Immune recovery vitritis associated with inactive cytomegalovirus retinitis: a new syndrome.

OBJECTIVE: To describe a syndrome of posterior segment intraocular inflammation that causes visual loss in patients with acquired immunodeficiency syndrome and cytomegalovirus retinitis. This syndrome was associated with immune recovery mediated by combination antiretroviral treatment including protease inhibitors. DESIGN: A case-control study at 2 university medical centers. PARTICIPANTS: One hundred thirty patients with acquired immunodeficiency syndrome and cytomegalovirus retinitis were examined at 2 medical centers for 15 months. In addition, the medical records of 509 patients examined at 1 center for 11 years before the initiation of protease inhibitor therapy were analyzed retrospectively. RESULTS: Five patients with symptomatic vitritis and papillitis with cystoid macular edema or epiretinal membrane formation were documented. In each patient there was inactive cytomegalovirus retinitis that had not caused visual decrease before the onset of inflammation. All patients had elevated CD4+ T lymphocyte levels (median increase, 86x10(6)/L [86 cells/mm3]) after combination treatment including protease inhibitors. Two patients with cystoid macular edema were treated with corticosteroids and had resolution of the cystoid macular edema and an increase in visual acuity without reactivation of the retinitis. Retrospective analysis failed to disclose similar patients with intraocular inflammation in the era before the introduction of protease inhibitors. CONCLUSIONS: This newly described syndrome of posterior segment inflammation related to cytomegalovirus retinitis is a cause of visual morbidity in patients with acquired immunodeficiency syndrome. It is associated with increased immune competence as a result of combined antiretroviral treatment with protease inhibitors and may be amenable to corticosteroid therapy without reactivation of retinitis.

Corneal microsporidiosis in a patient with AIDS.

Microsporidia are obligate intracellular protozoan parasites that are becoming increasingly recognized as opportunistic pathogens in patients with AIDS. They have been associated with enteritis, hepatitis, and peritonitis and recently keratoconjunctivitis. Gram stain demonstrates the presence of these organisms on light microscopic sections. The specific diagnostic features that distinguish microsporidia from other small nonspore-forming organisms are best demonstrated by electron microscopy, which is also used to characterize the members of Microsporea. In this study, salient histopathologic features of microsporidia in corneal epithelium obtained from an HIV-seropositive individual who developed AIDS are illustrated and discussed.

Plasmapheresis treatment of central retinal vein occlusion in a young adult.

PURPOSE/METHODS: A 16-year-old girl with mixed connective tissue disease had central retinal vein occlusion. The patient was treated with plasmapheresis. RESULTS/CONCLUSION: Resolution of clinical findings of central retinal vein occlusion was documented by fluorescein angiography and visual field tests within 15 days of treatment. Plasmapheresis may be effective therapy in carefully selected cases of central retinal vein occlusion in young adults.

Optic nerve head neovascularization in a patient with inactive cytomegalovirus retinitis and immune recovery.

PURPOSE: To report a case of optic nerve head neovascularization in a patient with acquired immunodeficiency syndrome (AIDS) associated with inactive cytomegalovirus retinitis and immune recovery. METHOD: Case report. RESULTS: We examined a 29-year-old man with AIDS and inactive cytomegalovirus retinitis and found vitritis and prominent optic nerve head neovascularization. The patient had been treated with reverse transcriptase and protease inhibitors, resulting in a notable rise in CD4+ lymphocyte count and an undetectable human immunodeficiency virus (HIV)-RNA viral load. No cause of neovascularization other than intraocular inflammation was detected. CONCLUSION: Immune recovery in a setting of inactive cytomegalovirus retinitis can result in optic nerve head neovascularization, as seen in our patient.

Treatment of clinically resistant cytomegalovirus retinitis with combined intravitreal injections of ganciclovir and foscarnet.

