Human T-cell lymphotropic virus type I-associated retinal lymphoma. A clinicopathologic report.

Human T-cell lymphotropic virus type I has been associated with a wide range of ocular conditions, including neoplastic, infectious, and inflammatory lesions. We studied a patient infected with human T-cell lymphotrophic virus type I who presented with deep retinal and subretinal infiltrates but without cells in the vitreous. The differential diagnosis included intraocular lymphoma and fungus infection. A chorioretinal biopsy specimen obtained for tissue diagnosis disclosed large atypical mononuclear cells located primarily at the level of the retinal pigment epithelium but focally involving overlying retina. Electron microscopy of this infiltrate showed features consistent with adult T-cell lymphoma/leukemia. Infection by human T-cell lymphotropic virus type I was verified by polymerase chain reaction studies conducted on peripheral-blood mononuclear cells. This case emphasizes the occurrence of intraocular lesions in adult T-cell lymphoma/leukemia that clinically show some features similar to those of the usual ocular lymphoma (reticulum cell sarcoma); diagnosis can be established by chorioretinal biopsy, thereby allowing appropriate therapy.

Echographic findings in infectious endophthalmitis.

OBJECTIVE: To correlate the initial echographic findings in eyes with infectious endophthalmitis with the visual prognosis and causative microorganism. DESIGN: A retrospective review of the clinical and standardized ocular echographic findings in eyes with infectious endophthalmitis was performed. SETTING: University-based ophthalmology department. STUDY PARTICIPANTS: One hundred thirty-seven eyes (136 patients) with infectious endophthalmitis that were evaluated by the ocular echography service of the Doheny Eye Institute, Los Angeles, Calif, between January 1, 1981, and December 31, 1992. RESULTS: Four findings on initial echography were associated with poor initial vision: dense vitreous opacities, retinal detachment, macular detachment, and choroidal detachment. Five findings on initial echography correlated with poor final vision: dense vitreous opacities, vitreous membranes, the presence of retinal detachment, the extent of retinal detachment, and the presence of choroidal detachment. Change (decrease) in vision during the follow-up period was associated with the presence of combined vitreous and subhyaloid opacities, retinal detachment, and choroidal detachment. All eyes with initially clear vitreous on ocular echography had either early streptococcal or culture-negative endophthalmitis. Advanced streptococcal endophthalmitis correlated with the most severe vitreous inflammation, vitreous membranes, and the most extensive posterior vitreous detachment, whereas gram-negative endophthalmitis correlated with choroidal detachment on initial echography. Stepwise logistic regression analysis revealed that the presence of choroidal detachment, not gram-negative microorganisms, was the principal predictor of poor visual outcome in these eyes. CONCLUSION: Ocular echography is a useful method in the clinical evaluation and treatment of infectious endophthalmitis, especially in eyes with opaque media.

Juvenile xanthogranuloma masquerading as pediatric chronic uveitis: a clinicopathologic study.

Juvenile xanthogranuloma (JXG) is a rare, pediatric histiocytic skin disorder that may affect the eye. It can present with protean ocular manifestations, including masquerade uveitis, heterochromia, hyphema, or glaucoma. It very rarely involves the retina and posterior segment; indeed, posterior involvement has been documented histopathologically in only one case. We present the case of a 2-year-old child with ocular JXG presenting as chronic, refractive uveitis, without skin or systemic findings. The blind, painful eye was enucleated and found to harbor a diffuse histiocytic process that involved both the anterior and posterior segments, including the retina and subretinal space. Histological, immunohistochemical, and electron microscopic studies confirmed the diagnosis of JXG. The pathologic classification and differential diagnosis of systemic histiocytic disorders are discussed. Since JXG can present as masquerade pediatric uveitis, this entity should be considered in children with atypical uveitis. In rare instances, JXG may involve the posterior segment and the retina, leading to retinal detachment and blindness.

Ocular adnexal Kaposi's sarcoma in acquired immunodeficiency syndrome.

