Pediatric keratoconus - Current perspectives and clinical challenges

Abstract

Keratoconus is an ectatic corneal disease characterized by progressive stromal thinning, irregular astigmatism, and defective vision. It can be unilateral or bilateral with asymmetric presentation. It starts at puberty and either progresses rapidly to an advanced stage of the disease or stops in case of delayed onset and slow progression. Pediatric keratoconus is more aggressive than in adults and the management protocols differ because of various rationales such as accelerated progression, advanced stage of disease at the time of diagnosis and co-morbidities. It poses a burden to the society as it affects the quality of life, social, and educational development in children. Hence early diagnosis, recognition of progression, and timely intervention with collagen crosslinking is imperative to arrest the worsening. Association with systemic syndromes and ocular comorbidities can be of concern in pediatric keratoconus. Severe ocular allergy when associated hastens progress and complicates timely intervention of crosslinking treatment and compliance to contact lens wear. Keratoplasty in pediatric keratoconus has good outcomes but can encounter frequent suture-related concerns. This article discusses the epidemiology, etiopathogenesis, clinical challenges, and current perspectives of management of pediatric keratoconus.

Accelerated Corneal Crosslinking for Treatment of Keratoconus in Children and Adolescents under 18 Years of Age

“…Bis zur 6-wöchigen Nachbeobachtung wird eine "Pseudoprogression" beobachtet, die nicht auf die Langzeitergebnisse schließen lässt. more aggressive course of KC in young ages [3,9]. Out of the 41 eyes, in the case of 16 eyes of 9 patients, A-CXL was conducted without waiting for an evident KC progression.…”

“…It is characterized by a progressive thinning and protrusion of the cornea, which leads to high astigmatism and myopia, corneal scarring in some cases, and visual impairment [1,2]. The incidence varies according to the variation of genetic and environmental factors and is estimated to range from 50 to 230 per 100 000 in patients under 18 years of age, although this number seems to be affected by the progressively increasing use of new diagnostic tools [3]. Its onset is estimated to start during puberty and seems to stabilize after the fourth decade of life [2].…”

“…There are several studies that reveal the fact that young ages are associated with more severe KC forms and with faster progression of the disease [6 -8]. History of ocular allergy and atopy as well as eye rubbing are important risk factors for rapid progression [3,9]. Younger ages at the time of diagnosis are also linked to a greater risk of corneal opacification and scarring, leading to corneal transplantation [7,10,11].…”

Corneal Scheimpflug topography values to distinguish between normal eyes, ocular allergy, and keratoconus in children

“…Keratoconus (KC) is an ectatic corneal disease characterized by the progressive stromal thinning, protrusion of the cornea, irregular astigmatism, and vision impairment. The prevalence of KC in general population is estimated to be around 1 in 2000 1 , but it varies in different population, ethnicity, age, and studies according to different diagnose criteria, variance in genetics, environmental factors, nutrition, and diagnose tools 2 . Some studies report incidences between 5 and 23 in 10,000, and prevalence 4 and 60 out of 100,000 3 .…”

“…KC onset usually occurs during puberty 2 , 23 ; however, when it develops earlier, it reaches more advanced stages sooner and progresses faster 24 , leading to greater decreases in quality of life, worsened visual acuity, and negative impacts on social and educational development 25 . Childhood KC is also associated with an elevated risk of delayed diagnosis, corneal scarring, and penetrating keratoplasty 26 .…”