1 / 24

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

cameron-nicholson
Download Presentation

David Wolf, MD, PhD Pediatric Neurology Group 4

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related