David J. Forster

Rapidly Progressive Outer Retinal Necrosis in the Acquired Immunodeficiency Syndrome

Two patients, both seropositive for the human immunodeficiency virus, developed rapidly progressive retinal necrosis associated with a systemic herpes zoster infection. The retinitis in these patients was characterized by primary involvement of the outer retina, with sparing of the inner retina and retinal vasculature until late in the disease process; a rapidly progressive course; poor response to intravenous acyclovir; and development of rhegmatogenous retinal detachment. In one of the patients, the retinitis was initially multifocal. Electron microscopy of a retinal biopsy specimen from one of the patients demonstrated virus particles consistent with a herpesvirus, and polymerase chain reaction disclosed herpesvirus in a retinal biopsy specimen of the other patient. This entity may represent a distinct form of acute retinal necrosis that is seen in immunocompromised individuals.

Variations in clinical features of the Vogt-Koyanagi-Harada syndrome.

Forty-eight cases of the Vogt-Koyanagi-Harada (VKH) syndrome occurring in patients residing in southern California were reviewed. Thirty-six patients were Hispanic and 12 of other racial groups. Symptoms of meningismus, predominantly headache, were present in 32 (67%) cases, but the other characteristic neurologic symptoms, i.e., tinnitus and dysacusis, were present in only eight (17%) and six (13%) cases, respectively. Dermatologic changes were rare; vitiligo occurred in five (10%) patients, alopecia in six (13%), and poliosis in three (6%). In this patient population, extraocular signs and symptoms of the VKH syndrome, other than headache, were unusual. The ocular manifestations of the VKH syndrome are more constant and include iridocyclitis, vitritis, diffuse swelling of the choroid, serous retinal detachment, and optic disc hyperemia. Procedures that may aid in the diagnosis include lumbar puncture, fluorescein angiography, and standardized echography. The ophthalmologist must be prepared to make this diagnosis and initiate treatment with high-dose systemic steroids based on the typical ocular findings even in the absence of other (extraocular) manifestations of this disease.

Systemic viral infections and their retinal and choroidal manifestations

Viruses are one of the most common causes of infections involving the posterior segment of the eye. Such infections can occur either on a congenital or an acquired basis, and may affect primarily the retina or the choroid. Congenital cytomegalovirus (CMV) and rubella infections may result in retinitis. CMV retinitis is also the most common cause of acquired viral retinitis, primarily because of the acquired immunodeficiency syndrome (AIDS). Other types of viral retinitis, such as those caused by herpes simplex or herpes zoster, can occur in immunocompromised or immunocompetent individuals. Retinitis or choroiditis caused by viruses such as measles, influenza, Epstein-Barr virus, and Rift Valley fever virus, typically occurs subsequent to an acute viral systemic illness. The systemic and ocular manifestations, as well as the histopathology, laboratory tests, differential diagnoses, and treatment regimens for each of the individual viruses are discussed in detail.

Association between Vogt-Koyanagi-Harada Syndrome and HLA-DR1 and -DR4 in Hispanic Patients Living in Southern California

BACKGROUND Vogt-Koyanagi-Harada (VKH) syndrome is associated with human leukocyte antigen (HLA)-B54, -DR4, -DR beta 1*0405, -DQ4, and -DR53 in Japanese patients. Disease-associated HLA specificities may differ among races. This study examined HLA associations with VKH syndrome in Hispanic patients living in southern California, a racial subgroup at increased risk for the disease. METHODS Human leukocyte antigen specificities were determined on 25 Hispanic patients with VKH syndrome and compared with HLA specificities of 217 healthy Hispanic control subjects. Inclusion criteria for study patients were nontraumatic panuveitis with exudative retinal detachments, with or without extraocular manifestations. Tests were performed using standard cytotoxic assays. RESULTS HLA-DR4 was present in 14 (56%) patients with VKH syndrome and in 62(29%) control subjects (relative risk = 1.96). HLA-DR1 was present in 9 (36%) patients with VKH syndrome and in 19 (9%) control subjects (relative risk = 4.11). HLA-DR1 and DR4 share a common epitope within the DR beta 1 gene. HLA-DR1 and/or DR4 were present in 21 (84%) patients with VKH syndrome and in 76 (35%) control subjects (relative risk = 2.40). CONCLUSIONS HLA-DR1 and -DR4 were found in a significantly disproportionate number of Hispanic patients with VKH syndrome living in southern California. HLA-DR4, although not HLA-DR1, has been previously associated with VKH syndrome in other groups. These associations suggest a common immunogenic predisposition to VKH among different racial groups, and suggest that a common epitope shared by DR1 and DR4 may be involved in the pathogenesis of the disease.

