Pediatric Diseases in the Context of Infantile Anterior Segment Disorders

Disorders of the anteriorAnterior segment of the eye segment of the eyeAnterior segment of the eye encompass a variety of diseases affecting the cornea, the iris, and the lens. Developmental disorders of the anteriorAnterior segment of the eye segment of the eyeAnterior segment of the eye are often associated with systemic manifestations, such as posterior embryotoxon in Alagille syndrome, aniridia in WAGR syndrome, congenitalCongenital iris ectropion syndrome in Prader-Willi syndrome or neurofibromatosis. Further affections of the anteriorAnterior segment of the eye segment of the eyeAnterior segment of the eye are e.g. congenitalCongenital glaucoma and the corneal dystrophies (which can also be found in Alport syndrome) There are several risk factors (systemic disease and environmental factors) predisposing to keratoconusKeratoconus. Inherited metabolic disordersInherited metabolic disorders can initially present with symptoms affecting the eye (e.g., neonatal cataract in disorders of galactose metabolism), but eye disorders can also evolve later in the natural history of an already diagnosed metabolic disorder (e.g., cornea verticillata in Fabry disease). There are also infectious causes for diseases of the anterior segment, such as herpes keratitis, Chlamydia keratoconjunctivitis and gonococcal conjunctivitis which play an important role on a global scale. Other conditions associated with anomalies of the anterior segment are Goldenhar Gorlin syndrome where patients may show a dermoid and X-chromosomal ichthyosis.