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PURPOSE: Compared with the general population, patients with hydrocephalus are more likely to have strabismus. This study was undertaken to examine characteristics and outcomes of children with esotropia and ventricular-peritoneal shunt placement due to hydrocephalus. METHODS: This is a retrospective chart review of all pediatric patients with esotropia and a history of ventricular-peritoneal shunt placement seen by our pediatric ophthalmology service between January 2000 and December 2010. RESULTS: Sixteen patients between the age of 3 months and 5.6 years met study criteria. Nine were premature and all but one of the patients had developmental delay. Although all patients had a ventricular-peritoneal shunt, the diagnosis leading to shunt placement was intraventricular hemorrhage or congenital hydrocephalus in 75% of the patients. In all but 3 patients the hydrocephalus was diagnosed before the esotropia. Ten children had congenital esotropia and 6 had acquired esotropia. Eleven of the 16 children required glasses: 5 had a myopic prescription and 6 had a hyperopic prescription. Treatment of the esotropia resulted in 9 patients (56%) with successful ocular alignment (<10 prism diopters) on their last visit: 7 underwent strabismus surgery and 2 were treated with glasses only. Of the 9 patients who had strabismus surgery, 6 had congenital esotropia and 3 had acquired esotropia. Among patients who underwent strabismus surgery, 78% had successful ocular alignment at their last visit. CONCLUSIONS: While acquired accommodative esotropia is more common in the general population, children with ventricular-peritoneal shunts may be more likely to have congenital esotropia. Although developmental delay is very frequent, successful ocular alignment may be possible in this patient population.

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Eyedrops used for mydriasis and cycloplegia can be systemically absorbed, causing serious side effects, including oxygen desaturation, apnea, bradycardia, transient hypertension, delayed gastric emptying, and transient paralytic ileus. These effects can be more serious in infants because of their lower body mass and immature cardiovascular and nervous systems. We report a case of a 27-week-old infant who suffered a cardiopulmonary arrest after the administration of only Cyclomydril eyedrops (Alcon Laboratories, Fort Worth, TX) during an outpatient retinopathy of prematurity examination.

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PURPOSE: To describe the clinical characteristics, treatment, and subsequent clinical course of children with exotropia and high hyperopia. METHODS: The medical records of 26 patients seen between 1990 and 2009 who had an exotropia and ≥4.00 D of hyperopia were retrospectively reviewed. We analyzed the clinical characteristics, treatments, and subsequent alignment outcomes. RESULTS: A total of 26 patients between the ages of 2.5 months and 9 years were included. Of these, 15 had associated medical conditions or developmental delay. Of 22 patients with measured visual acuities, 19 had amblyopia (10 unilateral, 9 bilateral). None of the patients demonstrated fine stereoacuity. Twenty-three exotropic children were treated with spectacles: 15 were fully corrected, 10 of whose exotropia improved; 8 received partial correction of their hyperopia, 3 of whose exotropia improved. Six patients who presented with large, poorly controlled exotropia and did not improve with spectacle correction required strabismus surgery. CONCLUSIONS: Children with high hyperopia and exotropia are likely to have developmental delay or other systemic diseases, amblyopia, and poor stereopsis. Treatment of high hyperopia in exotropic children with their full cycloplegic refraction can result in excellent alignment.

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OBJECTIVE: To compare the performance on the American Board of Ophthalmology Written Qualifying Examination (WQE) with the performance on step 1 of the United States Medical Licensing Examination (USMLE) and the Ophthalmic Knowledge Assessment Program (OKAP) examination for residents in multiple residency programs. DESIGN: Comparative case series. PARTICIPANTS: Fifteen residency programs with 339 total residents participated in this study. The data were extracted from the 5-year American Board of Ophthalmology report to each participating program in 2009 and included residency graduating classes from 2003 through 2007. Residents were included if data were available for the USMLE, OKAP examination in ophthalmology years 1 through 3, and the WQE score. Residents were excluded if one or more of the test scores were not available. METHODS: Two-sample t tests, logistic regression analysis, and receiver operating characteristic (ROC) curves were used to examine the association of the various tests (USMLE, OKAP examination year 1, OKAP examination year 2, OKAP examination year 3, and maximum OKAP examination score) as a predictor for a passing or failing grade on the WQE. MAIN OUTCOME MEASURES: The primary outcome measure of this study was first time pass rate for the WQE. RESULTS: Using ROC analysis, the OKAP examination taken at the third year of ophthalmology residency best predicted performance on the WQE. For the OKAP examination taken during the third year of residency, the probability of passing the WQE was at least 80% for a score of 35 or higher and at least 95% for a score of 72 or higher. CONCLUSIONS: The OKAP examination, especially in the third year of residency, can be useful to residents to predict the likelihood of success on the high-stakes WQE examination.

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Extensive intraocular hemorrhage in young infants in the setting of acute brain injury and in the absence of a history of severe accidental trauma or underlying medical cause must be considered to be nonaccidental injury until otherwise proven. In the absence of any obvious explanation, the presence of any retinal hemorrhage should raise the possibility of abusive head trauma in the differential diagnosis and perhaps lead to such testing as skeletal radiography and neuroimaging, consultation with a child abuse specialist, as well as workup for other relevant systemic conditions. Physicians who treat infants and children are mandated to report suspected child abuse to child welfare agencies for investigation, and ophthalmologists who encounter children with ophthalmic manifestations of abuse need to ensure that the proper steps are taken to protect their patients from the potential for further harm. Ascertainment of abusive head trauma is critical to prevent a potentially fatal recurrence.