We examined histopathologically 18 ocular adnexal Kaposi's sarcoma lesions related to acquired immunodeficiency syndrome. These lesions were classified into three types. Type I consisted of thin, dilated vascular channels lined by flat endothelial cells with lumen-containing erythrocytes. Type II featured plump, fusiform, endothelial cells, often with a hyperchromatic nucleus and foci of immature spindle cells and occasional slit vessels. Type III was characterized by large aggregates of densely packed spindle cells with hyperchromatic nuclei, occasional mitotic figures, and abundant slit spaces often containing erythrocytes in between. Clinically, type I and type II tumors were patchy and flat (less than 3 mm in height) and of less than four months' duration. Type III tumors were nodular and elevated (greater than 3 mm in height). We describe the clinical and histopathologic types of Kaposi's sarcoma that may help in diagnosis.

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.

Clinicopathologic study of retinal and choroidal biopsies in intraocular inflammation.

PURPOSE: We reviewed the clinical and histopathologic features of 33 intraocular tissue biopsy specimens from 32 patients and assessed the value of retinal and chorioretinal biopsies performed in patients with intraocular inflammation. METHODS: Twenty-four endoretinal biopsies and nine chorioretinal or choroidal biopsies were performed. On the basis of clinical indications, the specimens were processed for light microscopy, electron microscopy, immunohistochemical staining, in situ DNA hybridization, and polymerase chain reaction. RESULTS: Of the 24 endoretinal biopsy specimens, 19 were from patients with clinical signs suggestive of viral retinitis. Overall, the diagnosis of viral retinitis was suggested by electron microscopy, immunohistochemical staining, in situ DNA hybridization, or polymerase chain reaction in 53% (ten of 19) biopsies. The preoperative diagnosis was confirmed in seven of ten biopsies in cases of suspected cytomegalovirus retinitis, in one of seven biopsies in cases of suspected acute retinal necrosis, and in two of two biopsies in cases of progressive outer retinal necrosis. The remaining five endoretinal biopsies disclosed Candida in one specimen, subretinal fibrosis in one, and chronic inflammation in three. Histologic examination of the nine chorioretinal or choroidal biopsies disclosed lymphoma in two specimens, a subretinal neovascular membrane in one, uveal melanocytic proliferation in one, toxoplasmic retinochoroiditis in one, viral retinitis in one, and long-standing inflammation in three. CONCLUSION: In select cases of intraocular inflammation, intraocular tissue biopsies may provide clinically useful information.

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.

Repair of retinal detachment caused by cytomegalovirus retinitis in patients with the acquired immunodeficiency syndrome.

Twenty-two eyes of 19 patients with the acquired immunodeficiency syndrome who had pars plana vitrectomy and silicone-oil injection after retinal detachment caused by cytomegalovirus retinitis were studied. All patients but one were monitored until time of death. The postoperative survival time and the factors that predicted anatomic success (retinal attachment) and functional success (visual acuity) were analyzed. No intraoperative complications were encountered. The mean survival time after surgery was four months. Of all of the preoperative and intraoperative factors studied, only the duration of cytomegalovirus retinitis was predictive of survival (P less than .03). The anatomic success rate was 89.5% (17 of 19 patients). None of the factors showed a trend or statistical significance in relation to anatomic success. Fifteen of 19 patients (79%) had lost at least two lines of Snellen visual acuity at time of death. Vision declined in a bimodal pattern (within the first postoperative month and after four months postoperatively). The optic nerve was pink and well perfused preoperatively in 16 of 19 patients (81.8%), but optic-nerve atrophy was observed postoperatively in 18 of 19 patients (95.5%). There was a trend for functional success to be influenced by increased intraocular pressure and optic-nerve atrophy, although our sample size was too small for statistical significance.

Mycobacterium fortuitum keratitis.

Two of four cases of Mycobacterium fortuitum keratitis occurred after corneal surgery with contact lens wear, one was associated with extended contact lens wear alone, and one occurred after a foreign body injury. All cases were characterized by pain, conjunctival hyperemia, stromal inflammation, and ulceration. Diagnosis was made by culture and acid-fast staining of corneal scrapings. On the basis of published experience with amikacin for the treatment of nonocular M. fortuitum infections, three patients were treated with topical amikacin. Two patients responded clinically, but histopathologic examination of a penetrating keratoplasty specimen in one of the two disclosed persistent infection. One patient was cured of early disease by debridement alone. Rapid diagnosis and absence of corticosteroid use were the two most important determinants of successful therapy. In advanced cases, infection may be cured and useful vision restored by penetrating keratoplasty.