PURPOSE: To determine the efficacy of combined ganciclovir and foscarnet intravitreal injections in controlling clinically resistant cytomegalovirus retinitis in a 37-year-old man with the acquired immunodeficiency syndrome who refused systemic therapy. METHODS: The patient refused systemic therapy and was treated with intravitreal injections of ganciclovir and foscarnet, which were then combined when the retinitis became resistant to either drug alone. RESULTS: The retinitis was initially controlled with bilateral intravitreal ganciclovir injections. After reactivation of retinitis in the left eye, intravitreal foscarnet was effective until recurrent retinitis threatened the center of the fovea. The retinitis continued to progress until combined intravitreal injections of ganciclovir and foscarnet were administered. CONCLUSIONS: Combined intravitreal injections of ganciclovir and foscarnet may be effective in treating cytomegalovirus retinitis when the infection is clinically resistant to either intravitreal drug alone. Intravitreal injections can be effective in controlling cytomegalovirus retinitis in patients who are intolerant of or refuse systemic therapy.

Phacoemulsification cataract extraction and posterior chamber lens implantation in patients with uveitis.

PURPOSE: This study reports outcomes of phacoemulsification cataract extraction and posterior chamber intraocular lens implantation within the capsular bag in patients with uveitis. METHODS: We retrospectively reviewed the charts of 32 patients (39 eyes) with uveitis who underwent phacoemulsification cataract extraction and posterior chamber intraocular lens implantation by two surgeons at The Cleveland Clinic Foundation from January 1990 to June 1998. Patients with less than 3 months of follow-up were excluded. RESULTS: Diagnoses of uveitis included idiopathic (15 eyes), sarcoidosis (10 eyes), pars planitis (four eyes), CMV retinitis (two eyes), Fuchs heterochromic iridocyclitis (two eyes), syphilis (two eyes), and one eye each of tuberculosis, Crohn's disease, HLA-B27 associated, and acute retinal necrosis. Average follow-up was 20 months (range, 3 to 63 months). Best-corrected visual acuity improved in 37 eyes (95%). Average improvement was 4 +/- 3 Snellen acuity lines (range, 1 to 10 lines). Thirty-four eyes (87%) attained final visual acuity better than or equal to 20/40. Visual loss occurred in one eye (3%) with CMV retinitis. No improvement in visual acuity was seen in one eye (3%) that developed a retinal pigment epithelial detachment. Posterior capsule opacification occurred in 24 eyes (62%), 12 of which required Nd:YAG capsulotomy (31%). Other postoperative complications included recurrence of uveitis (41%), cystoid macular edema (33%), epiretinal membrane formation (15%), and posterior synechiae (8%). CONCLUSIONS: Phacoemulsification cataract extraction with posterior chamber intraocular lens implantation is safe in patients with uveitis. The incidences of recurrence of uveitis, cystoid macular edema, epiretinal membrane, and posterior synechiae were lower than those reported previously for extracapsular cataract extraction.

Retinal vasculitis and posterior pole "hypopyons" as early signs of acute bacterial endophthalmitis.

PURPOSE: To report a case of acute postoperative bacterial endophthalmitis presenting with retinal vasculitis and posterior pole "hypopyons." DESIGN: Observational case report. METHODS: Observational case report. RESULTS: In a 73-year-old woman, acute postoperative endophthalmitis presented with retinal vasculitis, diffuse retinal hemorrhages, and several posterior pole "hypopyons" that were collections of yellow-white inflammatory debris. The inflammation was located primarily in the posterior one third of the vitreous, and except for severely impaired visual acuity, the classic signs of acute postoperative endophthalmitis were not present until the next day. An immediate pars plana vitrectomy with intravitreal injection of vancomycin, amikacin, and dexamethasone was performed. Vitreous and aqueous cultures grew coagulase-negative Staphylococcus species. After intravenous and topical therapy for endophthalmitis, the inflammation subsided. Eight weeks after the initial cataract surgery, the best corrected visual acuity of the patient was 20/25. CONCLUSION: Acute postoperative bacterial endophthalmitis may present with atypical clinical signs and inflammation located primarily in the retina and posterior vitreous.

