Journal of Mental Deficiency Research, 1990, 34, 41-48
Eye abnormalities in the mentally handicapped C. AITCHISON,'.2 D. L. EASTY1.2 AND J. JANCAR2 'Bristol Eye Hospital and ^Stoke Park Hospital, Bristol, England ABSTRACT. In 1987, a survey of eye abnormalities was undertaken of the mentally handicapped patients in Stoke Park Hospital, Bristol. The total population was 367 patients (262 females and 105 males). Of the 367 patients reviewed, 218 were found to have one or more eye anomalies (including the Down's Syndrome group), which shows that more than half the patients suffered from eye disorders (59%). The prevalence of the following anomalies is presented in detail: strabismus, refractive errors and cataracts, Comeal anomalies, nystagmus, retinopathy, glaucoma and other eye pathologies associated with various syndromes are noted. Eye anomalies in 31 patients sufFering from Down's syndrome in the sample are recorded. Preventative measures to ameliorate the defects of eyesight, particularly in the aging population, relevance of regular eye examination and medical or surgical treatment of eye disorders are discussed.
INTRODUCTION The Reverend Harold Nelson Burden was the founder of Stoke Park Colony, which was opened on 1 April 1909. Mr Burden was not content merely to offer custodial care for the mentally handicapped patients but made financial provision for and encouraged research into the causes, treatment and prevention of mental handicap. In 1930, he established the first multi-disciplinary research centre in the country under the leadership of Professor Berry. The colony was absorbed into the National Health Service in 1948 when it became known as the Stoke Park Hospital Group, consisting of Stoke Park, Purdown, Leigh Court and Hanham Hall Hospitals. In 1930, Mr A. E. lies was appointed as the first ophthalmologist to the research team. Since then, a visiting consultant opthalmologist has been permanently attached to the staff of the Stoke Park Group of Hospitals. Excellent notes on ophthalmic disorders have been preserved since 1930 which enable researchers to study eye anomalies, rare syndromes and genetically transmitted eye disorder (Jancar, 1981). Since 1980, the ophthalmic team, consisting of ophthalmologist, orthoptist and optician, has been responsible for the assessment and treatment of patients with eye problems. The orthoptist (C.A.), has been concerned principally with screening patients to assess their visual acuity, detect the presence of strabismus, and patients with other ocular conditions requiring further investigation have been referred to the ophthalmologist, or optician. Correspondence: Christina Aitchison DBO, Bristol Eye Hospital, Lower Maudlin Street, Bristol BSl 2LX, England. 41
42
C. Aitchison et al.
METHOD There was a considerable number of patients with eye anomalies and the increased aging population. When searching for papers on the subject of ophthalmic anomalies and mental handicap, it was found that there is a scarcity of literature, particularly concerning patients over 40 years of age with eye pathologies. However, some studies show the high incidence of squint and refractive error in mentally handicapped children (Bankes, 1974). Therefore, it was decided that a survey of the residential community of Stoke Park Hospital should be undertaken to identify the prevalence of a variety of eye anomalies found, and to institute the appropriate treatment where possible. The authors realise that only a selective number of patients was used for the survey; it was not possible to gain sufficient additional information, nor gain access to acquiring such from the total number of mentally handicapped living in the community of Frenchay Health District. However, they hope that the results found will stimulate wider interest and act as a basis for future research.
RESULTS The total population of patients reviewed in the survey was 367; 105 males and 262 females (Fig. 1). The ages of females ranged from 15 to 92 (mean age 50-16) and of males from 15 to 77 (mean age 36-89). Recorded IQs extended from 15 to 74 (mean IQ 20 32). Previously, the majority of female patients were admitted to Stoke Park Hospital, and the male patients to Purdown Hospital. This explains the greater number of female patients. Of the 367 patients, there were 31 with Down's syndrome (five males and 26 females). It was decided to look at these as a separate group, bearing in mind that Down's syndrome presents many ocular problems and this group was no exception. Of the 367 patients reviewed, 218 were found to have one or more eye anomalies (including Down's syndrome group) and this shows the high percentage overall: 59%. Excluding the Down's syndrome group, the percentage is still high: 54%. The number of eye anomalies was found to be higher in the aging group. There were 144 patients over 40 years of age of whom 42 suffered from more than one ocular defect (Fig. 2). Longevity in patients with Down's syndrome has increased by an average of 40 years and in other handicapped patients by more than 30 years. New antibiotics and anticonvulsants, along with better environment and care, have been major determinants of increased longevity (Carter & Jancar, 1983). It can be seen that there is a particularly high incidence of strabismus, refractive error, cataract, and a lesser number of patients with comeal defect, nystagmus, retinopathic conditions (i.e. macular degeneration) and miscellaneous conditions (i.e. ectropia, entropia and glaucoma).
