Optic Nerve Aplasia.

OBJECTIVE: Optic nerve aplasia (ONA) is a rare ocular anomaly. We report ophthalmologic, systemic, and genetic findings in ONA. METHODS: Patients were identified through an International Pediatric Ophthalmology listserv and from the practice of the senior author. Participating Listserv physicians completed a data collection sheet. Children of all ages were included. Neuroimaging findings were also recorded. RESULTS: Nine cases of ONA are reported. Patients' ages ranged from 10 days to 2 years (median 9 months). Seven cases were bilateral. All patients had absence of the optic nerve and retinal vessels in the affected eye or eyes. Ophthalmologic findings included glaucoma, microcornea, persistent pupillary membrane, iris coloboma, aniridia, retinal dysplasia, retinal atrophy, chorioretinal coloboma, and persistent fetal vasculature. Systemic findings included facial dysmorphism, cardiac, genitourinary, skeletal, and developmental defects. A BCOR mutation was found in one patient. One patient had rudimentary optic nerves and chiasm on imaging. CONCLUSION: ONA is a unilateral or bilateral condition that may be associated with anomalies of the anterior or posterior segment with or without systemic findings. Rudimentary optic nerve on neuroimaging in one case suggests that ONA is on the continuum of optic nerve hypoplasia.

Ocular Surface Disease in a PICU: Incidence and Outcomes With a Dynamic Eye Care Protocol.

OBJECTIVES: Sedated intensive care patients have impaired ocular protective mechanisms putting them at risk for ocular surface disease with potential vision loss. Historically, routine eye care has been limited to critically ill patients receiving neuromuscular blockade. The aim of this project was to determine the occurrence rate of ocular surface disease in sedated and ventilated children, identify risk factors, and determine the progression of injury with routine eye care. DESIGN: Prospective cohort study. SETTING: A tertiary care medical-surgical PICU. PATIENTS: All intubated patients admitted from May 2015 to December 2016. INTERVENTIONS: Staff education regarding corneal examination with fluorescein, and routine eye care as per a PICU eye care protocol. MEASUREMENTS AND MAIN RESULTS: We evaluated 479 patients (1,242 corneal exams) and found that 15% had ocular surface disease at admission to the PICU: keratopathy 62, abrasion 16. The highest incidence was in trauma patients (39.0%) and those intubated in the emergency department (22.2%) or prehospital setting (42.9%). Of the 245 patients with multiple ocular assessments, 32.2% displayed ocular surface disease at some point during their hospitalization: keratopathy 73, abrasion 24. Ourprotocol dictated increased frequency of eye care if ocular surface disease worsened. As a result, the overall incidence of ocular surface disease decreased to 8.6% by the last examination (keratopathy 19, mild abrasion 2), but more severe ocular abnormalities such as corneal infiltrates, ulcers, or scarring were not observed. Based on multivariate analysis, clinical factors associated with increased risk of ocular surface disease included primary diagnosis, and lagophthalmos (incomplete eyelid closure). CONCLUSIONS: Ocular surface disease is an under-recognized process in critically ill pediatric patients. A standardized and dynamic protocol may improve corneal health, which in turn may reduce injury, pain, infection, and long-term vision loss.

Retinal Findings in Young Children With Increased Intracranial Pressure From Nontraumatic Causes.

OBJECTIVES: Increased intracranial pressure (ICP) has been suggested in legal settings as an alternative cause of retinal hemorrhages (RHs) in young children who may have sustained abusive head trauma. We assessed the prevalence and characteristics of RHs in children with increased ICP. METHODS: We conducted a prospective, multicenter study of children <4 years old with newly diagnosed increased ICP as determined by using direct measurement and/or clinical criteria. Infants who were premature, neonates, and suspected survivors of abusive head trauma were excluded on the basis of nonocular findings. Fundus examinations were performed; extent, number, and type of RH in each of 4 distinct retinal zones were recorded. RESULTS: Fifty-six children (27 boys) were studied (mean age 15.4 months; range 1-43 months). All of the children had elevated ICP that required intervention. One child had papilledema. No child (0%; 95% confidence interval: 0%-6.4%) or eye (0%; 95% confidence interval: 0%-3.3%) was found to have an RH. Causes of increased ICP included hydrocephalus, intraventricular hemorrhage, congenital malformations, malfunctioning shunts, and the presence of intracranial space-occupying lesions. CONCLUSIONS: Although acute increased ICP can present in children with a pattern of peripapillary superficial RHs in the presence of papilledema, our study supports the conclusion that RHs rarely occur in the absence of optic disc swelling and do not present beyond the peripapillary area in the entities we have studied.

Cup-to-disc ratio in patients with idiopathic intracranial hypertension is smaller than that in normal subjects.

BACKGROUND: A small cup-to-disc (C:D) ratio is an established risk factor for nonarteritic anterior ischemic optic neuropathy. We sought to determine if a small C:D ratio was present in patients with idiopathic intracranial hypertension (IIH) as a potential risk factor for visual loss in that disorder. METHODS: We performed a retrospective review of 52 charts of patients diagnosed with IIH at Michigan State University from 1990 to 2003. Twenty-eight patients (55 eyes) met diagnostic inclusion criteria and had undergone fundus photography of sufficient quality to allow assessment of the C:D ratio after optic disc edema had become minimal or resolved. C:D ratio was measured from the digitized photographs. The data were placed into rank order categories (0.1 unit intervals) and compared to published normative C:D data. RESULTS: The average vertical C:D ratio was 0.143 (SD 0.061) in the right eye and 0.127 (SD 0.056) in the left eye. The average horizontal C:D ratio was 0.145 (SD 0.053) in the right eye and 0.133 (SD 0.053) in the left eye. The IIH group rank distribution data were compared to published normative C:D ratio data (chi-square test). In each case, the IIH population had a statistically significantly smaller C:D ratio (P < 0.0001) compared to normal subjects. CONCLUSIONS: The C:D ratio in our IIH population was smaller than that in published control populations. A small C:D ratio may lower the threshold for developing optic disc edema from ischemia, increased intracranial pressure, or other mechanisms. Additional studies are needed to confirm these findings.