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Visual impairment in people with low intellectual abilities
International Congress Series 1282 (2005) 152 – 156
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Visual impairment in people with low intellectual abilities M.F. Hoogslag-Bienfait a,b,*, L.J. Blanksmaa, T. Kiesstraa, B. Melis-Dankersb, N. Stellingwerf b a de Brink, Vries, The Netherlands Visio Noord-Nederland, Tadingastraat 4, 8916PJ Leeuwarden, The Netherlands
b
Abstract. Institute de Brink in the North of The Netherlands is a residence people with profound to severe intellectual disabilities (ID) and severe visual impairment (VI). Ophthalmologic records from all residents were kept over the passed 30 years. We have reviewed and classified 160 records according to the classification of F. Meire [1] [F.M. Meire. Genetics in ophthalmology. Bull. Soc. Belge Ophtalmol. 269 (1998) 3–4]. Data on visual acuity, refractive errors, strabismus and ocular diagnoses were assembled as well as ophthalmic problems met during residency. Also additional handicaps were recorded. In the whole group, 155 records were complete for use. 64% of the residents appeared to be functionally blind (WHO category 0020 vision b 0.05). In the group with functional vision, N = 55, nystagmus and strabismus were more frequently seen as compared with the normal population. Optic atrophy was found most of the time as an end stage of Cerebral Visual Impairment (CVI). Classification of the causes of the ophthalmic problems showed that in the nongenetic group pre- and perinatal problems were the main causes of VI and in the genetic group syndromal anomalies. Severe and profound ID are seen in combination with other handicaps like deafness and motor handicaps which ask for special care. In this group, residents with congenital cataract had a bad prognosis. Eye poking was another problem with complications leading to blindness. Behavioural therapy and stimulation of the resident may prevent this behaviour. D 2005 Elsevier B.V. All rights reserved. Keywords: Intellectual disability; Visual impairment; Congenital Cataract; Eye poking
* Corresponding author. Visio Noord-Nederland, Tadingastraat 4, 8916PJ Leeuwarden, The Netherlands. Tel.: +31 2843500. E-mail address: [email protected] (M.F. Hoogslag-Bienfait). 0531-5131/ D 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.ics.2005.05.150
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1. Introduction In 1964, Institute de Brink in the North of The Netherlands started as a residence for 4 children with profound intellectual disabilities (developmental age 18–24 months, IQ b 30) and severe visual impairment (VA b 0.3) who were unable to follow the special school for children with ID. During the following years, not only children but also adults were admitted and at the moment de Brink offers residential care for 160 people. Other forms of care provided for by de Brink are ambulant care and day care. Also a special de Brink/ Visio team performs visual screening in institutions for people with ID. This was initiated by the government as a result of the studies done by Evenhuis et al. [2], who showed that 50% of the people with ID had visual problem. 2. Methods One of us, L. Blanksma, had been ophthalmic consultant at de Brink for 30 years. He started records for every resident at the start of their residency and would examine the resident in case of problems or at a regular base when a resident had functional vision. From the 160 residents, data were complete for research in 155 cases: 102 men and 53 women. In September 2004, data were assembled on age in September 2004, visual acuity assessed with pictury cards, Cardiff cards and most of the time Teller Acuity Cards as most residents function at a low developmental age and are non verbal. Refraction if done was measured with retinoscopy or autorefractometer. The cause of the VI was classified according to the classification of F. M. Meire [1]. Data on the presence of nystagmus, strabismus, optic atrophy, cerebral visual impairment, congenital cataract, eye poking and atrophia bulbi were recorded. Other impairments like deafness, motor disabilities and epilepsy were recorded.
3. Results Age in September 2004 ranged from 4 to 63, mean age was 35.3 (SD 13.5). Three age groups were formed (Fig. 1), which shows that these persons with ID are in relatively older population. F. M. Meire [1] differentiates between nongenetic, genetic and unknown causes. The results of the classification can be seen in Table 1.
Fig. 1. Age of subjects, N = 155.