Eye abnormalities in the mentally handicapped
43
60 O--O MALE { n=105 ) • - • F E M A L E ( n=262 ) TOTAL=367
40
NUMBERS
20
10-19
20-29
30-39
40-49
50-59
60-69
70-79
80-89
90*
AGE RANGE
Fig. 1 Total hospital population MALE
FEMALE
STRABISMUS REFRACTIVE ERRORS CATARACT CORNEAL NYSTAGMUS RETINOPATHY MISCELLANEOUS GLAUCOMA 30
20
n
0
[ ^ UNDER 40
10
20 U
30
40
OVER 40
Fig. 2 Eye anomalies in the total population (excluding Down's syndrome patients).
44
C. Aitchison et al.
Strabismus Thirty-one per cent of the total population and 52% of the number with eye anomalies were found to have strabismus. TTiere were 124 cases: 57% convergent; 40*M) divergent; and 16% vertical. No one type of strabismus was found to be present more frequently in the over 40 age group of 60, but 18 had associated defects of refractive error and cataract. Two of the younger patients had undergone corrective surgery: one 34-yearold female for cosmetic reasons and one 16-year-old female who was attending school at the time. She had a gross alternating convergent squint, restricted abduction of both eyes and a high degree of myopic astigmatis. Provision of spectacle correction improved her visual awareness and corrective surgery to improve abduction was recommended. She had a right medial rectus recession and a right lateral rectus resection in 1981 and is now cosmetically acceptable with a small right convergent squint.
Refractive error (+3/-3 spheres, cyls)
This was the second commonest defect present, occurring in 30% of the total population and 31% of the number with eye anomalies. There was no prevalence of hypermetropia or myopia. The maiority of patients had been prescribed spectacles. In the over-40 age group of 60, there were 15 who had either not been prescribed spectacles or who refused to wear them. As with the previous number of patients with strabismus, there were associated conditions; in this case, strabismus and cataract in 32 cases, 25 over 40. Screening the patients to assess their visual acuity revealed that there were a number who were quite happily wearing the wrong spectacles, having picked up a pair at random or exchanged them. It is imponant that the frames should have an identification mark for the benefit of both patient and ward staff.
Cataract The third largest group of anomalies was cataract: llVo of total population and 19% of the number witb eye anomalies. The age of onset was mainly inconclusive. Two of the under 40s had congenital cataracts, which had been treated by needling during childhood. In the over 40s, 17 had undergone cataract extraction at the Bristol Eye Hospital. Four of these patients had been diagnosed when under 40, and had been kept under observation until surgery was indicated. There were five patients on the waiting list. Patients with unicular cataract and acceptable visual acuity of the unaffected eye are kept under observation and surgery is not normally recommended unless there is deterioration of vision in the good eye. Cases with bilateral cataracts had bilateral cataract extraction with intra-ocular lens implant.
Eye abnormalities in the mentally handicapped
45
Comeal anomalies
There were a small number in this group with kerataconus, lens opacities and comeal scarring following penetrating injury. Nystagmus
There were only 17 patients in this group, mainly associated with strabismus and/or cataract. Retinopathy
Conditions of macular degeneration, optic atrophy and retinal detachment were present in 12 patients. Glaucoma
One 37-year-old male was admitted to the Bristol Eye Hospital in 1981 for bilateral trabeculectomy and later registered blind. Two females are currently being treated witb Timoptol (Timolol meleate) drops. Blindness
Patients with congenital or acquired blindness are registered with the Royal Society for the Blind for relevant benefits. Miscellaneous
There were three patients who had undergone bilateral enucleation, a 62-yearold female who had an artificial right eye and six cases with entropic and ectropic lid anomalies. Operations
The incidence of surgical treatment has been covered, in the main, by the previous group surveillance. In addition, 12 patients have been admitted to the Bristol Eye Hospital for an examination under anaesthesia. Down's syndrome
There were 26 female and five male patients, ages ranging from 21 to 65, recorded IQ extended from
Eye abnormalities in the mentally handicapped C. AITCHISON,'.2 D. L. EASTY1.2 AND J. JANCAR2 'Bristol Eye Hospital and ^Stoke Park Hospital, Bristol, England ABSTRACT. In 1987, a survey of eye abnormalities was undertaken of the mentally handicapped patients in Stoke Park Hospital, Bristol. The total population was 367 patients (262 females and 105 males). Of the 367 patients reviewed, 218 were found to have one or more eye anomalies (including the Down's Syndrome group), which shows that more than half the patients suffered from eye disorders (59%). The prevalence of the following anomalies is presented in detail: strabismus, refractive errors and cataracts, Comeal anomalies, nystagmus, retinopathy, glaucoma and other eye pathologies associated with various syndromes are noted. Eye anomalies in 31 patients sufFering from Down's syndrome in the sample are recorded. Preventative measures to ameliorate the defects of eyesight, particularly in the aging population, relevance of regular eye examination and medical or surgical treatment of eye disorders are discussed.
