Correspondence
Herpetic acute anterior uveitis complicated by retinal vasculitis in an immunocompetent child Herpetic uveitis is traditionally described as anterior granulomatous uveitis.1 An involvement of the posterior segment, even if less frequent, when present, is represented by an acute necrotizing retinitis (ARN).2 Retinal vasculitis apart from necrotizing retinitis is rare, and it has been reported during varicella zoster virus (VZV) primary infection only.3,4 We report for the first time, to our knowledge, a retinal vasculitis complicating an acute anterior granulomatous uveitis as a VZV recurrence in an immunocompetent child. A 6-year-old Chinese female presented to our clinic describing pain, photophobia, redness, and decreased vision in the right eye since 3 days, worsened in the last 24 hours. The patient had no history of recent systemic illness. At the first visit, the best corrected visual acuity (BCVA) was 20/50 OD and 20/20 OS. At the slit-lamp examination in the right eye, a severe granulomatous anterior uveitis was present (Fig. 1A-I). A mild vitreous inflammations was detectable (cells þ). At fundus examination, a vascular sheathing was visible along retinal vessels especially in the
inferior quadrant. Intraocular pressure was not recordable because of very poor compliance of the patient. Left eye results were within normal limits. Considering the posterior segment involvement, we performed a combined fluorescein angiography (FA) and indocyanine green angiography (ICG). ICG did not show any significant alteration in both eyes, ruling out a choroidal involvement. On the contrary, in the right eye, a diffuse staining was detectable along the whole venous tree and a papillary hyperfluorescence was present in the late stages of the FA (Fig. 1B-I). Left eye angiogram was normal. A pediatric consultation was carried out. Physical examination was irrelevant. Complete blood count, angiotensin-converting enzyme level, and serum β2-microglobulin test results were within normal limits. According to the clinical observations, a tailored battery of serology tests for several pathogens (herpes simplex virus types 1 and 2 [HSV-1/2], VZV, Epstein–Barr virus, cytomegalovirus, Toxoplasma, Toxocara, Borrelia, and Treponema pallidum) and tuberculin skin test (PPD) were performed. The only positive results were for HSV-1 (IgG: 15 index, negative o 1.1; IgM: negative) and a VZV (IgG 559 mIU/mL, positive 4 165 mIU/mL; IgM: negative).
Fig. 1 — Right eye. Infrared picture of the anterior segment at patient presentation (A-I), 3 days after therapy onset (A-II), and at the end of the treatment (A-III). Fluorescein angiography of the retina before the therapy start (B-I) and 15 days after the therapy onset (B-II). Note the presence of multiple mutton-fat keratic precipitates (white arrowheads) gradually decreasing as a response to the treatment (A-II), up to their complete disappearance (A-III). The persistence of a posterior synechiae (arrow) has not been considered as a sign of permanent inflammation but just as a sequela of the pre-existent inflammatory condition. Angiographic signs of inflammation as the optic nerve head (dotted circled) hyperfluorescence combined with diffuse dye staining along the whole venous tree (black arrowheads) (B-I) turn back to normal 15 days after therapy onset (B-II). CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
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Correspondence Although HSV cause could not be excluded, in agreement with pediatricians, these results were considered strongly suggestive of a VZV reactivation. On the diagnosis of VZV uveitis, oral acyclovir was started at 20 mg/kg 4 times a day. Topical steroid and mydriatic drops were given to control anterior segment inflammation. Three days later, the patient showed a dramatic response to treatment. Granulomatous keratic precipitates (KPs) were considerably decreased (Fig. 1AII), anterior chamber showed mild inflammation (flare þ, cells þ), and the vascular sheathing was regressing. BCVA was increased to 20/25. Acyclovir was continued, whereas topical steroids were gradually decreased. Fifteen days after the therapy onset, anterior chamber was quiet, KPs had disappeared (Fig. 1A-III), and the FA had returned to normal (Fig. 1B-II). BCVA was restored to 20/20. Systemic antiviral treatment, as well as topical therapy, was gradually tapered off. After 3 months, when the treatment was discontinued, the patient was not showing any sign of active disease. In this report, we describe a case of retinal vasculitis complicating acute anterior uveitis likely related to a VZV recurrence in an immunocompetent child. VZV posterior segment involvement is usually represented by an ARN.2 In the last few years a new spectrum of herpetic disease manifestations named non-necrotizing posterior uveitis has been described. They include papillitis, vasculitis, and non-necrotizing retinitis. However, these are typically bilateral and chronic entities, often misdiagnosed.5 Our patient showed a mixed picture characterized by the typical features of a herpetic acute anterior uveitis accompanied by an isolate vasculitis without retinal necrosis. The same kind of perivenular sheathing with dye staining during the FA late phases was previously reported by Kuo et al.3 in a chicken pox–related uveitis and by Wimmersberger et al.4 in a VZV primary infection without systemic symptoms. However, to our knowledge, this is the first time that this finding is noted during a disease recurrence. The correct therapy regimen for VZV vasculitis is unknown. ARN requires intravenous or intravitreal therapy.2 This treatment could be considered for VZV vascular involvement as well. However, both the vasculitis we are reporting and the one presented by Kuo et al.3
