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Hypertensive retinopathy in the newborn infant.

Hypertension (blood pressure greater than 70 mm Hg on three days) was noted in 1.2% of all neonatal admissions to the intensive and intermediate care nurseries. During this investigation of neonatal hypertension, a retinopathy identical to that seen in hypertensive adults was noted. Indirect ophthalmoscopy was performed in 21 neonates with elevated blood pressure. Eleven of these patients demonstrated some or all of the following abnormalities: increased ratio of venous to arterial caliber, vascular tortuosity (including arteriovenous crossing changes), superficial and deep hemorrhages, and exudates. These findings appeared to resolve after control of the hypertension. The specificity of this constellation of lesions for hypertensive retinopathy and their possible clinical significance are explained in light of what is known about hypertensive retinopathy in adults.

Exudates and Transudates↗

Binocular vertical diplopia.

The assessment of a patient with binocular vertical diplopia begins with a thorough history and neuro-ophthalmologic examination. The neuro-ophthalmologic examination includes observation for a compensatory head, face, or chin position; ocular ductions and versions in the nine cardinal positions of gaze; the three-step test; the double Maddox rod test; indirect ophthalmoscopy to observe the location of the fovea in relationship to the optic nerve head to determine cyclodeviation; and the forced ductions test. Binocular vertical diplopia may be due to supranuclear processes, ocular motor nerve dysfunction, neuromuscular junction disease, diseases of eye muscle, mechanical processes causing vertical eye misalignment, and even retinal disease. In this article, the differential diagnosis of these processes is outlined.

Diplopia↗

Endogenous uveitis: current concepts of treatment.

The medical treatment of patients with uveitis often necessitates the use of systemically administered drugs, including corticosteroids, cytotoxic agents, and cyclosporine. Because of the potential side effects of these medications, physicians unfamiliar with inflammatory ocular disease may be asked to participate in the care of these patients. The most important criteria on which to base the decisions of when and how to treat patients with uveitis are the site and severity of the inflammation, the degree of visual acuity, and the potential for restoration of vision with treatment. Slit-lamp examination, indirect ophthalmoscopy, and fluorescein angiography are useful for evaluating the loss of vision in patients with endogenous uveitis. The main goal of treatment is to prevent permanent scarring and deterioration of vision as a result of the disease or the drug side effects. Frequent reassessment will help ensure adequate treatment and will minimize adverse drug effects.

Adrenal Cortex Hormones↗

Ophthalmoscopic evaluation of the optic nerve head.

Optic nerve diseases, such as the glaucomas, lead to changes in the intrapapillary and parapapillary region of the optic nerve head. These changes can be described by the following variables: size and shape of the optic disk; size, shape, and pallor of the neuroretinal rim; size of the optic cup in relation to the area of the disk; configuration and depth of the optic cup; ratios of cup-to-disk diameter and cup-to-disk area; position of the exit of the central retinal vessel trunk on the lamina cribrosa surface; presence and location of splinter-shaped hemorrhages; occurrence, size, configuration, and location of parapapillary chorioretinal atrophy; diffuse and/or focal decrease of the diameter of the retinal arterioles; and visibility of the retinal nerve fiber layer (RNFL). These variables can be assessed semiquantitively by ophthalmoscopy without applying sophisticated techniques. For the early detection of glaucomatous optic nerve damage in ocular hypertensive eyes before the development of visual field loss, the most important variables are neuroretinal rim shape, optic cup size in relation to optic disk size, diffusely or segmentally decreased visibility of the RNFL, occurrence of localized RNFL defects, and presence of disk hemorrhages.

Anthropometry↗

Microperimetry of localized retinal nerve fiber layer defects.

