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PubMed1979

Vascular obstructive alterations related to chorio-retinitis.

Different stages of retino-choroiditis, many of toxoplasmic origin, may develop vascular alterations. One of these pictures has been observed in relation to old scars of retino-choroiditis, where focal vasculitis may affect the venous return circulation sometimes leading to intraocular hemorrhages and retinal holes of variable severity. The natural history of this process is described, as well as the clinical characteristics and the results of treatment. Generally, the disease has a favorable course and a good prognosis. 18 cases are reported.

Adult↗
PubMedDate not supplied

Optic disk vasculitis associated with chronic active Epstein-Barr virus infection.

PURPOSE: To report two cases of optic disk vasculitis associated with chronic active Epstein-Barr virus infection (CAEBV). METHOD: We examined the eyes of two patients with CAEBV. RESULTS: In the first case, a 6-year-old boy, visual acuity was 20/20 in both eyes. Papillary and peripapillary exudates were observed in the right eye. Fluorescein angiography showed hyperfluorescence of the optic disk and a leakage from the peripapillary retinal vessels in the right eye. Two months later, the exudates increased and preretinal hemorrhages appeared in the right eye. Visual acuity decreased to 20/60. He was treated with systemic administration of corticosteroid, globulin and acyclovir. Visual acuity returned to 20/20, but peripapillary exudates remained in the right eye. In the second case, a 16-year-old girl, visual acuity was 20/20 in both eyes. The right eye showed optic disk swelling and dilated retinal veins. Fluorescein angiography showed hyperfluorescence of the optic disk but no leakage from the retinal vessels. Visual field examination revealed an enlarged blind spot in the right eye in both cases. These ocular manifestations are compatible with those of optic disk vasculitis, which shows swelling of the optic disk and dye leakage on and around the optic disk in fluorescein angiography, with an almost normal visual acuity and an enlargement of the blind spot. CONCLUSION: Persistent Epstein-Barr virus infection may cause optic disk vasculitis.

Acyclovir↗
PubMed1979

Evidence for vasculitis in acute posterior multifocal placoid pigment epitheliopathy.

A case of acute posterior multifocal placoid pigment epitheliopathy is presented with the unusual feature of the exposure of deep choroidal vessels which filled with dye in the early phase of the fluorescein angiogram in the center of many of the placoid lesions. Visualization of these vessels with surrounding hypofluorescence implies nonperfusion of choriocapillaris presumably due to inflammation, rather than retinal pigment epithelial blockage. The pathology of acute posterior multifocal placoid pigment epitheliopathy is probably a vasculitis of the choriocapillaries, which causes transient occlusion of these vessels and the initial hypofluorescence in the typical lesions during angiography. In many lesions the ischemia is mild enough to cause only temporary disturbance of visual function with subsequent recovery. Only in the center of some of the lesions is the inflammation severe enough to allow for the findings observed in the case presented and to account for the permanent visual defects found in some patients.

Adult↗
PubMed1994

Antiphospholipid antibodies in retinal vascular occlusions. A prospective study of 75 patients.

OBJECTIVE: To assess the prevalence of antiphospholipid antibodies in patients with occlusive retinal vascular disorders. PATIENTS: Seventy-five consecutive patients (44 with central retinal vein occlusions, 24 with branch venous occlusions, five with vasculitis plus branch venous occlusion, and two with arterial occlusions) were screened for antiphospholipid antibodies and compared with a control group composed of outpatients with similar systemic vascular disorders. RESULTS: The antibody assay for one patient was positive for lupus anticoagulant and the antibody assay for three other patients was positive for anticardiolipin antibodies. These four patients had central or branch retinal vein occlusion and presented with several vascular risk factors. Comparison of the retinal vascular occlusion and the control groups showed no difference in the levels of anticardiolipin antibodies or lupus anticoagulant. CONCLUSIONS: Antiphospholipid antibodies did not seem to be a feature of retinal vein occlusion, but in rare cases (5%) they may contribute to the occlusive phenomenon. A systematic screening does not seem to be justified, but it may be valuable to test for antiphospholipid antibodies in patients without conventional risk factors and in patients with clotting screen abnormalities, particularly if associated with lupus-like syndrome or other elements of the primary antiphospholipid syndrome.

Adult↗
PubMed2002

Acute posterior multifocal placoid pigment epitheliopathy and scleritis in a patient with pANCA-positive systemic vasculitis.

