Syphilitic posterior uveitis: correlative findings and significance.

Twenty-one patients with syphilitic posterior uveitis were investigated retrospectively to study the disease spectrum, associations with neurosyphilis, and therapeutic implications. Ophthalmologic manifestations of syphilitic posterior uveitis are differentiated into acute and chronic uveitides. The several distinct acute uveitic syndromes are usually florid and are associated with early syphilis, with VDRL-positive syphilitic meningitis, and frequently with human immunodeficiency virus coinfection. The chronic posterior uveitides are often insidious, a manifestation of late syphilis, and associated commonly with subclinical neurosyphilis. All patients with acute cases and 54% of patients with chronic cases in our study received penicillin therapy appropriate for neurosyphilis. The frequent association of syphilitic posterior uveitis with neurosyphilis and the analogous spirochetal sequestration beyond the blood-brain and the blood-ocular barriers suggest that all patients with syphilitic posterior uveitis, irrespective of ocular disease intensity, should undergo evaluation of cerebrospinal fluid and be treated with penicillin regimens appropriate for neurosyphilis.

Posterior uveitis in patients with positive serology for syphilis.

The clinical features and ophthalmologic findings of 20 patients with syphilitic posterior uveitis seen at the Detroit Medical Center from November 1993 through February 1996 were reviewed. The mean age was 58 years; 8 patients were male and 12 were female; and all patients were black. Three of 9 patients tested were HIV positive. Patients were divided into 2 groups: those with acute (8) and those with chronic (12) syphilitic posterior uveitis. Chorioretinitis was the predominant uveitic pattern (15/20). Eighteen patients presented with blurred vision. All patients had reactive serum fluorescent treponemal antibody, absorbed (FTA-ABS); 3 had nonreactive rapid plasma reagin (RPR). Mean RPR titer in the chronic uveitis group and in the acute uveitis group was 1:27.3 and 1:209.8, respectively. Seven patients had abnormal cerebrospinal fluid (CSF); CSF VDRL was reactive in 2 patients. All patients were treated with intravenous penicillin G. Eight of 14 patients seen at follow-up showed improvement of ophthalmologic findings. Syphilis should be considered in the differential diagnosis of posterior uveitis.

Ocular manifestations of cat-scratch disease.

Bartonella henselae has only recently been isolated, characterized, and found to be the principal cause of cat-scratch disease (CSD). The availability of specific serologic investigations has allowed the recognition of a spectrum of ocular CSD syndromes that previously were ill defined and considered idiopathic. The primary inoculation complex causing regional lymphadenopathy is represented in the eye by Parinaud's oculoglandular syndrome; B. henselae is the most common cause. Leber's neuroretinitis has been identified for 80 years, and new data suggest that it is commonly a manifestation of CSD; the extent of the association remains to be determined. CSD optic neuritis is also described. The vitreoretinal manifestations include anterior uveitis, vitritis, pars planitis, focal retinal vasculitis, a characteristic retinal white spot syndrome, Bartonella retinitis, branch retinal arteriolar or venular occlusions, focal choroiditis, serous retinal detachments, and peripapillary angiomatous lesions. The pattern of ocular disease in AIDS-associated B. henselae infections is poorly delineated; unusual manifestations include conjunctival and retinal bacillary angiomatosis. The benefit of antimicrobial therapy for CSD in immunocompetent individuals has been difficult to establish, partly because most infections are self limited. Empirically, azithromycin, ciprofloxacin, rifampin, parenteral gentamicin, or trimethoprim-sulfamethoxazole provide the best therapeutic choices to minimize damage to the eye.

Retinal and choroidal manifestations of cat-scratch disease.

