A. Tülin Berk

Ocular findings in 55 patients with Down's syndrome

Fifty-five patients with Downs syndrome were examined to evaluate the characteristics and frequency of ocular findings. Of these patients, 29 (52.7%) were hypermetropes, 7 (12.7%) were emmetropes, and 7 (12.7%) were myopes; astigmatism of more than 3.00 diopters was present in 7(12.7%) patients. Strabismus was observed in 12 (21.8%) patients. All but one of these 12 patients also had esotropia. Congenital nasolacrimal duct obstruction was present in 12 subjects (21.8%). Blepharitis was found in 19 (34.5%) cases. Nystagmus occurred in 7 (12.7%) patients. Brushfield spots were detected in only 20 (36.3%) of the patients and were more common in light-colored irides. Lens opacities were diagnosed in 11 (20%) patients, and two underwent successful cataract surgery. On fundus examination, an increased number of retinal vessels crossing the optic nerve head was detected in 21 (38.1%) cases. One of the patients had a retinal detachment. Significant but correctable ocular problems are present in patients with Downs syndrome and may interfere with the quality of life of the patient and with binocular vision. Surgical intervention may be needed for strabismus and for cataracts.

Uveitis in juvenile arthritis.

Purpose: To evaluate the clinical features of and determine the risk factors for uveitis in patients with juvenile arthritis. Methods: The prevalence and clinical characteristics of uveitis were studied retrospectively in 90 children diagnosed with arthritis. Patients with uveitis were compared with those who did not have eye involvement. Results: Uveitis was diagnosed in 11 patients (12.2%). Of these, seven (63.6%) had oligoarticular, two (36.4%) had polyarticular, and one (9.1%) had systemic-onset juvenile rheumatoid arthritis (JRA). One patient (9.1%) was diagnosed with enthesitis-related arthritis (ERA) (9.1%). The prevalence of uveitis was significantly higher in patients with oligoarticular JRA. The mean age at onset of arthritis in the uveitis patients was 4.39 years, which was significantly lower than in the non-uveitis group. There was no gender difference in the risk of developing uveitis. Antinuclear antibodies (ANA) was positive in seven (63.6%) of the 11 uveitis patients, confirming ANA as a significant determinant for uveitis in juvenile arthritis. Rheumatoid factor was not found to be a risk factor. One (9.1%) of the 11 patients developed serious sight-threatening complications during the follow-up period. Conclusion: This study confirmed that oligoarticular onset, ANA positivity, and young age are risk factors for developing uveitis in patients with juvenile arthritis. Gender was not found to be a determining factor. Prompt treatment of uveitis effectively decreased the prevalence of visual impairment.

Ocular and systemic findings associated with optic disc colobomas

PURPOSE To determine the ocular and systemic anomalies associated with optic disc colobomas. PATIENTS AND METHODS The records of patients with a diagnosis of isolated optic disc coloboma and chorioretinal coloboma with optic disc involvement were retrospectively reviewed. RESULTS Fifteen patients were included in the study. Of the 30 eyes, the optic disc and choroid were involved in 18, an isolated disc coloboma was present in 5, a normal optic disc was present in 4, and the optic disc could not be identified because of extreme microphthalmia in 1. Of the optic discs outside the fundus colobomas, 1 had an abnormal shape and 1 had optic atrophy and hypoplasia. Eight patients had bilateral but asymmetric involvement of the optic nerve with the coloboma. Fourteen eyes of 9 patients were microphthalmic. Nine eyes of 6 patients had microcornea. One patient had a nonrhegmatogenous retinal detachment at the time of diagnosis. One eye had a retrobulbar cyst with microphthalmia. Eight (53%) of the patients had sensory strabismus. Two patients had unilateral cortical lens opacities. Associated systemic findings were present in 6 (40%) of the 15 patients. CONCLUSIONS Optic disc colobomas have a wide variety of presentations. Poor visual acuity was observed mostly in eyes with macular involvement and microphthalmia.

Macular hole formation in Bietti's crystalline retinopathy: A case report

Three years after the initial diagnosis, a 21-year-old healthy man with Biettis crystalline retinopathy developed unilateral stage 4 macular hole with surrounding macular detachment. The mechanism of macular hole formation, which may or may not be a feature of Biettis crystalline dystrophy, is not clear.

Efficacy of Occlusion Treatment in Amblyopia and Clinical Risk Factors Affecting the Results of Treatment

Purpose: To evaluate the factors influencing visual outcome in strabismic, strabismic-anisometropic and anisometropic amblyopia following occlusion treatment. Methods: Records of 128 pediatric patients who had been treated for amblyopia by occlusion of the fellow eye between March 1992 and March 2003 were reviewed retrospectively. Age and level of visual acuity at initiation of treatment, occlusion time (full-time, part-time or minimal) and type of amblyopia were analyzed for the effect on visual outcome. Results: The mean age of the patients was 5.69 ± 2.01 years (3 to 12 years). Mean follow-up time was 3 years 2 months (6 months to 10 years). Mean visual acuity improvements were similar for the subtypes of amblyopia (strabismic amblyopia 0.38 ± 0.29 logMAR units, strabismic-anisometropic amblyopia 0.46 ± 0.40 logMAR units, anisometropic amblyopia 0.35 ± 0.24 logMAR units). Level of initial visual acuity, age at initiation of treatment and type of occlusion correlated with the final visual acuity (p = 0.000, p = 0.035, p = 0.012, respectively). When the analysis was performed according to the subtypes of amblyopia, initial visual acuity was the only factor associated with the final visual acuity in all types of amblyopia (p < 0.05). Conclusion: The level of initial visual acuity is the most significant factor determining the success of treatment in amblyopia.

