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Progressive outer retinal necrosis combined with vitreous hemorrhage in a patient with acquired immunodeficiency syndrome.

You YS, Lee SJ, Lee SH, Park CH, Kwon OW - Korean J Ophthalmol (2007)

Bottom Line: PORN has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with AIDS.Although the etiologic agent has been reported to be VZV, concurrent or combined etiologic agents can include HSV-1, HSV-2, and CMV in AIDS patients.Therefore, combined antiviral therapy with acyclovir and ganciclovir could be more reasonable as an initial therapy.

View Article: PubMed Central - PubMed

Affiliation: NUNE Eye Hospital, Seoul, Korea.

ABSTRACT

Purpose: To describe an unusual case of rapidly progressive outer retinal necrosis (PORN) with vitreous hemorrhage in a 41-year-old woman with acquired immunodeficiency syndrome (AIDS), who had retinitis developed from what was probably varicellar-zoster virus combined with cytomegalovirus (CMV) and herpes simplex type 1,2, as proven by the polymerase chain reaction restriction fragment length polymorphism method (PCR-RFLP).

Methods: This study is a case report detailing clinical follow-up and an aqueous humor test by PCR-RFLP.

Results: The deep, white retinal lesions coalesced and progressively expanded in a circumferential manner, with sparing of the perivascular retina. However, retinal and vitreous hemorrhages, unusual findings for PORN, could be noted around the optic nerve. Varicellar-zoster virus (VZV), cytomegalovirus (CMV), and herpes simplex types 1,2 (HSV-1,2) were detected in the aqueous humor by PCR.

Conclusions: PORN has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with AIDS. Although the etiologic agent has been reported to be VZV, concurrent or combined etiologic agents can include HSV-1, HSV-2, and CMV in AIDS patients. Therefore, combined antiviral therapy with acyclovir and ganciclovir could be more reasonable as an initial therapy.

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Related in: MedlinePlus

Subsequent fundus photographs of each eye. (A) Extensive deep retinal white opacification sparing perivascular area is visible. Note the retinal and vitreous hemorrhage around optic nerve. These findings are consistent with PORN in the left eye, except for that hemorrhage. Additional inferior retinal and choroidal detachments were found. (B) Multifocal deep retinal lesions are seen with the central macula showing a cherry-red spot in the right eye. This is consistent with PORN.
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Figure 2: Subsequent fundus photographs of each eye. (A) Extensive deep retinal white opacification sparing perivascular area is visible. Note the retinal and vitreous hemorrhage around optic nerve. These findings are consistent with PORN in the left eye, except for that hemorrhage. Additional inferior retinal and choroidal detachments were found. (B) Multifocal deep retinal lesions are seen with the central macula showing a cherry-red spot in the right eye. This is consistent with PORN.

Mentions: A 41-year-old Korean woman visited the ophthalmic clinic because of visual disturbance. She was determined to be a hepatitis B antigen carrier seven months prior to visiting the clinic and was treated for a mental disorder with psychiatric medication one year prior to visiting the clinic. In addition, she underwent a neck mass excision at a private clinic six months previously. A history of herpes zoster infection in the skin or elsewhere was not found. Visual acuity was 20/100 in the right eye and 20/200 in left eye. Ophthalmoscopic examination detected a deep, white outer retinal lesion in the mid-peripheral retina and posterior pole. Also, some retinal hemorrhages were noted around the major vascular arcade in the left eye (Fig. 1). Fluorescence angiography showed no active vascular leakage, but there was diffuse leakage from the subretinal area. The patient would have been referred to retinal service, but she was unreachable for one week for private reasons. When she returned to the eye clinics on June 12, 2000, her retina finding had markedly changed. The previous deep, white retinal lesions had coalesced and progressively expanded in a circumferential manner, with sparing of the perivascular retina. Retinal and vitreous hemorrhages could be noted around the optic nerve. Retinal detachment was noted at the inferior mid-periphery of the left eye. Visual acuity was 20/200 in the right eye and hand motion in the left eye (Fig. 2). Typical progressive outer retinal necrosis was considered, but would not explain the moderate retinal and vitreous hemorrhage around optic nerve. Thus, necrotizing herpetic retinopathy was considered. Intravenous treatment with acyclovir for presumed VZV or HSV retinitis was administered. A systemic work-up was evaluated, and anti-HIV antibodies and HBs Ag were detected in the serum. Anterior chamber paracentesis was taken for the identification of a specific causative agent. A 0.1 cc sample of aqueous humor was acquired for polymerase chain reaction. The PCR-RFLP analysis of the aqueous humor specimen was positive for VZV, HSV-1, HSV-2, and CMV DNA. At the same time, the patient was diagnosed with AIDS at the C3 stage (CDC classification) because of the decreased CD4+ cell count of 18 /ul and the presence of encephalopathy caused by AIDS. Because of the association between HIV infection and suspiciously combined CMV retinitis, intravenous treatment with ganciclovir was added. Forscarnet is not available in Korea. The patient was referred to Yonsei University Hospital for management of her HIV infection. After the diagnosis, she was treated with zidovudine, lamivudine, and indinavir. On June 16, in spite of medical therapy, the retinal necrosis had progressed (Fig. 3). Total retinal detachment and extensive full-thickness necrosis were found in the left eye, and inferior partial retinal and choroidal detachment were noted in the right eye. The patient's visual acuity was hand motion for both eyes.


