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David Wolf, MD, PhD Pediatric Neurology Group 4

Characterization of retinal thickness in children with neurofibromatosis type 1 and optic pathway gliomas using optical coherence tomography. David Wolf, MD, PhD Pediatric Neurology Group 4. Neurofibromatosis Type 1. Autosomal dominant neurocutaneous disorder Incidence of 1:3500

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David Wolf, MD, PhD Pediatric Neurology Group 4

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  1. Characterization of retinal thickness in children with neurofibromatosis type 1 and optic pathway gliomas using optical coherence tomography David Wolf, MD, PhD Pediatric Neurology Group 4

  2. Neurofibromatosis Type 1 • Autosomal dominant neurocutaneous disorder • Incidence of 1:3500 • Diagnosis based primarily on clinical criteria

  3. Optic Pathway Gliomas and NF1 • 15% of children with NF1 (Listernick et al, J Pediatr 1994) • Age <6 years • Can be seen into early adulthood • Present with: • Diminished visual acuity • Difficult to assess • Proptosis • Precocious puberty • Tumor causes compressive damage to optic nerve

  4. Current Guidelines • Yearly ophthalmologic exam • Acuity • Visual fields • Color vision • Follow growth curves closely • If optic pathway glioma suspected, MRI indicated • No indication for “screening” neuroimaging

  5. T2 T2 Post-contrast

  6. Problems with MRI • Can follow size, enhancement over time • Changes not associated with visual acuity • Requires general anesthesia • May be needed up to 8 times over 2 years

  7. Other testing modalities? • Noninvasive • Fast • Can be performed on children • Quantitative • Correlates with standard visual acuity testing

  8. Optical Coherence Tomography

  9. Optical Coherence Tomography • Noninvasive, no ionizing radiation • Gives quantitative, reproducible measurement of retinal thickness • Decreased retinal thickness correlates with poorer visual acuity • Tumors that compress the optic nerve decrease retinal thickness • OPGs have not been specifically examined

  10. Hypothesis • Retinal nerve layer thickness will be decreased in children with optic pathway glioma relative to children without optic pathway glioma • This is a feasibility study. In the future, we hope to use OCT as a tool to follow progression of OPGs

  11. Study design • Cross sectional study of children seen in the Johns Hopkins Outpatient Center

  12. Inclusion and Exclusion Criteria • Inclusion: Children aged 6-19 years • Exclusion • History of optic neuritis, papilledema • Chemotherapy for optic glioma • Glioma regression • Foster children • Unable to sit for exam • Myopia and hyperopia are NOT exclusion criteria

  13. Definition of three comparison groups • Neurofibromatosis Type 1 with OPG • First occurrence of OPG as assessed by MRI • Recruited through referral from neuro-oncology and neuro-ophthalmology • Neurofibromatosis Type 1 without OPG • Based on clinical diagnosis • Matched to cases with OPG on age (+/- 3 years) • Recruited through neurofibromatosis clinic • Non-NF1 controls recruited from general pediatric neurology clinic

  14. Assessment of Retinal Thickness • OCT performed by one person (me) on one machine • Complete retinal thickness measured in each eye once • Average retinal thickness will be used in analysis

  15. Additional Testing • Visual acuity • Snellen visual acuity testing • Sloan low contrast visual acuity • Will be used for secondary analysis • Demographic factors, medical history collected at time of exam • Prior imaging studies • Prior ophthalmologic exams

  16. Power calculation Mean (controls): 100 microns Mean (OPG): 90 microns SD (controls): 30 SD (OPG): 30 Alpha: 0.05 Power: 0.8 Sample size: 19 subjects per group

  17. Analytical Plan • ANOVA comparing retinal thickness between three groups • General linear regression to compare differences in retinal thickness between all three groups (using non-NF1 as reference group) while adjusting for age, sex, and other potential confounders • Correlation matrix of retinal thickness, visual acuity, and tumor size

  18. Power calculation Mean (controls): 100 microns Mean (OPG): 90 microns SD (controls): 30 SD (OPG): 30 Alpha: 0.05 Power: 0.8 Sample size: 19 subjects per group

  19. Significance • Children with OPGs require frequent MR imaging to monitor tumor progression • In young children requires general anesthesia • OCT can serve as non-invasive method to follow OPG and assess progression of tumor

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