Jane D. Kivlin

Prevalence of Myopia between 3 Months and 5 '/-- Years in Preterm Infants with and without Retinopathy of Prematurity

Purpose: The purpose of the study was to examine spherical equivalent refractive errors, especially myopia, at six ages between 3 months and 5’/, years post-term in preterm children with birth weights of less than 1251 g. Design: A cohort study. Participants: There were a total of 827 participants in the multicenter study of cryotherapy for retinopathy of prematurity (ROP). Approximately one third of the eyes did not develop ROP, whereas two thirds developed mildto-severe ROP. None of the eyes underwent ctyotherapy. Intervention: Refractive error was measured at 3 months, 1 year, and 5’/2 years after term due date at the five long-term follow-up centers. In most eyes, refractive error also was measured at 2, 3’/*, and 4’/* years. Main Outcome Measure: Myopia was defined as 0.25 diopter (D) or greater with high myopia as 5 D or greater. Results: The proportion of eyes with myopia in this preterm population was increased compared to published data on full-term children and was related to severity of both acute-phase and cicatricial-phase ROP. The percentage of eyes with myopia varied little across ages, ranging from 21.2% at 1 year to 15.7% at 4’1’~ years. The percentage of eyes with high myopia doubled from 1.8% to 3.9% between 3 months and 1 year and remained stable thereafter. The distribution of refractive errors in eyes with mild acute-phase ROP was similar to that of eyes with no ROP. In contrast, eyes with moderate or severe acute-phase ROP showed an increased prevalence of high myopia. The distribution of refractive errors changed between 3 months and 1 year with little change after 1 year. This pattern of refractive development differs from that of full-term infants. Birth weight, severity of ROP, and degree of myopia at 3 months predicted the presence of myopia and high myopia at 5’1, years of age. Conclusions: The distribution of refractive errors in preterm infants from age 3 months to 51/2 years varies with severity of acute-phase ROP and cicatricial disease. Changes in refractive error distribution occur primarily between 3 months and 1 year and involve a decrease in the proportion of eyes with hyperopia and an increase in the proportion with high degrees of myopia. Ophfhalmology 7998; 705:7292- 7300

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.

Therapy of Anisometropic Amblyopia

In 67 patients with strictly anisometropic amblyopia, we found statistically significant correlations among three measurable parameters: the vision before treatment, the amount of anisometropia and the vision after treatment. The predetermined factors associated with success of treatment were 1) lower amounts of anisometropia, particularly less than six diopters for myopes, 2) better initial vision, and 3) a younger patient for hyperopic differences. Patients with less than three diopters of anisometropia were more likely to succeed with the use of glasses alone and a trial of glasses did not prolong therapy time. Despite a substantial number of older patients with extreme amounts of anisometropia or very poor vision, the group as a whole did well with 70% achieving 20/40 or better. Thus therapy should be approached enthusiastically, even for highly myopic patients since other authors have reported success with them. The factors under the control of the ophthalmologist which will promote success in these patients are correct glasses prescriptions, particularly with full astigmatic correction, continued use of glasses as maintenance therapy, and patience, since these children required a median of eight months for therapy to be successful.

Does cryotherapy affect refractive error? Results from treated versus control eyes in the cryotherapy for retinopathy of prematurity trial

PURPOSE To evaluate the effect of cryotherapy on refractive error status between ages 3 months and 10 years in children with birth weights of less than 1251 g in whom severe retinopathy of prematurity (ROP) developed in one or both eyes during the neonatal period. DESIGN Randomized clinical trial. PARTICIPANTS Two hundred ninety-one children in whom severe ROP developed during the neonatal period. INTERVENTION Cryotherapy for ROP. MAIN OUTCOME MEASURES Cycloplegic Refraction METHODS The children underwent repeated follow-up eye examinations, including cycloplegic retinoscopy, between 3 months and 10 years after term due date. Refractive error data from all eyes that were randomized to cryotherapy were compared with data from all eyes that were randomized to serve as controls. Refractive error data were also compared for a subset of children who had both a treated and a control eye that could be refracted. RESULTS At all ages, the proportion of treated eyes that were unable to be refracted because of retinal detachment, media opacity, or pupillary miosis was approximately half the proportion of the control eyes that were unable to be refracted. When data from all eyes that could be refracted were considered, the distribution of refractive errors between fewer than 8 diopters (D) of myopia and more than 8 D of hyperopia was similar for treated and control eyes at all ages. The proportion of eyes with 8 D or more of myopia was much higher in treated than in control eyes at all ages after 3 months. In the subset of children who had a treated eye and a control eye that could be refracted, distributions of refractive errors in treated versus control eyes were similar at most ages. CONCLUSIONS In both treated and control eyes, there was an increase in the prevalence of high myopia between 3 and 12 months of age. Between 12 months and 10 years of age, there was little change in distribution of refractive error in treated or control eyes. The higher prevalence of myopia of 8 D or more in treated eyes, as compared with control eyes, may be the result of cryotherapys preservation of retinal structure in eyes that, in the absence of cryotherapy, would have progressed to retinal detachment.

