Bilateral Sequential Herpes Simplex Type 2 Panophthalmitis in an Adult with a Toll-Like Receptor 4 Mutation

To present the first reported case of bilateral sequential Herpes Simplex Virus Type 2 (HSV-2) panophthalmitis causing severe vision loss in a young adult with a toll-like receptor 4 (TLR-4) mutation, with a review of previously reported cases of HSV-2 panophthalmitis.

Retrospective case report.

A 29-year-old man presented with a severely painful, swollen left eye and vision loss. Visual acuity was no light perception in both eyes. Six years earlier, he had developed right panophthalmitis, the cause confirmed as Herpes Simplex Virus Type 2 (HSV-2) by polymerase chain reaction (PCR) on aqueous humour (AH) sampling, with a poor outcome. At current presentation, PCR of AH and vitreous samples again confirmed HSV-2 infection. A poor initial response to high-dose intravenous antiviral led to the addition of high-dose systemic corticosteroid. Subsequent phacovitrectomy enabled partial visual recovery. Genetic testing revealed TLR-4 mutation rs4986790 A/G.

HSV-2 panophthalmitis is rare and visually destructive, and we present the first reported case of bilateral disease. We hypothesise that TLR-4 mutation, not previously reported in association with HSV ocular inflammation, may have contributed to excessive inflammation and severe ocular damage. We suggest comprehensive immunological evaluation in all patients with severe viral intraocular inflammation. Prompt diagnosis with PCR and immediate high-dose antiviral therapy is critical and immune abnormality may require amended management.