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.

Keywords: Allergy; DALK; atopy; corneal collagen crosslinking; genetics; keratoconus; pediatric; penetrating keratoplasty; vernal keratoconjunctivitis.

Figure 1

Clinical picture of pediatric keratoconus showing (a) Munson's sign, (b and c) apical scar, and (d) advanced ectasia

Flow Chart 1

Genetic mechanism involved in Keratoconus

Flow Chart 2

Pathogenesis of eye rubbing causing Keratoconus

Flow Chart 3

Pathogenesis of keratoconus in ocular allergies

Figure 2

Acute hydrops in a case of pediatric keratoconus

Figure 3

Clinical picture of GPC (a) and corneal opacity (b) in two different cases of pediatric keratoconus with vernal keratoconjunctivitis

Figure 4

Slit lamp photograph of a case of Brittle cornea syndrome bilateral keratoglobus with multiple repaired corneal tears (a) and healed hydrops (b)

Figure 5

Postoperative clinical picture of penetrating keratoplasty (a) and deep anterior lamellar keratoplasty (b) in pediatric keratoconus cases

Figure 6

Slit lamp photograph of post.DALK (at 3 months postoperative period) in a pediatric patient of VKC with keratoconus showing early suture loosening (a) [(note that differential wound healing had necessitated prior removal of few sutures (b)]; a different case of DALK in pediatric keratoconus with loose sutures (c)

Figure 7

Slit lamp photograph of DALK of a case of pediatric keratoconus (a) showing increased stromal edema (b), corneal haze (c) due to stromal graft rejection; (d) ASOCT picture of the same eye showing the increased stromal thickness due to edema as a result of immune rejection