Trabeculectomy and Molteno Implantation for Glaucomas Associated with Uvettis

PURPOSE This study compares the outcomes of trabeculectomy and Molteno implantation in the treatment of glaucomas associated with uveitis. METHODS Forty-five patients with uveitis, who had undergone filtering surgery for glaucomas associated with uveitis, were reviewed retrospectively. Successful outcome was defined as final intraocular pressure (IOP) of 6 to 21 mmHg, with a minimum follow-up of 6 months without visually devastating complications or loss of light perception. RESULTS One- and two-year life-table success rates, respectively, were 81% and 73% with trabeculectomy (16 patients); 53% and 31% with combined trabeculectomy and first-stage (reserve) Molteno implantation (19 patients); and 79% and 79% with one-stage Molteno implantation (10 patients). In 11 patients who underwent second-stage Molteno implantation after trabeculectomy failure, 1- and 2-year life-table success rates were 79% and 79%, respectively. Complications included surgically treated choroidal effusions (1/45; 2%), choroidal hemorrhages (3/45; 7%), and chronic hypotony (3/45; 7%). Follow-up in all groups ranged from 5 to 70 months (mean +/- standard deviation, 28 +/- 17 months). CONCLUSIONS Trabeculectomy provides surprisingly good results in glaucomas associated with uveitis (modulation of wound healing with antimetabolites probably would afford an even higher success rate). However, when significant, immediate postoperative and/or moderate chronic postoperative inflammation is likely, aqueous drainage devices appear more likely to control IOP.

Incidence and Management of Glaucoma in Vogt-Koyanagi-Harada Syndrome

BACKGROUND The Vogt-Koyanagi-Harada syndrome is a bilateral panuveitis associated with neurologic and dermatologic manifestations. METHODS The authors reviewed the charts of all patients with Vogt-Koyanagi-Harada syndrome seen at their institution over the past decade to determine the incidence of glaucoma, as well as the results of medical and surgical therapy for glaucoma, in this group of patients. FINDINGS Of 42 patients diagnosed with Vogt-Koyanagi-Harada syndrome, evidence of glaucoma requiring either medical or surgical intervention occurred in 16 patients (38.1%). Of these, nine (56.3%) had open-angle glaucoma and seven (43.7%) had angle-closure secondary to pupillary block. In 5 (31.3%) of the 16 patients, medical therapy alone was sufficient to control intraocular pressure. Eleven patients (68.7%) required surgical intervention, consisting of laser iridotomy, surgical iridectomy, trabeculectomy with or without 5-fluorouracil, and/or Molteno implantation. CONCLUSION The relative success of each of these procedures in this group of patients is discussed. Glaucoma is a common complication in the Vogt-Koyanagi-Harada syndrome, and one that is often difficult to control.

Anterior Uveitis and Hypopyon

We undertook a study to determine the incidence of hypopyon, as well as the most common anterior uveitis entities with which hypopyon is associated. A total of 216 patients with anterior uveitis were studied. The uveitis was acute in 155. Of the 155 patients, 11 (7.1%) had hypopyon. Nine of the 11 patients with hypopyon were positive for HLA B27. Of these nine, two had Reiters syndrome and one had ankylosing spondylitis; the other six had no confirmed systemic disease. Of the two patients with hypopyon who were HLA B27-negative, one had mixed connective-tissue vascular disease, and one had idiopathic anterior uveitis. Of the 155 patients with acute anterior uveitis, 62 were HLA B27-positive. Thus, the incidence of hypopyon uveitis among HLA B27-positive patients was 14.5% (nine of 62 patients), whereas the incidence among HLA B27-negative patients was only 2.2% (two of 93 patients). These results suggest that HLA B27-related anterior uveitis is the most common cause of hypopyon uveitis, and that most patients with anterior uveitis associated with hypopyon will test positive for HLA B27. Although these results reflect a referral population, they should be of benefit in the treatment of patients with anterior uveitis.

Pneumocystis Carinii Choroiditis After Long-Term Aerosolized Pentamidine Therapy

Pneumocystis carinii pneumonia is a major cause of morbidity and mortality in patients with the acquired immunodeficiency syndrome. When P. carinii is disseminated, the choroid may be involved and the infection is often fatal. We examined, treated, and followed up two patients who developed choroidal lesions typical of P. carinii while taking aerosolized pentamidine for prophylaxis. The choroidal lesions gradually resolved after three weeks of therapy with intravenous trimethoprim and sulfamethoxazole in one patient, and after three weeks of therapy with parenteral pentamidine in the other patient. The patients did not have clinical or laboratory evidence of P. carinii infection other than in the eye. It thus appears that early ophthalmologic examination may detect disease before it is threatening to sight and allow systemic therapy to be instituted before widely disseminated infection results in a fatal outcome.

Panuveitis and multifocal retinitis in a patient with leucocytoclastic vasculitis.

To our knowledge, bilateral panuveitis and multifocal retinitis/vasculitis have not been described previously in association with leucocytoclastic vasculitis. We describe a patient with bilateral panuveitis associated with multifocal retinitis/vasculitis who also had multiple purpuric, palpable skin lesions. Biopsies of the skin lesions were diagnostic of leucocytoclastic vasculitis. An extensive examination for systemic disease was otherwise negative. Prompt treatment with systemic corticosteroids resulted in dramatic improvement ofboth the ocular and the skin lesions.

Bull's Eye Retinopathy and Clofazimine

Excerpt To the Editors:Clofazimine, a red phenazine dye used to treat dapsone-resistant leprosy, has now become part of the standard multidrug regimen for the treatment of patients with the acquire...