Keratoconus in children

Keratoconus is a condition in which the normally round shape of the cornea (the clear outer layer of the eye) becomes cone-shaped. This results in distorted vision. Although keratoconus is most often diagnosed in adolescents and young adults between the ages of 10 and 25, it can also occur in younger children.

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Epidemiology

Keratoconus develops due to dystrophic stretching of the cornea, leading to thinning of its central and paracentral parts. The disease usually occurs in the second decade of life. The etiology of keratoconus is unknown, although there are assumptions about the important role of trauma in the origin of the disease. The significance of the hereditary factor is not determined, although in some patients a family history is clearly traced. Most cases are sporadic.

Causes keratoconus

The causes of keratoconus in children, as in adults, are not entirely clear, but there are several known risk factors and possible triggers that may contribute to the development of the disease:

1. Genetic predisposition: Keratoconus often runs in families, indicating a possible genetic component. If a parent or close relative has had keratoconus, the risk of children developing it increases.
2. Allergic conditions: Children with allergic conditions such as atopic dermatitis, allergic rhinitis or asthma may have a higher risk of developing keratoconus. This may be due to chronic eye rubbing caused by allergies.
3. Mechanical action: Frequent eye rubbing can contribute to corneal thinning and deformation, especially in children with pre-existing allergies.
4. Hormonal changes: Puberty is a time of significant hormonal changes, which can also affect the structure and metabolism of the cornea, increasing the risk of developing keratoconus.
5. Environment and lifestyle: Some studies suggest that ultraviolet radiation and other environmental factors, such as chronic eye inflammation or prolonged contact lens wear, may play a role in the development of keratoconus.
6. Oxidative stress: Damage to the cornea at the cellular level caused by oxidative stress may be one of the mechanisms contributing to the development of keratoconus.

However, even if one or more of these risk factors are present, not all children will develop keratoconus. A more accurate understanding of individual causes requires a comprehensive evaluation by a specialist, which may include family history, clinical examination, and diagnostic tests.

Pathogenesis

The clinical manifestations of keratoconus are initially related to its effect on visual acuity. Thinning of the cornea leads to the development of irregular astigmatism, which justifies the use of contact lenses. As the disease progresses, ruptures of the Descemet membrane occur, associated with hydration and leading to acute hydration of the cornea. In this condition, blurred vision caused by corneal edema is accompanied by severe pain.

The process stops spontaneously, leaving behind various cicatricial changes.

Symptoms keratoconus

• Blurred and distorted vision.
• Increased myopia and astigmatism, which is difficult to correct with standard glasses.
• Sensitivity to light, especially bright light.
• Frequent need to change glasses due to rapid changes in vision.

Keratoconus may accompany the following conditions:

• Apert syndrome;
• atopy;
• brachydactyly;
• Crouzon syndrome;
• Ehlers-Danlos syndrome;
• Lawrence-Moon-Biedl syndrome;
• Marfan syndrome;
• mitral valve prolapse;
• Noonan syndrome;
• osteogenesis imperfecta;
• Raynaud's syndrome;
• syndactyly;
• pigmented eczema;
• Leber's congenital amaurosis (and other congenital rod-cone dystrophies).

Diagnostics keratoconus

In children, keratoconus may go undiagnosed because of the difficulty in performing some types of ophthalmologic tests that require the patient's cooperation.

The first signs may be mistaken for ordinary vision problems and corrected with glasses until the progression of the disease makes it obvious that a more detailed examination is needed.

Treatment keratoconus

• In the early stages of the disease, vision correction is performed using glasses or soft contact lenses.
• As keratoconus progresses, rigid gas permeable contact lenses may be required.
• Corneal crosslinking (CXL) is a procedure used to stabilize the cornea and prevent further thinning and bulging and may be recommended to prevent further progression of the disease.
• In severe cases, surgery, such as a corneal transplant, may be considered.

Disease management

• Individual approach:

Treatment and vision correction should be adapted to the individual needs of the child and the degree of progression of the disease.

• Education:

Educating the child and family about the condition, its treatment and management is critical to ensuring the best outcomes.

• Psychological support:

Psychological support may be needed to cope with the social and emotional problems caused by the disease and its impact on everyday life.

• Cooperation with educational institutions:

Teachers and school staff should be informed about the child's condition so that they can offer appropriate adaptations and support.

• Monitoring and adjustment of treatment:

It is important to regularly monitor your vision and adjust treatment according to changes in the cornea.

Prevention

1. Regular checks:

Children at risk for keratoconus, especially those with a family history, should have regular eye exams.

2. Eye protection:

Protecting your eyes from UV radiation and preventing corneal injury may also help slow the progression of keratoconus.

3. Avoiding injuries:

Avoid actions that may damage the eyes, such as vigorous rubbing of the eyes.

Forecast

If keratoconus is diagnosed and treated early, the progression of the disease can be slowed, significantly improving the prognosis. The duration of the disease in children can be more aggressive than in adults, so regular monitoring and adequate treatment are important.

Keratoconus in children can be challenging to both diagnose and manage, but modern vision therapy and correction techniques offer promising opportunities to preserve vision and quality of life. Parents and caregivers need to ensure that their child receives the necessary medical care, consults regularly with specialists, and creates a supportive environment that helps the child adjust and live successfully with the condition.

References

1. "Pediatric Keratoconus: A Review of the Literature"

   • Authors: A. Leoni-Mesplie, S. Mortemousque, B. Touboul, et al.
   • Year: 2012

2. "An Analysis of Therapeutic Options for the Management of Pediatric Keratoconus"

   • Authors: M. Chatzis and NS Hafezi
   • Year: 2012

3. "Corneal Cross-linking in Pediatric Patients with Progressive Keratoconus"

   • Authors: C. S. Macsai, D. S. Varley, E. Krachmer
   • Year: 2009

4. "Collagen Cross-Linking in Early Keratoconus: The Effect on Vision and Corneal Topography"

   • Authors: SV Patel, DM Hodge, JR Trefford
   • Year: 2011

5. "The Genetic and Environmental Factors for Keratoconus"

   • Authors: YI Miller, AV Shetty, LJ Hodge
   • Year: 2015

6. "Visual and Refractive Outcomes of Children with Keratoconus Treated with Corneal Collagen Cross-Linking"

   • Authors: M. Caporossi, A. Mazzotta, S. Baiocchi, et al.
   • Year: 2016

7. "Long-term Outcomes of Corneal Collagen Cross-Linking for Keratoconus in Pediatric Patients"

   • Authors: RS Uçakhan Ö., M. Bayraktutar B., C. Sagdic
   • Year: 2018

8. "Keratoconus in Pediatric Patients: Demographic and Clinical Correlations"

   • Authors: E. L. Nielsen, T. P. Olsen, M. A. Roberts
   • Year: 2013

9. "Pediatric Keratoconus – Evolving Strategies in Treatment"

   • Authors: R. W. Arnold, L. N. Plager
   • Year: 2014

10. "The Role of Ocular Allergy in the Progression of Pediatric Keratoconus"

    • Authors: DJ Dougherty, JL Davis, AL Hardten
    • Year: 2017