Massive suprachoroidal hemorrhage during pars plana vitrectomy associated with Valsalva maneuver.

PURPOSE: To report the intraoperative occurrence of massive intraocular suprachoroidal hemorrhage associated with Valsalva maneuver. METHODS: Retrospective, multicenter study of patients who developed massive choroidal hemorrhage associated with Valsalva maneuver during vitrectomy. RESULTS: Massive intraoperative suprachoroidal hemorrhage in seven patients (seven eyes) involved three men and four women with a median age of 52 years (range, 26 to 82 years). General anesthesia was used in six of seven cases. Coughing or "bucking" on the endotracheal tube during general anesthesia or severe coughing during the one vitrectomy performed under local anesthesia was associated with massive suprachoroidal hemorrhage. In five of seven eyes, this occurred near the end of surgery, after air-fluid exchange but before sclerotomy closure. Scleral plugs were immediately placed, and sclerotomy closure was performed exigently. Immediate posterior sclerotomy was performed on five of seven eyes; an additional patient underwent posterior sclerotomy postoperatively. After median follow-up of 18 months (range, 3 to 36 months), final visual acuity was no light perception in four eyes, light perception in one eye, 20/250 in one eye, and 20/20 in one eye. Four eyes became phthisical. CONCLUSIONS: Valsalva maneuver during pars plana vitrectomy may result in massive suprachoroidal hemorrhage with disastrous visual consequences. Precautionary measures to prevent coughing or "bucking" on the endotracheal tube during general anesthesia, or a prolonged episode of coughing during local anesthesia, may prevent this potentially devastating complication.

Tumor necrosis factor-alpha (TNF-alpha)-induced optic neuropathy in rabbits.

Both in vitro and in vivo studies have implicated a role for tumor necrosis factor-alpha (TNF alpha) in various demyelinating diseases, including HIV-related encephalopathy. To investigate whether intravitreal TNF alpha can induce optic nerve axonal damage in a rabbit eye model, fifteen rabbit eyes were initially injected with TNF alpha (final concentrations: 2U, 20U, and 200U respectively) and studied at varying time intervals for up to 24 weeks post-injection, using light and electron microscopy. Control optic nerves (no injection or diluent injection only) had normal myelinated axons and glia; the myelinated regions, neural retina, retinal glia and vasculature of control retinas were normal. In TNF alpha-exposed optic nerves, intact, degenerating and demyelinated axons were interspersed. Astrogliosis was present, particularly from 8 weeks p.i. and was noted up to 24 weeks. Oligodendrocytes were not severely affected in TNF alpha-exposed optic nerves, and activated macrophages or microglia were not obvious. Axonal degeneration was visible among the more superficial myelinated fibers in TNF alpha-exposed retinas however the neural retina glia were unaffected. These observations suggest that the axonal degeneration induced in TNF alpha-exposed rabbit optic nerves over a 24 week period was most likely related to direct effects of TNF alpha on optic nerve axons, and not primarily due to anterograde degeneration from retinal lesions. In-so-far as neurological pathology in general, and optic nerve degeneration in particular, has been described in AIDS, and TNF alpha levels may be elevated in this disease, it is of great clinical significance that TNF alpha has the capacity to mediate neuronal or axonal injury. If so, strategies to block or inhibit TNF alpha can be pursued for treatment for the neurological symptoms of AIDS.

Mycobacterium fortuitum keratitis. Clinicopathologic correlates and corticosteroid effects in an animal model.

