Bradley V. Davitt

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.

Brief Report: Incidence of Ophthalmologic Disorders in Children with Autism.

Purpose To determine the incidence of ophthalmologic disorders in children with autism and related disorders. Design Retrospective chart review. Four hundred and seven children diagnosed with autism or a related disorder between 1998 and 2006. one hundred and fifty-four of these children completed a comprehensive ophthalmology exam by a pediatric ophthalmologist. Results Ophthalmologic pathology was found in 40% of patients with autism or a related disorder with 29% having significant refractive errors, 21% demonstrating strabismus, and 10% having amblyopia. Conclusions Children with autism or a related disorder will frequently have an ophthalmologic abnormality. Since cooperation with vision screening is understandably limited in these children, a comprehensive eye examination by a pediatric ophthalmologist is recommended for all such children.

Astigmatism in the Early Treatment for Retinopathy of Prematurity Study: findings to 3 years of age

PURPOSE To examine the prevalence of astigmatism (> or =1.00 diopter [D]) and high astigmatism (> or =2.00 D) at 6 and 9 months corrected age and 2 and 3 years postnatal age, in preterm children with birth weight of less than 1251 g in whom high-risk prethreshold retinopathy of prematurity (ROP) developed and who participated in the Early Treatment for Retinopathy of Prematurity (ETROP) Study. DESIGN Randomized, controlled clinical trial. PARTICIPANTS Four hundred one infants in whom prethreshold ROP developed in one or both eyes and who were randomized after they were determined to have a high risk (> or =15%) of poor structural outcome without treatment using the Risk Management of Retinopathy of Prematurity (RM-ROP2) program. Refractive error was measured by cycloplegic retinoscopy. Eyes with additional retinal, glaucoma, or cataract surgery were excluded. INTERVENTION Eyes were randomized to receive laser photocoagulation at high-risk prethreshold ROP (early treated [ET]) or to be conventionally managed (CM), receiving treatment only if threshold ROP developed. MAIN OUTCOME MEASURES Astigmatism and high astigmatism at each visit. Astigmatism was classified as with-the-rule (WTR; 75 degrees -105 degrees ), against-the-rule (ATR; 0 degrees -15 degrees and 165 degrees -180 degrees ), or oblique (OBL; 16 degrees -74 degrees and 106 degrees -164 degrees ). RESULTS The prevalence of astigmatism in ET and CM eyes was similar at each test age. For both groups, there was an increase in prevalence of astigmatism from approximately 32% at 6 months to approximately 42% by 3 years, mostly occurring between 6 and 9 months. Among eyes that could be refracted, astigmatism was not influenced by zone of acute-phase ROP, presence of plus disease, or retinal residua of ROP. Eyes with astigmatism and high astigmatism most often had WTR astigmatism. CONCLUSIONS By age 3 years, nearly 43% of eyes treated at high-risk prethreshold ROP developed astigmatism of > or =1.00 D and nearly 20% had astigmatism of > or =2.00 D. Presence of astigmatism was not influenced by timing of treatment of acute-phase ROP or by characteristics of acute-phase or cicatricial ROP. These findings reinforce the need for follow-up eye examinations in infants with high-risk prethreshold ROP. FINANCIAL DISCLOSURE(S) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Age and racial variation in central corneal thickness of preschool and school-aged children.

PURPOSE The purpose of this study was to determine variations in central corneal thickness (CCT) of preschool and school-aged African American and white children. Secondary aims were to assess possible correlations between CCT measurements and gender, axial length, intraocular pressure (IOP), family history of glaucoma, or history of prematurity. METHODS Contact ultrasound was used to measure CCT and axial length in 76 white and 60 African American children between the ages of 7 months and 18 years. A questionnaire was completed by the parents or guardians, including medical and family history. Statistically significant associations and differences were assessed using the independent t test, analysis of variance, and linear regression. All associations were defined as significant when the alpha value was less than 0.05 (two-tailed). RESULTS Mean CCT was thinner in African American children (535 +/- 35 microm) compared to white children (559 +/- 38 microm) (P < .001). The corneal thickness in children ages 10 to 18 years was significantly higher than in all other age groups in both African American (P = .03) and white (P < .005) children. No association was found between CCT and gender, axial length, IOP, or family history of glaucoma. Premature children had thinner CCT (536 +/- 40 pm) than full-term children (552 +/- 38 microm) (P = .009). CONCLUSIONS African American children have a thinner CCT compared to white children at all ages. Children of both racial groups have an increasing value of CCT with increasing age after approximately age 10 years. Children born prematurely have a thinner CCT than full-term children.

