Congenital Corneal Opacities

Corneal disease is among the most important causes of visual impairment in children worldwide. CongenitalCongenital and infantileInfantile corneal opacities have a narrow therapeutic window for intervention due to the risk of amblyopia, and are often complex. The causes and clinical presentation of congenitalCongenital corneal opacities are varied. The correct diagnosisDiagnosis should be made early and appropriate interdisciplinary involvement established as necessary. The most important carers for the affected child are his/her parents and close cooperation between the parents and the clinical team involved is therefore essential. The ophthalmic workup of patients with congenitalCongenital corneal opacities includes a thorough history with subsequent clinical examination, often performed under general anesthesia and supported by new imaging modalities such as linear ultrasound, high frequency ultrasound and optical coherence tomography. The structured classification of congenitalCongenital corneal opacities provides the basis for targeted therapyTherapy and should be focused on whether the disease is due to a primary corneal disorder or if the corneal disease is due to secondary corneal disorders.. However, in children, ophthalmologic surgery is surgically more demanding and the prognosis is generally worse. Surgical procedures include e.g. phototherapeutic keratectomy, optical iridectomy, and lamellar, penetrating, and autokeratoplasty procedures. Newer techniques of selective endothelial removal and the use of simple limbal epithelial transplant are also part of the armamentarium for the surgeon. An equally important part of therapyTherapy is represented by conservative treatment with bandage lenses, glasses, contact lenses, and occlusion if necessary. The present work provides an overview of evaluation including clinical, diagnostic molecular of congenitalCongenital corneal opacities and discusses current treatment.