Evaluation of Choroidal Thickness, Choroidal Vascularity Index and Peripapillary Retinal Nerve Fiber Layer in Patients with Juvenile Systemic Lupus Erythematosus.

OBJECTIVE: The aim of this study was to conduct a detailed ophthalmological examination in children with systemic lupus erythematosus (jSLE), including choroidal thickness (ChT), choroidal vascularity index (CVI) and peripapillary retinal nerve fiber layer (RNFL). METHODS: The study included all jSLE patients ( n = 21) diagnosed according to the Systemic Lupus International Collaborating Clinics classification criteria between January 2017 and April 2017, and an age- and gender-matched control group ( n = 21). The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was used to assess disease activity. After routine eye examinations, ChT at five points (750 µ and 1500 µ from the center of the fovea both in the temporal and nasal quadrants and under the fovea), total subfoveal choroidal area (TCA), luminal area (LA), stromal area (SA), CVI and RNFL thickness at the optic disc were evaluated. RESULTS: One patient had active ocular involvement in the form of episcleritis. Another patient had corticosteroid-induced cataract. The median age of the patients was 16 years (6-19 years). ChT at five points, TCA, LA and SA were found to be higher in patients with jSLE, whereas RNFL thickness and CVI were similar to those of the healthy control individuals. No correlation was determined between optical coherence tomography findings, SLEDAI and the immunological parameters (antinuclear antibodies, anti-double-stranded DNA, complements 3 and 4, extracted nuclear antigen antibody, antiphospholipid antibody). Intraretinal and subretinal fluid was not present in any of the patients. CONCLUSION: The choroid was thicker in patients with jSLE than in the control group. The study results suggest that jSLE may affect the choroid. Ophthalmological evaluation is important in SLE patients, even in the absence of relevant complaints.

Intravitreal pegaptanib sodium for choroidal neovascularisation secondary to age-related macular degeneration: Pan-European experience.

PURPOSE: To evaluate visual outcomes in patients with neovascular age-related macular degeneration (NV-AMD) who were treated with pegaptanib sodium in European clinical ophthalmology practices. METHODS: Thirteen centres in eight European countries participated in this retrospective study. Medical records for patients with any angiographic subtype of subfoveal choroidal neovascularisation secondary to NV-AMD with visual acuities (study eye) of 20/40-20/320 treated with 0.3 mg pegaptanib as first-line treatment and with at least 24 weeks of follow-up were identified. Anonymised data reflecting at least 24 and up to 54 weeks of follow-up were recorded. Primary end points were visual acuity outcomes at weeks 24 and 54 compared with those reported at week 54 in the vascular endothelial growth factor (VEGF) Inhibition Study in Ocular Neovascularisation (VISION) trial. RESULTS: In all, 253 patients were followed for at least 24 weeks; 62 patients completed 54 weeks of follow-up. A mean of 4.4 (SD, 1.8) pegaptanib injections were administered through 24 weeks. Compared with the VISION trial, the European experience showed that >90% of patients in the current cohort lost <15 letters from baseline at both time points compared with 70% in the VISION trial at 54 weeks. Pegaptanib was well tolerated with no reported cases of endophthalmitis, traumatic cataract, or iatrogenic retinal detachment. CONCLUSIONS: Pegaptanib was found to stabilise vision in a greater percentage of patients and produced greater overall visual improvement in this group of treatment-naive patients with NV-AMD compared with outcomes reported in the VISION trial; however, interpretation of these results should be tempered given the differences in design between this retrospective study and the prospective controlled trial.

Sympathetic ophthalmia associated with ocular and cerebral vasculitis: an angiographic and radiologic study.

PURPOSE: To describe a case of sympathetic ophthalmia (SO) associated with ocular and cerebral vasculitis. METHODS: The authors report a 38-year-old woman who presented with bilateral uveitis 7 years after a penetrating trauma to the right eye. Ocular examination included fundus fluorescein and indocyanine green angiography. Medical history disclosed an episode of dysarthria and right-sided weakness. RESULTS: Anterior uveitis was present in the previously injured pseudophakic right eye, which harbored a piece of glass stuck in the lower temporal iris. In the left eye, vitreal inflammation with retinal sheathing and subretinal lesions was predominant. Besides retinal vasculitis, fundus angiography showed choroidal vasculitis. Neurologic studies were compatible with cerebral vasculitis. CONCLUSIONS: In SO, choroidal vasculitis that is invisible clinically can be demonstrated angiographically. Although rare, inflammation can also affect the nervous system.

