Limbal Cell Deficiency Associated with K.I.D. Syndrome

Abstract

Abstract: : Purpose: To describe and to characterize the clinical findings and treatments of patients with limbal stem cell deficiency (LSCD) in KID ( Keratitis-Ichthiosis-Deafness) syndrome. Methods: We report the case of a 30 year old woman with K.I.D. syndrome consulting for therapeutic advice concerning a massive corneal neovascularization. She already underwent two unfruitful penetrating keratoplasties in her left eye. Results: Visual acuity was limited to a simple light perception because of the opacity and neovascularization of the graft. In the light of this case of limbal cell deficiency, we will discuss the ophthalmologic manifestations of KID Syndrome, its clinical characteristics, its physiopathology and its mode of transmission. Severity of LSCD was correlated with the presence of goblet cells by impression cytology. Conclusions: KID syndrome is a rare and as yet poorly recognized entity responsible for LSCD. Correct understanding of the physiopathological mechanisms may prevent unnecessary surgeries. Impression cytology is a simple tool in order to appreciate the severity of the disease.

Keywords: cornea: clinical science • neovascularization • apoptosis/cell death