Bilateral inferior rectus muscle recession for correction of hypotropia in dysthyroid ophthalmopathy.

PURPOSE The restrictive myopathy of dysthyroid ophthalmopathy frequently results in strabismus and diplopia. The most common deviation is hypotropia. Ipsilateral inferior rectus muscle recession, the generally accepted treatment, may lead to a progressive marked overcorrection. The purpose of this study is to evaluate the results of bilateral asymmetric inferior rectus muscle operations with regard to late progressive overcorrection. METHODS A retrospective review of all patients undergoing bilateral inferior rectus muscle recession for dysthyroid ophthalmopathy between 1993 and 1997 found 8 patients with hypotropia resulting from dysthyroid ophthalmopathy. Alignment and motility were assessed preoperatively and postoperatively in all patients. Bilateral asymmetric inferior rectus muscle recession was performed on all patients with an adjustable suture performed on the hypotropic eye. A successful, long-term, postoperative result was defined as orthophoria or a vertical misalignment of less than or equal to 5 PD in primary gaze. RESULTS Data were collected from 8 patients at 4 to 6 weeks postoperatively. Six patients had successful alignment, and 2 patients remained undercorrected. Seven patients were successfully aligned, and 1 patient was undercorrected at the latest postoperative examination (mean, 18 months). CONCLUSION In our series, bilateral asymmetric inferior rectus muscle recession resulted in successful correction of hypotropia without late postoperative overcorrections in patients with dysthyroid ophthalmopathy.

Astigmatism progression in the early treatment for retinopathy of prematurity study to 6 years of age.

PURPOSE To examine the prevalence of astigmatism (≥ 1.00 diopter [D]) and high astigmatism (≥ 2.00 D) from 6 months after term due date to 6 years of age in preterm children with birth weight of less than 1251 g in whom high-risk prethreshold retinopathy of prematurity (ROP) developed and who participated in the Early Treatment for ROP study. DESIGN Observational cohort study. PARTICIPANTS Four hundred one infants in whom high-risk prethreshold ROP developed in 1 or both eyes and were randomized to early treatment (ET) versus conventional management (CM). Refractive error was measured by cycloplegic retinoscopy. Eyes were excluded if they underwent additional retinal, glaucoma, or cataract surgery. INTERVENTION Eyes were randomized to receive laser photocoagulation at high-risk prethreshold ROP or to receive treatment only if threshold ROP developed. MAIN OUTCOME MEASURES Astigmatism and high astigmatism at each study visit. RESULTS For both ET and CM eyes, there was a consistent increase in prevalence of astigmatism over time, increasing from 42% at 4 years to 52% by 6 years for the group of ET eyes and from 47% to 54%, respectively, in the CM eyes. There was no statistically significant difference between the slopes (rate of change per month) of the ET and CM eyes for both astigmatism and high astigmatism (P = 0.75). CONCLUSIONS By 6 years of age, astigmatism of 1.00 D or more developed in more than 50% of eyes with high-risk prethreshold ROP, and nearly 25% of such eyes had high astigmatism (≥ 2.00 D). Presence of astigmatism was not influenced by timing of treatment, zone of acute-phase ROP, or presence of plus disease. However, there was a trend toward higher prevalence of astigmatism and high astigmatism in eyes with ROP residua. Most astigmatism was with-the-rule (75°-105°). More eyes with type 2 than type 1 ROP had astigmatism by 6 years. These findings reinforce the need for follow-up eye examinations through early grade school years in infants with high-risk prethreshold ROP. FINANCIAL DISCLOSURE(S) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Sleep disorders in children with congenital anophthalmia and microphthalmia

