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Retinal and optic nerve findings in Goldenhar-Gorlin syndrome.

Involvement of the posterior segment of the eye in Goldenhar-Gorlin syndrome is more common than is generally appreciated. We examined seven patients with this syndrome. Abnormalities included diminished visual acuity, tilted optic disc, optic nerve hypoplasia, tortuous retinal vessels, macular hypoplasia and heterotopia, microphthalmia and anophthalmia. In one case, pathologic study showed agenesis of the optic nerve. It is proposed that retinal, optic nerve and craniofacial abnormalities in this condition may reflect an asynchrony in the migration of the neural crest cells in the early stages of embryonal development.

Adolescent↗

Increase of pallor and fluorescein-filling defects of the optic disc in the follow-up of ocular hypertensives measured by computerized image analysis.

In 24 ocular hypertensive patients, two optic disc fluorescein angiograms were obtained a mean of 3.9 years apart. Using computerized image analysis, we measured the area of pallor and the fluorescein-filling defect area within the disc and the fluorescein circulation parameters representing the filling rates of optic disc, choroid, and retinal vessels. The results indicated that increase in pallor and fluorescein-filling defect areas, as well as a slowed filling rate of the retinal veins, correlated with glaucomatous progression.

Aged↗

[Medical treatment for experimental retinal vein occlusion--thrombolytic effect of nasaruplase].

The anti-thrombolytic effect of urokinase (UK), nasaruplase, and argatroban was studied using an experimental rabbit model of retinal vein occlusion (RVO). Experimental RVO was induced by transadventitial thrombin instillation. Five or fourteen days after thrombin application, the rabbits were injected with gelatin-fluorescein sodium and their eyeballs were enucleated for microscopic observation and flat preparation of the retina. Occlusion of retinal vessels was found to be less in number and distribution in the nasaruplase group than in the control group. On microscopic observation, the retinal arteries and veins of the nasaruplase group showed a vascular subendothelial space formation which suggested a process of thrombogenesis and thrombolysis in the space. The coefficiency of beraprost sodium, aspirin, and ozagrel hydrochloride to UK therapy was studied, but there was no significant difference between these groups and the control group with no after-treatment (the UK group). This result shows the effectiveness of nasaruplase for the thrombolytic therapy of RVO.

Animals↗

Clinical and histopathologic features of canine oxygen-induced proliferative retinopathy.

PURPOSE: In previous studies the morphologic features of the acute vaso-obliterative and vasoproliferative stages of oxygen-induced retinopathy (OIR) were quantified and described in the dog model of retinopathy of prematurity (ROP). In the present study the sequelae of these events were examined using fluorescein angiography and histologic, enzyme, and immunohistochemical techniques. METHODS: Thirty newborn animals were exposed to 95% to 100% oxygen for 4 days and returned to room air until they were 22 to 45 days of age. Before death some animals were anesthetized, and fluorescein angiography was performed. Retina and vitreous from some animals were processed for adenosine diphosphatase (ADPase) flat-embedding. In other cases, eyes were prepared for full-thickness eyewall sectioning or frozen for histochemical analysis. RESULTS: Fluorescein angiography, funduscopic examination, and ADPase preparations showed dilated and tortuous retinal vessels, pigmentary changes, incomplete vascularization of peripheral retina, vitreous hemorrhage, and persistence of massive intravitreal neovascularization. Full-thickness eyewall sections showed tractional retinal folds, tented intravitreal vascularized membranes, and vitreous synchysis. Immunohistochemical analysis showed inner retinal astrogliosis. Enzyme histochemistry showed high alpha glycerophosphate dehydrogenase activity in poorly differentiated neovascular formations and low activity in formations with mature pericytes and endothelial cells. CONCLUSIONS: End-stage OIR in the neonatal dog shares many features with the chronic human disease. These results provide additional support for the use of this model in experimental studies of ROP.

Animals↗

[Systemic fibrinolytic therapy using urokinase in central retinal artery occlusion. A case study].

