Introduction

Several benign and malignant ocular tumors can occur in childhood. Tumors in the ocular region can lead to loss of vision, loss of the eye and, in the case of malignant neoplasms, to loss of life. Therefore, it is important for the clinician to recognize childhood ocular tumors and to refer affected patients for further diagnostic studies and appropriate management. Based on the authors’ extensive clinical experience with ocular tumors during the last 35 years, some general concepts of childhood eye tumors are reviewed and the clinical manifestations of selected specific tumors of the eyelid, conjunctiva, intraocular structures and orbit in children are discussed.1–5

Clinical signs

of childhood ocular tumors

The clinical characteristics of childhood ocular tumors vary as to whether the tumor is located in the eyelids, conjunctiva, intraocular tissues, or the orbit.

Eyelid and conjunctiva

Eyelid and conjunctival tumors are generally quite evident, prompting an early visit to a physician. Since most tumors in the ocular area have characteristic features, an accurate diagnosis of eyelid and conjunctival tumors can usually be made with inspection alone. Therefore, additional diagnostic studies are often unnecessary.

Intraocular tumors

Unlike tumors of the eyelids and conjunctiva, intraocular tumors are not readily visible. Infants and very young children do not complain of visual loss and their visual acuity is difficult to assess. However, there are several features that should alert the pediatrician to consider the possibility of an intraocular tumor and prompt a timely referral.

LEUKOCORIA

One of the more important signs of an intraocular tumor in children is leukocoria, or a white pupillary reflex (271). There are many causes of leukocoria in children.2,4–7 The more common causes include congenital cataract, retinal detachment due to retinopathy of prematurity, persistent hyperplastic primary vitreous, and Coats’ disease. Retinoblastoma is probably the most serious condition to cause leukocoria in children. Any child with leukocoria should be referred promptly to an ophthalmologist for further diagnostic evaluation.