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Table 1 Causes of VI, N = 155 ! Nongenetic (N = 93; 60%) –Pre-natal –Peri-natal –Post-natal ! Genetic (N = 47; 30%) –Eye in total –Anterior segment –Posterior segment –Part of a syndrome ! Unknown (N = 14; 10%)
22.6% 25.8% 12.9% 6.5% 3.2% 3.9% 15.5%
In our group, nongenetic causes formed 60% of the subjects, genetic 30.3% and unknown 9.7%. Nongenetic causes can be subclassified in prenatal (rubella, toxoplasma infections or cerebral developmental disorders), perinatal (prematurity, dysmaturity and poor perinatal condition) and postnatal (infections and others). Genetic causes were nominated to eye in total (anophthalmia, microphthalmia), anterior segment disorders (corneal disorders, congenital cataract, aniridia) or posterior segment disorders (mainly M. Leber) or part of a syndrome. Especially in the older population, the mode of inheritance was not always recorded. Autosomally recessive was the most frequent recorded inheritance mode. Visual acuity was recorded following WHO categories and is shown in Table 2. As can be seen a very large group, 2/3 of the population is functionally blind. The residents with visual acuity N 0.3 were residents with Down syndrome or Batten Spielmayer Vogts disease. None of the residents had a visual acuity higher than 0.5. In the group with visual function (N = 55), strabismus was found in 20 cases, 36% and nystagmus in 16 cases 29%. Refraction was not always recorded, but shows a tendency for myopia. High myopia was found in 6 cases, 11%. Keratoconus was especially described in Down patients. This was seen with slitlamp but could not accurately be measured. In the whole group (N = 155) optic atrophy was found in 53 cases, 34% and cerebral visual impairment (CVI) in 20 cases 12.9%. CVI was only mentioned if there could not be found another diagnosis. Probably it plays a role in more residents as the visual performance of certain residents is more severely impaired than expected from the ophthalmic problem. Congenital cataract was seen in 32 cases (20.6%), as part of rubella syndrome in 9 cases, due to perinatal problems in 7 cases, due to syndromal anomalies in 8 cases and others 8. Long-term results of cataract operated on residents show a lot of problems with after-cataract, glaucoma, atrophia bulbi and eye poking. The persons in this study were operated on with intacapsular and extracapsular cataract extraction procedures. Hopefully, phaco emulsification technique will show better results in
Table 2 Visual acuity (WHO) N = 155 N 1: 2: 3: 4: 5:
VA z 0.3 0.1 V VA b 0.3 0.05 V VA b 0.1 0 b VA b 0.05 VA= 0
11 39 5 23 77
7% 25% 3% 15% 50%
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the future. Still extreme care should be given before operating this difficult group of people with severe ID. Eye poking was seen in 29 cases, 18.7%. In 3 cases, it led to atrophic bulbi, which had to be enucleated. Bulbar atrophy was seen in 35 eyes, 19 cases, 12.2%. Main cause was congenital cataract (7 cases). In the whole group, 25% shows hearing problems. Complete deafness was found in 18 residents, 11.6%. Epilepsy was seen in 40 cases, 25.8%. Motor disabilities were not well recorded in the ophthalmologic records. At least 20, 12.9% residents have tetraplegia.
4. Discussion In the literature VI in people with ID is described by several authors. M. Warburg [3] has made a review of all the literature on this topic. In most of the known studies, research was done in centres for ID. Our de Brink group is a selected group of residents with low ID and severe VI. Evenhuis et al. [2] and Warburg [4] show that people with severe ID show more VI than people with moderate ID. This is confirmed by our study in which 64.5% of the residents have no functional vision. Causes in of VI in our group cannot be compared to other groups because a comparable group could not be found in the literature. Nongenetic causes form with 60% the largest group, mainly due to pre or perinatal problems. In the genetic group, syndromal anomalies are most frequently the cause of the ophthalmic disorders. Nystagmus 29% and strabismus 36% of the residents with visual function (N = 55) are frequently met. These numbers are comparable to numbers mentioned by Warburg [4] and van Splunder et al. [5]. Special problems in our group form the residents with congenital cataract. Although intraocular surgery had been carefully performed, most eyes developed complications and ended with very low visual acuity. Jacobson [6] describes in his article the operative results of a subgroup of mentally retarded adults who needed intraocular surgery. She shows better results, but she shows results of operations for senile cataract and follow up is maximally 32 months. Woodruff et al. [7] describe another group of mentally handicapped with cataract surgery, but give no results of the operations. Eye poking is another great problem with severe consequences. In our group, it was recorded in 18.7%. In 3 cases, it led to atrophic bulbi, which had to be enucleated. Usually, it is due to lack of stimulation. Our neuropsychologist, T. Kiesstra, suggests that it is more often seen in our group in people with CVI and in people with developmental age 18–24 months, an age at which toddlers start to develop the world. Fazzi et al. [8] advise changes in the daily environment and activities to change this behaviour. This is also done at de Brink. Other impairments met were hearing problems. In our group, a hearing problem was found in 25% of the cases. Evenhuis [2] also showed 20% hearing problems in people with severe to profound ID. The de Brink group had 18 residents with complete deafness.
