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Impressum
Unilateral acute retinal necrosis syndrome in common variable immundeficiency (CVID)
1Grünauer-Kloevekorn C., 1Reichel M. B., 2Borte M., 1Wiedemann P.
1Klinik für Augenheilkunde der Universität Leipzig, University Leipzig, 2Klinik und Poliklinik für Kinder und Jugendliche, University Leipzig
Common variable immundeficiency (CVID, incidence 1:10000 to 1:50000)
includes several different immundeficiencies with hypogammaglobulinemia
and a defect in the production of antibodies. The genetic basis is unknown
and there is no inheritence pattern. Most patients suffer from recurrent
respiratory infection, some of meningoencephalitis or gastrointestinal
infections. Furthermore through hyperactive immune systeme CVID can cause
autoimmune and lymphoproliferative diseases. However no ophthalmological
findings are described to date. Case: An 18 year old patient was
hospitalised with iridocyclitis on her left eye 14 days after first treatment
with lokal cortisol and mydriatic. She suffered from CVID. Visual acuity
was 1,0 on the right eye, 0,25 on the left one. The ophthamological examination
of her right eye was normal. On the left eye there were cells in the anterior
chamber and vitreous and papillar edema. We saw vasculitis of the retinal
vessels and a serous retinal detachment in the periphery.
Results: Laboratory examination revealed that herpes simplex virus
titer in the blood was raised (IgG 13300 U/ml, IgM 0,1). In the aqueous
HSV could be demonstrated with PCR. After intravenous Foscavir therapy
there was decrease of cells in anterior chamber and vitreous and demarkation
of the now necrotic retinal area. There was never any affection of the
right eye. The visual acuity now is 0,6 on the left eye.
Discussion: Due to mostly bacterial inflammations in CVID and constant
substitution of immunglobulines, there are acute viral infections. Here
we present a case with acute retinal necrosis syndrome. With high dose
antiviral therapy a sufficient visual acuity was achieved and infection
of the other eye prevented.