The aim of this study was to determine the sensitivity of retinal areas involved in a localized retinal nerve fiber layer (RNFL) defect and to assess correlations between microperimetry and the standard full threshold central 30 deg visual field test. Twenty-five patients with focal RNFL defects, evaluated by means of Argon-blue scanning laser ophthalmoscopy (SLO), underwent an automated 30 deg central visual field examination and a microperimetry with SLO. Microperimetry was performed according to standard procedures (infrared laser for fundus imaging; HeNe laser for 10 candles/m2 background illumination, fixation aid and generation of stimuli; manual fundus tracking). The size of stimuli was Goldmann III with 0.1 sec duration. In eyes with focal RNFL defects a deep microperimetric scotoma of at least 5 dB was found in 12 cases and a mild scotoma (1-4 dB) in 13 cases. These scotomas were mainly located throughout the whole defect or grouped in the temporal or nasal sides of the defect and were characterized by sharp and well-defined borders. With automated perimetry, a scotoma, defined by a single point depression of at least 10 dB or a depression of at least 5 dB in two or more contiguous points corresponding to the RNFL, defect, was found in only 14 out of 25 eyes with microperimetric defect. Focal RNFL defects correspond to localized areas of depressed retinal sensitivity as evaluated by microperimetry. The close correspondence between structural and microperimetric findings suggests that, in hypertensive eyes also, localized RNFL defects correspond to visual dysfunction possibly associated with substantial atrophy of ganglion cells.

Adult↗

Frequency of choroidal abnormalities in neurofibromatosis type 1.

BACKGROUND: Choroidal neurofibromatosis is thought to be a rare form of neurofibromatosis that involves the eyes. The development of infrared light examination with a scanning laser ophthalmoscope (SLO) and indocyanine-green fundus angiography has allowed examination of the choroid. We studied choroidal abnormalities in patients with neurofibromatosis 1 and compared their frequency with that of other ocular abnormalities. METHODS: We examined 33 eyes of 17 consecutive patients diagnosed with neurofibromatosis 1 by conventional ophthalmoscopy and by non-invasive infrared monochromatic light with confocal SLO. 76 eyes of 39 age-matched controls were examined similarly by confocal SLO. 21 digital fluorescein and indocyanine-green angiographies were obtained from 11 adult patients, and 77 angiograms were obtained from age-matched controls. FINDINGS: Infrared monochromatic light examination by confocal SLO showed bright multiple patchy regions at and around the entire posterior pole of all 33 eyes examined. All bright patchy regions seen in adult patients corresponded to hypofluorescent areas on their indocyanine-green angiograms. However, no abnormalities were noted in any patient at corresponding areas under conventional ophthalmoscopic examination or fluorescein angiography. In SLO and indocyanine-green studies, controls and control angiograms showed no choroidal abnormalities. Iris nodules were noted in 25 eyes (76%) of 14 patients (82%) and eyelid neurofibroma in five patients (29%). INTERPRETATION: The bright patchy regions noted under infrared fundus examination and the corresponding hypofluorescent areas seen on indocyanine-green angiograms are probably of choroidal origin. The high frequency (100%) of these abnormalities suggests that the choroid is one of the structures most commonly affected by neurofibromatosis 1.

Adult↗

Ophthalmoscopic detectability of the parafoveal annular reflex in the evaluation of the optic nerve: an experimental study in rhesus monkeys.