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5. Conclusion From 160 residents with low ID and severe VI 155 ophthalmologic records were reviewed. 60.4% of the residents were according to WHO classification functionally blind (VA b 0.05). Causes of VI were classified after the classification of F. M. Meire. In the nongenetic group, pre- and perinatal problems are the main causes of the impairments. In the genetic group, syndromal anomalies are most frequently seen. Severe and profound ID were seen in combination with other handicaps like deafness, epilepsy and motor handicaps, which ask for special care. Residents with congenital cataract, operated on with classic intra- or extracapsular cataract procedures showed a bad prognosis. Newer techniques may ameliorate the results. Eye poking is a great problem in people with severe ID and VI and asks for special care. References [1] F.M. Meire, Genetics in ophthalmology, Bulletin de la Socie´te´ Belge d’Ophtalmologie 269 (1998) 3 – 4. [2] H.M. Evenhuis, et al., Prevalence of visual and hearing impairment in a Dutch institutionalized population with intellectual disability, Journal of Intellectual Disability Research 45 (2001) 457 – 464 (Part 5). [3] M. Warburg, Visual impairment in adult people with intellectual disability: literature review, Journal of Intellectual Disability Research 45 (2001) 424 – 438 (Part 5). [4] M. Warburg, Visual impairment in adult people with moderate, severe, and profound intellectual disability, Acta Ophthalmologica Scandinavica 79 (2001) 450 – 454. [5] J. van Splunder, et al., Ophthalmology 11 (2004) 1457 – 1463. [6] L. Jacobson, Ophthalmology in mentally retarded adults, Acta Ophthalmologica 66 (1988) 457 – 462. [7] M.E. Woodruff, T.E. Cleary, C. Bader, The prevalence of refractive and ocular anomalies among 1242 institutionalized. Mentally retarded persons, American Journal of Optometry and Physiological Optics 57 (2) (1980) 70 – 84. [8] E. Fazzi, et al., Stereotyped behaviours in blind children, Brain and Development 21 (1999) 522 – 528.
www.ics-elsevier.com
Visual impairment in people with low intellectual abilities M.F. Hoogslag-Bienfait a,b,*, L.J. Blanksmaa, T. Kiesstraa, B. Melis-Dankersb, N. Stellingwerf b a de Brink, Vries, The Netherlands Visio Noord-Nederland, Tadingastraat 4, 8916PJ Leeuwarden, The Netherlands
b
Abstract. Institute de Brink in the North of The Netherlands is a residence people with profound to severe intellectual disabilities (ID) and severe visual impairment (VI). Ophthalmologic records from all residents were kept over the passed 30 years. We have reviewed and classified 160 records according to the classification of F. Meire [1] [F.M. Meire. Genetics in ophthalmology. Bull. Soc. Belge Ophtalmol. 269 (1998) 3–4]. Data on visual acuity, refractive errors, strabismus and ocular diagnoses were assembled as well as ophthalmic problems met during residency. Also additional handicaps were recorded. In the whole group, 155 records were complete for use. 64% of the residents appeared to be functionally blind (WHO category 0020 vision b 0.05). In the group with functional vision, N = 55, nystagmus and strabismus were more frequently seen as compared with the normal population. Optic atrophy was found most of the time as an end stage of Cerebral Visual Impairment (CVI). Classification of the causes of the ophthalmic problems showed that in the nongenetic group pre- and perinatal problems were the main causes of VI and in the genetic group syndromal anomalies. Severe and profound ID are seen in combination with other handicaps like deafness and motor handicaps which ask for special care. In this group, residents with congenital cataract had a bad prognosis. Eye poking was another problem with complications leading to blindness. Behavioural therapy and stimulation of the resident may prevent this behaviour. D 2005 Elsevier B.V. All rights reserved. Keywords: Intellectual disability; Visual impairment; Congenital Cataract; Eye poking
* Corresponding author. Visio Noord-Nederland, Tadingastraat 4, 8916PJ Leeuwarden, The Netherlands. Tel.: +31 2843500. E-mail address: [email protected] (M.F. Hoogslag-Bienfait). 0531-5131/ D 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.ics.2005.05.150
M.F. Hoogslag-Bienfait et al. / International Congress Series 1282 (2005) 152–156
153
1. Introduction In 1964, Institute de Brink in the North of The Netherlands started as a residence for 4 children with profound intellectual disabilities (developmental age 18–24 months, IQ b 30) and severe visual impairment (VA b 0.3) who were unable to follow the special school for children with ID. During the following years, not only children but also adults were admitted and at the moment de Brink offers residential care for 160 people. Other forms of care provided for by de Brink are ambulant care and day care. Also a special de Brink/ Visio team performs visual screening in institutions for people with ID. This was initiated by the government as a result of the studies done by Evenhuis et al. [2], who showed that 50% of the people with ID had visual problem. 2. Methods One of us, L. Blanksma, had been ophthalmic consultant at de Brink for 30 years. He started records for every resident at the start of their residency and would examine the resident in case of problems or at a regular base when a resident had functional vision. From the 160 residents, data were complete for research in 155 cases: 102 men and 53 women. In September 2004, data were assembled on age in September 2004, visual acuity assessed with pictury cards, Cardiff cards and most of the time Teller Acuity Cards as most residents function at a low developmental age and are non verbal. Refraction if done was measured with retinoscopy or autorefractometer. The cause of the VI was classified according to the classification of F. M. Meire [1]. Data on the presence of nystagmus, strabismus, optic atrophy, cerebral visual impairment, congenital cataract, eye poking and atrophia bulbi were recorded. Other impairments like deafness, motor disabilities and epilepsy were recorded.
