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Background: The mucopolysaccharidoses (MPS) are an inhomogeneous group of disorders of errors in the carbohydrate metabolism with severe ocular involvement (corneal opacification, retinal degeneration, optic atrophy)., Patient Presentation: We report on a boy aged 12 years, with Morquio A (MPS IV A) syndrome. Ocular findings: progressive pseudoexophthalmus due to shallow orbits, increasing corneal stromal clouding, intermittent dissociated manifest nystagmus of the left eye, nyctalopia. Visual acuity OD cc = 0.16, OS cc = 0.05. Electrophysiology: changes suggesting a symptomatic tapetoretinal degeneration and optic atrophy. TREATMENT AND COURSE OF DISEASE: OS: perforating keratoplasty. Postoperative improvement of visual acuity to 0.25 for nearly a year, followed by progressive reopacification of the corneal graft. Both eyes: progressive signs of tapetoretinal degeneration and optic atrophy. Visual acuity now reduced to OD 0.05, OS 0.1., Conclusions: Success of a keratoplasty is limited by (1) reopacification of the cornea, (2) visual impairment due to (a) retinal degeneration and (b) optic atrophy. The indication for perforating keratoplasty has to be thought about very carefully in these multimorbid patients. In our patient, beside progressive visual impairment there is a progressive deafness which dominates his social and school life. Attending school is severely complicated by the double handicap. Perforating keratoplasty enabled the boy to attend a school for physically handicapped without a special low-vision care for another year. Progressive visual loss without further treatment options now renders optical and electronic low-vision aids necessary. Although the time of improved visual acuity lasted less than a year, we think patients with a life expectancy of less than 20 years should have every possible improvement of their situation - even if it does not last permanently. We therefore propose perforating keratoplasty in spite of insufficient long-term results.

Background: There are controversies concerning the necessity of pre-school vision screening., Aim of the Study: evaluation of the prevalence of pathologic ophthalmologic findings in kindergarten children., Materials and Methods: 1030 families were offered a vision screening. Of these, a total of 948 children, aged 3 to 6 years, voluntarily underwent a screening for strabismus, amblyopia and refractive anomalies. The examination was performed in the kindergarten in the absence of the parents. METHODS OF EXAMINATION: A questionnaire concerning general and ophthalmologic history of the child and of the family was evaluated. Visual acuity, cover-uncover-test, Lang-stereotest, retinoscopy, ophthalmoscopy (undilated pupils) were performed and the glasses were evaluated., Results: The screening was highly accepted by the parents and 92% of the families (n = 948) took part. The compliance of the children was very good. A total of 38.7% (n = 381) of the children showed one or more abnormal parameters. 21.4% (n = 229) showed a reduced visual acuity. Strabismus was found in 3.7%. Half of the children with abnormal findings already had had a vision screening, but only 25% had received ophthalmologic treatment. Of those who possessed glasses, 25% came without them, and another 25% had a reduced visual acuity even with their glasses. The main problems were many false-positive results and high costs., Conclusions: Ophthalmologic and orthoptic screening in kindergarten is technically easy and conclusive in experienced hands. Ideas to reduce costs and to avoid overreferrals are an age-related lowering of the visual acuity limit and a rescreening of suspected children in a screening-setting a second time before sending them to an ophthalmologist. Another possibility to reduce costs would be to perform examinations not by ophthalmologists but by "screening-orthoptists" who should be trained in retinoscopy and ophthalmoscopy.

Background: The aim of the study was the evaluation of how ophthalmological diagnoses and the proportion of multiply handicapped children has changed within the last 20 years at a state school for visually handicapped and blind children., Patients and Methods: A profile investigation was conducted on all 105 children at the Landesschule für Blinde und Sehbehinderte des Saarlandes and compared to the results of an examination from 1975., Results: The predominant ophthalmological diagnoses were: optic atrophy (17.5%), ocular albinism (11.9%), scar-stage IV and V of retinopathy of prematurity (11.1%), as well as tapetoretinal dystrophies with related syndromes (8.7%) and myopia magna (7.9%). Blind: 10.3% (1975: 36.4%); visually handicapped: 47.1% (1975: 49.2%); multiply handicapped: 42.5% (1975: 14.4%)., Conclusions: (1) The diseases that dominated in earlier years in schools for the visually handicapped have become rare (cataract, aphakia, buphthalmia, macular dystrophy--all less than 5%); (2) the proportion of completely blind pupils has become much smaller; (3) there is an increasing tendency to educate visually handicapped pupils in regular schools with integrative aids; (4) there is also an increasing proportion of multiply handicapped children (school and kindergarten: 42%, early patronage 74%).