Leyla S. Atmaca

Fundus changes associated with Behçet's disease

Between 1973 and 1987 we recorded 300 cases of Behçets disease with fundus changes. Our routine preview of each case included examination with the Goldman three-mirror contact lens, ophthalmoscopy, referral for laboratory tests, color fundus photography and, most importantly, fluorescein angiography. The ophthalmological findings led to the proper diagnosis in 254 (85%) of our cases. Fluorescein angiography revealed incipient fundus changes in 38 eyes of 19 medically diagnosed patients with no visual complaints and normal fundi on ophthalmoscopy. Eighty-six percent of our cases had or developed diverse fundus changes bilaterally. The fundus changes we encountered most frequently were hyperemia of the optic disc, macula edema, retinal edema, vascular sheathing, retinal exudate, and retinal hemorrhage. With the exception of eyes with very poor prognoses, only 184 of the 403 eyes that were followed up for 3–120 months (median 12) experienced new insults in the posterior segment; 40 of these became totally blind while 59 maintained their visual status. Early diagnosis is necessary for proper treatment patients with Behçets disease. Fluorescein angiography is required for early diagnosis and for monitoring the posterior segment involvement closely. This is the most characteristic and often the most serious consequence of Behçets disease; it may also the first indicator of the disease.

Clinical Features and Prognosis in Ocular Toxoplasmosis

PurposeTo evaluate retrospectively the clinical characteristics, complications, and prognosis in patients with ocular toxoplasmosis.Patients and MethodsWe reviewed the records of 189 patients (243 eyes) with ocular toxoplasmosis who were examined between 1972 and 1999. Color fundus photography and, in some patients, fluorescein angiography and indocyanine green angiography were performed. There were 98 male (52%) and 91 female (48%) patients with a mean age of 22.8 ± 8.9 years.ResultsOf the patients, 140 (74%) had congenital and 49 (26%) had acquired toxoplasmosis. At the initial examination, there were active lesions in 65 eyes and inactive lesions in 178 eyes. Active lesions included retinochoroiditis in 59 (91%), papillitis in 2 (3%), and neuroretinitis in 4 (6%) eyes. There was also an inactive scar in 17 eyes with active retinochoroiditis. Localisation of the active retinochoroiditis was the macula in 44 (74%), the macula and peripheral retina in 3 (5%), the peripheral retina in 9 (15%) and the peripapillary retina in 3 (5%) eyes. Optic atrophy, pigment epithelial detachment, choroidal neovascularization, lamellar macular hole, and retinal neovascularization were seen during the follow-up period.ConclusionsOcular toxoplasmosis commonly affects the macula and seriously impairs visual acuity. The prevention of acquired and congenital infections is very important in controlling ocular toxoplasmosis. Patients should be followed to avoid late complications.

The Efficacy of Cyclosporin-A in the Treatment of Behçet's Disease

We used cyclosporin A (CA) to treat 14 patients (25 eyes) with Behçets disease characterized by severe retinal vasculitis and active intraocular inflammation. All of the patients had been treated previously with corticosteroids, colchicine, and immunosuppressives, without satisfactory results. The patients were given an initial oral dose of 5 mg/kg/day. All medication was tapered and eventually completely stopped after 12 months. The number and severity of ocular attacks (anterior and/or posterior uveitis with vitreous haze) were significantly reduced below pretreatment levels during therapy (Kolmogorov Smirnov two-pair test: P < .05). Visual acuity improved in 32% and remained unchanged in 44%. No ocular attacks recurred in 11 eyes; they recurred 1 time in 8 eyes; 2 times in 3 eyes; and 3 times in 3 eyes during CA therapy. There were no significant changes in the level of retinal vasculitis. In two cases with vaso-obstructive changes, treatment with CA was not effective and laser photocoagulation was performed. Based on our study, we recommend that an initial dose of 5 mg/kg/day CA be used in the systemic medical treatment of ocular Behçets disease. The CA can be continued at this low dosage for an unspecified time. If intraocular inflammation does not totally resolve at this dosage or the inflammatory process recurs, combining the CA with low doses of a steroid should be considered.

