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JAMA Neurology Clinical Challenge
September 2018

Proptosis and Double Vision in a Child

Author Affiliations
  • 1Department of Ophthalmology and Visual Science, Yale School of Medicine, New Haven, Connecticut
  • 2Department of Neurology, Yale School of Medicine, New Haven, Connecticut
JAMA Neurol. 2018;75(9):1142-1143. doi:10.1001/jamaneurol.2018.1271

An 8-year-old boy presented to the pediatric emergency department with 2 weeks of fatigue and 3 days of double vision, ptosis, and painless periorbital swelling of the left eye. Medical history was unremarkable with no recent weight loss, travel, or trauma. Visual acuity without correction was 20/20 OU. The right pupil was 3.5 mm and briskly reactive, while the left pupil was 8.5 mm and unreactive to light or near effort; there was no relative afferent pupillary defect. Results of testing with the use of an Amsler grid and Ishihara color plates were within normal limits. Visual fields were full to confrontation. The optic nerves and retina were normal in both eyes. External examination was notable for left periorbital swelling, proptosis, and moderate ptosis of the upper eyelid (Figure 1A). Extraocular motility examination results were normal in the right eye but demonstrated severe ophthalmoparesis of the left eye: 0% supraduction (Figure 1B), 20% of normal abduction/adduction, and 10% of normal infraduction (not pictured). Corneal reflex was normal in the right eye but diminished in the left eye, and facial strength was normal bilaterally. The remainder of the neurologic examination results were normal.