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Orbital MRI versus fundus photography in the diagnosis of optic nerve hypoplasia and prediction of vision
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  • Published on:
    Orbital MRI versus fundus photography and OPTOS in the diagnosis of optic nerve hypoplasia and prediction of vision
    • Robert W Arnold, Pediatric Ophthalmologist Alaska Children's EYE & Strabismus

    Kruglyakova, et al recently published an excellent paper about visually pertinent correlation of optic nerve hypoplasia (ONH) with intra-operative photographic measurements1. We recently reported similar findings without subjecting children to general anesthesia if ultra-widefield imaging (OPTOS; Dunfermline, UK) is available2. We agree that a MD/DD ratio greater than 3.22 (≥3.51) is consistent with clinical optic nerve hypoplasia but our direct measure of horizontal optic nerve size was even more predictive utilizing our definition of logMAR for pediatric and low vision patients3. Instead of starting from the temporal edge of the optic nerve to determine MD (macula-disk) distance, we found the center of the optic nerve more uniform. In addition, we have also noted a worrisome relationship between ONH and threshold retinopathy of prematurity4 and wonder if the authors also found any association between the two common pediatric blinding conditions ONH and ROP?

    1. Kruglyakova J, Garcia-Filion P, Nelson M, Borchert M. Orbital MRI versus fundus photography in the diagnosis of optic nerve hypoplasia and prediction of vision. Br J Ophthalmol. 2020;104(10):1458-1461.
    2. Arnold AW, Eller AM, Smith KA, Grendahl RL, Winkle RK, Arnold RW. Direct nerve size determination and prevalent optic nerve hypoplasia in Alaska. Clin Ophthalmol. 2020;14:491—499.
    3. Arnold RW. Digital values for alpha acuities. JPOS. 2020:In Press.
    4. Arnold RW. Opti...

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    Conflict of Interest:
    I am the president of Glacier Medical Software that makes cloud-based NICU monitoring software ROP-Check. I am president of PDI Check that markets vision screening game for Nintendo 3DS. I coordinate the Alaska Blind Child Discovery which has received discounted vision screen technology from several vendors.
  • Published on:
    Optic nerve hypoplasia is associated with neurodevelopmental abnormalities requiring neuroimaging and paediatric review for improved diagnosis
    • Ngozi Oluonye, Consultant Neurodevelopmental Paediatrician Moorfields Eye Hospital and Great Ormond Street Hospital for Children
    • Other Contributors:
      • Indran Davagnanam, Consultant Neuroradiologist
      • Jenefer Sargent, Consultant Developmental Paediatrician
      • Naz Raoof, Consultant Paediatric Ophthalmologist
      • Mariya Moosajee, Consultant Ophthalmologist and Associate Professor

    This is an informative article highlighting the difficulties in diagnosing optic nerve hypoplasia (ONH) in infants. Comparisons were made of the reliability of MRI orbit to that of fundal photography, with the authors concluding that the latter was superior to neuroimaging for diagnostic purposes. Whilst valid, this may imply that MRI brain scans are not indicated when a diagnosis of ONH is under consideration.

    ONH has an incidence of between 2-10.9 per 100,000 births, systemic associations include developmental delay and neurologic deficits in over 50% and endocrine dysfunction in just over one-quarter of patients.(1) Children may display midline structural defects (abnormalities of the septum pellucidum, corpus callosum and pituitary axis), in addition to other cortical abnormalities, thus requiring neuroimaging.(1,2) A diagnosis of septo-optic dysplasia becomes appropriate when two out of three features are present: ONH, midline abnormalities and pituitary insufficiency. As babies and infants with ONH present primarily with abnormal visual behaviour, nystagmus, strabismus or amblyopia, from as early as 3 months of age, the Ophthalmologist may be the first specialist to evaluate the patient, and so the importance of investigating the wider clinical and radiological features cannot be overstated. This was not mentioned in this paper by Kruglyakova et al.

    Whilst one can argue that a normal MRI brain scan in a child with ONH is not predictive of future end...

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    Conflict of Interest:
    None declared.