The progressive outer retinal necrosis syndrome. A variant of necrotizing herpetic retinopathy in patients with AIDS.
BACKGROUND
The progressive outer retinal necrosis syndrome is a recently recognized variant of necrotizing herpetic retinopathy. This report characterizes more fully its clinical features and course.


METHODS
Using standardized clinical criteria, patients with progressive outer retinal necrosis syndrome from four institutions were identified. Patient records were reviewed retrospectively for the following data: medical and demographic characteristics, presenting symptoms, physical findings, course, responses to treatment, and outcomes.


RESULTS
Thirty-eight patients (65 involved eyes) were studied. All had acquired immune deficiency syndrome. A known history of cutaneous zoster was documented in 22 (67%) of 33 patients. Median CD4 lymphocyte count was 21/mm3 (range, 0-130/mm3). Median follow-up was 12 weeks. The most common presenting symptom was unilateral decreased vision (35 of 65 eyes, 54%); median visual acuity at presentation was 20/30 (range, 20/20 to no light perception [NLP]). Anterior chamber and vitreous inflammatory reactions were absent or minimal in all patients. Typical retinal lesions were multifocal, deep opacities scattered throughout the periphery, although macular lesions also were present in 21 eyes (32%) at diagnosis. Lesions progressed rapidly to confluence. Initial intravenous antiviral therapy appeared to reduce disease activity in 17 (53%) of 32 eyes, but treatment did not alter final visual outcome. Visual acuity was NLP in 42 (67%) of 63 eyes within 4 weeks after diagnosis. Retinal detachment occurred in 43 (70%) of 61 eyes, including 13 (93%) of 14 eyes that received prophylactic laser retinopexy.


CONCLUSIONS
The progressive outer retinal necrosis syndrome is characterized by features that distinguish it from cytomegalovirus retinopathy, acute retinal necrosis syndrome, and other necrotizing herpetic retinopathies. Visual prognosis is poor with current therapies.
Engstrom
R E
RE
UCLA Ocular Inflammatory Disease Center.
Holland
G N
GN
Margolis
T P
TP
Muccioli
C
C
Lindley
J I
JI
Belfort
R
R
Holland
S P
SP
Johnston
W H
WH
Wolitz
R A
RA
Kreiger
A E
AE
D017088:AIDS-Related Opportunistic Infections / Q000473:pathology*; D000293:Adolescent; D000328:Adult; D000998:Antiviral Agents / Q000627:therapeutic use; D002648:Child; D004359:Drug Therapy, Combination; D005260:Female; D006679:HIV Seropositivity / Q000175:diagnosis; D006563:Herpes Zoster Ophthalmicus / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012163:Retinal Detachment / Q000209:etiology / Q000601:surgery; D015882:Retinal Necrosis Syndrome, Acute / Q000150:complications / Q000188:drug therapy / Q000382:microbiology / Q000473:pathology*; D012189:Retrospective Studies
D000998:Antiviral Agents