The present study illustrates the difficulties in examining severely disabled children. Consistent ophthalmic manifestations of CDG-Ia patients due to the R141H/F119L genotype were congenital esotropia, DVM, and a reduced rod response in ERG-examined patients. The vast majority of patients had reduced VA and developed myopia. We speculate that there is a relationship between the glycosylation defect in CDG-Ia and the development of myopia. We recommend that CDG-Ia patients be followed annually by an ophthalmologist.

During the period from March 1978 to November 1979, 232 consecutive patients with traumatic hyphaema were allocated by admission-date to conservative treatment and to treatment with the antifibrinolytic drug tranexamic acid. Secondary haemorrhage occurred in only two of 102 tranexamic acid treated patients, while secondary haemorrhage occurred in 12 out of 130 conservatively treated patients. This difference was statistically significant. Some clinical aspects of the rebleeding cases are presented and briefly discussed.

Current pattern of blindness (i.e. visual acuity < or = 6/60) in Denmark was studied based on 1585 application forms for new membership to the Danish Society of the Blind during 1993. Statistics on blindness are very sensitive to the definitions used. A change of blindness definition to visual acuity < 6/60 reduced the number of formally blind subjects with 32%, and by using the definition of WHO (visual acuity < 3/60) only 562 subjects (35%) would have been considered blind. The prevailing causes of blindness were age-related macular degeneration with 1132 cases (71.4%), followed by diabetic retinopathy 133 cases (8.4%), and glaucoma 80 cases (5.0%). Among the younger subjects (133 persons) aged 20-59 years diabetic retinopathy comprised 36% and lesions of the optic pathways 26%, while myopia and retinitis pigmentosa accounted for 5% each. The majority of the applicants (92%) were > or = 60 years old. In this group, age-related macular degeneration was the main cause of blindness in 78%. Figures from 1993 were compared with six similar studies on newly registered blindness from the last 35 years. The annual number of registrations was doubled during the last 25 years. The annual number of registered blind due to diabetic retinopathy fell during the 60's and 70's followed by a constant rate during the last decades. Glaucoma blindness fell with a factor two, and declined from a relative frequency of 15% among the causes of blindness to 5%. The impact of age related macular degeneration increased from 20% to 70% during the same period.

Membership applications to the Danish Society of the Blind were used as a register source of legal blindness (visual acuity < or = 6/60). Based on application forms completed by specialists in ophthalmology 1585 subjects were recorded as blind in 1993. 1132 subjects (71.4%) had age-related macular degeneration. Only 5% of the registered subjects with age-related macular degeneration were below 70 years of age. The median age was 82, equal for both gender. A female overrepresentation of 2.8:1 was found. Five-year age-specific incidence rates demonstrated an exponential rise of registered blindness due to age-related macular degeneration from age 60 to 90. A decline in incidence after the age of 90 is assumed to reflect underregistration of very old persons. This tendency of non-registration was particularly pronounced in elderly males. Estimated prevalence rates of registered blindness due to age-related macular degeneration increased 100-fold from the age group 60-64 to the age group 80-84. The age specific incidence rate for the age group 60-99 years was 140:100,000 for females and only 66:100,000 for males. The corresponding incidence rate for both sexes was 108:100,000. It is still a matter of dispute whether 'true' prevalence rates of blindness are higher in females than in males. The sex difference seems not to be explained by differences in 'visual impairment threshold of registration' among the registered persons.