Allan E. Kreiger

The progressive outer retinal necrosis syndrome. A variant of necrotizing herpetic retinopathy in patients with AIDS.

BACKGROUNDnThe progressive outer retinal necrosis syndrome is a recently recognized variant of necrotizing herpetic retinopathy. This report characterizes more fully its clinical features and course.nnnMETHODSnUsing 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.nnnRESULTSnThirty-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.nnnCONCLUSIONnThe 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.

Management of giant retinal tears without scleral buckling. Use of radical dissection of the vitreous base and perfluoro-octane and intraocular tamponade.

The introduction of intraoperative perfluorocarbon liquids in the management of giant retinal tears has allowed exact anatomic reapposition of the retina and the giant retinal tear. Therefore, the authors managed 11 eyes with giant retinal tears and no proliferative vitreoretinopathy without scleral buckling. Maximum removal of the basal vitreous gel was performed in all cases, and perfluoro-octane was used intraoperatively to unfold the inverted posterior flap and reattach the retina completely. Endophotocoagulation was applied to the edges of the tear and perfluoropropane or silicone oil was used as an intraocular tamponade. Although 1 eye required reoperation for a redetachment from posterior proliferative vitreoretinopathy, all retinas were reattached with final visual acuity of 5/200 or better in 9 eyes. Eight additional major procedures were necessary in seven eyes: removal of silicone oil in four (with removal of perisilicone proliferation in three), repeat vitrectomy and membrane removal with photocoagulation twice in one eye, cataract extraction in one eye, and removal of a macular pucker in one eye. Four eyes required postoperative fluid-gas exchange and three eyes had tissue plasminogen activator injections into the anterior chamber for postoperative fibrin reactions.

Rhegmatogenous Retinal Detachments in Patients with AIDS and Necrotizing Retinal Infections

Rhegmatogenous retinal detachments can occur in patients with acquired immune deficiency syndrome (AIDS) and necrotizing retinal infections. Of 68 patients with AIDS and necrotizing retinal infections seen between 1983 and 1987, rhegmatogenous retinal detachments developed in 16 patients (27 eyes). In this group, cytomegalovirus retinopathy was present in 75% (12 of 16) of patients, 18.8% (3 of 16) had probable herpes simplex virus retinopathy, and 6.2% (1 of 16) had toxoplasmic retinochoroiditis. Retinal detachment was bilateral in 68.8% (11 of 16) of patients. The retina was reattached successfully in 91% (10 of 11) of operated eyes. Proliferative vitreoretinopathy was present preoperatively in 95% of these eyes. Seven of 11 operated eyes had initial improvement in visual acuity. However, 5 of 11 continued to lose vision despite successful reattachment. Techniques included pneumatic reattachment, scleral buckle, vitrectomy, and silicone oil injection. Complicated retinal detachments in AIDS patients with rhegmatogenous retinal detachments and necrotizing retinal infections are common and can be repaired, but the prognosis is guarded in many patients.

Posterior Vitreous Detachment in Diabetic Subjects

Posterior vitreous detachment (PVD) and vitreous synchysis were studied in autopsied diabetic subjects and compared with general autopsy population. PVD was significantly more common in diabetics, including eyes with no retinopathy. Synchysis in diabetics increased with age and with PVD, but was less than in non-diabetics for given age and PVD status. We conclude that synchysis contributes to the precocious and exaggerated PVD in diabetics, but other factors are operative.

An Association Between Acute Retinal Necrosis Syndrome and HLA-DQw7 and Phenotype Bw62,DR4

Human leukocyte antigen (HLA) typing was performed on 27 white patients with acute retinal necrosis syndrome. Antigens for the HLA-A, -B, -C, -DR and -DQ loci were determined, and frequencies were compared with racially matched controls. There was a statistically significant increase in the frequency of HLA-DQw7 (11 of 20 [55%] of patients vs 294 of 1546 [19%] of controls, P = .0004, relative risk 5.20) that remained significant at the P = .05 level when the P value was multiplied by the number of antigens tested. The HLA phenotype Bw62, DR4 is also more frequent than in normal control populations (4 of 25 [16%] of patients vs 26 of 1023 [2.6%] of controls, relative risk 7.49). These results support an association between the acute retinal necrosis syndrome and certain HLA specificities and suggest a possible immunogenetic predisposition to the syndrome in some patients.

