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A Child With Acute Eyelid Edema and Proptosis

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 2-year-old girl presented to the emergency department with 2 days of left upper eyelid swelling that started 1 day after an episode of vomiting due to a viral illness. She had no associated fever, chills, upper respiratory tract symptoms, lethargy, or changes in behavior. She had an unremarkable ocular and medical history. On examination, visual acuity was fix-and-follow in each eye. Both eyes were soft to palpation. Pupils were round, symmetric, and reactive to light without an afferent pupillary defect. Ocular movements were full, and the patient had grossly full visual fields to confrontation. External examination revealed mild left-sided ptosis, axial proptosis, and upper eyelid fullness with no mass or tenderness to palpation (Figure, A). Slitlamp and fundus examination results were unremarkable, and there was no left optic nerve edema or pallor. Magnetic resonance imaging (MRI) of the orbits revealed a left intraconal mass that was isointense relative to rectus muscles on T1-weighted images and hypointense with a hyperintense rim on T2-weighted images (Figure, B).

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Orbital venolymphatic malformation with acute intralesional hemorrhage

B. Obtain computed tomographic scan of the orbits

The differential diagnosis for orbital masses in children differs from that in adults and includes dermoid cyst, vascular malformation, optic nerve glioma, rhabdomyosarcoma, and orbital metastasis. Neuroimaging modalities, such as MRI or computed tomography (CT), can be very helpful in differentiating among these lesion types. This patient’s MRI, however, did not show distinguishing features to assist in narrowing the differential diagnosis. It would be inappropriate to observe a new and undifferentiated orbital mass that could potentially be life threatening, vision threatening, or both (choice A). As recommended by the neuroradiology service, CT scanning was performed to further characterize the mass and make an accurate diagnosis (choice B). Although biopsy is the criterion standard for diagnosis, multimodal imaging can sometimes accurately identify a benign mass and make an invasive biopsy unnecessary (choice C). This minimizes the risk of complications such as hemorrhage, which is especially true in vascular lesions. Aggressive treatment with chemotherapy and radiotherapy should not be initiated until after a diagnosis is established because these treatments are appropriate only for certain malignant lesions, such as rhabdomyosarcoma, optic nerve glioblastoma, and orbital metastasis (choice D).

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Article Information

Corresponding Author: Kara M. Cavuoto, MD, Bascom Palmer Eye Institute, Attn: Mr. Jose Aponte, 900 NW 17th St, Miami, FL 33136 (kcavuoto@med.miami.edu).

Published Online: November 25, 2020. doi:10.1001/jamaophthalmol.2020.4067

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s family for granting permission to publish this information.

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