PURPOSE: To present acute posterior multifocal placoid pigment epitheliopathy and scleritis occurring in association with pANCA (perinuclear antineutrophil cytoplasmic antibody)-positive systemic vasculitis. DESIGN: Interventional case report. METHODS: A 69-year-old woman developed bilateral acute posterior multifocal placoid pigment epitheliopathy concurrent with rapidly progressive glomerulonephritis and bronchioalveolitis in both lungs. RESULTS: The patient showed a high titer of pANCA (myeloperoxidase-ANCA). She then developed scleritis in the left eye and multiple gastric ulcers. CONCLUSION: Acute posterior multifocal placoid pigment epitheliopathy and scleritis in this patient are manifestations that are part of pANCA-positive systemic vasculitis. This association further supports that acute posterior multifocal placoid pigment epitheliopathy is caused by vasculitic inflammation of the choriocapillaris.

Acute Disease↗
PubMed2006

Combined central retinal artery and vein occlusion in Churg-Strauss syndrome: case report.

PURPOSE: To describe a rare case of Churg-Strauss syndrome presenting with severe visual loss due to a combined central retinal vein and artery occlusion. METHODS: A 42-year old man with a medical history of asthma and blood hypereosinophilia developed a sudden loss of vision in his right eye. We describe the clinical features and evolution of the case after treatment. RESULTS: A combined occlusion of the central retinal artery and central retinal vein was diagnosed by the funduscopic appearance of retinal whitening, macular cherry-red spot, papilloedema, retinal haemorrhages in all four quadrants and dilated and tortuous veins. The diagnosis was confirmed by a fluorescein angiogram showing absence of retinal filling and normal choroidal filling. Churg-Strauss syndrome was diagnosed based on the necessary presence of four of six criteria for the disease proposed by the American College of Rheumatology. Corticosteroid therapy was initiated. However, during the following year when tapering off the daily dosage, the patient experienced two relapses, with pulmonary symptoms and hypereosinophilia, and the corticosteroid dosage had to be augmented. The patient presented with neovascular glaucoma 7 weeks after the vascular occlusion and experienced no visual improvement. CONCLUSION: Combined central retinal artery and vein occlusion can occur in Churg-Strauss syndrome. We suggest that regional vasculitis may be the pathological mechanism underlying the vascular occlusions observed in our case. The condition carries a very poor prognosis for vision, due to the resulting retinal ischaemia, and a poor general prognosis due to the late stage of the systemic disease. Corticosteroids should be instigated promptly in order to prevent further systemic or ocular vasculitis.

Adult↗
PubMed2000

Retinal disease in patients with systemic lupus erythematosus.

OBJECTIVE: To investigate the incidence of retinopathy in systemic lupus erythematosus (SLE) and to clarify its significance in relation to other clinical manifestations. METHODS: A cross sectional study on lupus retinopathy was made in 69 patients with SLE. One expert ophthalmologist examined the ocular fundi of the lupus patients without any information of their disease state. Clinical and laboratory findings in the patients with retinopathy and those without were compared. RESULTS: Retinopathy was found in 7/69 (10%) patients. The findings included haemorrhages, vasculitis, cotton wool spots, and hard exudates, all of which were considered to reflect vascular damage. Retinopathy was found to be associated with the presence of anticardiolipin antibody (p<0.05) and with central nervous system lupus (p<0.01). The patients with retinopathy had higher levels of serum creatinine than the patients without retinopathy (p<0.01). The disease activity of lupus, as assessed by the maximum SLE disease activity index (SLEDAI) score of the patients, was also significantly higher in the patients with retinopathy (p<0.03). CONCLUSION: Incidence of retinopathy in SLE was similar to that in previous reports and it may reflect tissue microangiopathy, particularly associated with vasculitis or anticardiolipin antibodies, or both.

Adult↗
PubMed1996

[Rhegmatogenous retinal detachment in atrophia gyrata: pars plana vitrectomy with silicone oil instillation].