Progressive outer retinal necrosis combined with vitreous hemorrhage in a patient with acquired immunodeficiency syndrome.

You YS, Lee SJ, Lee SH, Park CH, Kwon OW - Korean J Ophthalmol (2007)

Subsequent fundus photographs of each eye. (A) Extensive deep retinal white opacification sparing perivascular area is visible. Note the retinal and vitreous hemorrhage around optic nerve. These findings are consistent with PORN in the left eye, except for that hemorrhage. Additional inferior retinal and choroidal detachments were found. (B) Multifocal deep retinal lesions are seen with the central macula showing a cherry-red spot in the right eye. This is consistent with PORN.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2629688&req=5

Figure 2: Subsequent fundus photographs of each eye. (A) Extensive deep retinal white opacification sparing perivascular area is visible. Note the retinal and vitreous hemorrhage around optic nerve. These findings are consistent with PORN in the left eye, except for that hemorrhage. Additional inferior retinal and choroidal detachments were found. (B) Multifocal deep retinal lesions are seen with the central macula showing a cherry-red spot in the right eye. This is consistent with PORN.
Mentions: A 41-year-old Korean woman visited the ophthalmic clinic because of visual disturbance. She was determined to be a hepatitis B antigen carrier seven months prior to visiting the clinic and was treated for a mental disorder with psychiatric medication one year prior to visiting the clinic. In addition, she underwent a neck mass excision at a private clinic six months previously. A history of herpes zoster infection in the skin or elsewhere was not found. Visual acuity was 20/100 in the right eye and 20/200 in left eye. Ophthalmoscopic examination detected a deep, white outer retinal lesion in the mid-peripheral retina and posterior pole. Also, some retinal hemorrhages were noted around the major vascular arcade in the left eye (Fig. 1). Fluorescence angiography showed no active vascular leakage, but there was diffuse leakage from the subretinal area. The patient would have been referred to retinal service, but she was unreachable for one week for private reasons. When she returned to the eye clinics on June 12, 2000, her retina finding had markedly changed. The previous deep, white retinal lesions had coalesced and progressively expanded in a circumferential manner, with sparing of the perivascular retina. Retinal and vitreous hemorrhages could be noted around the optic nerve. Retinal detachment was noted at the inferior mid-periphery of the left eye. Visual acuity was 20/200 in the right eye and hand motion in the left eye (Fig. 2). Typical progressive outer retinal necrosis was considered, but would not explain the moderate retinal and vitreous hemorrhage around optic nerve. Thus, necrotizing herpetic retinopathy was considered. Intravenous treatment with acyclovir for presumed VZV or HSV retinitis was administered. A systemic work-up was evaluated, and anti-HIV antibodies and HBs Ag were detected in the serum. Anterior chamber paracentesis was taken for the identification of a specific causative agent. A 0.1 cc sample of aqueous humor was acquired for polymerase chain reaction. The PCR-RFLP analysis of the aqueous humor specimen was positive for VZV, HSV-1, HSV-2, and CMV DNA. At the same time, the patient was diagnosed with AIDS at the C3 stage (CDC classification) because of the decreased CD4+ cell count of 18 /ul and the presence of encephalopathy caused by AIDS. Because of the association between HIV infection and suspiciously combined CMV retinitis, intravenous treatment with ganciclovir was added. Forscarnet is not available in Korea. The patient was referred to Yonsei University Hospital for management of her HIV infection. After the diagnosis, she was treated with zidovudine, lamivudine, and indinavir. On June 16, in spite of medical therapy, the retinal necrosis had progressed (Fig. 3). Total retinal detachment and extensive full-thickness necrosis were found in the left eye, and inferior partial retinal and choroidal detachment were noted in the right eye. The patient's visual acuity was hand motion for both eyes.

Bottom Line: PORN has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with AIDS.Although the etiologic agent has been reported to be VZV, concurrent or combined etiologic agents can include HSV-1, HSV-2, and CMV in AIDS patients.Therefore, combined antiviral therapy with acyclovir and ganciclovir could be more reasonable as an initial therapy.

View Article: PubMed Central - PubMed

Affiliation: NUNE Eye Hospital, Seoul, Korea.

ABSTRACT

Purpose: To describe an unusual case of rapidly progressive outer retinal necrosis (PORN) with vitreous hemorrhage in a 41-year-old woman with acquired immunodeficiency syndrome (AIDS), who had retinitis developed from what was probably varicellar-zoster virus combined with cytomegalovirus (CMV) and herpes simplex type 1,2, as proven by the polymerase chain reaction restriction fragment length polymorphism method (PCR-RFLP).

Methods: This study is a case report detailing clinical follow-up and an aqueous humor test by PCR-RFLP.

Results: The deep, white retinal lesions coalesced and progressively expanded in a circumferential manner, with sparing of the perivascular retina. However, retinal and vitreous hemorrhages, unusual findings for PORN, could be noted around the optic nerve. Varicellar-zoster virus (VZV), cytomegalovirus (CMV), and herpes simplex types 1,2 (HSV-1,2) were detected in the aqueous humor by PCR.

Conclusions: PORN has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with AIDS. Although the etiologic agent has been reported to be VZV, concurrent or combined etiologic agents can include HSV-1, HSV-2, and CMV in AIDS patients. Therefore, combined antiviral therapy with acyclovir and ganciclovir could be more reasonable as an initial therapy.

Show MeSH
Related in: MedlinePlus