Scedosporium apiospermum chorioretinitis.

PURPOSE: A case of a 24-year-old immunocompetent woman who developed unilateral Scedosporium apiospermum chorioretinitis and diffuse cervical lymphadenitis 10 years after facial steroid injection is described. METHODS: The patient was initially treated for the misdiagnosis of tuberculous lymphadenitis. Contemporaneous to the enlargement of her lymph nodes, she experienced gradual loss of vision in her left eye. She subsequently underwent lymph node biopsy. RESULTS: Histopathologic sections stained with Grocott's methenamine silver showed branched septate hyphae and cultures on Sabouraud's agar yielded Scedosporium apiospermum. The patient received oral itraconazole 200 mg twice daily. Lymphadenitis regressed within a year and chorioretinitis scarred within two years. CONCLUSION: Scedosporium apiospermum may cause isolated chorioretinitis in an immunocompetent individual years after local surgery or trauma and result in significant visual morbidity. Treatment may require prolonged use of systemic itraconazole.

Retinitis pigmentosa associated with peripheral sea fan neovascularization.

PURPOSE: To describe a case with retinitis pigmentosa associated with sea fan type retinal neovascularization. METHODS: Complete ocular examination including fluorescein angiography was performed in a 9-year-old girl. RESULTS: Ophthalmoscopically, in addition to arteriolar narrowing and bone corpuscular pigmentation of both retinae, a vascular lesion with surrounding intraretinal exudation was noted in the upper equatorial region of the right eye. On fluorescein angiography, the lesion stained in the form of a sea fan neovascularization. CONCLUSION: Sea fan type of neovascularization can be seen in association with retinitis pigmentosa. Fluorescein angiography is important in identifying the exact nature of such a lesion.

Interferon alfa-2b, colchicine, and benzathine penicillin versus colchicine and benzathine penicillin in Behçet's disease: a randomised trial.

BACKGROUND: Sight-threatening eye involvement is a serious complication of Behçet's disease. Extraocular complications such as arthritis, vascular occlusive disorders, mucocutaneous lesions, and central-nervous-system disease may lead to morbidity and even death. We designed a prospective study in newly diagnosed patients without previous eye disease to assess whether prevention of eye involvement and extraocular manifestations, and preservation of visual acuity are possible with combination treatments with and without interferon alfa-2b. METHODS: Patients were randomly assigned 3 million units interferon alfa-2b subcutaneously every other day for the first 6 months plus 1.5 mg colchicine orally daily and 1.2 million units benzathine penicillin intramuscularly every 3 weeks (n=67), or colchicine and benzathine penicillin alone (n=68). The primary endpoint was visual-acuity loss. Analysis was by intention to treat. FINDINGS: Significantly fewer patients who were treated with interferon had eye involvement than did patients who did not receive interferon (eight vs 27, relative risk 0.21 [95% CI 0.09-0.50], p<0.001). Ocular attack rate was 0.2 (SD 0.62) per year with interferon therapy and 1.02 (1.13) without interferon therapy (p=0.0001). Visual-acuity loss was significantly lower among patients treated with interferon than in those without interferon (two vs 13, relative risk 0.13 [95% CI 0.03-0.60], p=0.003). Arthritis episodes, vascular events, and mucocutaneous lesions were also less frequent in patients treated with interferon than in those not receiving interferon. No serious side-effects were reported. INTERPRETATION: Therapy with interferon alfa-2b, colchicine, and benzathine penicillin seems to be an effective regimen in Behçet's disease for the prevention of recurrent eye attacks and extraocular complications, and for the protection of vision.

Clinical features of pediatric Behçet's disease.

OBJECTIVE: The aim of this study is to determine the clinical features and outcome of Behçet's disease in the pediatric age group. METHOD: Twenty cases within a pediatric age group whose ages differ from 5 to 15 years were studied retrospectively to determine the age of onset, the initial signs, and the progress of the disease. RESULTS: In 18 of the patients whose average age were 13.3 years (5 to 18 years), the initial symptoms associated with Behçet's disease were oral aphtous lesions or genital ulcers. Among them, 16 developed ocular symptoms in a later stage, at an average of 15.5 years of age (11 to 18 years). Posterior uveitis was the most common manifestation, detected in 75% of the cases. Three patients had neurobehçet's disease, and the neurologic manifestations were dural thrombosis, pseudotumor cerebri, and quadriparesis. CONCLUSIONS: In this study, after an average 4 years' follow up (6 months to 13 years) the clinical progress of Behçet's disease in the pediatric age group was similar to that found in adult disease. Both the ocular signs (i.e., cataracts and glaucoma) and the systemic features (i.e., aphtous stomatitis, genital ulcers, erythema nodosum, arthritis, and neurologic signs) were manifested at the onset or during the progress of the disease.