OBJECTIVE: To evaluate whether assessment of the ophthalmoscopic visibility of the parafoveal annular reflex is helpful in the detection of optic nerve fiber and optic nerve damage. DESIGN: Experimental animal study. ANIMALS: The study was performed in rhesus monkeys, divided into four study groups, with no significant difference in ages of the animals in the various groups. Three of these groups had (1) experimental chronic high-pressure glaucoma (n = 37 monkeys), (2) experimental temporary occlusion of the central retinal artery (n = 19 monkeys), and (3) systemic atherosclerosis and arterial hypertension (n = 13 monkeys). The fourth group was the normal control group (n = 10 monkeys). METHODS: In 60-degree color fundus photographs, taken at baseline and at the end of the study, the ophthalmoscopic detectability of the parafoveal annular reflex was graded, the visibility of the retinal nerve fiber layer was assessed, and neuroretinal rim and parapapillary atrophy were measured. MAIN OUTCOME MEASURES: Ophthalmoscopic detectability of the parafoveal annular reflex; visibility of the retinal nerve fiber layer; neuroretinal rim area; size of parapapillary atrophy. RESULTS: The parafoveal annular reflex was significantly (P < 0.0001) better detectable at baseline of the study than at the end of the study. In all study groups, detectability of the parafoveal annular reflex decreased significantly (P < 0.0001) with decreasing visibility of the retinal nerve fiber layer. In the glaucoma group, parafoveal annular reflex detectability additionally decreased significantly (P < 0.001) with increasing area of beta zone of parapapillary atrophy and with decreasing neuroretinal rim area. In the normal group, the parafoveal annular reflex detectability decreased with increasing age, parallel to a loss in the visibility of the retinal nerve fiber layer. In 23 of 25 (92%) eyes with a complete loss of the visibility of the retinal nerve fiber layer at the end of the study, the parafoveal annular reflex was no longer detectable. CONCLUSIONS: The findings suggest that evaluation of the detectability of the parafoveal annular reflex in routine ophthalmoscopy is a useful additional tool in the qualitative assessment of the retinal nerve fiber layer and optic nerve.

Animals↗

Ophthalmologic findings in Cohen syndrome. A long-term follow-up.

OBJECTIVE: To determine the nature and course of ophthalmologic abnormalities and their clinical significance in Cohen syndrome. STUDY DESIGN: Observational case series. PARTICIPANTS: Twenty-two Cohen syndrome patients aged 2 to 57 years were examined, and a retrospective review of ophthalmologic records was carried out for 14 of them. All but one were part of the Finnish study of refined mapping of the Cohen syndrome gene by linkage disequilibrium in chromosome 8. MAIN OUTCOME MEASURES: Visual acuity (VA), cycloplegic refraction, biomicroscopy, lens opacitometry, ophthalmoscopy, and fundus photography. RESULTS: With the exception of the two youngest patients, all had symptoms such as nyctalopia, impaired vision, and visual field loss. Progressive, often high-grade myopia, astigmatism, and retinochoroidal dystrophy resembling retinitis pigmentosa occurred in all, except for the youngest patients. The earliest fundus changes were pale disc and pale fundus with or without pigment granularity, followed by narrowed vessels, pigment clumps, and bone spiculelike pigment accumulations by 10 to 20 years of age. Pigment deposits increased and approached the posterior pole by 35 to 40 years of age. Patients more than 45 years of age had severe retinochoroidal atrophy. A bull's-eye macula was seen in most patients. Teenagers had peripheral lens opacities, and young adults had early nuclear sclerosis confirmed by lens opacitometry. Older patients also had posterior subcapsular cataracts, iris atrophy, and iridophacodonesis. Vision started to deteriorate at the age of 6 to 10 years, but remained relatively good (VA 0.5-0.1) in most patients until 30 and, in one case, 46 years of age. Older patients were severely visually handicapped (VA hand motion to light perception), but none were completely blind. CONCLUSIONS: Progressive myopia and retinochoroidal dystrophy are essential features in Cohen syndrome and, together with early lens opacities, lead to deterioration of vision. Cohen syndrome patients need careful ophthalmologic follow-up at all ages. Nyctalopia and restricted visual fields should be considered when planning the patient's daily activities.

Adolescent↗

Incidence and rate of disappearance of retinal hemorrhage in newborns.

