Journal of Neuro-Ophthalmology, June 2009, Volume 28, Issue 2

Juxtapapillary Cytomegalovirus Retinitis with Optic Neuritis

A 49-year-old man with AIDS developed acute monocular visual loss and an ipsilateral swollen optic disc with a large right relative afferent pupillary defect, a nerve fiber bundle visual field defect, and a peripapillary retinal infiltrate. Lumbar puncture disclosed cytomegalovirus (CMV) DNA on polymerase chain reaction (PCR). Treatment with oral valganciclovir produced complete resolution of the visual deficits and the fundus abnormality. This case differs from previously reported cases of CMV optic neuritis in which visual function has been irreversibly lost.

PHOTO ESSAY Juxtapapillary Cytomegalovirus Retinitis With Optic Neuritis Alexander S. Ioannidis, MBBS, MRCOphth, James Bacon, MBBS, and Peggy Frith, MD, FRCP, FRCOphth FIG. 1. A. At presentation, there is a juxtapapillary focus of retinitis temporal to the optic disc. B. Four weeks after initiation of treatment, the retinitis is resolving. C. Four weeks after initiation of treatment, there is a dense inferior altitudinal defect in the right eye. The visual field of the left eye, not shown, was normal. D. Eight weeks after initiation of treatment, there are only mild residual exudates nasal and temporal to the optic disc. Department of Ophthalmology, University College London Hospitals NHS Trust, London, United Kingdom. Address correspondence to Alexander S. Ioannidis, MBBS, University College Hospitals NHS Trust, 235 Euston Road, London NW1 2BU, UK; E- mail: , ik'Mti, niiiuli<.. f Ir.' im. nl nini 128 J Neuro- Ophthalmol, Vol. 28, No. 2, 2008 Cytomegalovirus Optic Neuritis J Neuro- Ophthalmol, Vol. 28, No. 2, 2008 Abstract: A 49- year- old man with AIDS developed acute monocular visual loss and an ipsilateral swollen optic disc with a large right relative afferent pupillary defect, a nerve fiber bundle visual field defect, and a peripapillary retinal infiltrate. Lumbar puncture dis­closed cytomegalovirus ( CMV) DNA on polymerase chain reaction ( PCR). Treatment with oral valganci-clovir produced complete resolution of the visual deficits and the fundus abnormality. This case differs from previously reported cases of CMV optic neuritis in which visual function has been irreversibly lost. (/ Neuro- Ophthalmol 2008; 28: 128- 130) A49- year- old HIV- positive man with a past diagnosis of AIDS complained of a 3- day history of visual loss in the right eye. He had had a previous diagnosis of Burkitt lymphoma limited to the groin lymph nodes. He had been managed with highly active antiretroviral therapy ( HAART) but had been poorly compliant. Best- corrected visual acuity was 20/ 60 in the right eye and 20/ 40 in the left eye. A right relative afferent pupillary defect was present. Ishihara color plate vision was 0/ 17 in the right eye and 17/ 17 in the left eye. To confrontation, there was a central scotoma in the visual field of the right eye. The biomicroscopic examination was normal. The right optic disc was grossly swollen with focal retinitis temporally Til ( Fig. 1A). Small spot hemorrhages were also evident at the disc margin. There were no fundus abnormalities elsewhere. The left fundus was normal. The CD4 count was 120 cells/ mm3, and the HIV viral load was 32,500. Serologic test results for syphilis and toxoplasma were negative. Brain MRI was normal. A lumbar puncture showed a clear and colorless cerebrospinal fluid ( CSF) with a red blood and white blood cell count of less than 1 cell/ mm3. The total CSF protein was 0.40 g/ L and the CSF glucose was 2.8 mmol/ L ( blood glucose was 5 mmol/ L). The India ink stain for cryptococci was negative. CSF microscopy and culture results were negative. Cytomegalovirus ( CMV) DNA was identified on poly­merase chain reaction ( PCR). The diagnosis was juxtapapillary CMV retinitis with optic neuritis. Twice daily induction therapy with 900 mg of oral valganciclovir was initiated. Four weeks later, best-corrected visual acuity was 20/ 60 in the right eye, and color vision had improved to 3/ 17 Ishihara plates. The right optic disc was less swollen and the peripapillary retinitis was reduced. Exudates around the disc were attributed to resolving edema ( Fig. IB). The retinal periphery remained normal. A Humphrey 30- 2 field revealed a dense inferior altirudinal visual field defect in the right eye ( Fig. 1C). Two months after presentation, best- corrected visual acuity in the right eye had improved to 20/ 20, and color vision in that eye had improved to 16/ 17 Ishihara plates. There was a subjective improvement in the visual field ( no formal visual field was performed). There was complete resolution of the peripapillary retinitis ( Fig. ID). The visual field was not formally retested. The valganciclovir dose was reduced to 900 mg/ day The typical natural history of CMV optic neuritis ( papillitis) is progressive visual loss leading to blindness. Five previously reported cases ( 1- 5) have all ended with no light perception in the affected eye. Although ganciclovir is considered the " gold standard" for treatment of CMV valganciclovir, a well- absorbed valine ester product of ganciclovir, is equally effective and can be given orally to initiate treatment ( 6,7). In addition, treatment of the under­lying HIV infection with zidovudine and the ensuing im­provements in CD4 counts have been shown to halt the progression of CMV optic neuritis with partial recovery of vision reported in a single patient ( 8). As with CMV retinitis, CMV optic neuritis can relapse, so therapy should be maintained for a period of time depending on the disease severity and patient's immune status ( 9). Monitoring of these patients is best achieved with periodic visual field assessment as ophthalmoscopy is less reliable because of the presence of established disc pallor. One study ( 9) demonstrated that one third of treated patients showed worsening of visual field as an early sign of relapse and that three fourths of these patients had no other signs of relapse. In our patient there was a solitary focus of CMV retinitis adjacent to the optic disc with no evidence of peripheral CMV After treatment, the resolution of the retinitis above the disc was complete with no residual scarring or retinal discoloration. In such patients it is important to differentiate CMV optic neuritis from other causes of optic neuropathy such as syphilis, toxoplasmosis, cryptococcosis, bacterial and mycobacterial meningitis, neoplastic infiltration, and ethambutol toxicity. Patients who have recently started HAART are also at risk of developing immune reconstirution disease, which may present with optic disc edema ( 10). REFERENCES 1. Neuwirth J, Gutman I, Hofeldt AJ, et al. 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