Driving performance among bioptic telescope users with low vision two years after obtaining their driver's license: a quasi-experimental study.

This study sought to compare road safety of new drivers with low vision who have followed a specific pilot bioptic training program with other groups of drivers all matched for age and driving experience. A quasi-experimental design was used two years after drivers obtained their license. Drivers were classified in the experimental group (n = 10, they followed a pilot bioptic training program and had license restrictions: weight of the car, requirement of a yearly medical exams, requirement to wear glasses/contacts, use of a bioptic telescope), the comparison group (n = 17, similar license restrictions except the use of a bioptic telescope) and the regional population (n = 1,690, no license restriction). The number of new drivers involved in at least one accident and who committed at least one offense is not greater for users of a bioptic telescope than for drivers of in the other groups. The results of this study indicate that driving with a bioptic telescope does not increase the risk of accidents and offenses, with more scientific evidence than in previous studies, among drivers aged between 25 and 35 who have a congenital visual impairment and who have completed an eight-week pilot bioptic training program.

Two patients with spinocerebellar ataxia type 7 presenting with profound binocular visual loss yet minimal ophthalmoscopic findings.

Two patients with genetically confirmed spinocerebellar ataxia type 7 (SCA7) presented with progressive visual loss. Examination disclosed substantial visual acuity loss, central scotomas, and marked dyschromatopsia. Ophthalmoscopic abnormalities were subtle, with only mild retinal artery attenuation and minimal foveal region pigmentary abnormalities. Both patients had slow saccades and partially limited ductions, although neither reported diplopia. One patient had obvious extremity and gait ataxia, but the other had only an unsteady tandem gait. Results of electroretinography (ERG) were abnormal in both patients. These cases illustrate that SCA7 may present with profound visual loss yet minimal ophthalmoscopic findings and sometimes minimal ataxia. The clues to diagnosis are the abnormal color vision, retinal artery attenuation, abnormal eye movements, and a family history of similar manifestations, which may have gone undiagnosed. Full-field or multifocal ERG will always disclose photoreceptor dysfunction. Genetic testing is now available to confirm the diagnosis.

Comparison of vision-related quality of life and mental health between congenital and acquired low-vision patients.

PURPOSE: To evaluate the impact of the age of onset of low vision on patients' vision-related quality of life (VR-QoL) and mental health. METHODS: Low-vision patients who visited Chung-Ang University hospital from January 2012 to December 2014 were included. Patients were divided into the congenital low-vision (CLV) and acquired low-vision (ALV) groups according to the age of disease onset. People with normal visual function comprised the control group. VR-QoL was estimated with the National Eye Institute Visual Function Questionnaire (NEI VFQ-25), while mental health was assessed through the Beck Depression Inventory (BDI) and the Beck Anxiety Inventory (BAI). The mean scores of each questionnaire were compared between the groups in independent t-tests. RESULTS: Overall, 125 low-vision patients (55 CLV and 70 ALV) and 71 control subjects were included. Although the subscale and composite scores of the NEI VFQ-25 were lower in the ALV group than in the CLV group, the differences were not significant. However, the BDI and BAI scores were significantly higher in the ALV group than in the CLV group (12.07 ± 11.97 vs. 7.67 ± 9.04, P = 0.021; 9.11 ± 10.51 vs. 5.69 ± 6.85, P = 0.030, respectively). Also, the number of patients requiring expert consultation for depression was higher in the ALV group than in the CLV group (P = 0.010). CONCLUSION: ALV patients have more vulnerable mental health states than CLV patients. Therefore, assessment of the age of onset of low vision and mental health plays a critical role in successful rehabilitation.

Visual disorders in children with brain lesions: 2. Visual impairment associated with cerebral palsy.

Disorders of visual function are a common finding in children with cerebral palsy. In some cases they are secondary to ophthalmologic abnormalities such as cataract or retinopathy, but more often they are due to damage of the central visual pathway. We review the literature on the prevalence and distribution of visual abnormalities in children with cerebral palsy and their relation to cognitive, motor and emotional development.

Características clínicas en niños con baja visión. Sancti Spíritus 2001-2012 / Clinical characteristics in children with low vision. Sancti Spíritus 2001-2012

Fundamento: Las consecuencias del aumento creciente de la discapacidad visual afecta a los diferentes grupos etarios. En el niño limita el conocimiento del mundo y con ello sus avances académicos, el desarrollo sicomotor y la formación de su personalidad. Objetivo: Determinar las características clínicas en niños con baja visión en la provincia Sancti Spíritus desde el 2001 hasta el 2012. Metodología: Se realizó un estudio descriptivo, en el período de enero 2001 hasta diciembre 2012, en la consulta de baja visión del Hospital General Camilo Cienfuegos a los 87 niños entre 0 y 18 años, atendidos en intervención precoz y estudiantes de la escuela especial Miguel Ángel Echemendía. Resultados: Hubo un predominio del sexo masculino, en el grupo de pacientes atendidos en la consulta de intervención precoz, el motivo fundamental fue la retinopatía de la prematuridad y las principales causas de discapacidad en edad escolar fueron las anomalías congénitas, con 96,4 % de rehabilitación visual. Conclusiones: La estimulación visual en niños constituye un factor importante para contribuir al desarrollo de su visión. Se ofrece una atención diferenciada a este grupo etario para garantizar su inserción a la sociedad(AU)

[Septo-optic dysplasia (de Morsier syndrome)]. / Septooptische Dysplasie (de-Morsier-Syndrom).