OBJECTIVE: To determine the prevalance, associated biometric factors, and rate of disappearance of neonatal retinal hemorrhage. DESIGN: Cross-sectional and natural history study. METHODS: Healthy newborns (n = 149) at an urban hospital were examined using indirect ophthalmoscopy within 30 hours of birth. Newborns with retinal hemorrhage were reexamined biweekly until hemorrhage resolved. MAIN OUTCOME MEASURES: Neonatal and maternal biometric factors, and incidence and rate of resolution of retinal hemorrhage. RESULTS: Intraretinal hemorrhage was present in 34% of newborns and varied from a single dot hemorrhage in one eye to bilateral widespread hemorrhages, occasionally with white centers. The incidence of hemorrhage was higher for vacuum-assisted (75%) than for spontaneous vaginal deliveries (33%) and was least for infants delivered by cesarean section (7%). The mean maternal age was greater for infants with retinal hemorrhage. By 2 weeks after birth, retinal hemorrhage resolved in 86% of eyes, and at 4 weeks no intraretinal hemorrhage was detected, although a single subretinal hemorrhage persisted until 6 weeks after birth. CONCLUSIONS: Intraretinal hemorrhages are common in the immediate postnatal period and resolve by 1 month of age. Retinal hemorrhage in infants older than 1 month should heighten suspicion that the hemorrhage is associated with factors other than birth.

Connecticut↗

Ocular manifestations of tuberculosis.

OBJECTIVE: To present the clinical and histopathologic findings in five cases of tuberculosis (TB) with various ocular manifestations. DESIGN: Observational case series. METHODS: Retrospective review of clinical findings, course, and treatment of five patients. Diagnostic techniques, including biomicroscopic, histopathologic, and molecular biologic test results, are presented. MAIN OUTCOME MEASURES: Visual acuity, slit-lamp biomicroscopy, indirect ophthalmoscopy, and fluorescein angiography results. RESULTS: The ocular manifestations of TB in our patients included panophthalmitis, endophthalmitis, posterior uveitis with choroidal tubercles, keratitis, and a lid mass. Mycobacterium tuberculosis was identified in four cases in ocular specimens using acid-fast bacilli microscopy and in three cases by culture. Rapid diagnosis using polymerase chain reaction was obtained in one case. Extraocular foci of TB were identified in three cases with an intraocular infection at presentation. No patients had the human immunodeficiency virus (HIV) and none were immunocompromised. Two eyes could not be saved using antituberculous treatment because of delayed diagnosis and treatment. CONCLUSIONS: In this age of the HIV pandemic, TB is becoming more common. Because it is curable, heightened awareness and better understanding of the disease's ocular manifestations should be of concern to all ophthalmologists.

Aged↗

Benefits and complications of photodynamic therapy of papillary capillary hemangiomas.

OBJECTIVE: To evaluate the potential benefit and risks of photodynamic therapy (PDT) in the treatment of papillary capillary hemangioma. DESIGN: Prospective, noncomparative, interventional case series. PARTICIPANTS: Five patients with solitary capillary hemangioma on the temporal portion of the optic nerve presenting with exudative decompensation and decrease in visual acuity (VA). METHODS: All eyes received a standardized PDT treatment with 6 mg/kg body surface area verteporfin and application of 100 J/cm(2) light at 692 nm. One to three PDT courses were performed until resolution of exudation was achieved. A continuous follow-up was provided with documentation 1 week before and at 4 to 6 weeks, 3 months, and 12 months after the last treatment application. MAIN OUTCOME MEASURES: Functional parameters included best-refracted VA (Early Treatment Diabetic Retinopathy Study), and central scanning laser ophthalmoscope (SLO) scotometry and peripheral (automated perimetry) visual fields; anatomic parameters were presence of retinal edema or serous detachment (ophthalmoscopy) and tumor size (ultrasonography). RESULTS: Pretreatment VA levels ranged from 20/40 to 20/800; posttreatment levels ranged from 20/64 to 20/2000. Tumor regression with resolution of macular exudate and serous retinal detachment was obtained in all eyes. A decline in VA of 1, 3, and 10 lines, respectively, was documented in three patients. Complications included transient decompensation of vascular permeability, occlusion of retinal vessels, and ischemia of the optic nerve. CONCLUSIONS: PDT is successful in reducing tumor size and exudative activity. Vaso-occlusive effects at the level of the retina and optic nerve compromise the functional benefit. Parameters proven safe in choroidal neovascularization may be inappropriate in retinal capillary lesions of the optic nerve.