Mycobacterium fortuitum keratitis is an indolent infection of traumatized corneas in humans. To study this disorder in an animal model, 10(4) M fortuitum organisms (10 microliters) were inoculated into the stroma of both corneas of 16 New Zealand albino rabbits. Eight of the rabbits were also given bilateral subconjunctival injections of methylprednisolone acetate (20 mg in 0.5 ml) at the time of inoculation. Two corticosteroid-treated and two untreated rabbits were selected each week after inoculation for histopathological examination and quantitative cultures. Corneal lesions in corticosteroid-treated eyes were characterized clinically by indolent ulcerations and satellite lesions that slowly enlarged; on histopathologic examination at each week, acute inflammation and microorganisms were consistently present. Corneal lesions in untreated eyes were characterized clinically by small infiltrates that progressed little over time; at weeks 1 and 2, light microscopic examination showed intrastromal granulomatous and/or mixed acute and chronic inflammation with focal intrastromal necrosis, but at weeks 3 and 4 there was no evidence of active disease. Organisms could not be identified microscopically in corneas of any untreated rabbits. Mean values for quantitative cultures of corneas were higher in corticosteroid-treated rabbits after week 1, although standard deviations were large. These results suggest that M fortuitum keratitis in rabbits is made worse by corticosteroid use. Clinical and histopathologic changes were compared with human disease and found to be similar in corticosteroid-treated rabbits.

Opportunistic intraocular infections in AIDS.

In conclusion, this clinicopathologic study has shown that CMV ocular infection is present in about 16% of terminal AIDS patients. The treatment of CMV retinitis reduces the number of CMV-infected nonocular organs and may also lessen the severity and control the spread of concurrent nonocular infection, both of which may prolong survival in AIDS patients. Other opportunistic infections, involving primarily the choroid, were also seen in a number of patients, some of whom had concurrent intraocular infections with CMV and P carinii, M avium-intracellulare, C neoformans. In addition, all of these choroidal infections were components of disseminated infection, underscoring the increasingly important role of the ophthalmologist in the diagnosis and treatment of disseminated opportunistic infections in AIDS.

Infectious multifocal choroiditis in patients with acquired immune deficiency syndrome.

PURPOSE: The purposes of this study are to determine the incidence of infectious opportunistic choroiditis in patients with the acquired immune deficiency syndrome (AIDS), to study the association of these choroidal infections with systemic dissemination, and to investigate the life expectancy and cause of death in patients with infectious opportunistic choroiditis. METHODS: A total of 470 eyes of 235 consecutive autopsies of patients with AIDS were examined by histopathologic methods. The clinical charts and autopsy reports of these patients were subsequently reviewed for presence of systemic dissemination of various infectious agents. RESULTS: Of the 235 patients, 18 were found to have infectious choroiditis. The etiologic agents found were: Cryptococcus neoformans, Pneumocystis carinii, Mycobacterium tuberculosis, Histoplasma capsulatum, Candida, Aspergillus fumigatus, Toxoplasma gondii, and Mycobacterium avium-intracellulare. In 15 of these 18 patients, the cause of death was considered to be due to systemic dissemination of the organism causing the choroiditis. Only 4 of the 18 cases of infectious choroiditis were diagnosed during life, and the survival time of these patients after diagnosis was only 25 days. Five of the 18 patients also were found to have cytomegalovirus (CMV) infection of the retina. CONCLUSION: Multifocal choroiditis due to endogenous infectious emboli in patients with AIDS reflects systemic dissemination and localization of infectious agents predominantly in the choriocapillaris. Early diagnosis and treatment are imperative and may be life-saving.

Treatment of ocular adnexal Kaposi's sarcoma in acquired immune deficiency syndrome.

