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Clinical Challenge
March 2018

Facial Pain and Diplopia in a Young Boy

Author Affiliations
  • 1Department of Otolaryngology, SUNY Downstate Medical Center, Brooklyn, New York
JAMA Otolaryngol Head Neck Surg. 2018;144(3):270-271. doi:10.1001/jamaoto.2017.2906

A young boy presented to the emergency department with a 2-week history of left facial and eye pain with intermittent diplopia on upward and lateral gaze. He was otherwise healthy and exhibited no constitutional complaints. His remaining medical, surgical, family, and social history was noncontributory. Examination showed a healthy-appearing child with left abducens nerve palsy and an afferent pupillary defect. There was no chemosis, exophthalmos, or enophthalmos. Findings from nasal endoscopy were unremarkable. A computed tomographic scan of the sinuses without contrast showed a well-circumscribed posterior-medial extraconal mass extending into the orbital apex with smooth, nonaggressive bony remodeling and expansion of the left superior orbital fissure with further dorsal extension into the left cavernous sinus (Figure, A). Magnetic resonance imaging of the orbits showed that orbital component was inseparable from medial rectus muscle. Tumor also resulted in lateral displacement and compression of the left optic nerve (Figure, B). The patient underwent an image-guided left endoscopic orbital decompression with biopsy of the orbital mass. Representative hematoxylin-eosin–stained sections are shown in the Figure, C and D. The tumor was negative for FUS (FUsed in Sarcoma; also termed TLS, Translocated in LipoSarcoma)-CHOP (C/EBP HOmologous Protein; also termed DDIT3, DNA Damage-Inducible Transcript 3) fusion oncogene on fluorescent in situ hybridization (FISH).

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