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A 12-Month-Old Boy With Bilateral Facial Swelling 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 12-month-old healthy boy was seen at the outpatient ophthalmology clinic with gradually worsening bilateral temporal facial swelling and proptosis that developed in the setting of a recent bilateral ear infection 10 days prior. He was currently being treated with oral amoxicillin. His mother reported a recent decrease in appetite, decreased feeding, increased irritability, and near-constant crying.

On examination, he was inconsolable and crying. His pulse rate was 112 beats/min, blood pressure was 122/68 mm Hg, and temperature was 38.7°C. He had soft, bitemporal facial masses (Figure, A). He was proptotic and demonstrated a substantial change in his ocular appearance compared with photographs taken 4 weeks earlier that his mother brought for our review. He appeared to be orthophoric in primary gaze but had bilateral abduction deficits. His pupils were round and reactive, without an afferent pupillary defect. His anterior and posterior segment examination results were normal, without evidence of optic nerve edema or pallor. His pediatrician had obtained a complete blood cell count and head radiograph 10 days prior that were both normal.

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Metastatic neuroblastoma

C. Admit for urgent imaging and inpatient workup

Acute-onset proptosis in a child is always concerning, and urgent imaging should be obtained. Several underlying etiologies are possible, including an orbital infection or a neoplastic process (neuroblastoma, rhabdomyosarcoma, leukemia, and Langerhans cell histiocytosis). Because these processes can rapidly become vision- or life-threatening, urgent imaging is warranted, and observation, further workup as an outpatient, and broadening antibiotic coverage are not appropriate. Other orbital processes that lead to subacute or chronic proptosis in children include capillary hemangioma, optic nerve glioma, venolymphatic malformation, dermoid cyst, orbital pseudotumor or other inflammatory process, and thyroid eye disease.1,2

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Article Information

Corresponding Author: Karen E. Revere, MD, Division of Ophthalmology, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, CHOP 9-Main, Philadelphia, PA 19104 (reverek@email.chop.edu).

Published Online: April 12, 2018. doi:10.1001/jamaophthalmol.2017.5804

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest, and none were reported.

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

References
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