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A Mass in the Greater Wing of the Sphenoid in a Pediatric Patient

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

A 4-year-old girl with no previous medical or ocular history presented with 10 days of right painless, periorbital swelling and erythema. The patient was previously treated with an oral combination of amoxicillin and clavulanic acid and topical gentamicin therapy without any improvement. The patient’s visual acuity, color vision, and pupillary examination results were within normal limits, and intraocular pressures were 28 mm Hg OD and 20 mm Hg OS. The extraocular movements and confrontational visual fields were normal. There was right upper and lower eyelid edema with mild erythema and 3 mm of proptosis. The eyelids were nontender to palpation. There was trace vasodilation and chemosis of the conjunctiva in the right eye. The remainder of the slitlamp and dilated fundus examination results were within normal limits. Computed tomography of the orbits and magnetic resonance imaging (MRI) of the brain and orbits showed a large lesion of the right greater wing of the sphenoid bone extending toward the orbital apex with edema and enhancement of the right temporal lobe of the brain (Figure 1).

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Langerhans cell histiocytosis

D. Perform biopsy of lesion

Performing a biopsy (choice D) allows for proper diagnosis and management. A diagnostic biopsy through a lateral orbitotomy revealed histiocytic cells with abundant eosinophilic cytoplasm and enlarged nuclei with lobation of the nuclear membrane (Figure 2). The CD207 immunohistochemical stain was diffusely positive, consistent with Langerhans cell histiocytosis (LCH).

The differential diagnosis of a sphenoid wing mass in a child includes rhabdomyosarcoma, Ewing sarcoma, LCH, metastasis, and infection. Although the external examination results were suggestive of orbital cellulitis, the patient was afebrile and there was no obvious source of infection; thus, intravenous (IV) antibiotic therapy (choice B) would be inappropriate. An inflammatory lesion was possible; however, the extent of the process and involvement of the temporal lobe would make the use of IV corticosteroid therapy (choice C) inappropriate. Although a metastatic lesion was possible, a positron emission tomographic scan (choice A) prior to biopsy would not be desirable. Performing a biopsy (choice D) would be essential to determine the diagnosis.

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Article Information

Corresponding Author: Richard C. Allen, MD, PhD, Department of Ophthalmology, Baylor College of Medicine, 1977 Butler Blvd, BCM633, Houston, TX 77030 (richard.allen2@bcm.edu).

Published Online: March 26, 2020. doi:10.1001/jamaophthalmol.2020.0372

Conflict of Interest Disclosures: None reported.

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

References
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Schmitz  L, Favara  BE.  Nosology and pathology of Langerhans cell histiocytosis.  Hematol Oncol Clin North Am. 1998;12(2):221-246. doi:10.1016/S0889-8588(05)70507-4PubMedGoogle ScholarCrossref
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