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Frosted branch angiitis associated with cytomegalovirus retinitis.

We examined three patients with acquired immunodeficiency syndrome who had frosted branch angiitis associated with small patches of cytomegalovirus retinitis. Each patient had a low CD4-helper T-lymphocyte count and a T-lymphocyte helper-suppressor ratio of less than 0.1. Treatment with intravenous anticytomegalovirus antibiotics caused the vascular sheathing to resolve within two weeks in all three patients, but each patient continued to have a smoldering retinitis. Retinal biopsy in one of the patients demonstrated virions whose morphologic characteristics were consistent with cytomegalovirus on electron microscopy and the identity of which was confirmed by immunohistochemistry. Although frosted branch angiitis in otherwise healthy patients responds to corticosteroids, similar treatment with corticosteroids for frosted branch angiitis associated with cytomegalovirus retinitis in patients with AIDS does not seem to be indicated. Before corticosteroid treatment is started for a patient with the clinical signs and symptoms of frosted branch angiitis, careful medical examination of the patient is necessary.

Acquired Immunodeficiency Syndrome↗

Cerebroretinal vasculopathy. A new hereditary syndrome.

A new hereditary syndrome characterized by a frontoparietal lobe pseudotumor and retinal capillary abnormalities is described. A pedigree is presented in which characteristic ophthalmic findings have been found in ten family members and are suspected in eight additional family members spanning a total of four generations. Typical retinal findings include perifoveal capillary obliteration, peripheral focal capillary occlusion, and microvascular abnormalities, particularly involving the posterior pole. Eight patients spanning three generations had a central nervous system pseudotumor with identical histopathology. Histopathologic analysis of brain tissue shows a characteristic pattern of an unusual vasculopathy without vasculitis characterized by fibrinoid necrosis and resulting in necrosis of white matter with sparing of cortical brain tissue. The pedigree shows an apparent autosomal dominant pattern of inheritance with delayed expression of abnormalities. Of note, two patients unrelated to the pedigree having similar neuropathologic and retinal findings also have been seen at the authors' institution.

Adult↗

Angiographic features after photodynamic therapy for choroidal neovascularisation in age related macular degeneration and pathological myopia.

AIM: To describe the angiographic features after photodynamic therapy (PDT) with verteporfin in choroidal neovascularisation (CNV) associated both with age related macular degeneration (AMD) and pathological myopia (PM). METHODS: 36 patients affected by subfoveal CNV in AMD and 25 patients with subfoveal CNV in PM underwent an ophthalmological examination including fluorescein angiography (FA) and indocyanine green angiography (ICGA) using the IMAGEnet System. Post-PDT examinations were performed 7, 30, and 90 days later. RESULTS: The typical angiographic aspect after PDT for AMD related CNV was a round hypofluorescence visible both on FA and on ICGA, which included both CNV and the surrounding tissues and corresponded to the area exposed to laser light. In PM the CNV appeared hypofluorescent during the early phases and gradually became hyperfluorescent during the late phases on FA, whereas on ICGA it was detectable in its whole extension as a hyperfluorescent lesion since the early phases. Differently from AMD, there was no round hypofluorescence surrounding the CNV on FA or on ICGA. Moreover, five patients in the AMD group showed hot spots on ICGA, which spontaneously disappeared during the follow up. Classic and occult components of the AMD related CNV revealed a different angiographic response to PDT, showing with the latter only a partial closure 1 week after PDT followed by a complete reopening at the first month in 100% of cases. CONCLUSION: The post-PDT hypofluorescence typical of AMD related CNV, especially visible on FA, might be secondary to a combination of choriocapillary occlusion and masking effect due to swelling of retinal pigment epithelium cells. Hot spots in the AMD affected patients could be interpreted as the expression of a non-thermal choroidal vasculitis secondary to PDT.

Choroid↗

Posterior scleritis: a clinical and histological survey.

The clinical course of 47 patients with posterior scleritis is reviewed. Though clinical presentation varied widely, 73% of the patients presented with a visual acuity of 6/18 or less. Because the posterior scleritis was not always associated with pain or with anterior scleritis, the diagnosis was often not considered when the patient was first seen. The most common findings in the fundus were disc swelling, retinal detachment, and macular oedema and the most useful investigation was B scan ultrasound. No common aetiology was found, although 60% had a systemic disorder which was accompanied by a vasculitis. Those who were diagnosed and treated with the minimum delay had the most satisfactory visual outcome. However, there appears to be a group of patients with no underlying systemic disease who fail to respond to intensive therapy, and lose vision. A new sub-group of West Indians with the disease is described. The histopathology of 7 cases confirmed the presence of scleral vasculitis of the vessels in and around the sclera in all the specimens. Other significant findings include inflammatory swelling and focal loss of pigment epithelium together with choroidal vascular closure. This could account for the fluorescein angiographic findings.

Adolescent↗

Ocular findings in human babesiosis (Nantucket fever).