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Ophthalmic manifestations are noted in about 50% of adults with Wegener's granulomatosis. Wegener's granulomatosis is a rare disease in the pediatric age group. The disease may present initially with ocular manifestations. We report the largest series to date of children whose ocular findings were the initial presenting signs of Wegener's granulomatosis.

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Retinal hemorrhages are the most commonly reported ocular findings in nonaccidental trauma in children. Other reported ocular findings include perimacular folds, traumatic retinoschisis, choroidal hemorrhages, and retinal detachments. We report the clinical and pathologic findings in a case of a 10-month-old boy who sustained nonaccidental trauma and whose clinical presentation was characteristic of a hemorrhagic choroidal detachment. Postmortem examination revealed a large subretinal hemorrhage, with no evidence of choroidal hemorrhage.

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INTRODUCTION: Visual acuity in albinism can vary and is difficult to predict. We undertook this study to investigate whether patients with albinism with absent or minimal nystagmus have better visual acuity and ocular alignment than patients with albinism and obvious nystagmus. METHODS: Our retrospective chart review of 38 pediatric patients with albinism yielded 16 patients with absent or minimal nystagmus and 22 patients with obvious nystagmus. RESULTS: Patients with albinism and absent or minimal nystagmus had best-corrected visual acuities ranging from 20/20 to 20/50, with a mean visual acuity of 20/33. Those patients with albinism and obvious nystagmus had best-corrected visual acuities ranging from 20/30 to 20/200, with a mean visual acuity of 20/80. Visual acuity was significantly better in the group with absent or minimal nystagmus (P < 0.001). Of the 16 patients with absent or minimal nystagmus, 6 were orthophoric and 10 had a strabismic deviation of 12 prism diopters (PD) or less. None of these patients required strabismus surgery. Of the 22 patients with nystagmus, 16 were orthophoric, 1 had a horizontal deviation of less than 12 PD, and 5 had larger angle horizontal deviations ranging from 20 to 50 PD. CONCLUSION: Our findings suggest that pediatric albinism patients with absent or minimal nystagmus can be expected to demonstrate better visual acuity and are less likely to show a strabismic deviation of greater than 12 PD compared with those with obvious nystagmus (P = 0.0003).

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Noonan syndrome is a rare syndrome with both ocular findings and multiple organ involvement. We retrospectively identified all of the pediatric patients with Noonan syndrome seen at our institution and reviewed the ocular findings to determine specific ocular manifestations in pediatric Noonan syndrome patients.

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PURPOSE: To identify risk factors in children admitted with preseptal or orbital cellulitis with associated intracranial infection. METHODS: A retrospective chart review identified 10 patients (< or = 18 years) with a diagnosis of preseptal or orbital cellulitis and a concurrent or subsequent diagnosis of intracranial infection. RESULTS: Diagnoses confirmed by imaging included sinusitis (n = 10), preseptal cellulitis (n = 4), orbital cellulitis (n = 6), orbital subperiosteal abscess (n = 5), Pott's puffy tumor (n = 4), epidural empyema (n = 2), epidural abscess (n = 6), and brain abscess (n = 2). The timing of diagnosis of intracranial infection ranged from hospital day 1 to 21. All but 1 patient had positive microbial cultures. Seven of 10 patients had positive microbial cultures from two or more sites, 70% of which were polymicrobial; Streptococcus species and Staphylococcus species were the most commonly isolated bacterial pathogens. All patients required both medical and surgical therapy; all 10 patients underwent sinus surgery; 8 patients required neurosurgical craniotomy; and 5 patients underwent orbital surgery. There were no deaths. CONCLUSION: Intracranial involvement should be suspected in any patient age > or = 7 years with preseptal or orbital cellulitis associated with orbital subperiosteal abscess, Pott's puffy tumor, concurrent sinusitis, complaints of headache, and continuing fever despite intravenous antibiotics. Given the high incidence of polymicrobial infection found on cultures in this series, broad-spectrum antibiotics are strongly indicated. When imaging the orbits and sinuses in such patients, we recommend including the brain to rule out intracranial involvement.

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BACKGROUND: Recently, it has been advocated to decrease the frequency of eye examinations to screen for uveitis in children with juvenile rheumatoid arthritis (JRA) because of the low yield of positive findings after an initial normal eye examination. This study was undertaken to determine the time interval for the development of uveitis after the diagnosis of JRA and to further describe patients who develop uveitis related to JRA. METHODS: This was a retrospective chart review of all patients with JRA examined by either of 2 pediatric ophthalmologists from August 1984 to June 2001. All patients were also under the care of the Pediatric Rheumatology Division at Schneider Children's Hospital. Age of diagnosis of JRA, disease onset subtype of JRA, antinuclear antibody titers, age of diagnosis of uveitis, and complications from uveitis were recorded. RESULTS: One hundred fifty eight patients with JRA had eye examinations; 39 (25%) developed uveitis. Sixteen patients had uveitis on the initial eye examination, and 23 subsequently developed uveitis. When uveitis was absent at the initial eye examination, the mean time to develop it was 20 months (range, 4-81 months). CONCLUSIONS: A normal initial eye examination does not preclude the development of uveitis in patients with JRA. We recommend continuing the current standards of ophthalmologic examinations to screen for uveitis in children with JRA as prescribed by the Section on Rheumatology and Ophthalmology of the American Academy of Pediatrics.

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