PURPOSE: Retinal images acquired by means of digital photography are often used for evaluation and documentation of the ocular fundus, especially in patients with diabetes, glaucoma or age-related macular degeneration. The clinical usefulness of an image is highly dependent on its quality. We set out to develop and evaluate an automatic method of evaluating the quality of digital fundus photographs. METHODS: A method for making a numerical quantification of image sharpness and illumination was developed using Matlab image analysis functions. Based on their sharpness and illumination measures, 1000 fundus photographs, randomly selected from a clinical database, were assigned to four predefined quality groups (not acceptable, acceptable, good, very good). Six independent observers, comprising three experienced ophthalmologists and three ophthalmic nurses with extensive experience in fundus image acquisition, classified a selection of 100 of these images into the corresponding quality groups. RESULTS: Automatic quality evaluation was more sensitive than evaluation by human observers in terms of ability to discriminate between good and very good images. The median concordance between the six human observers and the automatic evaluation was substantial (kappa = 0.64). CONCLUSIONS: The proposed method provides an objective quality assessment of digital fundus photographs which agrees well with evaluations made by qualified human observers and which may be useful in clinical practice.
Purpose: To report prevalence, ocular characteristics and coexisting behavioural problems in children and adolescents with optic nerve hypoplasia (ONH), which is a common cause of visual impairment in children in western countries, often associated with neurological or endocrinological problems and where autism has been reported in severe cases with blindness. Methods: This is a population-based cross-sectional study of patients <20 years of age who had been diagnosed with ONH and lived in the county of Stockholm in December 2009. Ophthalmological assessments including fundus photographs with optic disc analyses were made. A questionnaire was used to screen for behaviour and development. Results: The prevalence of ONH in all living children <18 years of age in Stockholm was 17.3/100 000 with a prevalence of visual impairment (<0.3) of 3.9/100 000. In total, 66 patients, median age 9.3 years (0.6-19.4), 36 with bilateral and 30 with unilateral ONH, were included in the current study; 53 were re-examined clinically, group A, and 13 agreed to retrospective analyses of existing medical records, group B. Analyses of the optic discs were made in fundus photographs from 53 patients comparing a semi-automated (Retinal Size Tool) and a manual method (Zeki). There was a strong curvilinear correlation (rS = -0.91 p < 0.0001 for both eyes). Behavioural problems were more common (p < 0.05) in bilateral ONH. Conclusion: Opticnerve hypoplasia is a common ocular malformation with a prevalence of 17.3/100 000 children and adolescents <18 years of age in Stockholm. Unilateral ONH seems as common as bilateral.
Purpose: To describe visual function and ocular manifestations in patients with onset of cholestasis during the neonatal period. Methods: Patients with neonatal cholestasis, either transitory or chronic, who came for assessment to our tertiary referral centre were included in a cross-sectional study and underwent ophthalmological examinations including fundus photography. A total of 57 patients (24 girls and 33 boys), aged 0.4-18.0 years, were included. Of these, 28 patients had biliary atresia, 11 had Alagille's syndrome, five had progressive familiar intrahepatic cholestasis and nine had different disorders such as pituitary insufficiency, alpha-1-antitrypsin deficiency, mitochondriopathy, congenital infections or cholestasis caused by unknown reasons. Results: Visual dysfunction and / or one or several ocularmanifestations occurred in 39 out of 57 patients. Major ocular malformations occurred in five patients. Out of three patients with biliary atresia, one patient had severe visual impairment caused by microphthalmia and chorioretinal coloboma, one patient with Cat Eye syndrome had bilateral uveochorioretinal coloboma and one patient had Rieger's anomaly. Two patients, both with pituitary deficiency and transient cholestasis, had severe unilateral visual impairment caused by optic nerve hypoplasia. Conclusion: The majority (68%) of the patients with cholestasis had ocular manifestations. Although the severity of ocular complications varied with diagnosis, and was most apparent among patients with biliary atresia or pituitary deficiency, no conclusion can be drawn regarding the connections between these conditions from the present study. Nevertheless, ocular assessment is important for diagnostic purposes and for early intervention in patients with cholestasis.
Purpose: Fanconi anaemia (FA) is a multisystemic disorder with ocular implications. This study aims to describe visual function, ocular characteristics andvisual processing skills in patients with FA after haematopoietic stem cell transplantation. Methods: Ten patients with FA, ages 8-17years, underwent ophthalmological examinations, corneal, periocular and fundus photography, visual evoked potentials (VEPs), visual fields and ocular motor and visual processing information tests. Results: Best-corrected decimal visual acuity was ≥0.65 in all 20 eyes. Microcornea was present in 18 of 18 eyes, short axial lengths in six of six eyes, steep corneal curvatures in four of six eyes, ptosis in 14 of 16 eyes, short palpebral fissures in 15 of 16 eyes and hypotelorism in three of seven patients. Optic disc areas were smaller in patients with FA compared to controls (p=0.0003 right, p=0.0003 left eye). Visual fields were abnormal in 4 of 18 eyes, while VEP was abnormal in 1 of 20 eyes. Eight patients had ocular motor dysfunction, while nine of 10 patients showed delay in visual processing skills. Conclusion: Patients with FA can present with microcornea, microphthalmia, ptosis, steep corneal curvatures, small optic discs, ptosis and delay in visual processing skills. Detailed ophthalmological and visual processing skills assessments and developmental investigations are important to detect impairments and facilitate appropriate support.