OBJECTIVE: The ability to diagnose cat-scratch disease (CSD) has been facilitated greatly by the recent isolation and characterization of Bartonella henselae (formerly genus Rochalimaea) and Afipia felis and by the subsequent development of specific enzyme-linked immunosorbent assay (ELISA) serologic tests. This study will help define the patterns of posterior segment ocular involvement in patients with confirmed CSD. DESIGN: The study design is a retrospective case study and literature review. PARTICIPANTS: Two consecutive patients with acute visual loss from retinal manifestations of CSD participated. INTERVENTIONS: The diagnosis was confirmed by B. henselae ELISA testing. Patients underwent extensive medical and ophthalmic investigations to exclude other causes of retinal and choroidal disease. Ophthalmic investigation included fluorescein angiography and visual field testing. One patient received antibiotic therapy with cefotaxime, then with ciprofloxacin, and was treated with oral prednisone. The other patient was improving for several weeks before oral doxycycline was given. MAIN OUTCOME MEASURES: The clinical syndromes observed were studied over time using visual acuity, visual field, and clinical findings. Data were collated with cases from the literature. RESULTS: Unilateral neuroretinitis and an unusual macular retinitis developed in patient 1, as did bilateral small intraretinal white spots and a unilateral choroidal infiltrate that continued to develop while the patient received antibiotic treatment. Patient 2 had a branch arteriolar occlusion in relation to a perivascular retinal infiltrate and a few small, bilateral, intraretinal white spots. There was gradual resolution with visual improvement while the patient received the antibiotic treatment, although therapeutic efficacy could not be determined. Patient 1 also received oral corticosteroids. A detailed analysis of the literature placed these findings in context. CONCLUSIONS: An unusual, well-defined retinal opacification with features of both multiple retinal arteriolar occlusions and a low-grade retinitis was described. Several features also may occur in posterior segment CSD, including neuroretinitis, a retinal white spot syndrome, and focal choroiditis.

Rapidly progressive herpetic retinal necrosis: a blinding disease characteristic of advanced AIDS.

Eleven patients with rapidly progressive herpetic retinal necrosis (RPHRN) complicating AIDS were investigated retrospectively to study the disease spectrum, systemic involvement, and therapy. The mean CD4 cell count was 24/microL. There was a characteristic disease pattern with rapid progression, 82% bilaterality, relative resistance to intravenous antiviral therapy, and 70% retinal detachment. Varicella-zoster virus was the probable cause in 10 patients (detected by polymerase chain reaction in two eyes investigated), and herpes simplex virus was the probable cause in one. Cutaneous zoster occurred previously in 73% but was not concurrent. Seventy-three percent had central nervous system disease, possibly virus-related. RPHRN may be a local herpetic recrudescence in an immune-privileged site with transneural spread. Only four of 20 affected eyes retained useful vision. Poor ocular bioavailability, retinal ischemia, acquired drug resistance, and strain pathogenicity may underlie treatment failure. Acyclovir therapy appears relatively ineffective. Combined intravenous and intravitreal therapy with foscarnet and ganciclovir may be the best current management. Research advances are needed urgently.

Chronic Propionibacterium acnes endophthalmitis as a cause of intermediate uveitis.

Although the pathogenesis in most cases of intermediate uveitis is unknown, a small minority of cases is associated with a variety of specific inflammatory etiologies: sarcoidosis; multiple sclerosis; Lyme disease; syphilis; ocular lymphoma; and as a rare manifestation of Behçet's disease and AIDS. A 61-year-old woman developed pars planitis after cataract surgery. A vitrectomy was performed after ten months when a white capsular plaque and an hypopyon developed. Propionibacterium acnes was isolated. The intermediate uveitis was not controlled until later removal of the intraocular lens and capsular remnants. Chronic propionibacterial endophthalmitis may be a cause of intermediate uveitis.

AIDS-associated intraocular lymphoma causing primary retinal vasculitis.