The Genetic Aspects of Neurofibromatosis

Although the genetic pattern in NF has been definitely established as autosomal dominant, more precise data regarding penetrance, natural history, prevalence, and heterogeneity are needed for the counseling of families. NF is the prototypic disorder for the study of the biologic mechanisms of variable expressivity. The widely cited prevalence figure of Crowe is probably too high; thus the mutation ratio estimation in NF is among the highest in man but close to other common Mendelian disorders. With the existing data on frequency of Lisch nodules and with future prospective date on café-au-lait spot development, an age-of-onset penetrance curve for NF could be constructed for genetic counseling purposes. The segmental form of NF is of interest as cases of this presentation may be helpful in studying the hypothesis of human somatic mutation when DNA analysis is available. Guidelines for routine evaluation and ongoing health supervision of individuals with neurofibromatosis need to be developed; multidisciplinary NF clinics and collaborative study groups are appropriate settings for this undertaking. Neurofibromatosis is an important disorder for the study of the psychodynamic processes that families experience in dealing with uncertainty.

Ocular findings after bone marrow transplantation in a pediatric population

PURPOSE With the increasing use of bone marrow transplantation (BMT) in children to treat diseases of the hematopoietic system, ocular complications have been recognized in greater numbers. The authors performed a retrospective study to determine the prevalence and types of ocular abnormalities in a pediatric population following BMT. DESIGN Retrospective noncomparative study. PARTICIPANTS One hundred and four consecutive pediatric patients with aplastic anemia and various hematologic malignancies who required BMT. INTERVENTION Bone marrow transplantation. MAIN OUTCOME MEASURES Visual acuity and slit-lamp biomicroscopic and funduscopic examinations. RESULTS Ocular changes developed in 51% of patients. The most frequent findings included dry eye syndrome (12.5%), cataract (23.0%), and posterior segment complications (13.5%). A final visual acuity of 20/40 or better was achieved in 95.7% of eyes. CONCLUSIONS Pediatric patients can develop severe and potentially vision-threatening complications following BMT. Despite the high incidence of anterior and posterior segment abnormalities, ocular symptoms were generally mild and the majority of patients retained excellent visual function. Factors associated with ocular complications included the underlying disease, total body irradiation, systemic chemotherapy, graft-versus-host disease, and immunosuppression. Familiarity with these risk factors and potential complications in pediatric patients is important for successful treatment.

Retinal hemorrhages in children following fatal motor vehicle crashes: a case series

OBJECTIVE To demonstrate the severity of ocular findings in young children who died of injuries due to motor vehicle crashes. METHODS Case series of 10 children younger than 3 years who were fatally injured in motor vehicle crashes between January 1, 1994, and December 31, 2002. All children underwent autopsy that included eye examination. All available medical and autopsy records, pathology slides and photographs, and police and traffic department reports were reviewed for each case. RESULTS Eight patients had retinal hemorrhages, which extended into the periphery in 13 eyes and were bilateral in 7 patients. Three patients had elevated circular retinal folds. Six patients had hemorrhages below the internal limiting membrane, but no patients had deeper splitting of the retina. Nine patients had optic nerve sheath hemorrhages. CONCLUSION The association of extensive, sometimes severe, ocular hemorrhages with fatal accidental trauma, compared with previous reports of accidental trauma with no or few hemorrhages, indicates the severity of injury required to cause hemorrhages of this magnitude.

Periocular Infection After Strabismus Surgery

Fewer than 15 cases of cellulitis and subconjunctival abscess after extraocular muscle surgery have been reported in detail. In an effort to provide greater information about this serious complication, the members of the American Association for Pediatric Ophthalmology and Strabismus (AAPO&S) were surveyed and contributed cases were analyzed. Among the 25 cases of cellulitis, there was a predominance of preschool age patients and Staphylococcus aureus cultures. Many cases presented after a normal initial postoperative visit. The most common symptoms were marked swelling and pain. Possible predisposing factors included unsuspected sinusitis, eye rubbing, and poor hygiene. Two patients had documented bacteremia, but no patient developed endophthalmitis. All cases resolved on antibiotics, but only five patients were successfully treated with oral antibiotics alone. Periocular infections after extraocular muscle surgery can present after a normal initial postoperative visit. Symptoms of marked swelling and pain are cause for closer inspection. S. aureus is the most common organism and oral antibiotics alone can fail to halt the infection.

Genetic analysis of NF1: Identification of close flanking markers on chromosome 17

The gene causing von Recklinghausen neurofibromatosis, or NF1, has been more precisely localized in the pericentromeric region of chromosome 17. Narrowing of the location for the disease became possible through the identification of eight new DNA probe genetic markers in the centromeric region. Markers that closely flank the centromere also closely flank the NF1 gene. Although there was evidence against this localization in one recombinant, a review of the clinical records revealed a borderline diagnosis of NF1. Significant sex differences in recombination were observed in the pericentric region, and odds for different orders were less discriminating when sex differences were considered in multilocus analyses. The location of the NF1 gene with respect to the centromere could not be determined because recombinants between NF1 and the centromere were not detected in the set of families tested.

Unusual cause of short stature.

A 24-year-old man evaluated for paresthesias and short stature was found to be hypocalcemic on initial presentation. Further evaluation showed that he had a low-normal parathormone level by amino-terminal assay, medullary stenosis of the long bones, and multiple ophthalmologic abnormalities. The remainder of his pituitary function, including growth hormone response to insulin-induced hypoglycemia, was normal. As no family history of similar findings was evident, a sporadic case of Kennys or Kenny-Caffey syndrome was diagnosed. He became normocalcemic in response to vitamin D and calcium carbonate therapy. The results of testing in this patient and the findings in other patients previously described with the Kenny-Caffey syndrome are reviewed.