Keratoconus is a progressive asymmetric disorder associated with structural changes in corneal collagen organization. The disease usually manifests in the second decade of life, resulting in corneal thinning. If left untreated, keratoconus leads to irregular astigmatism, progressive myopia, corneal scarring, and poor vision.

When diagnosed in pediatric patients, the disease stage and progression of keratoconus is more severe (Kankariya et al, 2013). Because the disease is more aggressive in pediatric populations, management and treatment should be pursued immediately (Tuft et al, 1994; Kankariya et al, 2013). This article discusses current management and treatment options for pediatric keratoconus.

Optical Correction

Uncorrected high myopia and astigmatism can lead to amblyopia in children. Thus, it is important to prescribe optical correction as soon as possible. In early keratoconus, more regular astigmatism is correctable with spectacles and soft toric contact lenses. GP contact lenses correct highly irregular astigmatism in more advanced keratoconus and in surgically altered corneas (Figure 1). Children are capable of handling contact lenses as early as age 4 to 5 years old (Figure 2), and they are often able to take complete responsibility of all aspects of contact lens care at 10 years of age (Walline and Sindt, 2014).

Surgical Options

Based on its success in adult populations, the efficacy of corneal cross-linking (CXL) for slowing disease progression in pediatric keratoconus was examined. The standard epithelium-off protocol performed in children showed stabilization in disease process for up to five years (Arora et al, 2012; Wise et al, 2016). Improvement of corrected distance vision and a decrease in maximum keratometry readings also were reported (Padmanabhan et al, 2017; Toprak et al, 2017).

Noted progression after CXL is attributed to the corneal structural difference in adults versus children. Natural cross-linking occurs with aging, leading to increased stabilization of keratoconus in adults (Chatzis and Hafezi, 2012). Progression after CXL is linked to persistent eye rubbing (Kankariya et al, 2013) and vernal keratoconjunctivitis (Rabinowitz, 1998) and should be considered for pediatric patients.

The implantation of an intrastromal corneal ring segment is a refractive procedure used to flatten the center of the cornea in adult keratoconus patients. Although safe and reversible in adults (Alio et al, 2006), it is not preferred in pediatrics due to rapid progression of disease and eye-rubbing tendencies (Kankariya et al, 2013). It may be considered for adolescent patients who have end-stage keratoconus and will most likely undergo keratoplasty (Kankariya et al, 2013). Finally, corneal transplants are advocated for pediatric patients only in very advanced disease (Mukhtar and Ambati, 2017).

Summary

Compared to adult-onset disease, keratoconus in children is more severe and requires more frequent follow-up visits to limit its progression and consequences. Because amblyopia is a risk, early intervention of keratoconus is imperative. CLS

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