Retinal and disc neovascularization in Behçet's disease and efficacy of laser photocoagulation

Abstract⊎ Background: The vasoocclusive episodes resulting from Behçets disease can cause capillary dropout and vascular remodeling. Retinal and disc neovascularizations, which occur as a result of occlusive vasculitis, can cause recurrent vitreal hemorrhages and neovascular glaucoma leading to severe visual impairment. ⊎ Methods: 1080 eyes of 540 patients with Behçets disease were examined between 1973 and 1993. Of the 912 eyes with posterior segment involvement, laser photocoagulation could be performed in 13 of 25 eyes with disc neovascularization (NVD), 12 of 22 eyes with retinal neovascularization (NVE), and 4 of 6 eyes with NVD and NVE. Laser was directed at areas of NVE and retinal capillary nonperfusion. In cases of NVD, panretinal photocoagulation was performed. ⊎ Results: The rate of regression of NVD was significantly greater in laser-treated eyes than in the untreated group. The results were similar in cases of NVD with NVE. In eyes with NVE which underwent laser photocoagulation, the NVE regressed. None of the treated eyes developed neovascular glaucoma during the follow-up period. Vitreous hemorrhage occurred in two laser-treated eyes. ⊎ Conclusion: Laser photocoagulation is successful in preventing complications of retinal and disc neovascularizations. Thus, in cases of occlusive vasculitis associated with Behçets disease, laser photocoagulation should be considered for prevention of complications such as vitreous hemorrhage and neovascular glaucoma.

A long-term follow-up of Eales’ disease

Purpose: To provide long-term follow-up information on Eales’ patients. Methods: Eales’ patients, who had been examined at varying periods between the years 1970 and 1991 with a minimum five-year follow-up, were included in the study. Results: A total of 130 patients were followed up for a minimum of five and a maximum of 26.5 years. The retinal lesions found during the first examination included vascular sheathing, disc and/or retinal neovascularization, vitreous hemorrhage, branch retinal vein occlusion, retinitis proliferans, and retinal detachment. Visual acuity improved in 37 (20%) of the 185 treated eyes, was maintained in 79 (43%), and worsened in 69 (37%). The complications were tractional detachment, cataract, rubeosis iridis, neovascular glaucoma, and phthisis bulbi. Conclusion: The most important elements in dealing with Eales’ disease are periodic follow-up, a good and adequate laser treatment, pars plana vitrectomy combined with procedures for nonclearing vitreous hemorrhage, and retinal vasoproliferative changes to stabilize the retinal lesions and maintain functional levels of vision.

Behçet Disease in Children

Purpose: To investigate the incidence and clinical characteristics of Behçet disease in children. Methods: The authors retrospectively reviewed the charts of 3382 patients with Behçet disease from October 1986 to December 2005 at Ankara University Medical School Behçet Unit and/or Atmaca private clinic. Results: 110 children were diagnosed with Behçet disease. The 110 children represented 3.3% of the total number of Behçet patients that were reviewed. 62.7% were girls and 37.3% were boys. The mean age at initial manifestation was 11.63 ± 3.46 years and at diagnosis was 14.15 ± 2.13 years. Ocular involvement was found in 30.9% of the children. Of these, 61.8% were bilateral and 38.2% were unilateral. Anterior uveitis was found in 18 eyes (32.8%), posterior uveitis in 24 eyes (43.6%), and panuveitis in 13 eyes (23.6%). Conclusion: Behçet disease should be considered in the differential diagnosis of childhood uveitis, particularly in endemic areas. The collaboration of pediatricians, rheumatologists, dermatologists, ophthalmologists, and other specialists when necessary is required in the diagnosis and management of children with Behçet disease.

Early and late visual prognosis in solar retinopathy.

