 |
|
Retinal Periphlebitis as Zoster Sine Herpete
Arch Ophthalmol. 2001;119:1550-1552.
INTRODUCTION
Although infection with varicella-zoster virus (VZV) has been known to cause occlusive retinal arteritis leading to acute retinal necrosis1 in both immunocompetent and immunocompromised patients, retinal periphlebitis is a rare manifestation of varicella-zoster ophthalmicus. We examined 2 immunocompetent patients with unilateral retinal periphlebitis and iridocyclitis without cutaneous eruptions associated with positive VZV genomes in the aqueous fluid. One patient had a fundus appearance compatible with that of frosted branch angiitis, suggesting a possible correlation between one form of frosted branch angiitis and a VZV infection.
Report of Cases
Case 1
A healthy 20-year-old woman noticed conjunctival redness in her left eye. Visual acuity was 20/20 OD and 20/200 OS. Intraocular pressure was 15 mm Hg OD and 33 mm Hg OS. Slitlamp biomicroscopy revealed ciliary vasodilation, diffuse corneal stromal edema, and mutton fat–like corneal precipitates in the left eye. Inflammatory cells were also observed in the anterior chamber of the left eye. Fundus examination allowed a blurred view of only the optic disc and major retinal vessels; no other details could be seen because of corneal cloudiness and diffuse vitreous opacity. Serological screening tests using enzyme immunoassay for VZV yielded an IgM level of 84 (normal, negative) and an IgG level of 2816 (normal, <42). Her blood cell count was within normal limits. Laboratory investigation results, including serum angiotensin-converting enzyme, were unremarkable. The treponema pallidum hemoagglutination test and antinuclear antibody test were negative. Her chest x-ray film had no abnormal shadow. Oral aphtha and other symptoms of Behçet disease were not seen. After oral administration of aciclovir and acetazolamide and topical instillation of steroids, mydriatics were started. Consequently, the inflammatory reaction gradually resolved and intraocular pressure decreased to a normal range, resulting in reduced corneal edema. Fundus examination revealed extensive diffuse sheathing of retinal veins in all quadrants in the left eye (Figure 1A). Fluorescein angiography results demonstrated diffuse leakage and tissue staining of retinal venous walls and the optic disc (Figure 1B). These findings were compatible with those of frosted branch angiitis except that inflammatory signs were seen unilaterally. The VZV genome was detected in the aqueous sample by polymerase chain reaction. The patient received 1500 mg of aciclovir intravenously combined with 30 mg of oral prednisolone. Both the phlebitis and vitreous opacity gradually disappeared and visual acuity improved to 20/20 OS. Two months after the initial onset, iris atrophy appeared. Neither abnormal findings in the right eye nor herpes skin eruptions were noted throughout the course.
|
|
|
|
Figure 1. A, Fundus appearance of case 1 shows severe periphlebitis. Vitreous opacity is also present. B, Fluorescein angiogram shows diffuse leakage and staining of retinal veins and optic disc.
|
|
|
Case 2
A healthy 33-year-old man noticed conjunctival redness and subsequent blurred vision in his left eye. His visual acuity was 20/10 OD and 20/25 OS. Intraocular pressure was 15 mm Hg OD and 21 mm Hg OS. Slitlamp biomicroscopy revealed ciliary vasodilation, fine corneal precipitates, and corneal edema. Inflammatory cells were seen in the left anterior chamber. Although the ocular media were initially so clouded that the detail of the left fundus was difficult to see, the media gradually cleared after treatment with topical instillation of steroid and oral administration of acetazolamide. Fundus examination then revealed segmental sheathings of retinal veins that showed diffuse leakage and tissue staining by fluorescein angiography (Figure 2). Results of a blood cell count, laboratory screening test, serum angiotensin-converting enzyme test, treponema pallidum hemoagglutination test, and antinuclear antibody test were unremarkable. Other mucocutaneous symptoms of Behçet disease were not seen either. Because the VZV genome was detected in the aqueous sample by polymerase chain reaction, systemic administration of 1500 mg of aciclovir and 30 mg of prednisolone was started. After the initiation of treatment, inflammation gradually resolved and periphlebitis disappeared. A serological screening test result for VZV revealed an IgG level of 704 mg/dL; no IgM could be detected. Iris atrophy appeared in 3 weeks. Neither abnormal findings in the right eye nor herpes skin eruptions were noted throughout the course.
|
|
|
|
Figure 2. Fluorescein angiogram shows diffuse leakage and staining of retinal veins.
