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Nyctalopia in Children

Nyctalopia - Overview

Definition

Nyctalopia, or night blindness, is impaired vision in dim light and darkness, primarily affecting dark adaptation.

Key Points

  • Can be congenital or acquired
  • Often first symptom of retinal dystrophies
  • May indicate systemic conditions
  • Critical for early childhood development
  • Can affect educational and social activities

Pathophysiology

Normal Dark Adaptation

  • Rod Photoreceptor Function
    • Rhodopsin cycling
    • Vitamin A dependent process
    • Neural signal transmission
  • Retinal Pigment Epithelium Role
    • Vitamin A metabolism
    • Visual cycle maintenance
    • Photoreceptor support

Pathological Changes

  • Rod Dysfunction
    • Genetic mutations
    • Nutritional deficiencies
    • Retinal degeneration
  • Visual Cycle Disruption
    • Vitamin A processing defects
    • RPE dysfunction
    • Metabolic disorders

Causes & Associations

Congenital Conditions

  • Retinitis Pigmentosa
    • Multiple genetic forms
    • Progressive rod-cone dystrophy
    • Early onset nyctalopia
  • Congenital Stationary Night Blindness
    • X-linked forms
    • Autosomal forms
    • Non-progressive
  • Leber Congenital Amaurosis
    • Severe early vision loss
    • Multiple genetic variants

Acquired Causes

  • Nutritional Deficiencies
    • Vitamin A deficiency (most common)
    • Zinc deficiency
    • Protein malnutrition
  • Retinal Diseases
    • Chorioretinitis
    • Central serous retinopathy
    • Diabetic retinopathy
  • Medications
    • Ethambutol
    • Isotretinoin
    • Some antipsychotics

Diagnosis & Assessment

Clinical Evaluation

  • History Taking
    • Age of onset
    • Progression pattern
    • Family history
    • Associated symptoms
  • Physical Examination
    • Visual acuity
    • Color vision
    • Visual fields
    • Fundus examination

Specialized Testing

  • Dark Adaptometry
    • Gold standard test
    • Measures adaptation threshold
    • Quantifies severity
  • Electroretinography (ERG)
    • Rod and cone responses
    • Disease classification
    • Prognosis assessment
  • Genetic Testing
    • Gene panel testing
    • Family screening
    • Treatment planning

Management Approach

Treatment Strategies

  • Nutritional Support
    • Vitamin A supplementation
    • Balanced diet
    • Regular monitoring
  • Genetic Counseling
    • Family planning
    • Prognosis discussion
    • Support group referral

Vision Rehabilitation

  • Adaptive Devices
    • Night vision devices
    • High-contrast materials
    • Mobility aids
  • Educational Support
    • School accommodations
    • Special lighting needs
    • Safety measures

Monitoring & Prevention

  • Regular Follow-up
    • Vision assessment
    • Disease progression
    • Complication screening
  • Preventive Measures
    • UV protection
    • Nutritional counseling
    • Lifestyle modifications
Further Reading


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