IRIS ECTROPION SYNDROME

Course overview

IRIS ECTROPION SYNDROME IS A RARE CONGENITAL ANOMALY. CONGENITAL IRIS ECTROPION IS CAUSED BY THE DEVELOPMENTAL ARREST OF THE NEURAL CREST, UNLIKE AXENFELD RIEGER SYNDROME AND PETERS ANOMALY, WHICH ARE CAUSED BY DISORDERS IN THE PROLIFERATION, MIGRATION, AND DIFFERENTIATION OF NEURAL CREST CELLS. IT HAS TO BE DIFFERENTIATED FROM THE IRIDOCORNEAL ENDOTHELIAL SYNDROME, WHICH DEVELOPS LATER IN LIFE. ASSOCIATION WITH NEUROFIBROMATOSIS TYPE 1 AND PRADER-WILLI SYNDROME MAY OCCUR. ASSOCIATED FEATURES SUCH AS TELECANTHUS, PTOSIS, AND NYSTAGMUS MAY BE SEEN. CAREFUL FOLLOW-UP IS REQUIRED, AS GLAUCOMA MAY DEVELOP AT ANY TIME IN THE LIFE OF THESE PATIENTS, WHICH MAY NEED SURGICAL MANAGEMENT. ACQUIRED ECTROPION UVEAE MAY OCCUR DUE TO THE FORMATION OF MEMBRANE ON THE IRIS DUE TO ADVANCED DIABETIC RETINOPATHY, VASCULAR OCCLUSIONS, OCULAR ISCHEMIC SYNDROMES, METASTASIS TO THE IRIS, IRIS MELANOMA, AND OTHER CAUSES OF NEOVASCULAR GLAUCOMA.