PURPOSE The increased incidence of sleep disorders among blind patients has been documented in the sleep medicine literature. Blind patients lack the normal regulatory control of retinal input over their circadian rhythms, which can lead to abnormalities in their sleep-wake cycles. Our study was conducted to determine the incidence of sleep disorders in children with anophthalmia or microphthalmia and to offer therapeutic alternatives. METHODS A 13-question survey was distributed to families of children with anophthalmia, microphthalmia, or both identified through the Anophthalmia/Microphthalmia Registry in Philadelphia, Pennsylvania. The survey included questions regarding the childrens medical and ocular histories and any sleep disorders they may have experienced. Questions regarding daily schedules, family history, and social history were also included. RESULTS Surveys were returned from 13 children with bilateral anophthalmia or microphthalmia. Ten of 13 (77%) anophthalmic/microphthalmic children were reported to have frequent early-morning waking and extensive daytime sleeping. Specific medical and social problems did not appear to be associated with the development of these sleep disorders. Strict daily schedules were often helpful in entraining the childrens sleep-wake cycles. CONCLUSION Without the contribution of retinal input to help regulate circadian rhythms, most children with bilateral anophthalmia or microphthalmia will experience sleep disorders. These children may benefit from the introduction of strict daily schedules, medical therapy (melatonin), or both. An attempt should also be made to preserve any existing light perception.

Congenital microcephaly and chorioretinopathy due to de novo heterozygous KIF11 mutations: Five novel mutations and review of the literature

The microcephaly‐lymphedema‐chorioretinal dysplasia (MLCRD) syndrome is a distinct microcephaly syndrome. The hallmark features, microcephaly, chorioretinopathy, and lymphedema are frequently recognized at birth. Another clinical entity, the chorioretinal dysplasia, microcephaly and mental retardation syndrome (CDMMR) is a highly overlapping syndrome characterized by more variable lymphedema. Recently, heterozygous mutations in KIF11, a gene encoding a critical spindle motor protein of the Kinesin family, have been reported in individuals with MLCRD, and in individuals with CDMMR. This finding is suggestive of a single clinically variable spectrum. Here, we report on de novo novel mutations of KIF11 in five individuals with severe microcephaly, marked simplification of the gyral pattern on neuroimaging, bilateral chorioretinopathy, and developmental delay. Three patients had congenital lymphedema, and one had congenital bilateral sensorineural hearing loss. This report, therefore, further expands the clinical and molecular spectrum of KIF11‐associated microcephaly.

Abnormal Head Postures: A Review

Patients frequently present with abnormal head postures including face turns, head tilts, and chin elevation or depression. These may be related to an ocular or non-ocular cause. Since the etiology is not always obvious, these patients must be carefully evaluated. Most patients adopt head postures to avoid diplopia caused by incomitant strabismus. Treatment addresses the underlying strabismus, aligning the eyes in primary position and optimizing the field of single binocular vision. For those with nystagmus, treatment often involves placing the eyes into the null point. Less common causes of ocular torticollis include compensation for refractive errors, visual field defects, eyelid anomalies and cosmetic reasons.

The decreasing prevalence of uveitis associated with juvenile rheumatoid arthritis: do NSAIDs play a role?

We studied the prevalence and characteristics of chronic uveitis in a population of children diagnosed with juvenile rheumatoid arthritis (JRA). Uveitis is one of the most important, potentially debilitating extra-articular manifestations of JRA and has been observed in as many as 20% of cases. The medical records of 230 patients diagnosed with JRA and treated at a tertiary care hospital ophthalmology clinic between 1992 and 2000 were retrospectively reviewed.Seventeen patients (7.4%) were found to have clinical features of uveitis. There was a preponderance of female patients (16/17) and pauciarticular disease (13/17). Only 12 of 17 were ANA positive. Six had uveitis at diagnosis. Patients who were receiving naproxen had less incidence of uveitis compared with those receiving other nonsteroidal antiinflammatory drugs. Despite a relatively low prevalence of uveitis, complications occurred in about 24% (4/17) of the patients, even with adequate treatment and close monitoring.The prevalence of uveitis in JRA seems to be decreasing and may be secondary to the increased use of naproxen. However, routine ophthalmologic screening should be continued in patients with JRA to avoid potential complications of chronic uveitis.