BACKGROUND AND OBJECTIVE: Systemic fibrinolysis has become an important therapeutical option in patients with thrombotic occlusion of coronary or pulmonary arteries. In view of the hemorrhagic risk systemic fibrinolytic therapy for retinal vessel occlusion has been discussed controversial. In the present case study results and complications of systemic fibrinolysis should be investigated in patients with central retinal artery occlusion. PATIENTS AND METHODS: From 1995 to 2002 a case series of 19 consecutive patients (8 female, 11 male, age: 63.2+/-14,3 years) with central retinal artery occlusion were treated by systemic application of urokinase using a standardized scheme. The latency from initial symptoms until the initiation of therapy and the medical history of the patients were documented. Visual acuity was determined on admission and before discharge and possible complications were documented. Additionally, screening investigations for genetic thrombophilia were performed. RESULTS: 15 patients showed an improvement of the visual acuity (79 %, 95 %-KI: 54 %-94 %). For 3 patients no improvement and for one patient a decrease of the visual acuity was determined. Hemorrhagic complications were observed in two patients (11 %, 95 %-KI: 1 %-33 %). As these minor bleedings were self-limiting the fibrinolytic therapy was discontinued only in one patient. As risk factors most commonly arterial hypertension (68 %) and smoking (26 %) were identified. In 4 patients a genetic thrombophilia was diagnosed. CONCLUSIONS: Considering the poor prognosis of central retinal artery occlusion and the disappointing results of conservative treatment, an improvement of the visual acuity in the absence of critical complications was observed with systemic fibrinolytic therapy in the presented case study. However, only controlled trials can provide proof for the effect of fibrinolysis versus spontaneous improvement.

Contraindications↗

[Leber congenital amaurosis: diagnosis, follow-up and differential diagnosis].

AIM OF THE STUDY: Leber's congenital amaurosis (LCA) had been diagnosed on/in 42 children between 1968 and 1996 at the Deptm. of Ophthalmology, University Hospital Zurich. We reexamined critically this rare diagnosis in retrospect and with new examinations where possible. PATIENTS AND METHODS: Clinical and electroretinographic (ERG) results, often obtained in general anesthesia, were re-evaluated and when possible repeated in new examinations. RESULTS: Thirty-three of the total 42 patients presented with an extinguished, 35 with markedly reduced, and 6 with minimal ERGs. A profound visual loss (from no light perception to 20/200), nystagmus and strabismus were the principal symptoms. The heterogeneity of retinal findings ranged from normal to salt and pepper or bone spicules pigmentation and pronounced chorioretinal atrophy. Vascular attenuation and rarification were frequent. Patients with nonocular findings such as mental retardation (n = 12), renal (n = 3) and skeletal (n = 4) abnormalities revealed no differing ERG- or retinal findings. The oculodigital sign (eye-poking) was found in 25%, and parental consanguinity was evident in 10% of the cases. In 16 patients that were reexamined, the progression of the disease was characterized by an increase in retinal pigmentary changes, attenuation of retinal vessel, and further diminuation of the visual acuity (n = 6). CONCLUSION: Upon review, the diagnosis had to be revised in 8 patients as juvenile retinitis pigmentosa and in one as infantile Refsum syndrome. Bilateral visual impairement in infants should be assessed clinically and electroretinographically within the first year. Neuropediatric and metabolic examinations meaningfully complement the diagnostic procedures.

Child↗

[Comparative study of beta irradiation (106Ru/106Rh) and gamma irradiation (125I) on the rabbit eye].

We compared beta irradiation (106Ru/106Rh) to gamma irradiation (125I) on the normal rabbit eye, using ophthalmic plaques to deliver doses similar to those recommended in man for choroidal melanoma treatment. A detailed dosimetry was performed and the animals were followed up by clinical and histological examinations during 1 year. The mean total doses were either comparable, or larger with iodine-125, but the globes treated with ruthenium-106 exhibited more damaging effects: total destruction of the chorioretina on the plaque site, abnormalities of the retinal vessels and of the close nerve fiber layers, cavernous atrophy of the optic nerve. In the eyes treated with iodine-125, only the external retinal layers were destroyed.

Animals↗

[Early neuro-ophthalmological symptoms of periarteritis nodosa. A case report (author's transl)].

A report is given on a 47-year-old woman with periarteritis nodosa. A unilateral retinopathy with disseminated cotton-wool-spots surrounded by retinal haemorrhages and edema was observed. Fluoresceinangiography showed a multifocal constriction of the retinal capillaries. This lesion of the retinal vessels can be distinguished from those caused by hypertension. An acute immunological reaction has been suggested as the pathogenesis. Some authors have described this retinal lesion in collagen diseases as a "toxic" retinopathy. We prefer the term acute immunological microangiopathy (AIM).

Eye Manifestations↗

[Fluorescence angiography in the diagnosis of pigment-free melanomas of the choroid].