BACKGROUND: There is an increasing association between ocular-central nervous system (CNS) lymphoma and the acquired immunodeficiency syndrome (AIDS). In this population, the disease generally occurs in a younger age group. The origin of these B-cell neoplasms remains unknown. METHOD: Case study of a 26-year-old AIDS patient with an incidental finding of localized retinal vasculitis and local vitritis. Disease progression and the failure of antiviral therapy led to early diagnostic vitrectomy with vitreal and retinal biopsy. Cerebrospinal fluid (CSF) evaluation, head magnetic resonance imaging (MRI), and brain biopsy were performed. RESULTS: The diagnosis of ocular non-Hodgkin lymphoma was made by vitreous cytology. Serial sectioning of a retinal biopsy showed no retinal neoplastic infiltration, only perivascular inflammatory cells. CSF was normal. MRI showed multicentric brain mass lesions that enhanced with intravenous contrast. Brain biopsy revealed an immunoblastic, angiocentric, B-cell non-Hodgkin lymphoma. The patient died within two months, despite cerebral and ocular irradiation. CONCLUSIONS: The segmentally localized retinal vasculitis-vitritis and absence of retinal infiltration suggested early primary involvement of the vitreous. Coarse perivascular sheathing is characteristic of the mixed retinal vasculitis in this disease. The diagnosis of ocular-CNS lymphoma must be considered in patients with AIDS, however young, with unexplained posterior segment inflammatory disease or subretinal masses.

Ophthalmologic manifestations of acquired immune deficiency syndrome-associated progressive multifocal leukoencephalopathy.

PURPOSE: Progressive multifocal leukoencephalopathy (PML) is increasingly described as a late complication of the acquired immune deficiency syndrome (AIDS). The purpose of this study is to evaluate retrospectively the ophthalmologic, clinical, and investigational aspects of AIDS-associated PML. METHODS: The authors evaluated ten patients in whom ophthalmologic manifestations developed in the course of AIDS-associated PML. Findings at clinical examination and their progression over time, neuroimaging correlates, the results of pathologic investigation, and visual outcomes were reviewed. RESULTS: Progressive multifocal leukoencephalopathy was the AIDS-defining illness in six of ten patients. Homonymous visual field defects were the presenting symptom in three patients and detected in six patients overall. Occipital blindness developed in one patient. Cerebellar signs and brain stem nuclear and supranuclear palsies also were common. Confluent white matter lesions with increased intensity on T2-weighted magnetic resonance imaging were supratentorial in seven patients and infratentorial in three patients. With incomplete data, the median survival time was 3 months from PML onset. Histopathologic confirmation of PML diagnosis was available for nine of the ten patients. CONCLUSIONS: The development of progressive retrochiasmal visual field defects, supranuclear and nuclear cranial nerve palsies, or nystagmus ataxia in the relatively young patient should alert the ophthalmologist to the possibility of PML, particularly in the presence of long-tract central nervous system signs or dementia. Progressive multifocal leukoencephalopathy will often be human immunodeficiency virus associated. Human immunodeficiency virus encephalopathy, cerebral toxoplasmosis, lymphoma, and infarction need to be discriminated. Effective therapy is required urgently for this devastating disease.

Dynamics of external ocular blood flow studied by scanning angiographic microscopy.

The scanning angiographic microscope (SAM) provides a solution to the considerable technical difficulties associated with conventional episcleral fluorescein angiography. Standardised anterior segment fluorescein videoangiograms were performed using the SAM in each episcleral quadrant of the right eye in 6 normal subjects; frame-by-frame analysis proved important. Centripetal flow was seen in all 37 scleral perforating arteries investigated. Other features were the marked individual variability, much larger vertical anterior ciliary arteries, the high frequency of arteriovenous anastomoses, the complex flow patterns, the absence of a 'watershed' zone between anterior ciliary and posterior episcleral circulations, a characteristic and discontinuous distribution of 'leaky' episcleral veins, and the primacy of venous drainage into the plexus of muscular veins. Reports of retrograde blood flow in the anterior ciliary arteries in most fluorescein angiographic studies are probably incorrect, the result of unappreciated methodological problems. The SAM is an important advance on previous anterior segment fluorescein angiography techniques.

Long-term outcomes after the surgical removal of advanced subfoveal neovascular membranes in age-related macular degeneration.

