similarly in both groups. The improvements in visual function were maintained 4 weeks after the termination of all treatment. Adverse side effects were minimal in both groups. CONCLUSION: The combination of levodopa-carbidopa and occlusion improves visual function more than levodopa-carbidopa alone in older amblyopic children.

Levodopa-carbidopa and childhood retinal disease.

PURPOSE: Our purpose was to determine the influence of levodopa-carbidopa on visual function in children with retinal disease. METHOD: Two studies were undertaken, a single-dose study and a longitudinal dosing study. A double-masked, placebo controlled single-dose study was undertaken of levodopa-carbidopa (2.08 mg/kg of body weight levodopa with 25% carbidopa) on monocular visual acuity in 14 children with retinal disease. Subjects received two capsules approximately 2.5 hours apart, and monocular visual acuity was measured 2 hours after each capsule ingestion. The second study was a double-masked, placebo-controlled 12-week longitudinal dosing (0.62 mg/kg of body weight) crossover study in which subjects received levodopa-carbidopa for 6 weeks and placebo for 6 weeks. RESULTS: The single-dose study revealed a small but statistically significant improvement in monocular visual acuity after levodopa-carbidopa ingestion. The longitudinal study revealed a small but statistically significant improvement in binocular visual acuity after levodopa ingestion. In both studies placebo had no significant effect on visual acuity. Six subjects participated in both studies and demonstrated a significant correlation (r = 0.76, p < 0.05) between change in visual acuity in the single-dose study and the longitudinal dosing study. CONCLUSION: The results are consistent with the hypothesis that dopamine influences the receptive field characteristics of retinal cells. The results also suggest that there may be low retinal dopamine levels in some types of retinal disease, which may be amenable to treatment.

A comparison of cryotherapy versus diode laser retinopexy in 100 consecutive infants treated for threshold retinopathy of prematurity.

PURPOSE: The purpose of this paper is to present a series of patients who were treated for threshold retinopathy of prematurity with either cryotherapy or diode laser. Complications and unfavorable outcomes during the first year after treatment will be compared for the two procedures. METHODS: The clinical courses of a consecutive series of 100 infants (192 eyes) were reviewed. All infants had their threshold status confirmed by a second examiner. Infants were treated with cryotherapy through 1993 and with diode laser thereafter. One hundred two eyes of 54 patients were treated with cryotherapy. Ninety eyes of 46 patients were treated with laser retinopexy. Two of the patients who were treated with laser (4 eyes) did not survive to the 3-month follow-up visit, and their results are not included here. The two groups of infants were comparable in their birth weight, adjusted gestational age at treatment, and severity of disease as determined by zone and sectors of stage 3 retinopathy of prematurity. RESULTS: Unfavorable outcome (total retinal detachment) was seen in 25.4% of eyes treated with cryotherapy (26 of 102), as compared with 15% of eyes treated with laser (13 of 86). Two cataracts were seen in our patients: one patient 22 weeks after cryotherapy, and the other 7 months after diode laser. CONCLUSIONS: No statistically significant difference was found in the rate of retinal detachments in the two groups (X2 = 3.05; P = .08).

Levodopa/carbidopa treatment for amblyopia in older children.

The purpose of the present study is to determine how long visual function improves during levodopa/carbidopa with part-time occlusion treatment in older amblyopic children. A 7-week open clinical trial of levodopa (0.55 mg/kg, three times daily) with 25% carbidopa combined with part-time occlusion (3 hours/day), was undertaken in 15 older (mean age, 8.87 years) amblyopic children. Visual acuity, fusion, and stereo acuity were measured at baseline, at weeks 1, 3, 5, and 7 during treatment, and 6 weeks following the end of all treatment. Health status was assessed by physical examination, questionnaire, and standard laboratory tests (CHEM 20, complete blood cell count [CBC], and differential). The results revealed that visual acuity in the amblyopic eye improved for 5 weeks, from 20/170 at baseline to 20/107, then stabilized for the last 2 weeks. Visual acuity also improved in the dominant eye by 0.6 lines, from 20/19 to 20/16. At the 6-week follow up, visual acuity in the amblyopic eye remained at 20/107 (paired t = 4.78, df = 14, P < .001), a 37% improvement. Thirty-three percent of the subjects demonstrated improved fusion from baseline to the 7-week trial that was maintained at follow up (chi 2 = 3.97, P < .05). Stereo acuity did not significantly change. Physical exam (blood pressure, body temperature, respiration, heart rate) and standard laboratory tests remained normal and side effects were minimal.(ABSTRACT TRUNCATED AT 250 WORDS)

Retinopathy of prematurity in discordant twins.