Adult↗

Correlation between intraocular pressure level and optic disc changes in high-tension glaucoma suspects.

PURPOSE: To assess the correlation between intraocular pressure (IOP) and future optic disc changes in eyes of patients suspected of having high-tension glaucoma and to determine the target pressure in eyes of patients suspected of having high-tension glaucoma. DESIGN: Prospective, comparative, observational case series. PARTICIPANTS: Fifty-seven eyes of 57 patients suspected of having high-tension glaucoma selected from 226 consecutive patients with glaucoma or suspected glaucoma. INTERVENTION: The status of the optic disc and visual fields was evaluated during an initial examination by confocal laser ophthalmoscopy and automatic perimetry, respectively. Fifty-seven eyes were followed up for at least 2.5 years and then underwent a final examination. IOP was measured every 3 months. MAIN OUTCOME MEASURES: The correlation between changes in optic disc status and IOP control during follow-up was determined. Changes in the optic disc were evaluated by using the percentage change in total contour area, neuroretinal rim area, and cup-to-disc ratio. The control IOPs were evaluated on the basis of the percentage of time during which IOP was <18 mmHg (%<18 mmHg control) and <21 mmHg (%<21 mmHg control) during follow-up in each eye. The correlation between visual field changes and IOP control was also analyzed. RESULTS: Results from 48 (84.2%) of 57 participants were included. Patients were followed up for a mean 4.4 years. The %<21 mmHg control was significantly correlated with changes in neuroretinal rim area (r = 0.40; P = 0.0055) and in cup-to-disc ratio (r = -0.40; P = 0.0051) by linear regression analysis. The correlations were also significant if the difference in the length of follow-up was adjusted by multivariate regression analysis. There was no significant correlation between %<18 mmHg control and changes in disc parameters. Seven (14.6%) of 48 eyes had glaucomatous visual field defects at the final examination. The %<21 mmHg control was significantly lower in eyes with visual field changes compared with eyes with no changes (P = 0.0153; unpaired t test). In contrast, %<18 mmHg control was not significantly different between eyes with and without visual field changes (P = 0.3886). CONCLUSIONS: The IOP level correlates with topographic changes in the optic disc in eyes of patients suspected of having high-tension glaucoma. The target pressure for such eyes may need to be between 18 and 21 mmHg, and <18 mmHg is a safe target level in the treatment of patients suspected of having high-tension glaucoma to delay topographic optic disc changes.

Adult↗

Infliximab in the treatment of refractory posterior uveitis.

PURPOSE: To determine the efficacy and safety of infliximab in the treatment of refractory posterior uveitis. DESIGN: Noncomparative interventional case series. PARTICIPANTS: Five patients with posterior uveitis were treated: 3 had Behçet's syndrome, and 2 had idiopathic posterior uveitis. INTERVENTIONS: Patients with sight-threatening uveitis refractory to other immunosuppressive agents were treated with infliximab. MAIN OUTCOME MEASURES: Intraocular inflammation, by using binocular indirect ophthalmoscopy score, retinal vasculitis, and visual acuity. Adverse effects of infliximab were documented. RESULTS: Within 2 weeks of the first infusion of infliximab, 4 of 5 patients showed marked improvement in vitreous haze and visual acuity. By the 6-month follow-up, the same four patients had achieved remission of posterior uveitis and had successfully withdrawn all other immunosuppressive therapy. Further infusions of infliximab were required in 3 patients. One patient developed ocular and systemic tuberculosis, which responded to antituberculous treatment. CONCLUSIONS: Infliximab is effective in the treatment of sight-threatening refractory posterior uveitis. However, patients should be thoroughly screened for tuberculosis before treatment and followed up closely during and after therapy with infliximab.

Adult↗

Examination of the premature infant.