BACKGROUND: The poor results of laser photocoagulation in patients with age-related macular degeneration who have subfoveal neovascular membranes, as reported by the Macular Photocoagulation Study Group, have posed the question as to whether the surgical removal of the neovascular membranes by subfoveal surgery might provide superior functional results, possibly in subgroups of patients. METHODS: The authors' first ten patients treated by subfoveal surgery were followed prospectively. Follow-up of a mean duration of 2 years is presented, with particular emphasis on visual and anatomic outcomes. Preoperative subfoveal choroidal neovascular membranes and postoperative retinal pigment epithelial defects were measured using digitized planimetry. RESULTS: Initial visual acuities were equal to or less than 20/400, with a mean duration of visual loss of 8 months. The mean choroidal neovascular membrane size was 7 disc areas. Eight of ten patients improved one to two lines of Snellen visual acuity postoperatively. One patient achieved visual acuity of 20/60 at 15 months before declining because of recurrent neovascularization. Surgically induced retinal pigment epithelial defects were invariable; the mean defect was 14 standard disc areas in size. Choriocapillaris atrophy and focal losses of deeper choroidal tissue also occurred. Surgical complications were frequent but responded to routine management. The authors observe a 2-year recurrence rate of 40%. Recurrences often are atypical, fibrous, and poorly vascularized. CONCLUSIONS: Although substantial visual improvements are common, long-term reading vision has not been achieved. Retinal pigment epithelial incorporation into late subfoveal membranes remains a major limiting factor. The role of early surgery and the role of surgery for patient subgroups need to be compared directly with the results of foveal laser treatment, using several visual outcomes.

Serious occurrences in the natural history of advanced climatic keratopathy.

BACKGROUND: Climatic or chronic actinic keratopathy is an important corneal degeneration occurring after prolonged climatic exposure. The advanced stages of disease are confined generally to tropical or arid localities (including the Arctic) with high levels of sunlight. After many years of disease evolution, the advent of stage 3 keratopathy often presages a rapid downhill course. The instability of advanced climatic keratopathy has received little attention. METHODS: Eighteen patients with advanced climatic keratopathy are described from the Transvaal region in South Africa and from Saudi Arabia. Patients with rapid disease progression, spontaneous sterile ulceration, and secondary microbial keratitis are described. RESULTS: The rapid progression characteristic of stage 3 climatic keratopathy is illustrated. Severe, focal, sterile ulceration of the devitalized corneal degeneration may be common. Secondary infection may occur, leading to rapid dissolution of the climatic keratopathy material. Corneal perforation may ensue. The occurrence of yellow or brown fragments of the climatic keratopathy within or adjacent to the corneal inflammatory infiltrate indicates the predisposing cause of the infection, as usually also with examination of the opposite eye. CONCLUSIONS: These observations emphasize the inherent instability of advanced climatic keratopathy, which frequently takes a relentless downhill course. In rural populations of the developing world, climatic keratopathy is an important cause of blindness. Disease pathogenesis, treatment, and prevention deserve greater study.

Endophthalmitis caused by the coagulase-negative staphylococci. 1. Disease spectrum and outcome.

PURPOSE: The coagulase-negative staphylococci are the most common causes of postoperative endophthalmitis. This study investigates the variability in the disease spectrum and visual outcome of coagulase-negative staphylococcal endophthalmitis in a large, single-center series. METHODS: Ninety consecutive cases of coagulase-negative staphylococcal endophthalmitis were investigated retrospectively from two time periods, 1978 to 1982 and 1985 to 1987, separated by a transitional period in cataract surgery technique. Using a detailed protocol, inpatient, outpatient, and microbiologic records were analyzed. Six-month visual acuity results were obtained. RESULTS: Diagnosis frequently was delayed, often suspected only after hypopyon development. Thirty-seven percent of patients presented more than 1 week after the inoculating event, and 13% presented after more than 1 month. Variable asymptomatic intervals and gradually worsening inflammatory prodromes are noted. Painless endophthalmitis occurred in 16%. Non-epidermidis infections comprised 28%. With vitrectomy/intraocular antibiotic management, 38% and 68% achieved visual acuities of 20/50 and 20/400, respectively. Overall, 10% of patients developed late retinal detachments. This occurred in only 4% of patients, with endophthalmitis occurring after cataract surgery. CONCLUSION: Ophthalmologists should become familiar with the emerging concepts of delayed-onset, chronic, and often painless endophthalmitis in which the coagulase-negative staphylococci play a prominent role.

