A 40-year-old male patient presented with sudden blurring of vision in the right eye that had persisted for 2 days. He reported that discomfort and redness had occurred in the eye, along with periorbital skin rashes over the affected side, almost 2 weeks prior to the visual obscuration. He had no history of systemic illnesses such as diabetes mellitus or bleeding disorders. He had not been in contact with persons with any skin disorders, and he had no history of drug abuse.
On examination, his best corrected visual acuity was hand motion in the right eye and 6/6 in the left eye. Crusted skin lesions were distributed along the ophthalmic division of the trigeminal nerve on the right side, as well as on the tip of the nose (Hutchinson's sign). A corneal dendritic ulcer accompanied by reduced corneal sensation was found in the right eye. The same eye showed a positive relative afferent pupillary defect [Figure 1a]. Fundus examination of this eye revealed a cherry-red spot surrounding pale retinal nerve fiber layers, box-carring over the superotemporal arcade, and attenuation of the retinal arteries [Figure 1b]. Anterior and posterior segment examinations of the left eye revealed no significant abnormalities. The intraocular pressure (IOP) in both eyes was 14 mmHg. The patient's blood pressure was 106/75 mmHg, while his random blood sugar was 7.0 mmol/L.
HZO with CRAO was diagnosed in the right eye. Intermittent ocular massage and breathing into a paper bag were performed. Oral acetazolamide (500 mg) and topical timolol (0.5%) were initiated immediately. The patient was also started on oral acyclovir (800 mg, 5 times daily) as well as acyclovir ophthalmic ointment in the affected eye (5 times daily). The next day, the vision in his right eye improved to counting fingers at 1 foot.
A number of investigations were performed during the patient's first presentation. Laboratory analyses showed a white cell count of 6.4 x 103/mul, hemoglobin of 14.3 g/dL, a platelet count of 219 x 103/mul, and an erythrocyte sedimentation rate of 14 mm/hr. Tests for anti-nuclear antibody, p- and c-antineutrophil cytoplasmic antibodies (ANCA), rheumatoid factor, thrombophilia, C-reactive protein, protein-C and protein-S, and human-immune-deficiency virus (HIV) were all negative or within normal limits, as was the venereal disease research laboratory (VDRL) test. The patient's fasting lipid profile and blood sugar were normal. The Mantoux test and chest X-ray showed no abnormalities, ruling out tuberculosis. Electrocardiogram, echocardiogram, and carotid Doppler examination were also within normal limits. The patient's ocular fluids were not analyzed using polymerase chain reaction for VZV because this facility was not available to us.
Subsequently, the patient defaulted with his appointment and came back 1 month later with pain, redness, and further deterioration in his vision. The vision in his right eye had dropped to perception of light, and the IOP was 56 mmHg. There was generalized corneal edema, flare, and cells in the anterior chamber, as well as prominent rubeosis iridis [Figure 2a]. Fundus examination revealed vitreous hemorrhage, as well as hemorrhage overlying the disc and sclerosed arteries [Figure 2b]. The patient's ocular movements were not restricted and no signs of orbital involvement were found. However, the patient now reported pain upon ocular movement, especially superior gaze.
A computerized tomography scan of the orbit was performed, revealing thickening of the right optic nerve upon contrast enhancement. A diagnosis of optic neuritis was made, most likely secondary to the VZV infection and NVG. The patient was started on intravenous acyclovir (10 mg/kg body weight, once daily for 10 days), followed by oral acyclovir (800 mg, 5 times daily for 6 weeks). He was also given intravenous methylprednisolone (250 mg, four times daily for 3 days) followed by oral prednisolone (1 mg/kg body weight, slowly tapered over 3 months). He was started on topical anti-glaucoma medications, and pan-retinal photocoagulation was also performed.
At his last follow-up, nearly 4 months after the first presentation, vision in the affected eye was hand motion. The eye was quiet, with regression of the corneal edema and rubeosis iridis. IOP of the right eye had decreased to 28 mmHg. The optic disc was fully cupped, with resolution of the hemorrhages [Figure 3].