Axial length and hyperopia in eyes with retinal vein occlusions.

We performed a prospective study in order to elucidate thepredisposing role of axial length and hyperopia in retinal veinocclusions. The study group comprised 39 patients with unilateralcentral retinal vein occlusion (CRVO), 50 patients with unilateralbranch retinal vein occlusion (BRVO), 13 patients with unilateralhemispheric retinal vein occlusion (HRVO) and 45 controleyes.The axial length of affected eyes was compared to fellow eyesand control eyes in each subgroup of patients with retinal veinocclusion. No statistical difference was noted for any of thesubgroups (p > 0.05). Hyperopia was detected in 12 of 39 eyes(31%) with CRVO, 14 of 50 eyes (28%) with BRVO, 4 of 13 eyes(31%) with HRVO and 15 of 45 eyes (33%) in the control group.No statistically significant difference was discovered (p> 0.05).In the light of our study, we believe that axial length andhyperopia may not be risk factors in retinal vein occlusions, incontrast to common belief.

Changing trends over the last decade in the aetiology of childhood blindness: a study from a tertiary referral centre

Aims To discern treatable and preventable causes of childhood blindness by evaluating the aetiologic factors, and to compare the distribution of the most commonly affected anatomic sites of severe visual impairment (SVI) with our previous published data. Methods The charts of 11 871 patients followed between June 2002 and May 2014 were reviewed retrospectively, and 695 patients (5.9%) who had SVI or blindness in accordance with WHO criteria were enrolled. The results of ophthalmologic examinations and coexistence of any systemic disease were documented and checked against our published clinic data concerning the aetiology of childhood blindness before 2002. χ2 test was used for statistics. Results Mean age was 47.0±51.9 months (median: 24 months). Cortical visual impairment (CVI) was present in 212 cases (30.5%) and 20.3% of those had a history of premature birth. The most common anatomic sites of SVI were retina (24.6%) and crystalline lens (17.1%). When compared with our previous data, we found a significant increase in the prevalence of CVI (p=0.046) and decrease in the frequency of SVI due to uveal disorders (p<0.001). Prevalence of blindness secondary to retinopathy of prematurity reduced by a third (p=0.280), and a significant decrease in aphakia-related SVI (p=0.028) was achieved within the last decade. Conclusions The prevalence of CVI was found to be relatively increased due to the significant reduction in the frequency of preventable causes of SVI. Furthermore our clinical practice for visual rehabilitation in aphakia has resulted in a considerable decrease in SVI in the last decade.

Choroidal detachment associated with direct spontaneous carotid-cavernous sinus fistula

A case of spontaneously occurring direct carotid-cavernous fistula complicated by choroidal detachment is presented. After thrombosis of the superior ophthalmic vein, paradoxical worsening of the clinical manifestations, recurrence of the choroidal detachment and subsequent resolution of symptoms were observed. We concluded that carotid-cavernous fistula should be kept in mind in the etiological evaluation of choroidal detachment.

Ocular disorders in children with spastic subtype of cerebral palsy

AIM To document common ocular abnormalities in children with spastic subtype of cerebral palsy (CP) and to find out whether any correlation exists between their occurance and etiologic factors. METHODS Totally 194 patients with the diagnosis of spastic type CP were enrolled in this retrospective study. Detailed ophthalmic examinations were performed. Demographic data and neuroradiological findings were documented. Kruskal-Wallis, Mann Whitney U, Pearson Chi-square tests and Students t tests were used in the statistical analysis. RESULTS The mean age was 64.7±44.2 months on the first ophthalmic examination. Prevalences of diplegia (47.4%) and tetraplegia (36.1%) were found to be higher than the frequency of hemiplegia (16.5%) in our study population. Etiologic factor was asphyxia in 60.8% of the patients. Abnormal ocular findings were present in 78.9% of the patients. Statistically significant poor vision was detected in tetraplegia group among all the spastic ubtypes of CP (P=0.000). Anisometropia and significant refractive error were found in 14.4% and 70.1% of the patients, respectively. Thirty-six children (18.6%) had nystagmus and 107 children (55.2%) had strabismus. Lower gestational age and birth weight were statistically higher in patients with esotropia than exotropia (P=0.009 and P=0.024, respectively). Abnormal morphology of the optic disc was present in 152 eyes (39.2%). Severe periventricular leukomalacia (PVL) was found in 48 patients and statistically significant poor vision was detected in the presence of PVL (P=0.000). CONCLUSION Spastic diplegic or tetraplegic CP patients with positive neuroradiological symptoms, younger gestational age and lower birth weight ought to have detailed ophthalmic examinations as early as possible to provide best visual rehabilitation.

Extraocular muscle imbalance after scleral buckling

Postoperative muscle imbalance was prospectively evaluated in 44 patients who underwent conventional scleral buckling operations. Strabismus was present in 27 of 44 patients (61%). Heterotropia resolved spontaneously during the first six postoperative months in nine of 27 patients (33%). Diplopia persisted in six patients (13.6%) and limitation of ductions occurred in 22 of 44 patients (50%). The risk of developing postoperative strabismus was 2.5 times longer if an implant was placed under a rectus muscle. Otherwise, presence, degree, and direction of the duction deficits did not correlate with placement of a local implant under the corresponding rectus muscles or their antagonists. Reoperation, preoperative detachment of macula and size of the local implant were not statistically correlated with extraocular muscle imbalance. We believe that decrease in postoperative swelling and increase in visual acuity that allows phoria adaptation are the main causes of spontaneous resolution.