Case

A 5-year-old Hispanic boy was referred to the University of Virginia Ophthalmology Clinic for evaluation of strabismus. His parents gave a 1-month history of intermittent crossed eyes. His ocular history was significant for astigmatism; however, the medical history was unremarkable. He was the product of a full-term uncomplicated pregnancy and had met all developmental milestones and immunisation requirements. Family history was significant for hypertension in his parents, and his review of systems was positive only for occasional headaches.

On examination, the best corrected visual acuity was 20/200 in the right eye and 20/50 in the left. External examination result was unremarkable. Motility examination showed 16 diopters of comitant esodeviation and overaction of the right inferior oblique with a small left face turn and left head tilt. The pupils were equally round without an afferent pupillary defect. Automated visual field testing revealed moderate diffuse depression in the right eye with an enlarged blind spot, inferior arcuate defect and superior Seidel scotoma. The left eye demonstrated similar abnormalities with diffuse depression, a cecocentral scotoma and superior arcuate changes. Slit lamp examination result was within normal limits, and there were no cells in the anterior chamber or vitreous. Intraocular pressures were normal. Dilated fundus examination showed extensive bilateral macular exudates and disc oedema with a few scattered intraretinal haemorrhages (figure 1).

A diagnosis of congenital fourth nerve palsy of the right eye was made, although his esodeviation remains unexplained. There was no evidence of sixth cranial nerve palsy. The aetiology of his fundus findings was unclear. Bartonella titres were negative. …