Handheld Optical Coherence Tomography During Sedation in Young Children With Optic Pathway Gliomas

Abstract

IMPORTANCE Monitoring young children with optic pathway gliomas (OPGs) for visual deterioration can be difficult owing to age-related noncompliance. Optical coherence tomography (OCT) measures of retinal nerve fiber layer (RNFL) thickness have been proposed as a surrogate marker of vision but this technique is also limited by patient cooperation.OBJECTIVE To determine whether measures of circumpapillary RNFL thickness, acquired with handheld OCT (HH-OCT) during sedation, can differentiate between young children with and without vision loss from OPGs. DESIGN, SETTING, AND PARTICIPANTSThis cross-sectional analysis of a prospective observational study was conducted at a tertiary-care children's hospital. Children with an OPG (sporadic or secondary to neurofibromatosis type 1) who were cooperative for visual acuity testing, but required sedation to complete magnetic resonance imaging, underwent HH-OCT imaging of the circumpapillary RNFL while sedated. MAIN OUTCOMES AND MEASURESArea under the curve of the receiver operating characteristic, sensitivity, specificity, positive predictive value, and negative predictive value of the average and quadrant-specific RNFL thicknesses.RESULTS Thirty-three children (64 eyes) met inclusion criteria (median age, 4.8 years; range, 1.8-12.6 years). In children with vision loss (abnormal visual acuity and/or visual field), RNFL thickness was decreased in all quadrants compared with the normal-vision group (P < .001 for all comparisons). Using abnormal criteria of less than 5% and less than 1%, the area under the curve was highest for the average RNFL thickness (0.96 and 0.97, respectively) compared with specific anatomic quadrants. The highest discrimination and predictive values were demonstrated for participants with 2 or more quadrants meeting less than 5% (sensitivity = 93.3; specificity = 97.9; positive predictive value = 93.3; and negative predictive value = 97.9) and less than 1% (sensitivity = 93.3; specificity = 100; positive predictive value = 100; and negative predictive value = 98.0) criteria. CONCLUSIONS AND RELEVANCEMeasures of RNFL thickness acquired with HH-OCT during sedation can differentiate between young children with and without vision loss from OPGs. For young children who do not cooperate with vision testing, HH-OCT measures may be a surrogate marker of vision. Longitudinal studies are needed to delineate the temporal relationship between RNFL decline and vision loss.

Reproducibility of circumpapillary retinal nerve fiber layer measurements using handheld optical coherence tomography in sedated children

“…The ability to monitor longitudinal circumpapillary RNFL changes could be useful in young children with optic pathway gliomas 24 and glaucoma. 29 The ophthalmologic monitoring and neuro-oncologic care of children with optic pathway gliomas, especially those with NF1-related optic pathway gliomas, is challenging for a number of reasons.…”

“…The development of a handheld SD-OCT has enabled pediatric practitioners to acquire high resolution images of the circumpapillary RNFL and macula in neonates, infants and young children. [13][14][15][16][17][18][19][20][21][22][23][24] While neonates and infants can be imaged while awake, the portability of the handheld OCT permits acquisition in toddlers and young children during sedation.…”

Pediatric Optical Coherence Tomography in Clinical Practice—Recent Progress

“…95 Optical coherence tomography also can detect RNFL loss from optic atrophy occurring secondary to optic pathway glioma in older children affected by neurofibromatosis type 1. 96 In addition, the potential of the HH-SDOCT to monitor progressive optic neuropathies such as optic nerve glioma in infants and young children unable to cooperate with traditional OCT testing devices has been established. 97 It has been demonstrated that measurements of the RNFL, GCL, and IPL thickness can discriminate between children with and without vision loss from their optic nerve pathway gliomas, thus suggesting that HH-SDOCT can be used as a surrogate marker of visual function in infants and young children.…”

“…97 It has been demonstrated that measurements of the RNFL, GCL, and IPL thickness can discriminate between children with and without vision loss from their optic nerve pathway gliomas, thus suggesting that HH-SDOCT can be used as a surrogate marker of visual function in infants and young children. 96,98 Similarly, OCT can be used to diagnose and monitor pediatric pseudotumor cerebri and is potentially useful as a visual prognostic tool in this condition. 99,100 An increased central macular, temporal, and superior RNFL thickness has been described in children affected by pseudotumor cerebri.…”

OCT and Compressive Optic Neuropathy

“…However, more recently Avery et al [ 29 ] used a hand-held OCT to obtain optic nerve and retinal thickness measurements during sedation in 33 young children with OPGs. The authors concluded that for young children who do not cooperate with vision testing, hand-held OCT measures may be a surrogate marker of vision.…”