Ophthalmoscopic examination of the premature infant requires binocular indirect ophthalmoscopy, use of an eyelid speculum, and wide pupillary dilatation. Normal and pathologic features unique to the infant eye often are encountered. Familiarity with these features is essential for accurate diagnosis and prevention of unnecessary therapeutic intervention. Supplemental oxygen is the mainstay of supportive therapy for idiopathic respiratory distress syndrome, a common cause for morbidity in premature infants. Arterial oxygen monitoring is essential for survival and for prevention of retrolental fibroplasia, but precise arterial oxygen levels associated with development of retrolental fibroplasia have not been established.

Humans↗

Technology: its role in our conception of glaucoma. The 35th Mark J. Schoenberg lecture.

Glaucoma is a medical notion encompassing many diverse entities. Our present conception of glaucoma is rooted in four major technological innovations: ophthalmoscopy, tonometry, perimetry, and gonioscopy. The results of our findings from these modalities determine how we diagnose and plan management strategies. In essence, we are bound to a series of numbers or values useful in clinical practice but which tell us little about the pathogenetic factor(s) causing glaucoma.

Glaucoma↗

Effects of the Nd:YAG laser on the primate retina and choroid.

The effects of the neodymium:YAG laser in the thermal mode on the subhuman primate retina were studied with ophthalmoscopy, intravenous fluorescein angiography, light microscopy, transmission electron microscopy, and scanning electron microscopy using a plastic injection-corrosion technique. Representative samples were examined at 24 hours, 14 days, and 28 days after treatment. The burns appeared to predominantly affect the outer half of the retina and the inner half of the choroid, although with higher energy levels the inner retinal layers were occasionally involved. In most instances Bruch's membrane was disrupted. The injured choriocapillaris underwent a reparative process during a four-week period in which the discrete lesions observed at 24 hours had disappeared.

Animals↗

Fundus lesions in malignant hypertension. VI. Hypertensive choroidopathy.

Experimental renovascular malignant arterial hypertension was produced, by modified Goldblatt's procedures, in 60 rhesus monkeys, and hypertensive fundus changes were studied in detail (by serial ophthalmoscopy and fluorescein fundus angiography in all monkeys on a long-term follow-up, and pathologically in 29 eyes). In hypertensive choroidopathy, retinal pigment epithelial (RPE) lesions and serous retinal detachment (RD) were the classic ophthalmoscopic lesions, whereas fluorescein fundus angiography and histopathologic studies revealed marked abnormalities in the choroidal vascular bed, in addition to the changes in the RPE. The RPE lesions could be subdivided into initial acute focal lesions (due to focal RPE infarction), and degenerative lesions, which developed later and were progressive in nature, maximally involving the macular and peripheral regions of the fundus. The RD developed most commonly in the posterior pole and infrequently involved the peripheral retina. The choroidal vascular bed showed impaired circulation and extensive occlusive and ischemic changes. These studies revealed that hypertensive choroidopathy is as important a fundus change as hypertensive retinopathy. The pathogenesis of hypertensive choroidopathy is discussed in detail; the evidence indicates that it is due to choroidal ischemia, and that hypertensive choroidopathy and retinopathy are two independent and unrelated manifestations of renovascular malignant hypertension.

Animals↗

Involutional macular thinning. A pre-macular hole condition.

We identified a group of 93 patients (102 eyes) with involutional macular thinning which is a condition that predisposes the affected eyes to developing idiopathic macular holes. The fovea in these patients shows specific architectural changes that can be identified on ophthalmoscopy. On follow-up (mean, 50 months), 26 eyes (27%) developed a macular hole compared to 0% in normal fellow eyes (P less than 0.00001). Patients with pigment epithelial window defects on fluorescein angiography (12 of 15 eyes or 80%), or with no posterior vitreous detachment (15 of 34 eyes or 44%) had the highest risk of developing macular holes. There were seven eyes with involutional macular thinning, pigment epithelial window defects on fluorescein angiography and no posterior vitreous detachment; six (86%) developed a macular hole. Macular degeneration, estrogen supplements, cystoid changes (with normal fluorescein angiography), and poor visual acuity did not increase risk. We present a theory for the pathogenesis of idiopathic macular holes.

Adult↗