|
|
|
Comment
Retinal periphlebitis is a rare manifestation of varicella-zoster ophthalmicus. Characteristic features seen in our healthy young adult patients without preceding symptoms or herpes cutaneous eruptions included the following: unilateral elevated intraocular pressure; iridocyclitis; retinal periphlebitis without retinal exudates, hemorrhages, or periarteritis; diffuse vitreous opacity; positive VZV genome in the aqueous humor; and iris atrophy in the later stage. Although ocular manifestations such as iridocyclitis, elevated intraocular pressure, and iris atrophy have recently been known to be associated with zoster sine herpete,2 retinal vasculitis has been reported in only 2 Japanese patients.3 To our knowledge, no case has ever been reported of periphlebitis resembling unilateral frosted branch angiitis associated with positive VZV, as seen in case 1. Although many cases with frosted branch angiitis have been reported among variable age groups since Ito et al4 first described a bilateral retinal panvasculitis of unknown etiology, usually seen in healthy young individuals, the only pathogen that has been directly detected in the lesion was herpes simplex virus in just one case.5 Chatzoulis et al5 have suggested a close correlation between frosted branch angiitis and herpes simplex infection because of the positivity of the herpes simplex virus genome in the aqueous sample of their case. Also, because clinical features of patients with frosted branch angiitis were reportedly variable, frosted branch angiitis itself may not be a single clinical entity but a group of retinal vasculitis diseases of heterogeneous origins. Therefore, detection of the VZV genome in the aqueous sample of our Case 1 suggests that this virus may be one of the causative agents of some forms of retinal periphlebitis similar to frosted branch angiitis.
Because necrotizing retinitis was not observed in either case, our patients did not fulfill the criteria for acute retinal necrosis. In our cases periphlebitis was characteristic, whereas arteritis is mainly observed in cases with acute retinal necrosis. Therefore, while VZV may cause cerebral vasculitis, our case reports also suggest that VZV may lead to either retinal periphlebitis or periarteritis, although the precise mechanism to explain these different manifestations is still uncertain.
Currently, we do not know why the clinical features were somewhat different between the 2 patients. Case 2 showed rather segmental retinal periphlebitis vs the diffuse periphlebitis seen in case 1. One possible explanation is the different pattern of infection between the patients. Case 2 had a history of varicella infection (chickenpox), while case 1 did not. Patterns of serological screening results were also different, indicating that case 1 had an initial infection of VZV and case 2 had a reactivated VZV infection. These differences may have resulted in different clinical features. As Brown and Mendis6 speculated that minor fundus changes might be more common than was believed in herpes zoster ophthalmicus, our cases suggest that some forms of retinal periphlebitis associated with anterior uveitis, for which no other cause is known, may be caused by a VZV infection, even in cases without herpes eruption (zoster sine herpete). Because systemic administration of aciclovir combined with steroids is effective treatment for a VZV infection, a correct diagnosis should be made without delay. Molecular diagnosis using polymerase chain reaction is beneficial.
AUTHOR INFORMATION
Yasuko Noda, MD;
Mitsuru Nakazawa, MD, PhD;
Daisuke Takahashi, MD, PhD;
Tomoko Tsuruya, MD, PhD;
Makoto Saito, MD;
Miho Sekine, MD
Hirosaki, Japan
Corresponding author: Mitsuru Nakazawa, MD, Department of Ophthalmology, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki, 036-8562, Japan (e-mail: mitsuru{at}cc.hirosaki-u.ac.jp).
REFERENCES
1. Culbertson WW, Blumenkranz MS, Pepose JS, Stewart JA, Curtin VT. Varicella zoster virus is a cause of the acute retinal necrosis syndrome. Ophthalmology. 1986;93:559-569.
ISI
| PUBMED
2. Stravrou P, Michell SM, Fox JD, Hope-Ross MW, Murray PI. Detection of varicella-zoster virus DNA in ocular samples from patients with uveitis but no cutaneous eruption. Eye. 1994;5:684-687.
3. Sakai J-i, Usui N, Usui M, Imai S, Osato T. Detection of varicella zoster virus DNA by polymerase chain reaction in 4 uveitis patients. J Eye (Atarashii Ganka). 1992;9:447-452.
4. Ito Y, Nakano M, Kyu N, Takeuchi M. Frosted branch angiitis in a child. Jpn J Clin Ophthalmol. 1976;30:797-803.
5. Chatzoulis DM, Theodosiadis PG, Apostolopoulos MN, Drakoulis N, Markomichelakis NN. Retinal perivasculitis in an immunocompetent patient with systemic herpes simplex infection. Am J Ophthalmol. 1997;123:699-702.
PUBMED
6. Brown RM, Mendis U. Retinal arteritis complicating herpes zoster ophthalmicus. Br J Ophthalmol. 1973;57:344-346.
FREE FULL TEXT
SECTION EDITOR: W. RICHARD GREEN, MD
|