BACKGROUND: Gyrate atrophy is a form of choroidal dystrophia characterized by a genetic defect of the mitochondrial matrix enzyme ornithine ketoacidoaminotransferase on chromosome 10. Apart from rare myopathic problems, cases of choroidal, iridal atrophy, complicated subcapsular cataract, optic atrophy, macular edema, reticular retinoschisis, vasculitis and perivasculitis could be observed so far. To our knowledge, rheumatogenous retinal detachment has been described before. We present a 34-year-old female subject with a retinal detachment of the right eye over 3 quadrants with 3 small round foramina in the area 10 to 11 o'clock. PATIENTS: Concentric defects of the visual field had been known since the age of 4; the patient was slightly myopic. At the age of 31, the patient had undergone cataract extraction with implantation of posterior chamber lenses in both eyes. The ophthalmological examination revealed large areas of choroidal atrophia in both eyes. A determination of visual acuity resulted in hand movements in the right eye and 0.1 in the left eye. The ERG was extinguished. RESULTS: The retina could be fully reattached by pars plana vitrectomy and instillation of silicone oil. At the same time, we performed an excision of secondary cataract. On demission, the visual acuity of the right eye was finger counting. Because a brother of the patient had also suffering from a progressive choroidal atrophy since his childhood, an autosomal recessive disease was suspected. The only abnormality detected was strongly elevated plasma levels of ornithin (7.5 to 14 mg/dl, compared to levels of 0.6 to 2.0 in controls). Urinary excretion of ornithin was also strongly increased. DISCUSSION: To our knowledge there was no previous description of a case of atrophia gyrata in association with rhegmatogenous retinal detachment after intraocular surgery. As the pigment epithelium is missing, intraoperative endolaser-coagulation would be useless. For this reason, and since a tendency for reattachment is lacking consistent with one of the characteristics of this disease, the only approach to this case is intraocular tamponade with silicone oil.

Adult↗
PubMed2004

Posterior scleritis in Cogan's syndrome.

PURPOSE: To describe an unusual case of atypical Cogan's syndrome with features of posterior scleritis. METHODS: Interventional case report. RESULTS: A 53-year-old man presented with bilateral posterior scleritis, along with sensorineural hearing loss and systemic vasculitis. Systemic corticosteroid therapy was started with resolution of ophthalmic findings and improvement of vestibuloauditory symptoms. CONCLUSIONS: In the setting of atypical Cogan's syndrome, ophthalmic manifestations may be the first sign of disease.

Administration, Oral↗
PubMed1996

[Vision disorders in inflammatory-rheumatic diseases].

The association of visual disturbances and rheumatic disease has been known for centuries. This review provides a synopsis of the ocular conditions that are associated with inflammatory rheumatic disease. The major ophthalmic manifestations of the rheumatic diseases include keratoconjunctivitis sicca, ulcerative keratitis, scleritis, uveitis, retinal vascular disease, and neuro-ophthalmic lesions. Each of these ocular conditions is most characteristically associated with a few, but not all, of the rheumatic disorders. Scleritis, for example, is most often seen with rheumatoid arthritis or with vasculitis. Acute anterior uveitis is most often seen with the seronegative spondylarthropathies. Retinal vascular and neuro-ophthalmic lesions are seen with disorders having either a vaso-occlusive component, such as systemic lupus erythematosus, or with one of the vasculitides. Important considerations for a successful collaboration between ophthalmologists and physicians/rheumatologists are discussed.

Adult↗
PubMed1979

Microangiopathy of the brain and retina.

Two women (26 and 40 years old) developed an unusual microangiopathy that affected the brain and retina. Psychiatric symptoms initially overshadowed the subacute features of the progressive neurologic disorder. Ophthalmoscopic findings of multifocal branch retinal artery occlusions provided clinical evidence of vasculopathy. Laboratory data did not reveal evidence of the known vasculitides, including systemic lupus erythematosus (SLE) and syphilis. Cerebral angiography suggested vasculitis in the younger patient. Brain biopsy in the older patient (after 3 months of steroid therapy) revealed a sclerosis of the small pial and cortical vessels that was consistent with a "healed" angitis. Both patients seemed to respond to steroid therapy.

Adult↗
PubMed2005

Parry-Romberg syndrome.

The authors report an unusual case of Parry-Romberg syndrome (PRS). Magnetic resonance imaging and computed tomography scan of the craniofacial region and surgical correction of enophthalmos were performed. Results after the operative intervention included persistent palpebral edema and ecchymosis and transient choroid vasculitis of the right eye, highlighted with echography and fluorescein angiography. The fundus oculi examination showed retinal choroid folds. Immunological test results were weakly positive. The authors conclude enophthalmos, associated with right side hemi atrophy, and the transient choroids vasculitis support the diagnosis of PRS. Furthermore, it is suggested the case had an autoimmune etiology, rather than a hemi facial asymmetry caused by a facial trauma that occurred in puberty.