Human babesiosis (Nantucket fever) is a rare, tick-borne intraerythrocytic parasitic disease characterized by fever, lymphadenopathy, arthralgias, and hemolytic anemia. A 34-year-old woman, who had previously undergone surgical removal of her spleen, was hospitalized because of presumed hepatitis. The many retinal nerve fiber layer infarcts and serologic abnormalities suggested collagen disease. The diagnosis of babesiosis was established by the demonstration of intraerythrocytic parasites and a greatly elevated titer to Babesia microti. The infection was treated with chloroquine, orally administered quinine, and pyramethamine, and her symptoms resolved within one month. The retinopathy was probably the result of focal vasculitis secondary to immune complex disease caused by chronic infection.

Adult↗

Indocyanine green and fundus fluorescein angiographic findings in patients with active ocular Behcet's disease.

PURPOSE: To define the indocyanine green (ICG) and fundus fluorescein angiographic (FFA) features of Behçet's disease. METHODS: The study included 49 eyes of 25 patients (19 males, 6 females; mean age: 34.1 years; age range: 14-68 years) with Behçet's disease and active ocular involvement that fulfilled the criteria of the International Study Group for Behçet's Disease. In each patient, FFA and ICG angiography were performed consecutively in the same session using a TRC-50I/A fundus camera (Topcon, Tokyo, Japan) coupled to an Image Net digitizing system. All images were analyzed and FFA, ICG, and clinical findings summarized. RESULTS: The mean duration of ocular involvement was 52.4 months (range: 2-240 months). FFA showed staining and dye leakage at the optic disc in 44 (89.8%) eyes and diffuse vasculitis in 36 (73.5%) eyes. Macular edema and ischemia were observed in 31 (63.3%) and 3 (6.1%) eyes, respectively. Eight (16.3%) eyes exhibited detectable retinal FFA alterations, but no abnormalities on ICG angiography. Disc ICG hyperfluorescence was observed in 23 (46.9%) eyes. ICG angiography revealed choroidal fuzziness in 16 (32.6%) eyes, hyperfluorescent spots in 13 (26.5%) eyes, and hypofluorescent plaques in 12 (24.5%) eyes. Eleven eyes (22.4%) showed no abnormal findings on ICG angiography. CONCLUSION: ICG and FFA complement each other as tools for diagnosing patients with Behçet's disease. FFA often serves as a fairly reliable guide. Although some ICG findings cannot be demonstrated by FFA, they are not specific or pathognomonic. We do not recommend performing both procedures for Behçet's disease.

Adolescent↗

Metabolic syndrome and central retinal artery occlusion.

BACKGROUND: The accumulation of risk factors for central retinal artery occlusion can be seen in a single person and might be explained by the metabolic syndrome. CASE REPORT: We presented the case of a 52-year-old man with no light perception in his right eye. The visual loss was monocular and painless, fundoscopy showed central retinal artery occlusion and the laboratory investigation showed the raised erythrocyte sedimentation rate of 105 mm/h and the raised C-reactive protein of 22 mg/l. Specific laboratory investigations and fluorescein angiography excluded the presence of vasculitis, collagen vascular diseases, hypercoagulable state and antiphospholipid syndrome. CONCLUSION: The patient met all the five of the National Cholesterol Education Program (NCEP) criteria for the metabolic syndrome: hypertension, abnormal lipid profile, abnormal glucose metabolism, obesity and hyperuricemia. Measurement of C-reactive protein is useful for the assessment of therapeutic systemic effect on any abnormality in the metabolic syndrome. Individual therapy for all risk factors in the metabolic syndrome is necessary to prevent complications such as cardiovascular, retinal vascular diseases and stroke.

Fluorescein Angiography↗

Polyarteritis presenting with thrombocytosis and central retinal vein thrombosis.

We report a case of polyarteritis in a 54 year old woman who presented with marked thrombocytosis and acute blindness in one eye secondary to central retinal vein thrombosis. She also developed bilateral pulmonary infiltrates and renal failure. The diagnosis was confirmed by histological changes in the renal biopsy. Treatment with immunosuppressives, plasma exchange and antiplatelet drugs led to rapid clinical improvement and recovery of renal function and prevented further thromboembolic episodes. Plasma exchange and antiplatelet drugs should be considered in polyarteritis group of systemic vasculitis especially in the presence of thromboembolic complications or thrombocytosis.

Female↗

Are inflammatory factors related to retinal vessel caliber? The Beaver Dam Eye Study.

OBJECTIVE: To examine the relationship of systemic markers of inflammation, endothelial dysfunction, and serum folate level to retinal vessel diameter. METHODS: Cross-sectional analyses were completed for data from a random sample of 396 persons aged 50 to 86 years who underwent a baseline examination from 1988 to 1990. Standardized protocols for blood collection and measurement of markers were used. Diameters of arterioles and venules were measured from digitized photographs. Standard univariate and multivariate analyses were performed. RESULTS: While controlling for age, smoking status, diabetes status, serum high-density lipoprotein cholesterol, and hematocrit, wider retinal venular diameters were associated with higher serum high-sensitivity C-reactive protein, interleukin 6, and amyloid A levels. While controlling for age, systolic and diastolic blood pressure, smoking, serum high-density lipoprotein cholesterol level, and gout, smaller arteriolar diameters were associated with higher serum amyloid A and lower serum albumin and folate levels but not high-sensitivity C-reactive protein or interleukin 6 levels. Levels of serum soluble intercellular adhesion molecule-1 and serum soluble E-selectin, markers of endothelial dysfunction, were not associated with retinal arteriolar or venular diameters. CONCLUSIONS: These data show an association of inflammatory markers with larger retinal venular diameter, suggesting that retinal venular caliber may be a marker of systemic inflammation.