Septo-optic dysplasia (or de Morsier syndrome) is a congenital disorder characterised by anomalies in cerebral midline structures, optic nerve hypoplasia, and hormonal deficiencies. Diagnosis should be made early, due to the possibility of treating the hormonal disturbances. We describe here a case with decreased visual acuity, one-sided hemianopia, nystagmus und agenesis of the septum pellucidum and discuss the heterogeneous appearance of this syndrome. There are two theories regarding its pathogenesis. The first postulates simultaneous damage to both cerebral structures and optic nerve development around the 6th week of gestation, while the other favours secondary degeneration of optic nerve fibres due to a cerebral lesion.

Visual characteristics of low vision children.

A retrospective survey of pediatric clinical files from the Kooyong Low Vision Clinic (LVC) showed that the major causes of low vision were congenital or inherited conditions and most children had 6/60 (20/200) or better distance acuities. A classroom evaluation of these children showed that overall, the clinically determined visual acuity corresponded with classroom performance. A high rate of use of prescribed low vision aids was found, and a reading evaluation on a standardized test showed poor performance with respect to reading speed and comprehension, but almost all the children had adequate reading accuracy. Considering the importance of reading in education, greater emphasis on reading evaluations in routine low vision examinations is recommended.

Head and eye movements in children with low vision.

BACKGROUND: Early childhood nystagmus may herald blindness, brain tumors, benign idiopathic motor nystagmus or spasmus nutans. Nystagmus unique to low vision was sought. METHODS: Videotapes and head/ eye movements of 18 congenitally visually impaired subjects were analyzed. RESULTS: Nystagmus of congenitally visually impaired subjects was characterized as small horizontal or vertical movements superimposed on larger oscillations. Small and large nystagmus movements were of pendular or jerk type. Slow-phase jerk nystagmus had increasing, constant and decreasing velocities. Fast-phase nystagmus changed direction periodically. Pendular nystagmus was out of phase and evolved to jerk nystagmus in two subjects. All subjects displayed head nodding, and one stabilized gaze with head movements. CONCLUSION: Eye movement recordings allow characterization and differentiation of subjects with nystagmus and low vision from other nystagmus forms.

Haptic perception of the horizontal by blind and low-vision individuals.

We examined haptic perception of the horizontal in visually impaired people. Blind people (late blind and congenitally blind), persons with very low vision, and blindfolded sighted individuals felt raised-line drawings of jars at four angles. They had to demonstrate their understanding that water remains horizontal, despite jar tilt, by selecting the correct raised-line drawing given four choices. Low-vision subjects, with near perfect scores, performed significantly better than the other groups of subjects. While the late-blind and blindfolded sighted subjects performed slightly better than the congenitally blind participants, the difference between the late-blind and congenitally blind groups was nonsignificant. The performance of the congenitally blind subjects indicates that visual experience is not necessary for the development of an understanding that water level stays horizontal, given container tilt.

Congenital and hereditary visual impairment in Greenland.

Thirty-eight cases of congenital visual impairment (CVI) were reported in the Inuit population of Greenland over a period of 40 years (1950-1989), corresponding to a frequency of 86 per 100,000 live born children. The two most common causes of blindness were optic atrophy and cerebral visual impairment due to brain disorders of various etiologies. This finding is in accordance with recently published data from the Nordic countries. On the other hand, retinopathy of prematurity and congenital cataract were rare causes of CVI in Greenland. Fifteen out of the 38 cases had unknown etiology. Genetic disorders accounted for 7/38 of the cases. A separate examination of registered cases with probable genetic visual impairment, irrespective of birth year, disclosed 25 patients. New mutations seemed to be the most reasonable explanation for isolated cases of aniridia, lens ectopia, and Down syndrome, while inbreeding was a possible contributory factor in a few autosomal recessive conditions.

Septo-optic dysplasia: case report

The clinical triad of septo-optic dysplasia (SOD) comprises the abscence of the septum pellucidum, congenital optic nerve dysplasia, and multiple endocrine disorders. When any two of these factors are present, the condition is defined as an imcomplete form of SOD. The authors report the case of an imcomplete form of SOD in a 9-year old boy with low vision and nystagmus present from birth. The bilateral ophthalmoscopic examination revealed small papillae with double countour images. Magnetic resonance imaging showed hypoplasia of the optic nerve bilaterally, chiasm and absence of the septum pellucidum.