Endophthalmitis caused by the coagulase-negative staphylococci. 2. Factors influencing presentation after cataract surgery.

PURPOSE: This study, comprising 60 patients with coagulase-negative staphylococcal endophthalmitis which occurred after cataract surgery, was designed to define the variation in disease presentation and visual outcome and to evaluate statistically the role of the primary surgery and its management. METHODS: An intensive evaluation of microbiological, inpatient, outpatient, and cataract surgery charts was made retrospectively using a standardized protocol. The predictive value of surgical, iatrogenic, and clinical factors was analyzed for their influence on defined aspects of the disease pattern and of the visual results using multiple regression models, via a stepwise technique. RESULTS: There was commonly a significant asymptomatic latent period after cataract surgery. The median diagnostic delay was 7 days; 22% of patients presented after 2 weeks and 12% after 1 month. Symptoms progressed longer than 3 days in 25% of patients. Ten percent had no pain. Clinical variation proved largely unrelated to cataract surgery events and postoperative management; bacterial factors were implicated. Good visual outcome was associated statistically with intensive topical corticosteroid in the symptomatic period, but was negatively associated with operative subconjunctival corticosteroid. CONCLUSIONS: The clinical variation in cases of postoperative coagulase-negative staphylococcal endophthalmitis poses particular problems for diagnosis in the outpatient setting. Surgical and perioperative events (except corticosteroid use) probably can be disregarded in studies of endophthalmitis management.

Scleral flap necrosis and infectious endophthalmitis after cataract surgery with a scleral tunnel incision.

BACKGROUND: Long scleral tunnel dissection techniques have been developed for cataract surgery incisions. These incisions reduce postoperative astigmatism and keratorefractive instability. If fashioned correctly, the internal lip of the incision produces a tight seal to the anterior chamber, permissible of sutureless surgery. The behavior of such a wound during intraocular infection is unknown. METHODS: The authors describe two elderly patients in whom postoperative bacterial endophthalmitis was accompanied by infectious scleritis, infectious sclerokeratitis or keratitis, and rapid scleral flap necrosis. One of the patients had a painless disease process. RESULTS: Intraocular isolates of Staphylococcus aureus and Streptococcus equinus were recovered from the two patients, respectively. Management was complicated by loss of tectonic integrity that followed scleral flap necrosis, by impaired vitreoretinal visualization associated with rapidly progressive sclerokeratitis or keratitis, and by bacterial scleritis in the base of the scleral flap. No light perception was retained in either eye. CONCLUSION: Scleral tunnel incisions create a potential abscess cavity. Although a rare occurrence, postoperative endophthalmitis in such an eye may present major surgical and therapeutic problems intrinsic to the wound design.

Eicosanoid modulation and epithelial wound healing kinetics of the alkali-burned cornea.

The cyclooxygenase and lipoxygenase enzyme systems can metabolize a number of C20 polyunsaturated fatty acids. Although arachidonic acid is the usual substrate for these pathways, the eicosanoid precursor pool can be nutritionally manipulated by supplementation with alternative precursors, often generating less active or down-regulatory metabolic products. Prefeeding with gamma-linolenic acid, eicosapentaenoic acid, or a combination of both failed to influence the lagphase, epithelial migration rate, or epithelial wound healing kinetics after either 1N or 4N NaOH alkali burning of the rabbit cornea. Initial epithelial wound healing probably does not involve eicosanoid-mediated processes. Essentially linear epithelial healing kinetics continued to closure without a late decrease in wound edge velocity.