Discordant twins may be at increased risk for retinopathy of prematurity (ROP) because of factors related to their unequal growth. Discordancy is defined as a difference of 15% or more in the birth weights of the two infants. We examined the data in 26 sets of discordant twins from six NICUs, including birthweight, gestational age, sex, and highest grade of ROP. Thirty-eight percent (10) of the lower birthweight infants had higher grades of ROP than their twin. Twenty-three percent (6) of the heavier birthweight twins had higher grades of ROP than their smaller siblings. Three infants reached threshold, and five were prethreshold. In every case, their twin siblings had mild or no ROP. Thirty-eight percent of the twins (10) had the same ROP outcome.

Flash visual evoked response binocular summation in normal subjects and in patients with early-onset esotropia before and after surgery.

Flash visual evoked responses were recorded and visual evoked response binocular summation was assessed in normal children between the ages of 1 and 58 months, in normal adults and in children with early-onset esotropia before and longitudinally for 1 year after surgical binocular alignment. Normal flash visual evoked response binocular summation started in the range of facilitation (> 2.0) at 1 month of age and decreased to adult levels by 3.7 months of age. The shape of the flash visual evoked response binocular summation function obtained from the patients with early-onset esotropia, appeared similar to that of normal subjects; however, the rapid decrease in flash visual evoked response binocular summation from facilitation to normal adult levels occurred after surgical binocular alignment. In normal adults, flash visual evoked response binocular summation was significantly reduced by a 40-diopter base-in prism, suggesting that binocular misalignment was not the reason for the facilitation in flash visual evoked response binocular summation in either childhood population. It is proposed that this facilitation may reflect a process that leads to binocularity and that develops rapidly with binocular alignment.

SKILL card results in amblyopic children.

Two experiments were undertaken to assess high contrast-high luminance (HCHL) and low contrast-low luminance (LCLL) visual acuity as measured with the Smith-Kettlewell Institute Low Luminance (SKILL) card, in childhood amblyopia. In experiment 1, HCHL and LCLL visual acuity was measured in 11 amblyopic children and in 12 normal children of similar age. The results revealed that HCHL visual acuity was similar in the normal eyes and in the amblyopes' dominant eyes, and was abnormal in 91% of the amblyopic eyes. LCLL visual acuity was significantly worse in normal eyes compared to the amblyopes' dominant eyes, and was abnormal in only 64% of the amblyopic eyes. In experiment 2, HCHL and LCLL visual acuity was measured in seven previously tested amblyopic children, after a 5-month interval, and in six additional amblyopic children. In the amblyopic children who were tested twice, significant test-retest reliability for HCHL and LCLL visual acuity was found and visual acuity varied by 3.6%. The results of experiment 2 corroborated the results of experiment 1 from the amblyopic children. It is concluded that visual acuity in the amblyopic eye tends to normalize under low contrast-low luminance conditions. In addition, the mechanism(s) underlying amblyopia appear also to influence the dominant eye, resulting in superior visual acuity under low contrast-low luminance conditions.

Longitudinal study of levodopa/carbidopa for childhood amblyopia.

To determine the tolerability and efficacy of levodopa/carbidopa combined with occlusion therapy for childhood amblyopia, a double-masked placebo-controlled randomized longitudinal study was performed on 10 amblyopic children between 6 and 14 years of age. Subjects received, on average, 20/5 mg levodopa/carbidopa or 20 mg of placebo three times per day combined with part-time occlusion over a 3-week period. Visual function was assessed by Early Treatment Diabetic Retinopathy Study (ETDRS) visual acuity charts and by contrast sensitivity. Tolerability was assessed by questionnaire, SMAC (standard laboratory tests; consists of a chem 20 and complete blood count [CBC]) analysis, and physical examination. Compliance with occlusion and capsule consumption were assessed by questionnaire and by capsule count, respectively. At the end of the dosing regimen, the levodopa/carbidopa group significantly improved in visual acuity by 2.7 lines and in mean contrast sensitivity by 72% in the amblyopic eye. The placebo group improved in visual acuity by 1.6 lines in the amblyopic eye. Tolerability and occlusion compliance were similar between groups; however, capsule ingestion compliance was significantly lower in the levodopa/carbidopa group. One month after the termination of treatment, the levodopa/carbidopa group maintained a significant 1.2-line improvement in visual acuity and 74% improvement in contrast sensitivity in the amblyopic eye. The placebo group did not maintain an improvement in visual acuity between the eyes. It is concluded that levodopa/carbidopa, at an average of 0.48/0.12 mg/kg, is well tolerated and, when combined with part-time occlusion, is efficacious in improving visual function in amblyopic children.

