Differential Diagnosis of Leukocoria

Leukocoria is the most common presenting sign of intraocular retinoblastoma in developed countries. Directed by the available demographic and historical data, a comprehensive clinical and ultrasound examination in the office is usually sufficient to make the correct diagnosis. Occasionally, an examination under anesthesia may be necessary to distinguish retinoblastoma (RB) from simulating conditions, such as Coats’ disease, persistent hyperplastic primary vitreous (PHPV), retinal dysplasia, or astrocytic hamartoma. Clinical findings associated with the commonly diagnosed conditions are summarized in this chapter.