Several pediatric malignancies may occur in the orbit. Benign adnexal masses, which may threaten vision, are common in the pediatric population.
Diagnosis of space-occupying lesions in the orbit is particularly challenging because the clinical manifestations are both nonspecific and relatively limited:
proptosis or other displacement of the globe
swelling or discoloration of the eyelids
palpable subcutaneous mass
Many orbital processes may cause rapid onset of symptoms. These include trauma, which may occur without a reliable history. Mild or moderate proptosis can be difficult to detect in an uncooperative child with associated eyelid swelling. Nevertheless, typical presentations of the more common benign orbital and periorbital masses in infants and children (eg, hemangioma and dermoid cyst, discussed later) are sufficiently distinctive to permit confident clinical diagnosis in most cases. A malignant process should be suspected when proptosis and eyelid swelling suggestive of cellulitis are not accompanied by signs of inflammation or when periorbital ecchymosis or hematoma develops in the absence of a history of trauma. Pseudoproptosis can result when the volume of the globe exceeds the capacity of the orbit (eg, patients with primary congenital glaucoma or high myopia).
High-quality imaging allows orbital masses to be differentiated noninvasively in many cases. Magnetic resonance imaging (MRI) is the preferred modality for most patients. Computed tomography (CT) is superior at detecting bone abnormalities but exposes the child to radiation and thus should be avoided unless necessary. Ultrasonography may be useful.
Definitive diagnosis often requires biopsy. A pediatric oncologist should be consulted when appropriate. A metastatic workup should be considered prior to orbital surgery, because other, more easily accessible sites can sometimes be biopsied.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.