Levodopa/carbidopa for childhood amblyopia.

PURPOSE: To evaluate the efficacy and tolerance of two low doses of levodopa/carbidopa (25/6.25 mg, 50/12.5 mg) and placebo (Tums) in 20 children with amblyopia between the ages of 4 and 14 years. METHODS: A double-masked placebo-controlled randomized 8-hour study was performed during which subjects received one of two doses of levodopa/carbidopa or placebo, combined with occlusion of the dominant eye. Visual acuity was measured at baseline and at 1 and 5 hours after capsule ingestion. Tolerance was assessed by questionnaire and physical examination. RESULTS: Visual acuity significantly improved by one line, from an overall average of 20/121 to 20/96, in the amblyopic eyes of both groups that received levodopa/carbidopa. Visual acuity did not significantly change in the placebo group. Tolerance was similar among all three groups. CONCLUSION: Average dose levels of 0.95/0.24 mg/kg and 1.94/0.49 mg/kg of levodopa/carbidopa were found to be well tolerated and efficacious at temporarily improving visual acuity in amblyopic eyes of children.

A prospective study of ocular abnormalities in hearing impaired and deaf students.

In a prospective study of 505 hearing impaired and deaf students conducted at Children's Hospital Eye Clinic, 48.7% were found to have significant ocular abnormalities. The prevalence of myopia, astigmatism, and pathological intraocular changes was found to be significantly increased over the general population. In addition, the prevalence of ocular abnormalities generally increased with the severity of the hearing loss. Of importance was the high prevalence of rubella-consistent abnormalities in the hearing impaired and deaf student populations. Rubella syndrome may be responsible for a major portion of high refractive errors and ocular pathological changes. It is recommended that ENT specialists and pediatricians be aware of the nearly 50% chance of ocular abnormality in hearing impaired and deaf students, and that ophthalmologic examination may be valuable for identification or confirmation of the etiology of hearing impairment.

Levodopa and childhood amblyopia.

A pilot study was undertaken to address the tolerance and efficacy of levodopa/carbidopa treatment for amblyopia in older amblyopic children who failed to respond to conventional occlusion therapy. Five amblyopic children, between the ages of 7 and 12 years, and two normal adults were given between 100 mg/25 mg and 400 mg/100 mg of levodopa/carbidopa, respectively, depending on body weight. A symptoms questionnaire was completed, with temperature, respiration, heart rate, and blood pressure taken periodically to assess tolerance. Blood samples were taken, via a heparin well, to assess the pharmacokinetics of levodopa, dopamine, noradrenaline, and DOPAC. Snellen visual acuity, contrast sensitivity, stereo acuity, and pattern VERs were measured periodically to assess efficacy. The results revealed a high prevalence of side effects including emesis and nausea (four of seven subjects). Pharmacokinetics revealed that maximum serum levels of levodopa occurred 30 minutes to 1 hour after drug ingestion and decreased by 50% after 2 to 4 hours. One hour after drug ingestion, Snellen visual acuity temporarily improved from an average of 20/159 to 20/83 in the amblyopic eyes. Contrast sensitivity and pattern VERs (10-minute checks) temporarily improved in both dominant and amblyopic eyes, whereas visual function remained stable in normal eyes. The improvements in visual function started to decrease 5 hours after drug ingestion. The results are discussed in the context of developing a therapeutic trial of levodopa/carbidopa for childhood amblyopia.

Electro-oculogram in vitamin A deficiency associated with cystic fibrosis. Short communication.

Electro-oculograms (EOGs) were recorded in a patient with cystic fibrosis and vitamin A deficiency before and during vitamin A supplementation (25,000 IU/day). Before vitamin A supplementation the EOG Arden light/dark ratio was 1.27. After seven months of vitamin A supplementation the Arden ratio increased to 3.0. These results reveal that vitamin A deficiency can cause an abnormal EOG.

Loss of contrast sensitivity in cystic fibrosis.