Aged↗

An ocular cat-scratch disease patient positive for cytoplasmic anti-neutrophil cytoplasmic antibody.

BACKGROUND: We report a case of ocular cat-scratch disease with permanent vision reduction in a patient who was cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) positive. METHODS: Case report and review of the literature. RESULTS: While taking steroids and antibiotics, a 52-year-old man with uveitis associated with cat-scratch disease developed retinal vein occlusion and a macular exudate. His final visual acuity was poor because of residual macular degeneration and optic atrophy. Serum C-ANCA increased and decreased in parallel with ocular inflammatory activity. CONCLUSION: C-ANCA is an indicator of vasculitis and may be useful as an indicator of severe cat-scratch disease.

Ampicillin↗

SLE retinopathy: evaluation by fluorescein angiography.

Fifty-two patients with systemic lupus erythematosus (SLE) were examined by fluorescein angiography, and retinopathy was detected in 15. Three patterns of retinopathy were discerned: 4 patients had disc vasculitis, 6 had multiple cotton-wool spots, and 5 had a normal fundal appearance but fluorescein leakage on angiography. One patient had arterial occlusive disease with retinal neovascularisation and another had extensive venous disease. With 3 exceptions retinopathy was found only in patients with active SLE. No association was discovered between retinopathy and cerebral disease; in particular, fluorescein angiography did no assist the diagnosis of mild cerebral lupus.

Adult↗

Use of split-thickness dermal grafts to repair corneal and scleral defects--a study of 10 patients.

The use of split-thickness dermal grafts for successful repair of corneal and scleral defects is reported in 10 patients (11 eyes) who had non-infectious, impending, or overt ocular perforation. In all patients, traditional methods of reconstruction were deemed inappropriate or had already failed. Corneo-scleral defects occurred after various operations: pterygium excision, retinal detachment repair, insertion of a keratoprosthesis (Cardona implant) into an opaque, vascularised cornea, and penetrating keratoplasty. Other causes of corneoscleral defects were scleromalacia perforans, idiopathic systemic vasculitis, alkali burn, ocular cicatricial pemphigoid, and band keratopathy with recurrent erosion following intraocular metallic foreign body. We propose the use of split-thickness grafts: (1) when adjacent conjunctiva is inadequate to cover a corneoscleral defect owing to its large size or great depth or to conjunctival scarring from previous operations, injury, or ocular cicatricial pemphigoid; or (2) as an alternative to autogenous grafts such as conjunctiva, cartilage, fascia lata, tibial periosteum, or mucous membrane as well as to homologous scleral and lamellar grafts. Dermal grafts are advantageous in that they are autogenous, non-antigenic, survive on avascular surfaces, and self-epithelialise and, thus, need not be covered by conjunctiva. Furthermore, they are pliable, have excellent tensile strength, provide ample tectile support, and are abundantly available. Dermal grafts are harvested from the dermal bed of the thigh after an epidermal flap is hinged at one end.

Adult↗

Histopathological changes in the eyes in systemic lupus erythematosus: an electron microscope and immunohistochemical study.

This paper reports the histopathological findings in the eyes of a 26-year-old female patient diagnosed with systemic lupus erythematosus (SLE) with peripheral neuropathy. The patient had no significant ocular problems. She died of pneumonia after two years of suffering. The eyeballs were procured at autopsy and the retina, choroid and optic nerve processed for light and electron microscopy, and immunohistochemistry for immunoglobulin G (IgG), glial fibrillary acidic protein (GFAP), calbindin and parvalbumin. Histologically, there was haemorrhaging in the retinal nerve fibre layer. Ultrastructurally, the axons of this layer were swollen, and contained an unusual accumulation of microtubules and smooth endoplasmic reticulum. There were degenerative changes in the pericytes and smooth muscle cells of blood vessels. The capillary lumen was partially obliterated, and contained IgG, which was also detected throughout the choroid and wall of choroidal arterioles. The latter and Bruch's membrane showed fibrin deposits. The optic nerve showed infiltrated mononuclear cells near the degenerated axons, these axons lacked immunoreactivity to calbindin and parvalbumin. Compared to the control, the connective tissue sheaths of the central retinal vessels possessed a vast number of proliferated fibroblast cells, and trichrome staining showed transmural vessel scarring. Dense GFAP immunoreactivity was observed surrounding the vessel wall. These pathological changes are due to impaired blood circulation caused by haemorrhaging and vasculitis, and vessel occlusion by fibrin. The nature of the changes observed tends to indicate that a regular, thorough ophthalmic examination should be conducted even in the absence of significant ocular symptoms in SLE.

Adult↗