Background:

The rapidly progressive proptosis in neonates is not frequent, it comprises a broad spectrum of pathologies that might endanger life and ocular function.

Case description:

A 12-day male began with right rapidly progressive proptosis and palpebral edema. On examination, displaced proptosis was observed towards the superior temporal region, edema of both eyelids and chemosis in the right eye. A CT was performed evidencing a neoproliferative intraorbital lesion that reinforces with contrast. A BAAF was taken, evidencing neoplastic cells. The tumor was resected and histopathological study identified vascular neoplasia with a definitive diagnosis of epithelioid hemangioendothelioma. He received treatment with 6 courses of vincristine, actinomycin and cisplatin; in the re-evaluation, a residual tumor was found, therefore enucleation of the right eye was performed due to failure in the response to treatment. Currently, the patient is under surveillance.

Discussion:

The causes of ocular proptosis in the neonate are similar to those of other ages. Orbit primary tumors represent a large percentage of malignant causes. The epithelioid hemangioendothelioma is a vascular neoplasm which behavior is controversial, some cases are indolent and others present progression and metastasis. The treatment is the complete resection of the tumor; neither radiation therapy nor chemotherapy alone are curative options. Orbital malignancies in the child can be treated effectively with a timely diagnosis. The pediatrician in these cases should have an adequate suspicion and opportune referral.

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