Acute multifocal inner retinitis.

Two patients developed acute changes in vision two to four weeks after a febrile illness. On ophthalmic examination, each patient had bilateral vitreitis without anterior segment inflammation and multiple, bilateral, round, yellow-white inner retinal lesions that were located in the posterior pole and midperiphery. Laboratory tests did not contribute to a diagnosis. Symptomatic visual loss was caused by neuroretinitis and serous retinal detachment in one patient and by an occluded branch retinal artery in the other. The multifocal retinal lesions resolved gradually without treatment over several months with minimal or no residual retinal changes. Acute multifocal inner retinal lesions may be associated with a preceding nonspecific viral illness and may cause a sudden change in vision if associated with neuroretinitis, serous retinal detachment, or retinal vessel occlusion. We have termed this constellation of ophthalmic findings acute multifocal inner retinitis.

CD8+ T lymphocytes and cytomegalovirus retinitis in patients with the acquired immunodeficiency syndrome.

PURPOSE: We compared the levels of CD8+ and CD4+ cells in human immunodeficiency virus (HIV)-seropositive patients who had normal eye examinations, microvasculopathy, or ocular infections other than cytomegalovirus retinitis to those of patients with cytomegalovirus retinitis, to determine whether lymphocyte counts other than CD4+ are predictive of cytomegalovirus retinitis. METHODS: The records of HIV-positive patients who had a lymphocyte subset analysis within three months of a complete eye examination were reviewed for age, gender, mode of HIV transmission, stage of disease, ocular findings, and absolute lymphocyte counts. Data for patients without cytomegalovirus retinitis were compared with those for patients with cytomegalovirus retinitis. RESULTS: Ninety-three HIV-positive patients had a lymphocyte subset analysis within three months of a complete eye examination; 76 patients had no cytomegalovirus retinitis and 17 had cytomegalovirus retinitis. Patients without cytomegalovirus retinitis and those with cytomegalovirus retinitis had the following median cell counts: CD4+, 76.0 and 15.0 cells/microliters; CD8+, 634.5 and 280.0 cells/microliters, respectively. Patients with cytomegalovirus retinitis had significantly lower CD4+ and CD8+ cell counts than those without cytomegalovirus retinitis (P < .001). CD4+ and CD8+ cells are significantly correlated with each other, and the correlation is much higher in patients with cytomegalovirus retinitis (r = .80, P < .001) than in patients without cytomegalovirus retinitis (r = .57, P < .001). Stepwise logistic regression analysis showed that CD8+ cell counts were also predictive of cytomegalovirus retinitis. CONCLUSION: Patients with low CD4+ cell counts are known to be at high risk for cytomegalovirus retinitis. We showed that patients with low CD8+ cell counts are also at high risk for cytomegalovirus retinitis.

Cicatrizing conjunctivitis associated with paraneoplastic lichen planus.

PURPOSE: To report two cases of cicatrizing conjunctivitis associated with paraneoplastic lichen planus. METHODS: Case reports. RESULTS: Two patients were examined because of redness and discomfort in both eyes. A 63-year-old woman with follicular, small-cleaved cell lymphoma had cicatrizing conjunctivitis, stomatitis, vulvitis, and skin lesions. A 25-year-old man with malignant thymoma had cicatrizing conjunctivitis, erosive stomatitis, and penile papules. Histopathologic studies of conjunctiva and skin biopsy specimens in the first patient and labial biopsy specimens in the second revealed lichen planus. CONCLUSION: Paraneoplastic lichen planus is a possible cause of cicatrizing conjunctivitis associated with inflammatory skin and mucous membrane disease.

Chest computed tomography and mediastinoscopy in the diagnosis of sarcoidosis-associated uveitis.

