Pediatric limbal stem cell deficiency: An overview of a rarely studied pathology

Abstract

The cornea is the outermost transparent layer of the eye, and the continuous renewal of the corneal epithelium is important for its transparency. This process is primarily facilitated by corneal stem cells, most of which are found at the limbus. However, any deterioration or damage in this region leads to corneal conjunctivalization and consequent limbal stem cell deficiency (LSCD), which compromises corneal transparency. LSCD is an important condition, especially in the pediatric population, as it can lead to corneal vascularization, opacity and ultimately loss of vision and subsequent amblyopia, unlike adults. Although pediatric LSCD is often due to chemical injuries, as in adults, it is also caused by conditions such as congenital aniridia, Steven Johnson Syndrome and various other immunological disorders. Appropriate and timely treatment in pediatric LSCD is of particular importance in preventing progression to amblyopia, unlike adults. Accurate staging of the disease is essential for the necessary medical and surgical treatment decision. While medical approaches are at the forefront to eliminate the causative agent and improve the ocular surface in reversible cases, it is essential to replace the limbal stem cells lost in advanced disease. For these replacement procedures, it is noteworthy that there is a tendency for minimally invasive methods compared to adults to avoid possible complications due to long life expectancy in children. In conclusion, although there are various reviews on limbal stem cell deficiency (LSCD) in adults, we believe that this review for childhood LSCD will make an important contribution to the literature, since the relevant literature currently reported for the pediatric population is limited.

Keywords: Limbal stem cells; limbal stem cell transplantation; pediatric limbal stem cell deficiency.