Commentary: Keratoconus in pre-teen children: Demographics and clinical profile

We read with great interest the manuscript titled “Keratoconus in pre-teen children: Demographics and clinical profile” by the authors.[1] The manuscript describes in detail the clinical profile of keratoconic children, less than 12 years of age.

The study includes a large cohort of keratoconic children (586 eyes) without any other active comorbid conditions of the eye. Majority studies in the literature evaluating the outcomes of pediatric keratoconus include children with age less than 17–19 years of age. The study here includes children specifically below 12 years of age. The title chosen by the authors “Keratoconus in pre-teen children” attracts attention and adds an entirely new dimension to the original spectrum of the disease.

As far as the disease course and progression is concerned, the classical description says that Keratoconus usually manifests in the second decade of life with puberty as the starting point, although cases as young as 4 years old have also been reported.[2,3] When it comes to children, it takes an aggressive course, due to the elastic nature of the juvenile cornea.[4] Progression has been seen to involve as high as 88% of study patients, over a period of 1 year.[5] As rightly pointed by the authors, since children have to deal with many more years of potential progression, there is a need to actively monitor and chase the course of the disease.

The current principles of management and choice of treatment for managing keratoconic children cannot be directly replicated from the adults. Corneal cross linking (CXL) has been seen to be safe and effective in arresting the progression of keratoconus in pediatric patients, besides allowing improvement in refractive, topographic, and corneal aberrometric parameters.[6] Also studies report comparable improvement in topographic indices between children and adults.[7]

However, this is also not a straight road ahead, with various concerns such as deciding the exact timing of CXL, choosing the cutoff for deciding actual progression, the chances of continued progression after the procedure, a higher rate of cooccurrence of ocular allergy in keratoconic children, and the relative efficacy of various protocols. Achiron et al.[8] in their systemic review found a 10% risk of progression after CXL in children. More detailed studies analyzing the dynamic properties of cornea in this age group will provide an answer to this situation.

Considering the technical difficulties of performing and following up of pediatric corneal transplants, all attempts should be made to halt the progression of disease. The first diagnosis of the occurrence of a refractive error in a child should prompt the physicians to carefully evaluate and rule out an ectatic corneal pathology. Another possible way to pick up preteen keratoconus right at its inception is to advise regular screening, for children of patients with diagnosed keratoconus in view of the established genetic nature of the disease.[9]

To conclude, the subcategory of Keratoconus under 12 years of age needs to be separately understood from the “teenage” keratoconus. Understanding and comparing the biomechanical properties of cornea among normal teens and preteens versus keratoconic teens and preteens versus patients with a simple myopic refractive error should shed further light on the understanding of this disease. The study published by the authors is an important addition to the literature and shows a new path which should be explored and its dimensions be widened.