Case Presentation: Varicella zoster virus (VZV) retinitis is a rapidly progressive infection of the retina, most commonly presenting as acute retinal necrosis (ARN). ARN leads to severe inflammation, retinal necrosis, retinal detachment and permanent vision loss. It can occur in both immunocompetent and immunocompromised individuals, often following herpes zoster, and demands prompt antiviral treatment to preserve visual function. Here we present a case of a 47-year-old female with a past medical history of spontaneous coronary artery dissection (SCAD) status post CABG on DAPT and traumatic subdural hematoma who presented with the chief complaint of left eye vision loss. The patient reported decreased vision from her left eye starting one month prior to presentation. A week prior to presentation, her vision decreased to the point of only being able to detect shadows and light. Of note, the patient denied eye pain, headache, confusion, mental status changes and rash during this time. On presentation to our hospital, the patient was hypertensive but overall well-appearing. Her ophthalmologic exam was grossly notable for left eye injection and perception only to light, with her retinal exam revealing subtle inferotemporal retinal whitening. Initial lab work was unremarkable. MRI orbit and brain demonstrated possible left optic nerve enhancement, subacute infarct in lower gangliocapsular region, and scattered leptomeningeal enhancement. The initial differential was extensive including infectious, embolic or thrombotic, or inflammatory causes. Infectious work-up was unrevealing with negative serum testing for HIV, CMV, HSV, Lyme, syphilis, HBV and HCV. QuantiFERON testing was positive but deemed unlikely in the absence of risk factors and normal chest imaging. Rheumatologic work-up was also unrevealing with negative ESR, ANA, anti-CCP, anti-SS-A/B, MPO, NMO, MOG and CTA abdomen/chest. Hypercoagulability work-up and pan-scan for malignancy were negative. She was started on prophylactic acyclovir. On day 4 vitreous fluid PCR resulted positive for VZV. The following day lumbar puncture was preformed after completion of clopidogrel wash-out and CSF PCR was also positive for VZV. Ultimately, the patient was discharged on 14 days IV acyclovir and close follow-up with retina clinic.
Discussion: We present this case in the context of challenging clinical diagnosis of VZV retinitis, specifically complicated by the abnormal presentation of acute retinal necrosis and patient’s past medical history. VZV retinitis in immunocompetent individuals typically occurs over days, not weeks. The patient’s extended clinical course, minimal findings on initial retinal exam and lack of preceding characteristic rash was abnormal. Concern for a primary rheumatologic process, such as vasculitis, given her recent SCAD and cerebral infarction further obscured the clinical picture. Finally, consideration must be given to the CSF VZV positivity which suggests a possible concomitant or preceding subclinical meningoencephalitis. We ponder that in the absence of cutaneous VZV infection, a subacute VZV meningoencephalitis could have served as the driver to retinal disease.
Conclusions: Our case emphasizes the importance of maintaining a high level of clinical suspicion for VZV retinitis even with a slow progression of disease. Finally, consideration should be given to performing CSF analysis for retinitis in the absence of a more obvious nidus.