Herpesvirus Antibody Levels in the Etiologic Diagnosis of the Acute Retinal Necrosis Syndrome

Quantitative antibody levels to three herpesviruses in acute and chronic sera from six patients with clinical signs of the acute retinal necrosis syndrome were consistent with a specific etiologic diagnosis only in the two cases associated with cutaneous herpes zoster. Available data on acute and convalescent antibody titers to herpes group viruses from these six patients in addition to data from 27 acute retinal necrosis cases from the literature disclosed that only 13 of the 33 patients (39%) had a diagnostic increase or decrease in herpes group viral antibody levels on serial sampling. Three patients had nondiagnostic changes in viral antibody levels despite positive vitreous cultures for herpesviruses. In contrast, a review of 25 cases from the literature with paired antiviral serum and intraocular fluid antibody levels suggested a more promising approach to the etiologic diagnosis of the acute retinal necrosis syndrome. By calculating the ratio of antiviral antibodies in intraocular fluid and serum, an etiologic diagnosis could be made in 12 of 14 (86%) of subacute and convalescent samples. The sensitivity of this method decreased to 72% (13 of 18) when fluids were obtained earlier in the course of the disease.

Vitrectomy techniques in late-stage coats'-like exudative retinal detachment

Retinal telangiectasia is the hallmark of Coats disease. In the late stages, leakage from these abnormal vessels can result in a total, bullous exudative retinal detachment with cholesterol-laden subretinal fluid. Secondary angle-closure glaucoma may result in a blind and painful eye which may require enucleation or evisceration. Surgical reattachment of the retina and destruction of the retinal telangiectasia may preserve these eyes. We have found that vitrectomy, internal drainage of subretinal fluid and cholesterol, direct treatment of the retinal telangiectasia with intraocular diathermy and intravitreal gas or silicone oil injection are effective surgical techniques for salvaging these severely damaged eyes.

Immunocytologic Localization of Herpes Simplex Type 1 Viral Antigens in Herpetic Retinitis and Encephalitis in an Adult

An immunoperoxidase technique was utilized to identify herpes simplex type I viral antigens in the retina, optic nerve and brain of an adult with herpetic retinitis and encephalitis. Viral antigens were demonstrated in all layers of retina, retinal pigment epithelium and to a lesser extent, in choroid. Oligodendroglia in the right optic nerve and neuronal and glial cells in the grey and white matter of the left frontal, inferior parietal and temporal lobes of the brain also expressed herpes simplex antigens. The sensitive immunoperoxidase method allowed detection of viral antigens in many cells without intranuclear inclusions or surrounding inflammation, and thereby added valuable information regarding the anatomic and cellular localization of herpetic infection. The clinicopathologic features that characterize herpes simplex retinitis in the adult are compared to cytomegalovirus retinopathy.

Topical indomethacin in the treatment of chronic cystoid macular edema

Eighty percent of 30 eyes with chronic cystoid macular edema (CME) after cataract extraction achieved improved visual acuity of three or more lines when treated with 1% indomethacin eye drops. Of these patients 53% demonstrated an “on/off” phenomenon induced by the initiation and cessation of treatment documented by visual acuity measurements and fluorescein angiography. This “on/off” phenomenon suggests that there is a direct relationship between the use of 1% indomethacin eye drops and the resolution of chronic CME after cataract extraction. Previous studies on the treatment of CME with indomethacin have not addressed the use of indomethacin eye drops for chronic CME.

Pars plana incisions of four patients : histopathology and electron microscopy

The pathology of pars plana incisions of four patients is described: three with light microscopy and one with light and electron microscopy. Two eyes were removed because of choroidal melanoma, immediately and 8 days after vitrectomy and transvitreous retinal biopsy. Considerable disruption of tissues surrounding the pars plana incisions was observed. Vitreous was incarcerated in the wounds, which healed with granulation tissue. One eye was examined 4 months after vitrectomy for diabetic retinopathy and a failed pars plana filtering operation. It contained fibrovascular ingrowth from all the incisions, infiltrating the vitreous base with granulation tissue and causing vitreous haemorrhage and retinal detachment. One eye was removed 1 year after vitrectomy for anterior hyaloidal fibrovascular proliferation and early phthisis. The wound had fibrous ingrowth histologically and evidence of active fibroplasia.