PURPOSE: To report the usefulness of chest computed tomography and mediastinoscopy in diagnosing sarcoidosis in elderly patients with uveitis. METHODS: Case reports. Two Caucasian women, aged 73 and 70 years, underwent evaluations for uveitis, which included chest computed tomography and mediastinoscopy. RESULTS: A chest computed tomographic scan of each woman disclosed mediastinal lymphadenopathy and warranted mediastinal lymph node biopsy by mediastinoscopy. In both patients, histopathologic and microbiologic studies demonstrated sterile noncaseating granulomas consistent with the diagnosis of sarcoidosis. CONCLUSIONS: The diagnosis of sarcoid-associated uveitis in the elderly may be facilitated by chest computed tomography, which may be more sensitive than conventional chest roentgenography. Laboratory studies of biopsied mediastinal nodes obtained by mediastinoscopy can confirm the diagnosis.

Acute posterior multifocal placoid pigment epitheliopathy after acute group A streptococcal infection.

PURPOSE: We studied a case of acute posterior multifocal placoid pigment epitheliopathy in a 40-year-old man who had had an acute febrile illness. METHODS: The medical record was reviewed for clinical manifestations, course of disease, and laboratory findings, including results of fluorescein and indocyanine green angiography. RESULTS: The patient had the typical clinical course of acute posterior multifocal placoid pigment epitheliopathy with spontaneous resolution of posterior pole lesions and improvement in visual acuity from 20/60 to 20/20. The laboratory evaluation was remarkable for a rise in the anti-DNAse B antibody titer between initial and convalescent-phase serum samples, providing evidence of recent group A streptococcal infection. CONCLUSION: Although acute posterior multifocal placoid pigment epitheliopathy is often attributed to a postviral condition, this syndrome may also develop after an acute group A streptococcal infection.

Choroidal neovascularization secondary to Candida albicans chorioretinitis.

PURPOSE: To study the clinical histories and courses of six patients with choroidal neovascularization secondary to endogenous Candida albicans chorioretinitis. METHODS: The medical records, fundus photographs, and fluorescein angiograms of six patients who developed C. albicans chorioretinitis secondary to candidemia and who subsequently developed choroidal neovascularization in one or both eyes were reviewed. RESULTS: The six patients ranged in age from 18 to 79 years. Four were women and two men; all but one showed evidence of bilateral chorioretinal scarring secondary to C. albicans chorioretinitis. All patients had been treated successfully with systemic antifungal therapy (amphotericin B). Two weeks to two years after the chorioretinitis, choroidal neovascularization developed in one eye (four cases) or both eyes (two cases). The neovascularization on initial examination was subfoveal in four eyes, extrafoveal in three eyes, and juxtafoveal in one eye. Laser photocoagulation was used in four of the eight involved eyes. In these cases, the active choroidal neovascularization was brought under control. In one eye, the patient had submacular surgery for excision of the choroidal neovascular membrane. Final visual acuities ranged from 20/20 to 20/200 in treated eyes and from 20/50 to 20/400 in untreated eyes. CONCLUSION: Choroidal neovascularization is a potential cause of late visual loss in patients who have had C. albicans sepsis and endogenous C. albicans chorioretinitis. Eyes that have chorioretinal scarring from C. albicans chorioretinitis should be watched for the development of choroidal neovascularization. Laser photocoagulation or perhaps surgical excision of the neovascular complex may be of benefit in selected cases.

Microsporidial keratoconjunctivitis caused by Septata intestinalis in a patient with acquired immunodeficiency syndrome.

PURPOSE: To examine and treat a patient with acquired immunodeficiency syndrome (AIDS) who had mildly hyperemic conjunctiva and epithelial keratopathy in both eyes. METHODS: The patient underwent conjunctival biopsy. The specimen was examined by transmission electron microscopy. RESULTS: Septata intestinalis was demonstrated to be the cause of keratoconjunctivitis in the patient. The keratoconjunctivitis resolved after three weeks of therapy with topical fumagillin. No organisms were seen on repeat conjunctival biopsy. CONCLUSIONS: Microsporidial keratoconjunctivitis in patients with AIDS can be caused by S. intestinalis. This condition appears to respond to topical fumagillin.

Posterior scleritis with annular ciliochoroidal detachment.