PURPOSE: To determine the most effective, safe, and cost-efficient treatment for ocular adnexal Kaposi's sarcoma in the acquired immune deficiency syndrome. METHODS: Eighty-two patients with ocular adnexal Kaposi's sarcoma related to the acquired immune deficiency syndrome were examined, and 25 were selected to participate in a 3-year study that treated the ocular lesions based on stage of development and location. RESULTS: Of 14 patients with bulbar conjunctival Kaposi's sarcoma treated with surgical excision, 2 stage III lesions recurred during a follow-up that ranged from 8 to 31 months; no stage I or stage II lesion recurred. Of 7 patients with eyelid Kaposi's sarcoma treated with cryotherapy, 2 stage III lesions recurred during a follow-up that ranged from 9 to 24 months; no stage I or stage II lesion recurred. Four patients with stage III Kaposi's sarcoma of the bulbar conjunctiva were treated with fluorescein angiography-based surgical excision. None of these lesions recurred during a follow-up that ranged from 4 to 8 months. CONCLUSION: A treatment regimen for ocular adnexal Kaposi's sarcoma related to the acquired immune deficiency syndrome based on tumor stage and location, using cryotherapy and surgical excision with or without fluorescein angiography, is effective, safe, and cost-efficient.

Pars plana vitrectomy for intraocular inflammation-related cystoid macular edema unresponsive to corticosteroids. A preliminary study.

PURPOSE: To determine the role of pars plana vitrectomy in patients with intraocular inflammation-related cystoid macular edema that is unresponsive to corticosteroids. METHODS: Eleven eyes of nine patients underwent a standard three-port pars plana vitrectomy. The primary indication was intraocular inflammation-related cystoid macular edema that was unresponsive to oral, sub-Tenon's, and topical corticosteroids. Preoperative follow-up ranged from 20 months to 144 months (average, 70 months). Postoperative follow-up ranged from 3 months to 108 months (average, 21 months). RESULTS: Seven eyes (64%) improved 4 or more lines of Snellen visual acuity within 4 weeks. Two eyes (18%) remained unchanged and 2 eyes (18%) worsened. Cystoid macular edema improved by clinical examination and fluorescein angiography in 9 eyes (82%) and by clinical examination alone in 2 eyes (18%). No intraoperative complications were noted. Postoperative complications consisted of cataract formation in 1 eye (9%), glaucoma in 2 eyes (18%), and epiretinal membrane formation in 1 eye (9%). CONCLUSION: Pars plana vitrectomy may have a role in the treatment of intraocular inflammation-related cystoid macular edema that fails to respond to corticosteroids. The subgroup of patients who benefit most remains to be identified.

Macular hole surgery with internal-limiting membrane peeling and intravitreous air.

OBJECTIVE: To examine the results of macular hole surgery using pars plana vitrectomy, internal-limiting membrane peeling, and intravitreous air in a series of consecutive patients. DESIGN: A retrospective, interventional, noncomparative case series. PATIENTS: Fifty consecutive patients (58 eyes) with full-thickness macular holes. INTERVENTION: All eyes underwent a pars plana vitrectomy with internal-limiting membrane peeling and intravitreous air, and patients were asked to position face-down for only 4 days. MAIN OUTCOME MEASURES: Status of macular holes, visual acuity, and associated findings and complications. RESULTS: All patients had postsurgical follow-up of 6 months or greater. Eight eyes (14%) presented with stage-2 macular holes, 48 eyes (83%) with stage-3 macular holes, and 2 eyes (3%) with stage-4 macular holes. Only 26 eyes (45%) had a macular epiretinal membrane seen before surgery. Fifty-three (91 %) of the 58 macular holes were closed with 1 operation, and 55 (95%) had closure of the macular holes with subsequent operations. Five (9%) of 58 eyes had an initial visual acuity of 20/50 or better, and 31 eyes (53%) had a final visual acuity of 20/50 or better. Of the 45 eyes with symptoms of less than 6 months' duration, 44 (98%) had macular holes that were closed with 1 operation and 27 (60%) had a final visual acuity of 20/50 or better. Of the 13 eyes with symptoms of 6 months' duration or longer, 9 (69%) had macular holes that were closed with 1 operation and 4 (31 %) had a final visual acuity of 20/50 or better. Complications attributed to the operation included retinal tears, retinal detachments, postoperative macular puckers, and macular light toxicity. CONCLUSIONS: The anatomic and visual results in this series are good. The current technique is similar to that of conventional macular hole surgery except for the use of intravitreous air, internal-limiting membrane peeling in all eyes, and only 4 days of postoperative positioning. This study would suggest that peeling of the internal-limiting membrane is an important adjuvant for successful closure of macular holes.