3. Results Age in September 2004 ranged from 4 to 63, mean age was 35.3 (SD 13.5). Three age groups were formed (Fig. 1), which shows that these persons with ID are in relatively older population. F. M. Meire [1] differentiates between nongenetic, genetic and unknown causes. The results of the classification can be seen in Table 1.
Fig. 1. Age of subjects, N = 155.
154
M.F. Hoogslag-Bienfait et al. / International Congress Series 1282 (2005) 152–156
Table 1 Causes of VI, N = 155 ! Nongenetic (N = 93; 60%) –Pre-natal –Peri-natal –Post-natal ! Genetic (N = 47; 30%) –Eye in total –Anterior segment –Posterior segment –Part of a syndrome ! Unknown (N = 14; 10%)
22.6% 25.8% 12.9% 6.5% 3.2% 3.9% 15.5%
In our group, nongenetic causes formed 60% of the subjects, genetic 30.3% and unknown 9.7%. Nongenetic causes can be subclassified in prenatal (rubella, toxoplasma infections or cerebral developmental disorders), perinatal (prematurity, dysmaturity and poor perinatal condition) and postnatal (infections and others). Genetic causes were nominated to eye in total (anophthalmia, microphthalmia), anterior segment disorders (corneal disorders, congenital cataract, aniridia) or posterior segment disorders (mainly M. Leber) or part of a syndrome. Especially in the older population, the mode of inheritance was not always recorded. Autosomally recessive was the most frequent recorded inheritance mode. Visual acuity was recorded following WHO categories and is shown in Table 2. As can be seen a very large group, 2/3 of the population is functionally blind. The residents with visual acuity N 0.3 were residents with Down syndrome or Batten Spielmayer Vogts disease. None of the residents had a visual acuity higher than 0.5. In the group with visual function (N = 55), strabismus was found in 20 cases, 36% and nystagmus in 16 cases 29%. Refraction was not always recorded, but shows a tendency for myopia. High myopia was found in 6 cases, 11%. Keratoconus was especially described in Down patients. This was seen with slitlamp but could not accurately be measured. In the whole group (N = 155) optic atrophy was found in 53 cases, 34% and cerebral visual impairment (CVI) in 20 cases 12.9%. CVI was only mentioned if there could not be found another diagnosis. Probably it plays a role in more residents as the visual performance of certain residents is more severely impaired than expected from the ophthalmic problem. Congenital cataract was seen in 32 cases (20.6%), as part of rubella syndrome in 9 cases, due to perinatal problems in 7 cases, due to syndromal anomalies in 8 cases and others 8. Long-term results of cataract operated on residents show a lot of problems with after-cataract, glaucoma, atrophia bulbi and eye poking. The persons in this study were operated on with intacapsular and extracapsular cataract extraction procedures. Hopefully, phaco emulsification technique will show better results in
Table 2 Visual acuity (WHO) N = 155 N 1: 2: 3: 4: 5:
VA z 0.3 0.1 V VA b 0.3 0.05 V VA b 0.1 0 b VA b 0.05 VA= 0
11 39 5 23 77
7% 25% 3% 15% 50%
M.F. Hoogslag-Bienfait et al. / International Congress Series 1282 (2005) 152–156
155
the future. Still extreme care should be given before operating this difficult group of people with severe ID. Eye poking was seen in 29 cases, 18.7%. In 3 cases, it led to atrophic bulbi, which had to be enucleated. Bulbar atrophy was seen in 35 eyes, 19 cases, 12.2%. Main cause was congenital cataract (7 cases). In the whole group, 25% shows hearing problems. Complete deafness was found in 18 residents, 11.6%. Epilepsy was seen in 40 cases, 25.8%. Motor disabilities were not well recorded in the ophthalmologic records. At least 20, 12.9% residents have tetraplegia.