Management of the hypotonous cyclodialysis cleft.

Authoritative experience in the management of hypotonous cyclodialysis clefts is difficult to obtain because of their rarity. In this study, the authors describe nine patients with hypotonous cyclodialyses clefts: six patients were treated successfully with argon laser photocoagulation to the cleft surfaces; in one patient, cleft diathermy was used to seal an incompletely closed cleft after a single argon laser treatment; one patient responded to conservative management; another patient did not require treatment. Four cases followed ocular trauma and five occurred after extracapsular cataract extraction and posterior chamber intraocular lens implantation. In three patients, the anterior chamber was too shallow to permit gonioscopy; sodium hyaluronate (Healon) was used to reform the anterior chamber, to delineate the extent of the cyclodialysis cleft, and to provide maximal access for the argon laser treatment. In another patient, laser cleft consolidation was successful only after fully opening the cleft with sodium hyaluronate. The evolution of the laser photocoagulation technique used by the authors is described. Laser cyclodialysis cleft consolidation can be repeated easily and safely. The authors recommend argon laser photocoagulation as the primary management approach. Intracameral viscoelastic agents are useful adjuncts. The complications of cleft lasering are minor, although a hypertensive episode commonly occurs in the early postoperative period as the cleft closes. Major intraocular surgeries usually can be avoided.

Infectious crystalline keratopathy. Role of nutritionally variant streptococci and other bacterial factors.

Infectious crystalline keratopathy (ICK) is a chronic corneal infection characterized by interlamellar plaques of gram-positive coccal bacteria in the absence of inflammatory cells. It generally occurs within a corneal graft. Viridans streptococci are usually isolated, but the clinical response to antibiotics is poor and disparate with the in vitro antimicrobial sensitivities. These features suggest the possibility of unusual bacterial factors in pathogenesis. Four cases caused by nutritionally variant viridans streptococci are described. The organisms were fully characterized. They have a rare nutritional requirement for pyridoxal and require defined culture conditions and specific identification. Nutritional variant streptococci (NVS) are principally described as causing endocarditis, another infection involving an avascular collagenous tissue, and exhibiting similar biologic behavior. Electronmicrographic evidence is also adduced that suggests the possible importance of intracorneal glycocalyx deposition. Such factors might explain the anomalous clinical characteristics of this condition.

Practical anterior segment fluorescein angiography.

Anterior segment fluorescein angiography is a useful investigative technique for diagnosing and assessing anterior segment disease. However, a number of technical problems are inherent in current methodology, such as confounding reflexes, off-axis illumination, and shallow depth of field. We developed a simple technique using readily available equipment, polarized light, and an easy-to-construct auxiliary bracket to provide an inexpensive and optimal photographic system for anterior segment fluorescein angiography.

Effects of altering the eicosanoid precursor pool on neovascularization and inflammation in the alkali-burned rabbit cornea.

The effects of altering the eicosanoid precursor pool on several aspects of the nonimmunologically mediated inflammatory and angiogenic processes that follow 1N and 4N NaOH alkali burning of the rabbit cornea were compared with controls. Diets were supplemented with several dosages of oils containing either gamma-linolenic acid (GLA) (borage oil), eicosapentaenoic acid (EPA) (sardine oil), or a combination of the two in a dose-response protocol. Significant changes in serum fatty acid composition were demonstrated. Gamma-linolenic acid proved consistently superior to EPA in modulating the neovascular response judged by three neovascular indices. At 14 days, GLA significantly reduced the polymorphonuclear leukocyte and macrophage inflammatory infiltrate and EPA reduced the macrophage component, both with high dose; EPA also reduced keratocyte proliferation. Wound-healing parameters were unaffected. Evidence for GLA-EPA synergism was modest. Prolonged neovascular responses and chronic inflammation occurring in the clinically relevant context of severe structural damage can be modulated by nutritional alteration of the eicosanoid precursor pool.