Progressive outer retinal necrosis is an emergency
Lam et al[1] provide an excellent overview of ophthalmic varicella
virus (VZV) reactivation presentation and management. Although VZV rarely
manifests as progressive outer retinal necrosis (PORN), usually in immune
suppressed patients, PORN can occur in apparently immune competent
patients,[2, 3] with the outcome in most a visual acuity of no light
perception.
In contrast to the relatively older patients Lam et al[1] describe
(>50 year olds), PORN tends to occur in younger patients, often
associated with a rash. Rapidly progressive unilateral or bilateral visual
loss (to no light perception within days to weeks) may be associated with
or progress to optic neuritis,[4] retinal detachment, vitreous haemorrhage
and meningoencephalitis. It is most often seen in HIV positive patients
but also in those with haematological malignancies, organ or bone marrow
transplant, steroid therapy,[3, 4] anti-TNF therapy,[3] rituximab therapy,
lupus, rheumatoid arthritis[3]and nephrotic syndrome.
Management approaches include combinations of oral, intravenous and
intravitreal therapy.[2-5] Agents include highly active antiretroviral
therapy, valganciclovir, bromovinyldeoxyuridine, aciclovir, ganciclovir,
foscarnet and gammaglobulin (IVIg). Multiple surgical procedures are often
required, mainly for treating complications, including vitrectomy with
silicone oil tamponade, retinopexy, optic nerve fenestration, laser
photocoagulation and ganciclovir implant. However, profound visual
disability usually persists.
References
[1] Lam FC, Law A, Wykes W. Herpes zoster ophthalmicus. BMJ.
2009;339:b2624.
[2] Carrillo-Pacheco S, Vazquez-Marouschek MC, Lopez-Checa F, Sanchez
-Roman J. Progressive outer retinal necrosis in an immunocompetent
patient. Acta Ophthalmol Scand. 1996;74:506-8.
[3] Benz MS, Glaser JS, Davis JL. Progressive outer retinal necrosis
in immunocompetent patients treated initially for optic neuropathy with
systemic corticosteroids. Am J Ophthalmol. 2003;135:551-3.
[5] Pérez-Blázquez E, Traspas R, Méndez Marín I, Montero M.
Intravitreal ganciclovir treatment in progressive outer retinal necrosis.
Am J Ophthalmol. 1997;124:418-21.
Competing interests:
None declared
Competing interests:
No competing interests
26 August 2009
Amit Patel
NIHR Academic Clinical Fellow
Dept. of Haematology, Imperial College London, Hammersmith Hospital, Du Cane Rd, London, W12 0NN
Rapid Response:
Progressive outer retinal necrosis is an emergency
Lam et al[1] provide an excellent overview of ophthalmic varicella
virus (VZV) reactivation presentation and management. Although VZV rarely
manifests as progressive outer retinal necrosis (PORN), usually in immune
suppressed patients, PORN can occur in apparently immune competent
patients,[2, 3] with the outcome in most a visual acuity of no light
perception.
In contrast to the relatively older patients Lam et al[1] describe
(>50 year olds), PORN tends to occur in younger patients, often
associated with a rash. Rapidly progressive unilateral or bilateral visual
loss (to no light perception within days to weeks) may be associated with
or progress to optic neuritis,[4] retinal detachment, vitreous haemorrhage
and meningoencephalitis. It is most often seen in HIV positive patients
but also in those with haematological malignancies, organ or bone marrow
transplant, steroid therapy,[3, 4] anti-TNF therapy,[3] rituximab therapy,
lupus, rheumatoid arthritis[3]and nephrotic syndrome.
Management approaches include combinations of oral, intravenous and
intravitreal therapy.[2-5] Agents include highly active antiretroviral
therapy, valganciclovir, bromovinyldeoxyuridine, aciclovir, ganciclovir,
foscarnet and gammaglobulin (IVIg). Multiple surgical procedures are often
required, mainly for treating complications, including vitrectomy with
silicone oil tamponade, retinopexy, optic nerve fenestration, laser
photocoagulation and ganciclovir implant. However, profound visual
disability usually persists.
References
[1] Lam FC, Law A, Wykes W. Herpes zoster ophthalmicus. BMJ.
2009;339:b2624.
[2] Carrillo-Pacheco S, Vazquez-Marouschek MC, Lopez-Checa F, Sanchez
-Roman J. Progressive outer retinal necrosis in an immunocompetent
patient. Acta Ophthalmol Scand. 1996;74:506-8.
[3] Benz MS, Glaser JS, Davis JL. Progressive outer retinal necrosis
in immunocompetent patients treated initially for optic neuropathy with
systemic corticosteroids. Am J Ophthalmol. 2003;135:551-3.
[4] Nakamoto BK, Dorotheo EU, Biousse V, Tang RA, Schiffman JS,
Newman NJ. Progressive outer retinal necrosis presenting with isolated
optic neuropathy. Neurology. 2004;63:2423-5.
[5] Pérez-Blázquez E, Traspas R, Méndez Marín I, Montero M.
Intravitreal ganciclovir treatment in progressive outer retinal necrosis.
Am J Ophthalmol. 1997;124:418-21.
Competing interests:
None declared
Competing interests: No competing interests