We measured the contrast sensitivity function in a 16-year-old boy with cystic fibrosis, before and during vitamin A supplementation. Before vitamin A supplementation, serum levels of vitamin A were abnormally low, the electroretinogram was reduced, and contrast sensitivity was abnormally low at all spatial frequencies. During vitamin A supplementation (25,000 IU/day), serum levels of vitamin A became low normal, the electroretinogram returned to normal, and the overall contrast sensitivity function improved by 94%. We propose that the contrast sensitivity function may be abnormal in patients with cystic fibrosis who have reduced retinal function secondary to vitamin A deficiency.

CSF interocular interactions in childhood ambylopia.

Contrast sensitivity functions (CSF's) were measured in the amblyopic and dominant eyes of 17 strabismic and 28 anisometropic children and in 19 similar age normal controls. A three-alternative forced-choice procedure was used to measure CSF's with the VCTS 6500. The results revealed reduced contrast sensitivity (CS) in both the amblyopic and dominant eyes of strabismic and anisometropic amblyopes compared to normal controls. Statistically significant intereye correlations of CS at each spatial frequency were found in all groups and in the presence of deep amblyopia, suggesting continued interocular interactions and binocularity. A separate longitudinal study of 7 of the amblyopes showed that, during the course of occlusion therapy, both the amblyopic and the dominant eyes improved in CSF. The results suggest that the amblyopic eye may influence CS in the dominant eye through interocular interactions. This process may serve to minimize CSF differences between the eyes and maximize binocular vision.

Contrast sensitivity of optokinetic nystagmus.

To determine the threshold characteristics of optokinetic nystagmus (OKN), contrast thresholds for involuntary OKN were measured for gratings of different spatial frequency to yield an OKN-contrast sensitivity function (OKN-CSF). The OKN-CSF resembled an inverted U-shaped function with temporal-to-nasal and nasal-to-temporal movement yielding similar functions. In addition, when psychophysical CSFs were determined for separate form and movement thresholds, it was discovered that the OKN-CSF approximated the psychophysical-movement CSF rather than the psychophysical-form CSF.

Visual-evoked response binocular summation in normal and strabismic infants. Defining the critical period.

Pattern visual-evoked response binocular summation (VERBS) was recorded in normal infants, between the ages of 1-58 months, and in similar-aged esotropic infants before and at various times after corrective surgery. The normal subjects had no significant VERBS at 1.5 months of age, developed a rapid acceleration of VERBS between 1.5-3 months, and then gradually declined in VERBS from 3-58 months. The peak of the VERBS by age function at 3 months was well in the facilitation range (greater than 2.0) and corresponded to the general age range for the onset of binocular eye alignment, fusion, and stereopsis. The results from the early-onset esotropic patients revealed a similar function to that found in normal subjects; however, the function was triggered by surgical eye alignment. The peak of the VERBS function for esotropic subjects was lower than normal, and the initial rise was less rapid. It is proposed that the VERBS function reflects the human critical period for the development of binocular vision. In this framework, data from both infant developmental studies and adult studies were clarified.

The predictability of infant visual-evoked response testing on future visual acuity.

We reviewed the records of 27 infants with abnormal eye examinations and visual-evoked response (VER) testing (mean age, 10.5 months) who subsequently underwent a long-term follow-up ophthalmology examination (mean duration, 41 months). The infants were initially diagnosed with various ocular disorders including cortical blindness (eight), optic nerve hypoplasia (six), congenital cataract (two), and retinopathy of prematurity (one). Standard optotype visual-acuity determinations were available in the follow-up records of 11 children (21 eyes), and fixation behavior was obtained in the remaining 16 children (32 eyes). Results revealed that pattern-reversal VER P1 latency was predictive (87%) of whether visual acuity was equal to (or better than) or worse than 20/100 and whether a patient would have good fixation behavior (fix and follow, FF) or poor fixation (no FF) (86%) (P less than .001). Although flash VER P1 latency was also predictive of later visual acuity or good fixation (73%), it was not statistically significant. Pattern VER P1 amplitude and flash VER P1 amplitude were not predictive of later visual function. The predictive power of pattern VER P1 latency for later visual function probably relates to its reflection of macular function and low variability. An analysis of the variability of each of the four VER factors in normal infants (n = 50) indicated that pattern VER P1 latency was the least variable, and consequently most sensitive, VER factor for detecting and quantifying pathology. Overall, the results of this retrospective study suggest that pattern VER P1 latency may have important predictive power for later visual function in infants with an initially abnormal ophthalmologic examination.