Adult↗
PubMed1992

Branch retinal vein occlusion and its management.

The natural course of Branch Retinal Vein Occlusion is determined by the site and completeness of the occlusion, the integrity of arterial perfusion to the affected sector and the efficiency of the developing collateral circulation. Most patients with tributary vein occlusion have some capillary fall out and microvascular incompetence in the distribution of the affected retina and vision is significantly compromised in over 50% of patients who have either chronic macular oedema or ischemia involving the perifoveolar arcades. Retinal and capillary vasoproliferation with or without intraretinal or preretinal membrane formation are common sequelae, where inner retinal ischemia is extensive and vitreous hemorrhage is often a distinct threat to vision. Treatment of the sequelae of vein occlusion are largely centered on laser photocoagulation to diseased retina to limit fluid accumulation and abolish preretinal and papillary neovascularization. Vitrectomy and retinal detachment procedures are occasionally required in patients with uncontrolled vitreous hemorrhage and retinal membrane formation which threatens the integrity of the macula. A small proportion of patients with vein occlusion subsequent to vasculitis require medical therapy.

Edema↗
PubMed1991

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.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Acute posterior multifocal placoid pigment epitheliopathy.

The pathogenesis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is not fully understood. Some believe it to be a primary dysfunction of the retinal pigment epithelium while others assume that its development follows a choroidal vasculitis. It generally presents as a bilateral condition and shows no sex predilection. The visual prognosis is generally good, though severe and permanent visual loss occasionally occurs. The funduscopic presentation consists of multiple flat, yellow-white lesions of the posterior pole at the level of the retinal pigment epithelium. This paper discusses a case of APMPPE and reviews and summarizes the current literature on this disorder.

Acute Disease↗
PubMed2001

[Retinal thrombosis in young patients. Immunological and clinical aspects].

OBJECTIVES: To ascertain preexisting medical conditions, clinical evolution of retinopathy, and associated immunological disorders in a series of young patients suffering from retinal thrombosis, and to determine the prevalence of antiphospholipid antibodies. METHODS: Twenty two patients younger than 50 years, who had presented an acute retinal thrombotic episode, were studied prospectively with a general physical, ophthalmoscopic and immunological examination, placing special emphasis on the detection of antiphospholipid antibodies (anticardiolipin antibodies and lupus anticoagulant). RESULTS: No baseline disease stood out significantly over the others, and the most frequent risk factor found was systemic arterial hypertension (5/22%). No associated risk factor was found in nine cases (41%), and more than two factors were found in six cases (27%). Most of the vascular occlusions affected the venous vessels (18/81%), and five of them were associated with vasculitis. The ophthalmologic follow-up showed a rapid evolution to retinal neovascularization in 11 cases. Our data show many immunologically altered values, there being nine cases (41%) of the series with more than four parameters altered. The antiphospholipid assay showed a high prevalence of anticardiolipin antibodies (5/23%), and two patients were diagnosed of primary antiphospholipid syndrome. The lupus anticoagulant was negative in all patients. CONCLUSIONS: The high prevalence of anticardiolipin antibodies and immunologic abnormalities found in the retinal thrombosis younger patients leads us to recommend the systematic immunological study in these subjects. It has relevant diagnostic and therapeutic implications in a population with no evident associated risk factors and a greater severity of retinopathy.

Adult↗
PubMedDate not supplied

The evaluation of patients with Eales' disease.

Thirty-two patients with documented Eales' disease who have been followed-up at Johns Hopkins Hospital for periods of as long as 37 years were investigated. The previously reported association of Eales' disease with tuberculoprotein hypersensitivity was confirmed in 10 of 21 patients tested. For the first time, associated vestibuloauditory dysfunction is described. The previously reported association with multiple sclerosis was not confirmed. Eales' disease is a specific vasculopathy that can affect both the retinal and vestibuloauditory circulations. Patients with suspected Eales' disease should also be examined to rule out other diseases such as diabetes, sarcoidosis, and connective tissue diseases including systemic lupus erythematosus. This can be done by obtaining such tests as fasting blood glucose, chest radiograph, erythrocyte sedimentation rate, antinuclear antibody, rheumatoid factor, and LE cell preparation. Tuberculoprotein sensitivity status should be ascertained and active tuberculosis should be ruled out. The patient should be questioned regarding hearing or balance problems, and if a history suggestive of abnormalities is elicited, referral for vestibuloauditory function testing should be made.

Adult↗