Management of moderate to advanced Coats' disease.

Sixteen patients (16 eyes) with Coats' disease who had either total bullous exudative retinal detachment or macular involvement and/or at least two quadrants of exudative retinal detachment were followed for a mean period of 60.6 months. Three patients with no light perception received no treatment. Ten eyes received cryotherapy on one or more occasions and two had laser photocoagulation. One eye underwent subretinal fluid drainage, intraocular infusion and cryotherapy. In those patients who could be tested, all the treated eyes retained their initial visual acuities. All 16 eyes had an acceptable cosmesis and none progressed to painful neovascular glaucoma or phthisis bulbi. Patients with late onset disease had a more benign course.

Paraneoplastic retinopathy associated with metastatic cutaneous melanoma of unknown primary site.

PURPOSE: To describe further the clinical and immunological features of cutaneous melanoma-associated retinopathy, which is an infrequent form of paraneoplastic syndrome. METHODS: We studied the salient clinical and immunological aspects of a 66-year-old man with metastatic cutaneous melanoma to lymph nodes of unknown primary site who developed melanoma-associated retinopathy. RESULTS: There was gradual loss of vision in the left eye. Colour vision and night vision were not affected. Visual fields showed arcuate defects. A full-field electroretinogram demonstrated attenuation of the b-wave amplitude in the left eye. The a-wave was intact. Indirect immunofluorescence techniques showed that the antibody reactions took place mainly in the outer plexiform layer of the retina. CONCLUSIONS: Bipolar cells seem to be the target in melanoma-associated retinopathy. Contrary to previous reports, night blindness may not be a universal finding.

Cerebellar ataxia associated with hypogonadotropic hypogonadism and chorioretinopathy: a poorly recognized association.

We report a male with cerebellar ataxia, hypogonadism and chorioretinopathy. The age of onset was 12. The parents were first cousins. Endocrinologic studies demonstrated hypogonadotropic hypogonadism due to pituitary dysfunction. The ocular disorder involved the choriocapillaris and the retina. The association may represent a separate syndrome, seldom recognized in the past.

Eales disease with internuclear ophthalmoplegia.

Eales disease is an idiopathic type of retinal perivasculitis characterized by recurrent retinal and vitreous hemorrhages. Neurologic involvement is rare. We report the case of a patient with Eales disease who had internuclear ophthalmoplegia thought to be a neurologic manifestation of this disease.

Long-term effects of cyclophosphamide and colchicine treatment in Behçet's disease.

We assessed the long-term effects of cyclophosphamide and colchicine on visual prognosis and attack frequency in 64 patients with Behcet's disease who were studied in two separate groups between the years of 1976 and 1989. No statistically significant positive changes in visual acuity or attack frequency which might be attributed to the treatment could be shown when the period of treatment was compared with the period after treatment.

Punctate inner choroidopathy and its differential diagnosis.

The diagnosis of chorioretinal diseases largely depends on their clinical presentation rather than etiologic or systemic investigations. We report on a patient whose examination revealed a peculiar fundus pattern, almost completely different from disorders such as multifocal choroiditis, presumed ocular histoplasmosis, myopic degeneration, acute multifocal placoid pigment epitheliopathy, vitiliginous chorioretinitis, and punctate outer retinal toxoplasmosis. It resembled punctate inner choroidopathy, described by Watzke in 1984.

Symmetry of disciform scars in bilateral age-related macular degeneration.

The size of the final macular scar in subretinal neovascularisation (SRNV) is one of the most important determinants of final visual function in patients with subfoveal disease. We studied patients with bilateral macular scars from age-related subretinal neovascular membranes retrospectively in order to determine whether or not fellow eyes behave similarly. We found a significant correlation between eyes in terms of final scar size (r = 0.50, p less than 0.01). We found that 50% of fellow eyes with large macular scars (greater than 3 x 10(6) microns2) had similar sized lesions, while only 16% of fellow eyes with small macular scars (less than 0.5 x 10(6) microns2) had large scars (p less than 0.01). We discuss the significance of these findings in relation to the pathogenesis of subretinal neovascular membranes, and their implications for treatment.