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responded to oral acyclovir, confirming the efficacy of this approach. On the contrary, Wimmersberger et al.4 treated the patient by intravenous drug administration. Independently from the clinicians’ choice, in such an uncertain scenario, we strongly suggest very close follow-up visits in the first days after therapy onset to verify the response to treatment. All the patients so far reported to have this VZV-related vascular inflammation were immunocompetent females. However, there are not enough data to speculate a correlation between any subject characteristic and disease manifestation. The cause of the retinal vascular involvement during VZV anterior uveitis in a restricted number of patients remains therefore unexplained. However, detection of this unusual clinical manifestation apart from the primary herpetic infection should alert the clinicians to carefully evaluate the posterior segment of the eye every time an anterior herpetic uveitis is suspected. Alessandro Invernizzi, * Chiara Mameli, † Vania Giacomet, † Paola Chiari, * Gian Vincenzo Zuccotti † * Eye Clinic, Department of Biomedical and Clinical Science, L. Sacco Hospital, University of Milan, Milan, Italy; † Department of Pediatrics, L. Sacco Hospital, University of Milan, Milan, Italy
Correspondence to: Alessandro Invernizzi, MD: [email protected] REFERENCES 1. Liesegang TJ. Herpes zoster virus infection. Curr Opin Ophthalmol.. 2004;15:531-6. 2. Wong R, Pavesio CE, Laidlaw DA, Williamson TH, Graham EM, Stanford MR. Acute retinal necrosis: the effects of intravitreal foscarnet and virus type on outcome. Ophthalmology. 2010;117: 556-60. 3. Kuo YH, Yip Y, Chen SN. Retinal vasculitis associated with chickenpox. Am J Ophthalmol. 2001;132:584-5. 4. Wimmersberger Y, Gervaix A, Baglivo E. VZV retinal vasculitis without systemic infection: diagnosis and monitoring with quantitative polymerase chain reaction. Int Ophthalmol. 2010;30:73-5. 5. Bodaghi B, Rozenberg F, Cassoux N, Fardeau C, LeHoang P. Nonnecrotizing herpetic retinopathies masquerading as severe posterior uveitis. Ophthalmology. 2003;110:1737-43.
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
Can J Ophthalmol 2013;48:e171–e172 0008-4182/13/$-see front matter & 2013 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.08.011
Herpetic acute anterior uveitis complicated by retinal vasculitis in an immunocompetent child Herpetic uveitis is traditionally described as anterior granulomatous uveitis.1 An involvement of the posterior segment, even if less frequent, when present, is represented by an acute necrotizing retinitis (ARN).2 Retinal vasculitis apart from necrotizing retinitis is rare, and it has been reported during varicella zoster virus (VZV) primary infection only.3,4 We report for the first time, to our knowledge, a retinal vasculitis complicating an acute anterior granulomatous uveitis as a VZV recurrence in an immunocompetent child. A 6-year-old Chinese female presented to our clinic describing pain, photophobia, redness, and decreased vision in the right eye since 3 days, worsened in the last 24 hours. The patient had no history of recent systemic illness. At the first visit, the best corrected visual acuity (BCVA) was 20/50 OD and 20/20 OS. At the slit-lamp examination in the right eye, a severe granulomatous anterior uveitis was present (Fig. 1A-I). A mild vitreous inflammations was detectable (cells þ). At fundus examination, a vascular sheathing was visible along retinal vessels especially in the
inferior quadrant. Intraocular pressure was not recordable because of very poor compliance of the patient. Left eye results were within normal limits. Considering the posterior segment involvement, we performed a combined fluorescein angiography (FA) and indocyanine green angiography (ICG). ICG did not show any significant alteration in both eyes, ruling out a choroidal involvement. On the contrary, in the right eye, a diffuse staining was detectable along the whole venous tree and a papillary hyperfluorescence was present in the late stages of the FA (Fig. 1B-I). Left eye angiogram was normal. A pediatric consultation was carried out. Physical examination was irrelevant. Complete blood count, angiotensin-converting enzyme level, and serum β2-microglobulin test results were within normal limits. According to the clinical observations, a tailored battery of serology tests for several pathogens (herpes simplex virus types 1 and 2 [HSV-1/2], VZV, Epstein–Barr virus, cytomegalovirus, Toxoplasma, Toxocara, Borrelia, and Treponema pallidum) and tuberculin skin test (PPD) were performed. The only positive results were for HSV-1 (IgG: 15 index, negative o 1.1; IgM: negative) and a VZV (IgG 559 mIU/mL, positive 4 165 mIU/mL; IgM: negative).