4. Discussion In the literature VI in people with ID is described by several authors. M. Warburg [3] has made a review of all the literature on this topic. In most of the known studies, research was done in centres for ID. Our de Brink group is a selected group of residents with low ID and severe VI. Evenhuis et al. [2] and Warburg [4] show that people with severe ID show more VI than people with moderate ID. This is confirmed by our study in which 64.5% of the residents have no functional vision. Causes in of VI in our group cannot be compared to other groups because a comparable group could not be found in the literature. Nongenetic causes form with 60% the largest group, mainly due to pre or perinatal problems. In the genetic group, syndromal anomalies are most frequently the cause of the ophthalmic disorders. Nystagmus 29% and strabismus 36% of the residents with visual function (N = 55) are frequently met. These numbers are comparable to numbers mentioned by Warburg [4] and van Splunder et al. [5]. Special problems in our group form the residents with congenital cataract. Although intraocular surgery had been carefully performed, most eyes developed complications and ended with very low visual acuity. Jacobson [6] describes in his article the operative results of a subgroup of mentally retarded adults who needed intraocular surgery. She shows better results, but she shows results of operations for senile cataract and follow up is maximally 32 months. Woodruff et al. [7] describe another group of mentally handicapped with cataract surgery, but give no results of the operations. Eye poking is another great problem with severe consequences. In our group, it was recorded in 18.7%. In 3 cases, it led to atrophic bulbi, which had to be enucleated. Usually, it is due to lack of stimulation. Our neuropsychologist, T. Kiesstra, suggests that it is more often seen in our group in people with CVI and in people with developmental age 18–24 months, an age at which toddlers start to develop the world. Fazzi et al. [8] advise changes in the daily environment and activities to change this behaviour. This is also done at de Brink. Other impairments met were hearing problems. In our group, a hearing problem was found in 25% of the cases. Evenhuis [2] also showed 20% hearing problems in people with severe to profound ID. The de Brink group had 18 residents with complete deafness.
156
M.F. Hoogslag-Bienfait et al. / International Congress Series 1282 (2005) 152–156
5. Conclusion From 160 residents with low ID and severe VI 155 ophthalmologic records were reviewed. 60.4% of the residents were according to WHO classification functionally blind (VA b 0.05). Causes of VI were classified after the classification of F. M. Meire. In the nongenetic group, pre- and perinatal problems are the main causes of the impairments. In the genetic group, syndromal anomalies are most frequently seen. Severe and profound ID were seen in combination with other handicaps like deafness, epilepsy and motor handicaps, which ask for special care. Residents with congenital cataract, operated on with classic intra- or extracapsular cataract procedures showed a bad prognosis. Newer techniques may ameliorate the results. Eye poking is a great problem in people with severe ID and VI and asks for special care. References [1] F.M. Meire, Genetics in ophthalmology, Bulletin de la Socie´te´ Belge d’Ophtalmologie 269 (1998) 3 – 4. [2] H.M. Evenhuis, et al., Prevalence of visual and hearing impairment in a Dutch institutionalized population with intellectual disability, Journal of Intellectual Disability Research 45 (2001) 457 – 464 (Part 5). [3] M. Warburg, Visual impairment in adult people with intellectual disability: literature review, Journal of Intellectual Disability Research 45 (2001) 424 – 438 (Part 5). [4] M. Warburg, Visual impairment in adult people with moderate, severe, and profound intellectual disability, Acta Ophthalmologica Scandinavica 79 (2001) 450 – 454. [5] J. van Splunder, et al., Ophthalmology 11 (2004) 1457 – 1463. [6] L. Jacobson, Ophthalmology in mentally retarded adults, Acta Ophthalmologica 66 (1988) 457 – 462. [7] M.E. Woodruff, T.E. Cleary, C. Bader, The prevalence of refractive and ocular anomalies among 1242 institutionalized. Mentally retarded persons, American Journal of Optometry and Physiological Optics 57 (2) (1980) 70 – 84. [8] E. Fazzi, et al., Stereotyped behaviours in blind children, Brain and Development 21 (1999) 522 – 528.