Unilateral frosted branch angiitis.

We examined two patients with monocular frosted branch angiitis. The patients were young and healthy; they rapidly developed severe visual loss with thick, white sheathing of the retinal veins and responded promptly to systemic corticosteroids. The fluorescein angiograms showed late leakage from the retinal veins, without evidence of stasis or occlusion. Frosted branch angiitis can be either a unilateral or a bilateral condition. We believe the potential for visual loss and the prompt response to systemic corticosteroids make early, accurate diagnosis and institution of therapy desirable.

Diagnosis and treatment of cytomegalovirus retinitis.

Cytomegalovirus (CMV) retinitis is the most common ocular opportunistic infection in patients with the acquired immune deficiency syndrome (AIDS), in whom it may cause loss of vision. The early diagnosis of CMV retinitis depends on patient awareness of often subtle clinical symptoms; screening examinations may be of benefit. Aggressive treatment of the condition is imperative. Ganciclovir administered intravenously has been shown to be effective therapy, but treatment must be continued indefinitely and is associated with a risk of neutropenia. Careful monitoring during treatment is required. Investigational methods of treatment, including intravitreal injection of ganciclovir or use of foscarnet, another antiviral agent, may be of benefit if intravenous ganciclovir therapy must be discontinued.

Retinopathy before the diagnosis of AIDS.

We reviewed the records of ocular examinations of patients referred or examined for inflammatory retinal disease over a two-year period and found 18 cases in which retinopathy was documented before the diagnosis of acquired immunodeficiency syndrome (AIDS). Seventeen patients were either homosexual men or intravenous drug abusers. Although 13 patients had AIDS-related complex, no patients met the Centers for Disease Control criteria for AIDS before their ophthalmologic examination. In all 18 cases, the diagnosis of severe immunodeficiency suggestive of infection by the human immunodeficiency virus (HIV) was made by the ophthalmologist. These ophthalmologic findings included five patients with asymptomatic retinal cotton-wool patches, two patients with endogenous Staphylococcus epidermidis bacterial endophthalmitis, and 11 cases of isolated cytomegalovirus retinitis. All patients were ambulatory outpatients at the time of ophthalmologic examination. Noninfectious retinopathy and intraocular opportunistic infections suggest the diagnosis of HIV infection and AIDS, and the ophthalmologist may play an important role in early diagnosis of this disease.

Prevalence and significance of acquired immunodeficiency syndrome-related retinal microvasculopathy.

We performed ophthalmologic examinations on 127 subjects with or at risk for human immunodeficiency virus (HIV) infection over a one-year period to determine the prevalence and significance of retinal cotton-wool spots and hemorrhages (AIDS-related retinal microvasculopathy). Of 26 asymptomatic homosexual men, of whom 13 were HIV seronegative and 13 were HIV seropositive, none manifested this retinopathy. Three of 34 patients (9%) with AIDS-related complex and 29 of 67 patients (43%) with AIDS manifested retinopathy on the initial examination. This difference in the prevalence of retinopathy between groups was statistically significant (P less than .05). Patients with AIDS demonstrated 7.2 times greater odds of manifesting retinopathy than patients with AIDS-related complex (P less than .05). Within the group of patients with AIDS, the T helper (CD4) to suppressor (CD8) cell ratio was significantly associated with retinopathy at the initial ocular examination. The CD4:CD8 ratio of the total group of AIDS and AIDS-related complex patients with retinopathy was significantly lower than that of patients without retinopathy (P less than .05). There was no significant association between retinopathy and any specific past or concurrent opportunistic infection or neoplasm. The presence of retinopathy was not associated with symptoms in any patient. The lesions of AIDS-related retinal microvasculopathy may be an important finding in the evaluation of patients suspected to have HIV-related disease.

Demonstration of cytomegalovirus retinitis by in situ DNA hybridization.

Five cases of the acquired immune deficiency syndrome (AIDS), each exhibiting retinitis, were studied by DNA hybridization technique to detect viral infection. Five involved eyes were obtained at the time of autopsy from three patients who had received no treatment for the retinitis. Retinal biopsy specimens were obtained at the time of surgery from two other patients who developed retinal detachments after being treated with ganciclovir (dihydroxy propoxymethyl guanine). DNA hybridization revealed cytomegalovirus in retina and retinal pigment epithelium in all five specimens from patients who had not been treated with ganciclovir. No hybridization occurred in the two retinal biopsy specimens obtained from the ganciclovir-treated patients. These results suggest that in situ DNA hybridization is a highly specific and easily interpretable means of establishing the tissue diagnosis of viral retinitis.

The significance of the pars plana exudate in pars planitis.

We divided 61 patients (111 eyes) with pars planitis into three groups based on clinical appearance. Patients in Group I had bilateral vitreous cells and bilateral pars plana exudates (36 patients, 72 eyes); patients in Group II had bilateral vitreous cells but a pars plana exudate in one eye only (14 patients, 28 eyes); and patients in Group III had vitreous cells and exudate formation in the single eye involved (11 patients, 11 eyes). The presence of a pars plana exudate was associated with more severe vitreous disease and increased incidence of cystoid macular edema.

Prevalence, pathophysiology, and treatment of rhegmatogenous retinal detachment in treated cytomegalovirus retinitis.

