Papilledema With Intracranial Hypertension and Ectopic Orbital Calcification During Hemodialysis: A Case Report

Abstract

Papilledema is optic disc swelling due to intracranial hypertension, which leads to progressive visual impairment. We report a rare case of papilledema with ectopic orbital calcification during hemodialysis. A 34-year-old woman with low body weight who was undergoing long-term hemodialysis presented with papilledema in both eyes, and her vision gradually deteriorated over the course of six months. The best corrected visual acuity was 20/16 in the right eye and 20/500 in the left eye, and fundus examination revealed significant optic disc swelling and visual field testing revealed nasal defects in both eyes and a central scotoma in the left eye. Computed tomography scan showed ectopic orbital calcification in the sclera and optic nerve margin. Orbital magnetic resonance imaging and magnetic resonance venography did not show optic neuritis or cerebral venous sinus thrombosis. Blood test results indicated hyperparathyroidism, which was considered to be a secondary change associated with long-term hemodialysis. Cerebrospinal fluid test confirmed intracranial hypertension, and treatment to reduce intracranial pressure was required to prevent the progression of visual impairment. Oral treatment was difficult, so surgical treatment was considered. Papilledema can be diagnosed from optic disc findings, and it is important to differentiate and search for various causes, including idiopathic intracranial hypertension, and to intervene at the appropriate time before visual impairment progresses.

Keywords: cerebrospinal pressure shunt surgery; ectopic calcification; intracranial hypertention; optic disc swelling; papilledema.

Figure 1. (A) Fundus photography at the initial visit showing optic disc swelling and redness in both eyes; (B) Autofluorescence showing no signs of optic disc drusen

Figure 2. (A) Optical coherence tomography showing optic disc swelling in both eyes; (B) No serous retinal detachment is seen in the macula; (C) The RFNL of both eyes, especially the left eye, is thickened circumferentially

RFNL: retinal nerve fiber layer: OD: oculus dexter (right eye); OS: oculus sinister (left eye)

Figure 3. Anterior corneal findings showing calcification resembling band-keratopathy (arrows) in both eyes

Figure 4. The visual field testing of Goldmann perimeter (GP).

GP at the initial visit revealed bilateral visual field defects on the inferior nasal side and a left central scotoma.

Figure 5. Orbital computed tomography (CT) images

The horizontal orbital CT images revealed ectopic calcification in the sclera of the eyeball wall (blue arrows) and optic canal stenosis due to bone thickening (red arrows) (A); The coronal images also showed ectopic calcification along the edge of the optic nerves and vascular walls (yellow arrows), mild enlargement of the diameter of the optic nerves (B), and mild thickening of the entire skull (C). The bone density of these calcification areas in the orbit was approximately 200-300 HU of CT values.

Figure 6. (A) Orbital MRI showing mild enlargement of the subarachnoid space around the optic nerve (arrows), but no optic neuritis, space-occupying intracranial lesions, or ventricular enlargement; (B) MRA showing no aneurysm or arterial occlusion; (C) MRV showing no signs of cerebral venous sinus thrombosis

MRA: magnetic resonance angiography; MRV: magnetic resonance venography

Figure 7. Fundus findings and Goldmann visual field six months after initial visit.

(A) The papilledema is slightly decreased, and the left optic disc is turning pale and entering the atrophy stage; (B) The right central vision is preserved, while the left central visual field loss remain