The ophthalmoscopic and angiographic patterns of 52 patients with pigment-free melanomas of the choroid, aged 27 to 73 were investigated. the mean age of examinees was 52. The prominence of tumors ranged from 1.3 to 6.5 mm. All patients underwent standard ophthalmoscopic general-clinical examinations. The tumor sizes were verified echographically. Fluorescence angiography was made according to the commonly used technique of serial pictures with computer processing. Diagnostically valuable angiographic signs were defined: contrasting of tumor vessels, spotty nature of fluorescence, beginning of fluorescence (arterial phase), pick of fluorescence (late venous phase), intensification velocity of fluorescence, late fluorescence, angiopathy of retinal vessels, large choroidal vessels and tumor-associated epitheliopathy of retina (small pigment foci, druses and hypofluorescence). Considering the research results, fluorescence angiography can be regarded as a principle diagnostic tool in pigment-free melanomas of the choroid.

Adult↗

[The immediate and late results of the combined treatment of open-angle glaucoma].

The paper analyses immediate and remote results after medicamentous-surgical treatment of open-angle glaucoma of stages II and III with preliminary vasosection of facial vessels of the ophthalmic artery basin in 110 patients, aged under 60 years (21), from 61 to 70 (43), above 70 (46). Atherosclerosis of retinal vessels was recorded in 83.6% of patients, hypertonic disease--in 61.8%. The results of a 5-year period of observation have shown that in glaucoma of stage II visual functions remained at the initial level in 84% of patients, and of stage III--in 78.2%. Positive results of vasosection used as a means for improvement of hemodynamics in the internal coats of the eye and the optic nerve after antiglaucomatous operations confirm that, besides antiglaucomatous operations, it is necessary to use a complex of preparations and vasosection, thus improving the hemodynamics of the eye and providing preservation of visual functions.

Aged↗

Experimental retinal vascular occlusion. III. An ultrastructural study of simultaneous occlusion of central retinal vein and artery.

The retinal changes following the simultaneous occlusion of retinal vessels were examined at an ultrastructural level. The preservation of endothelial cells that prevented leakage of blood and horseradish peroxidase tracer material in the surrounding retinal tissues explained the lack of hemorrhages observed clinically. Mueller cell death resulted in severe structural disorganization of the retina, with disruption of the inner limiting membrane, involvement of the outer retinal layers, and fleurette formation. The retinal macrocysts were derived from ischemic infarction.

Animals↗

Bilateral acute retinal necrosis.

Four patients who experienced the sudden onset of anterior uveitis with large keratic precipitates and dense vitreous opacities developed confluent yellow-white swellings and exudates in the peripheral retina and sheathing and obliteration of retinal arteries. After absorption of the exudates, atrophic patches in the peripheral retina and a funnel-shaped retinal detachment with many tears appeared. Angiograms showed retinal edema, fluorescein leakage from the choroid in the affected areas, and perivasculitis of the retinal arteries. Although treatment seemed unable to alter the course of the disease, in one case the retina reattachment after vitrectomy and filling of the vitreous space with silicone oil. Histopathologic studies disclosed extensive atrophy and degeneration of the outer retinal layers and pigment epithelium and occlusion of the retinal vessels. An infection may trigger the uveitis, leading to an autoimmune sensitization against rods and cones that causes severe local immune-complex disease and retinal vasculitis.

Adult↗

In vivo rabbit eyecup preparation for use in retinal research.

PURPOSE: To develop an in vivo rabbit eyecup preparation that preserves neuronal and vascular connections with normal posterior segment contour, permitting direct access to the retina and facilitating retinal microsurgical and neuroscience research. METHODS: Cyanoacrylate glue was applied to the anterior sclera of six Dutch-belted rabbits before open-sky vitrectomy. The glue was used to harden the compliant scleral wall and to fix it to the surrounding periorbital tissues. RESULTS: A normal contour of the posterior segment was retained in all cases. Vitrectomy under air was successfully accomplished and an extensive removal of the vitreous gel was achieved. Fluorescein angiography revealed normal vascular patency of the retinal vessels after vitrectomy. CONCLUSIONS: The proposed modification of the rabbit eyecup retains the normal neurovascular connections and configuration of the retina, making it suitable for retinal microsurgical maneuvers or any procedures in which easy access to anatomically undisturbed retina is required.

Animals↗

[A case of metastatic carcinoma to the retina].

The authors report a case of metastatic carcinoma to the retina. The patient was a 61-year-old man who had an operation for a well-differentiated adenocarcinoma of the rectum. Ophthalmoscopic examination disclosed a single, white, elevated mass lesion surrounded by serous retinal detachment located in the upper part of the macula of the right eye. A few retinal hemorrhages existed around the lesion. Fluorescein angiography revealed partially obscured retinal vessels due to compression by the tumor and arteriovenous anastomosis. Postmortem pathologic examination confirmed metastases to the brain, lung and retina. Microscopic examination showed a retinal lesion and tumor cells in the right eye. Tumor cells, similar to the carcinoma of the rectum, were present only in the neurosensory retina and did not invade the pigmented epithelium or choroid.