Seventeen patients with the acquired immune deficiency syndrome and cytomegalovirus retinitis were treated with the antiviral drug ganciclovir (9-[1,3-dihydroxy-2-propoxy-methyl]-guanine, DHPG). Eight eyes of five patients developed rhegmatogenous retinal detachment after initiation of treatment. Multiple breaks in areas of peripheral, healed, atrophic retina accounted for the detachments. All seven eyes that underwent surgery had extensive retinal detachments that were reattached with vitrectomy and silicone oil. Retinotomy and retinal tacks were necessary in two cases that were complicated by severe proliferative vitreoretinopathy. In the fellow eye of one patient, laser treatment was used prophylactically to wall off a peripheral patch of healed retinitis. Endoretinal biopsies and culture were taken in five eyes; evidence of persistent cytomegalovirus was seen in two cases despite concurrent and clinically effective antiviral therapy.

Cytomegalovirus retinitis and response to therapy with ganciclovir.

A 15-month prospective study of 109 patients with the acquired immune deficiency syndrome (AIDS) or AIDS-related complex (ARC) was conducted. Cytomegalovirus (CMV) retinitis developed in 18 of these patients; they were treated with ganciclovir. Five other patients with CMV retinitis who were not part of the prospective study were also treated with ganciclovir. CMV retinitis frequently involved the peripheral retina. All 23 patients treated with ganciclovir showed clinical regression of retinitis, although breakthrough recurrence of CMV retinitis occurred in seven patients (30.4%) while on maintenance therapy with ganciclovir. During treatment, neutropenia (less than 1000 leukocytes/mm3) developed in three patients (13%). Ganciclovir is an effective means of therapy for CMV retinitis, but it must be given chronically to prevent reactivation. Breakthrough recurrences while on maintenance therapy are not uncommon, but can be successfully treated with more aggressive treatment with ganciclovir. In addition, the prognosis for survival of AIDS patients being treated with ganciclovir is improved when compared with that of untreated patients.

Cytomegalovirus retinitis as the initial manifestation of the acquired immune deficiency syndrome.

We studied eight patients in whom cytomegalovirus retinitis was an initial manifestation of AIDS. Six of these patients had cytomegalovirus retinitis alone at the time of diagnosis and two patients had simultaneous cytomegalovirus retinitis with other AIDS associated diseases (Kaposi's sarcoma in one patient and cytomegalovirus colitis in one patient). Treatment with ganciclovir (dihydroxy propoxymethyl guanine), a new antiviral medication related to acyclovir, resulted in regression of retinitis in all five patients treated with this drug. Our study shows that cytomegalovirus retinitis may be the initial manifestation of AIDS and that ganciclovir is a clinically effective antiviral agent for cytomegalovirus.

Changing patterns of uveitis.

We conducted a retrospective analysis of 600 patients with uveitis seen at the Estelle Doheny Eye Center to determine the frequency of occurrence of the various forms of uveitis and to see if the causes of uveitis have changed as compared with previous studies. In 402 cases (67.0%) we established a specific diagnosis based on history, physical findings, and laboratory studies: 167 cases (27.8%) involved primarily the anterior segment, 230 (38.4%) the posterior segment, and 111 (18.4%) occurred as panuveitis. Intermediate uveitis (pars planitis) was the single most frequently diagnosed uveitic entity and accounted for 92 cases (15.4%). We compared our findings with those of previously published studies and found that, as new diseases occur and improved diagnostic techniques become available, the differential diagnosis of uveitis continues to change.

Cryptococcal chorioretinitis and endophthalmitis.

The diagnosis of endogenous ophthalmic fungal infection may be difficult. A patient initially presented with hydrocephalus and later developed a progressive bilateral intraocular inflammation unresponsive to corticosteroids or antituberculous therapy. Further evaluation with vitreous biopsy study and lumbar puncture led to the diagnosis of cryptococcal chorioretinitis, endophthalmitis, and meningitis. Initial treatment with intravenous amphotericin failed to control the intraocular infections. Vitrectomy and intravitreal injections of amphotericin combined with the systemic intravenous amphotericin halted progression of the intraocular disease. This case demonstrates the difficulty in making the diagnosis of an endogenous fungal endophthalmitis and the value of diagnostic vitreous biopsy study in cases of increasing intraocular inflammation unresponsive to medical therapy. It also suggests the usefulness of vitrectomy combined with intravitreal amphotericin in cases of fungal endophthalmitis that are unresponsive to systemic antifungal agents.

Pars planitis.

The records on thirty-three patients (58 eyes) with the diagnosis of pars planitis were reviewed. The patients were divided into 4 groups: Group 1 had bilateral vitreous cells and pars plana 'snowbank' formation (13 patients, 26 eyes), Group 2 had bilateral vitreous cells and 'snowbank' formation in one eye only (8 patients, 16 eyes), Group 3 had vitreous cells and 'snowbank' formation in one eye only (8 patients, 8 eyes), and Group 4 had bilateral vitreous cells without 'snowbank' formation (4 patients, 8 eyes). Frequent findings and complications were tabulated; cystoid macular oedema was the most common cause of decreased vision. Although pars plana 'snowbank' exudates are usually present in pars planitis, they may not be necessary to make the diagnosis. An approach to therapy is outlined.