Abstract• Background: Solar retinopathy was observed in a total of 86 eyes of 58 patients following the solar eclipse over Turkey in April 1976. The visual prognosis and the presence of late complications were evaluated at the early and late periods. • Methods: Of the 58 patients, 34 (51 eyes) presented during the first week and came for follow-up examination in the succeeding week, also after 1, 3, 12 and 18 months. After that they were examined at yearly intervals (mean 4.2 years). Twenty-four patients (35 eyes) presented during the period between 1 and 11 years post-eclipse and were followed up for a mean period of 3.4 years. After a period of 15 years, all of the patients were invited for re-examination and nine patients (14 eyes) attended. • Results: The improvement in visual acuity was observed to have taken place mostly during the first 2 weeks to 1 month after the eclipse. Further improvement in visual acuity was not observed in any of the eyes after the 18-month examination. The improvement in visual acuity was more prominent and earlier in the eyes that had visual acuity of 0.2 or better initially. Only the eyes with initial visual acuity equal to or better than 0.4 had a chance to improve their acuity to 10/10. Having observed the 51 eyes for mean period of 4.2 years and the 35 eyes for 3.4 years, no change in visual acuity was observed. Among the total of 86 eyes, 9 were found to have pseudolamellar macular holes. • Conclusion: Correlation was found between initial visual acuity and the funduscopic appearance after the 2nd week. Fluorescein angiography was not found to be a conclusive test in solar retinopathy. No late complications were observed.

Pseudoxanthoma elasticum: a rare cause of hypertension in children.

Abstract. We describe an 11-year-old girl with severe renin-dependent hypertension. She had characteristic cutaneous, ocular, and vascular signs of pseudoxanthoma elasticum (PXE), and the diagnosis was confirmed histologically. Because PXE is a heterogeneous heritable disorder, the family was evaluated for this condition. Since the patient’s mother and brother showed some manifestations of PXE, autosomal dominant inheritance is the most likely inheritance pattern in this family.

Evaluation of the Optic Nerve Head with the Heidelberg Retina Tomograph in Diabetes Mellitus

Background: To evaluate the optic nerve head by means of the Heidelberg retina tomograph (HRT) in patients with diabetes mellitus (DM). Methods: The study group consisted of 47 patients with DM [group 1; 29 patients without diabetic retinopathy (DR) and 18 patients with non-proliferative DR] and 50 normal subjects (group 2). All patients and controls underwent a complete ophthalmological examination, and the optic nerve head topography of both eyes was evaluated by using HRT-I. One eye of these cases was randomly selected for statistical analysis. Glycosylated haemoglobin (HbA1c) levels of all the study participants were measured. Results: The HRT parameters were similar between diabetic and control groups (p > 0.05). In group 1, in the patients with duration of diabetes ≤10 years, when compared with the patients with duration of diabetes >10 years, we did not detect any statistically significant difference between the HRT parameters (p > 0.05). There was no statistically significant correlation between HBA1c levels and rim volume (r = –0.078, p = 0.601), and mean retinal nerve fibre layer thickness (r = 0.058, p = 0.700) in DM patients. Conclusion: These results suggest that non-glaucomatous diabetic patients had no decreased neuroretinal rim when compared with non-diabetic patients.

EALES DISEASE: Medical, Laser, and Surgical Treatments

Eales disease is an idiopathic retinal vasculitis that affects predominantly young men. The major manifestations of Eales disease are the inflammation of retinal veins with or without arteritis papillitis anterior posterior uveitis or pars planitis. The prognosis usually depends on the degree of vitreous macular circulatory failure proliferative retinopathy. The involvement is mostly bilateral at the peripheral retina. The fundus lesions that can be observed vary among retinal neovascularization (fig.1) vascular sheathing (fig.2) retinal vitreous hemorrhage retinitis proliferans (fig.3) disc neovascularization (fig.4) branch central retinal vein occlusion tractional retinal etachment rubeosis iridis neovascular glaucoma. In addition to these lesions the engorged tortous retinal veins leakage from these veins macular retinal edema shunt vessels hypoxic areas microaneurysms (fig. 5) can be seen more easily with fluorescein angiography.