PURPOSE: We studied an unusual case of posterior scleritis in a patient with sarcoidosis. METHOD: The medical record was reviewed for clinical manifestation, course, and tests performed, including laboratory evaluations, fluorescein angiography, and ultrasonography. RESULTS: The patient had posterior scleritis and unilateral angle-closure glaucoma caused by an annular ciliochoroidal detachment. Sarcoidosis was confirmed by biopsy of an enlarged parotid gland. CONCLUSION: The mechanism of angle-closure glaucoma may not be clinically or echographically apparent for a week or more in patients who develop annular ciliochoroidal detachment. We also found an unusual association of sarcoidosis and annular ciliochoroidal detachment secondary to posterior scleritis.

Corticosteroid therapy for optic disc neovascularization secondary to chronic uveitis.

PURPOSE: To report successful corticosteroid treatment of optic disc neovascularization associated with uveitis. METHODS: Retrospective review of medical records. RESULTS: Nine patients were identified with chronic uveitis and optic disc neovascularization without clinical or angiographic evidence of retinal ischemia. Ages ranged from 14 to 37 years (median age, 27). All patients were treated with either oral and/or subtenon's corticosteroids. Partial regression of the neovascularization was observed in all patients within 2 to 6 weeks (median, 5 weeks) after initiating treatment. Eight of nine patients had complete resolution of disc neovascularization at a median of 3 months (range, 2 to 42 months) after initiation of treatment and a median follow-up of 24 months (range, 7 to 144 months). Recurrence of disc neovascularization occurred in two patients, but it regressed again after further corticosteroid therapy. CONCLUSIONS: Optic disc neovascularization may occur in patients with chronic uveitis in the absence of retinal ischemia. This neovascularization can be successfully treated with corticosteroids.

Varicella-zoster virus retinitis in patients with the acquired immunodeficiency syndrome.

We examined five patients infected with the human immunodeficiency virus who developed a rapidly progressive necrotizing retinitis characterized by early patchy choroidal and deep retinal lesions and late diffuse thickening of the retina. In all but one case, the retinitis began in the posterior pole with little or no clinical evidence of vasculitis. All five patients had relentless progression of disease and were left with atrophic and necrotic retinae, pale optic-nerve heads, and narrowed vasculature. None of the patients developed aqueous or vitreal inflammation or retinal detachment. Clinical and laboratory evidence suggested that varicella-zoster virus was the causal agent in all five cases. First, the onset of retinitis in four cases either succeeded or was coincident with an eruption of dermatomal zoster. Second, varicella-zoster virus was cultured from the two chorioretinal specimens and varicella-zoster virus antigen was detected in the vitreal aspirate from one case. Third, by means of immunocytochemistry, varicella-zoster virus antigen was found in the outer retinae of both enucleation specimens. Fourth, viral capsids with the size and shape of herpesviridae were found in the outer retinae of both enucleation specimens. The clinical features observed in this study are distinct from those described for the acute retinal necrosis syndrome and appear to constitute a new and highly characteristic pattern of varicella-zoster virus-induced disease.

Haemophilus influenzae associated scleritis.

AIMS: To describe the clinical course and treatment of Haemophilus influenzae associated scleritis. METHODS: Retrospective case series. RESULTS: Three patients developed scleritis associated with ocular H influenzae infection. Past medical history, review of systems, and laboratory testing for underlying collagen vascular disorders were negative in two patients. One patient had arthritis associated with an antinuclear antibody titre of 1:160 and a Westergren erythrocyte sedimentation rate of 83 mm in the first hour. Each patient had ocular surgery more than 6 months before developing scleritis. Two had cataract extraction and one had strabismus surgery. Nodular abscesses associated with areas of scleral necrosis were present in each case. Culture of these abscesses revealed H influenzae in all patients. Treatments included topical, subconjunctival, and systemic antibiotics. Scleral inflammation resolved and visual acuity improved in each case. CONCLUSION: H influenzae infection may be associated with scleritis. Accurate diagnosis and treatment may preserve ocular integrity and good visual acuity.