Adenocarcinoma↗

Retinal and choroidal vessel closure using phthalocyanine photodynamic therapy.

Chloro-aluminum sulfonated phthalocyanine (CASPc) is a photoactive dye capable of generating photochemical reactions when excited with 675 nm light. We used CASPc to produce photochemical closure of retinal medullary ray vessels and choroidal vessels in normal rabbits. Irradiation prior to CASPc injection produced no photographic, angiographic, or histologic lesions in any eyes. Identical irradiation of medullary ray and choroidal vessels after CASPc injection produced complete vessel closure in all eyes. Histopathologic examination showed marked thrombosis of medullary ray and choroidal vessels, with minimal damage to contiguous tissues including the neurosensory retina. We conclude that CASPc can produce profound closure of normal retinal and choroidal vessels with minimal deleterious effect on surrounding tissues.

Aluminum↗

Optic nerve degeneration in the DBA/2NNia mouse: is the lamina cribrosa important in the development of glaucomatous optic neuropathy?

To study optic nerve (ON) degeneration in the DBA/2NNia (DBA) mouse, a species lacking a lamina cribrosa and a model for secondary angle-closure glaucoma, serial semi- and ultra-thin sectioning of the myelinated ON and of the ON head was performed and sections evaluated qualitatively and quantitatively by light and electron microscopy. Immunohistochemistry was performed using antibodies against collagen type I, III, VI, laminin, and connexin43. The major finding on the myelinated ON was a significant decrease in cross section area during ON degeneration which was paralleled by a loss of axons and an increase in microglia. The number of astrocytes and blood vessels did not change. The major findings on the ON papilla were that ON heads with only mild degeneration showed a pronounced focal degeneration around the central retinal artery. In more severely degenerated ON, newly formed bundles of collagen type VI were located between astrocyte processes within the ON head. In a species that has no lamina cribrosa, DBA mice can develop typical signs of glaucomatous optic neuropathy. The entrance of the central retinal vessels into the ONH seems to be a preferentially vulnerable region for axon loss in this mouse model. In addition, astrocytes in the ON head form extracellular material similar to that found in human glaucomatous eyes.

Animals↗

Congenital retinal macrovessel.

Congenital retinal macrovessel is a rare condition in which anomalous retinal vessels cross the macula. Visual impairment can result if the anomalous vessels pass across the foveola, if foveolar cysts form, or if hemorrhage occurs. A now 17-year-old African-American female has an anomalous retinal vein that crossed the macular region beyond the horizontal raphe in her left eye. This patient exhibited the classic characteristics of congenital macrovessels. Her visual acuity remained unaffected after a 5-year follow-up and therefore treatment was not required.

Adolescent↗

Intraobserver reproducibility of a two-dimensional mapping of the optic nerve head perfusion.

PURPOSE: To evaluate the intraobserver reproducibility of a software designed to assess retinal blood flow with the Heidelberg Retina Flowmeter (HRF). METHODS: Ten subjects were consecutively recruited, and one eye of each patient was randomly selected for study. Blood flow measurements were analyzed by using an automatic full field perfusion image analysis (AFFPIA) program, which calculates the Doppler frequency shift and hemodynamic variables (flow, volume, and velocity) for each pixel. The resulting perfusion image is processed with respect to underexposed and overexposed pixels, saccades, and retinal vessel tree. Intraobserver reproducibility was calculated for the AFFPIA program. All the optic nerve heads were horizontally divided into three sections (superior, central, and inferior). The retinal blood flow was calculated in the superior and inferior section, and each section was further divided into three areas (temporal, nasal, and rim). The blood flow was evaluated for each area. RESULTS: When the same observer analyzed the same image five times (intraobserver intraimage reproducibility), the AFFPIA coefficient of variation ranged from 0.5% to 5% in the temporal area, from 0.1% to 5.3% in the nasal area, and from 0.5 to 28% in the rim area. When the same observer analyzed three different images of the same section once (intraobserver interimage reproducibility), the AFFPIA coefficient of variation of flow measurements ranged from 1% to 7.3% in the temporal area, from 1.5% to 10% in the nasal area, and from 2 to 30% in the rim area. CONCLUSION: Retinal blood flow measured by HRF and analyzed by AFFPIA had good intraobserver reproducibility. The reproducibility was significantly better in the temporal and nasal areas than in the rim area.

Aged↗