Aanpak van congenitaal cataract: een actueel overzicht

Current management of congenital cataract: an overview Congenital cataract, a congenital clouding of the lens of the eye, annually affects 20,000 to 40,000 neonates worldwide. An important clinical sign is complete or partial leukocoria, a white pupillary reflex, detected on a screening examination of the newborn in primary care or otherwise noticed by the parents. Congenital cataracts can occur uni- or bilaterally. Unilateral cataract is mostly due to a developmental disorder of the lens. Bilateral cataract is often isolated and caused by a genetic mutation, but can also be seen in an underlying syndrome, a metabolic disorder, a chromosomal disorder or a congenital infection, requiring further investigation by the pediatrician. It is crucial to recognize the condition and to refer the patient to a specialized center for pediatric cataract as early as possible to initiate an appropriate investigation and treatment. The mainstay of the therapy is cataract surgery. Its ideal timing varies individually, but in most cases the procedure is planned very early: ideally at the age of 6 to 8 weeks. Afterwards, an intensive and long-term treatment with contact lenses, glasses and often occlusion therapy is needed to stimulate the visual development. Lifelong follow-up is indicated. This literature review provides a comprehensive overview of the epidemiology, classification, diagnosis, treatment and prognosis of congenital cataract.