Bacterial contamination of anaerobic vitreous cultures: using techniques employed for endophthalmitis.

PURPOSE: To investigate the occurrence of contaminated cultures of vitreous specimens from non-infected eyes obtained using anaerobic techniques employed for endophthalmitis. METHODS: Vitreous specimens were obtained using meticulous sterile techniques employed for endophthalmitis from seventeen patients undergoing pars plana vitrectomy for non-infective indications: vitreous hemorrhage (12 eyes), retinal detachment (3), Coat's disease (1), and congenital dislocated lens(1). Vitreous specimens were inoculated in the operating room onto an anaerobic blood agar plate and into thioglycolate broth. Bacterial growth occurring before 10 days was considered positive. RESULTS: Three organisms were isolated from three separate eyes. One colony of Staphylococcus species was isolated on an anaerobic blood agar plate on day 3. A single colony of Propionibacterium acnes grew on an anaerobic blood agar plate on day 6. Alpha-hemolytic streptococci grew from thioglycolate broth on day 10. CONCLUSIONS: Growth as detected in this study might represent contaminating rather than an infecting organism in an eye suspected of having endophthalmitis.

American uveitis society meeting october 31, 1994 san francisco, california.

1 Ophthalmic manifestations of presumed rifabutin-related uveitis. Arevalo JF, Freeman WR, La Jolla, CA, USA. 2 Diagnosis of toxoplasmosis acute anterior uveitis by PCR. Cano J, Diaz M, Navee A, Maldonado M, Barcelona, Spain. 3 Neuroretinits in patients with AIDS. Berger B, Austin TX, USA. 4 Presumed varicella zoster retinitis in a pediatric patient with AIDS. El Baba F, Nachman S, Stony Brook, NY, USA. 5 ARN with hypopyon caused by EVB and herpes type VI viruses. Cano J, Diaz M, Navea A, Maldonado MJ, Barcelona, Spain. 6 CD8(+) T-lymphocytes and ocular infections in HIV(+) patients. Lowder CY, Butler CP, Dodds EM, Recillas-Gispert C, Cleveland, OH, USA. 7 Intravitreal foscanet for persistent CMV. Lieberman RM, Orellana J, New York, NY, USA. 8 Perfluorocarbon liquid versus air-fluid exchange during surgical repair of retinal detachment caused by cytomegalovirus retinitis in patients with AIDS. Sery T, Gomes J, Sando R, Dua H, Donoso L, Vrabec T, Philadelphia, PA, USA. 9 Endogenous ophthalmitis simulating retinoblastoma: a report of six cases. Shields J, Shields C, Eagle R, Barrett J, DePotter P, Philadelphia, PA, USA. 10 Ocular lymphoma resembling chronic postoperative endophthalmitis. Fox G, Chan CC, Whitcup SM, Nussenblatt R, Bethesda, MD, USA. 11 A phase II trial of combination chemotherapy for primary central nervous system lymphoma. Whitcup SM, Stark-Vanes V, Nussenblatt RB, Heiss H, Witte R, Bethesda, MD, USA. 12 Cancer-induced autoimmune retinopathy. Thirkill C, Sacramento, CA, USA. 13 Leukocytoclastic vasculitis. Tessler H. Chicago, IL, USA. 14 Bilateral choroidal neovascular membranes after Candida albicans chorioretinitis. Dodds E, Townsend-Pico W, Lowder CY, Lewis H, Cleveland, OH, USA. 15 An unusual complications of Toxoplasma retinochoroiditis. Gormley PD, Flaxel CJ, Pavesio CE, Conrad DK, Lightman S, London, UK. 16 Surgical removal of a choroidal neovascular membrane in sympahtetic ophthalmia. Conrad DK, McCluskey PJ, Schwartz S, Gregor Z. Lightman S, London, UK. 17 Peripheral laser scatter ablation in pars planitia. Park SE, Mieler WF, Pulido JS, Milwaukee, WI, USA.