Fig. 1 — Right eye. Infrared picture of the anterior segment at patient presentation (A-I), 3 days after therapy onset (A-II), and at the end of the treatment (A-III). Fluorescein angiography of the retina before the therapy start (B-I) and 15 days after the therapy onset (B-II). Note the presence of multiple mutton-fat keratic precipitates (white arrowheads) gradually decreasing as a response to the treatment (A-II), up to their complete disappearance (A-III). The persistence of a posterior synechiae (arrow) has not been considered as a sign of permanent inflammation but just as a sequela of the pre-existent inflammatory condition. Angiographic signs of inflammation as the optic nerve head (dotted circled) hyperfluorescence combined with diffuse dye staining along the whole venous tree (black arrowheads) (B-I) turn back to normal 15 days after therapy onset (B-II). CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
e171
Correspondence Although HSV cause could not be excluded, in agreement with pediatricians, these results were considered strongly suggestive of a VZV reactivation. On the diagnosis of VZV uveitis, oral acyclovir was started at 20 mg/kg 4 times a day. Topical steroid and mydriatic drops were given to control anterior segment inflammation. Three days later, the patient showed a dramatic response to treatment. Granulomatous keratic precipitates (KPs) were considerably decreased (Fig. 1AII), anterior chamber showed mild inflammation (flare þ, cells þ), and the vascular sheathing was regressing. BCVA was increased to 20/25. Acyclovir was continued, whereas topical steroids were gradually decreased. Fifteen days after the therapy onset, anterior chamber was quiet, KPs had disappeared (Fig. 1A-III), and the FA had returned to normal (Fig. 1B-II). BCVA was restored to 20/20. Systemic antiviral treatment, as well as topical therapy, was gradually tapered off. After 3 months, when the treatment was discontinued, the patient was not showing any sign of active disease. In this report, we describe a case of retinal vasculitis complicating acute anterior uveitis likely related to a VZV recurrence in an immunocompetent child. VZV posterior segment involvement is usually represented by an ARN.2 In the last few years a new spectrum of herpetic disease manifestations named non-necrotizing posterior uveitis has been described. They include papillitis, vasculitis, and non-necrotizing retinitis. However, these are typically bilateral and chronic entities, often misdiagnosed.5 Our patient showed a mixed picture characterized by the typical features of a herpetic acute anterior uveitis accompanied by an isolate vasculitis without retinal necrosis. The same kind of perivenular sheathing with dye staining during the FA late phases was previously reported by Kuo et al.3 in a chicken pox–related uveitis and by Wimmersberger et al.4 in a VZV primary infection without systemic symptoms. However, to our knowledge, this is the first time that this finding is noted during a disease recurrence. The correct therapy regimen for VZV vasculitis is unknown. ARN requires intravenous or intravitreal therapy.2 This treatment could be considered for VZV vascular involvement as well. However, both the vasculitis we are reporting and the one presented by Kuo et al.3
e172
responded to oral acyclovir, confirming the efficacy of this approach. On the contrary, Wimmersberger et al.4 treated the patient by intravenous drug administration. Independently from the clinicians’ choice, in such an uncertain scenario, we strongly suggest very close follow-up visits in the first days after therapy onset to verify the response to treatment. All the patients so far reported to have this VZV-related vascular inflammation were immunocompetent females. However, there are not enough data to speculate a correlation between any subject characteristic and disease manifestation. The cause of the retinal vascular involvement during VZV anterior uveitis in a restricted number of patients remains therefore unexplained. However, detection of this unusual clinical manifestation apart from the primary herpetic infection should alert the clinicians to carefully evaluate the posterior segment of the eye every time an anterior herpetic uveitis is suspected. Alessandro Invernizzi, * Chiara Mameli, † Vania Giacomet, † Paola Chiari, * Gian Vincenzo Zuccotti † * Eye Clinic, Department of Biomedical and Clinical Science, L. Sacco Hospital, University of Milan, Milan, Italy; † Department of Pediatrics, L. Sacco Hospital, University of Milan, Milan, Italy
Correspondence to: Alessandro Invernizzi, MD: [email protected] REFERENCES 1. Liesegang TJ. Herpes zoster virus infection. Curr Opin Ophthalmol.. 2004;15:531-6. 2. Wong R, Pavesio CE, Laidlaw DA, Williamson TH, Graham EM, Stanford MR. Acute retinal necrosis: the effects of intravitreal foscarnet and virus type on outcome. Ophthalmology. 2010;117: 556-60. 3. Kuo YH, Yip Y, Chen SN. Retinal vasculitis associated with chickenpox. Am J Ophthalmol. 2001;132:584-5. 4. Wimmersberger Y, Gervaix A, Baglivo E. VZV retinal vasculitis without systemic infection: diagnosis and monitoring with quantitative polymerase chain reaction. Int Ophthalmol. 2010;30:73-5. 5. Bodaghi B, Rozenberg F, Cassoux N, Fardeau C, LeHoang P. Nonnecrotizing herpetic retinopathies masquerading as severe posterior uveitis. Ophthalmology. 2003;110:1737-43.
CAN J OPHTHALMOL — VOL. 48, NO. 6, DECEMBER 2013
Can J Ophthalmol 2013;48:e171–e172 0008-4182/13/$-see front matter & 2013 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.08.011
