Evaluation of progressive pediatric myopia despite control lenses

Cases & Resources
Presentation
PART 1 - CASE PRESENTATION

    Clinical situation

    A 9-year-old child is referred for a second opinion for progressive myopia despite myopia-control spectacle lenses. 

    Refraction under cycloplegia performed 6 months previously: 

    • -7,50 (+1,75 à 60°)
    • -5,25 (+0,75 à 135°) 

    Visual Acuity (VA): 8/10 RE and LE (0.1 Log MAR) 

    When seen in the clinic, new refraction under cycloplegia reveals: 

    • -13 (+3 à 70°)
    • -10,75 (+1,75 à 100°) 

    Visual Acuity (VA): 7/10 RE and LE (0.2 Log MAR), i.e. + 5 / + 4,5 Spherical equivalent 

    At that stage, the most crucial examinations to perform are : 

    • Axial length measurement
    • Corneal topography
    • Slit lamp examination after full pupil dilation

    Title Axial lenght measurement: 23,83 / 23,66

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    Media
    Image Axial lenght measurement: 23,83 / 23,66

    Title Not an axial myopia… Refractive myopia => cornea, or lens

    Paragraph
    Media
    Image => Not an axial myopia… Refractive myopia => cornea, or lens

    Patient’s eye after cyclopentolate: Good cycloplegia, but suboptimal pupil dilation

    Title Refractive myopia

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    Media
    Image Refractive myopia

    cornea, or lens Fully dilate the pupils…

    Media
    Image Refractive myopia

    cornea, or lens Fully dilate the pupils…

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PART 2 - FINAL STRATEGY

    Considering the child has no dysmorphic features, no psychomotor delay, and no family or personal history apart from his visual symptoms: 

    •  An NGS panel for lens ectopia can be performed
    • The myopia-control spectacle lenses can be discontinued 

    Visual acuity remains good; surgery is not indicated at that stage.  
    Mild forms of homocystinuria can present with such a phenotype; metabolic tests are compulsory.  
    An NGS panel can be performed in addition.  
    Axial length is normal, and therefore myopia-control interventions aiming to slow down length elongation are not indicated

    Plasmatic homocysteinemia = 342 µmol/L (abnormally elevated) 

    The child was diagnosed with homocystinuria due to cystathionine beta-synthase (CBS) deficiency, requiring a metabolic follow up, with a risk of thrombosis and the necessity to get a plasmatic homocysteinemia < 50 µmol/L before any surgical intervention

Team
Pr Matthieu ROBERT
Pr Matthieu ROBERT
Scientific board

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