INTRODUCTION The Reverend Harold Nelson Burden was the founder of Stoke Park Colony, which was opened on 1 April 1909. Mr Burden was not content merely to offer custodial care for the mentally handicapped patients but made financial provision for and encouraged research into the causes, treatment and prevention of mental handicap. In 1930, he established the first multi-disciplinary research centre in the country under the leadership of Professor Berry. The colony was absorbed into the National Health Service in 1948 when it became known as the Stoke Park Hospital Group, consisting of Stoke Park, Purdown, Leigh Court and Hanham Hall Hospitals. In 1930, Mr A. E. lies was appointed as the first ophthalmologist to the research team. Since then, a visiting consultant opthalmologist has been permanently attached to the staff of the Stoke Park Group of Hospitals. Excellent notes on ophthalmic disorders have been preserved since 1930 which enable researchers to study eye anomalies, rare syndromes and genetically transmitted eye disorder (Jancar, 1981). Since 1980, the ophthalmic team, consisting of ophthalmologist, orthoptist and optician, has been responsible for the assessment and treatment of patients with eye problems. The orthoptist (C.A.), has been concerned principally with screening patients to assess their visual acuity, detect the presence of strabismus, and patients with other ocular conditions requiring further investigation have been referred to the ophthalmologist, or optician. Correspondence: Christina Aitchison DBO, Bristol Eye Hospital, Lower Maudlin Street, Bristol BSl 2LX, England. 41
42
C. Aitchison et al.
METHOD There was a considerable number of patients with eye anomalies and the increased aging population. When searching for papers on the subject of ophthalmic anomalies and mental handicap, it was found that there is a scarcity of literature, particularly concerning patients over 40 years of age with eye pathologies. However, some studies show the high incidence of squint and refractive error in mentally handicapped children (Bankes, 1974). Therefore, it was decided that a survey of the residential community of Stoke Park Hospital should be undertaken to identify the prevalence of a variety of eye anomalies found, and to institute the appropriate treatment where possible. The authors realise that only a selective number of patients was used for the survey; it was not possible to gain sufficient additional information, nor gain access to acquiring such from the total number of mentally handicapped living in the community of Frenchay Health District. However, they hope that the results found will stimulate wider interest and act as a basis for future research.
RESULTS The total population of patients reviewed in the survey was 367; 105 males and 262 females (Fig. 1). The ages of females ranged from 15 to 92 (mean age 50-16) and of males from 15 to 77 (mean age 36-89). Recorded IQs extended from 15 to 74 (mean IQ 20 32). Previously, the majority of female patients were admitted to Stoke Park Hospital, and the male patients to Purdown Hospital. This explains the greater number of female patients. Of the 367 patients, there were 31 with Down's syndrome (five males and 26 females). It was decided to look at these as a separate group, bearing in mind that Down's syndrome presents many ocular problems and this group was no exception. Of the 367 patients reviewed, 218 were found to have one or more eye anomalies (including Down's syndrome group) and this shows the high percentage overall: 59%. Excluding the Down's syndrome group, the percentage is still high: 54%. The number of eye anomalies was found to be higher in the aging group. There were 144 patients over 40 years of age of whom 42 suffered from more than one ocular defect (Fig. 2). Longevity in patients with Down's syndrome has increased by an average of 40 years and in other handicapped patients by more than 30 years. New antibiotics and anticonvulsants, along with better environment and care, have been major determinants of increased longevity (Carter & Jancar, 1983). It can be seen that there is a particularly high incidence of strabismus, refractive error, cataract, and a lesser number of patients with comeal defect, nystagmus, retinopathic conditions (i.e. macular degeneration) and miscellaneous conditions (i.e. ectropia, entropia and glaucoma).
Eye abnormalities in the mentally handicapped
43
60 O--O MALE { n=105 ) • - • F E M A L E ( n=262 ) TOTAL=367
40
NUMBERS
20
10-19
20-29
30-39
40-49
50-59
60-69
70-79
80-89
90*
AGE RANGE
Fig. 1 Total hospital population MALE
FEMALE
STRABISMUS REFRACTIVE ERRORS CATARACT CORNEAL NYSTAGMUS RETINOPATHY MISCELLANEOUS GLAUCOMA 30
20
n
0
[ ^ UNDER 40
10
20 U
30
40
OVER 40
Fig. 2 Eye anomalies in the total population (excluding Down's syndrome patients).
44
C. Aitchison et al.
Strabismus Thirty-one per cent of the total population and 52% of the number with eye anomalies were found to have strabismus. TTiere were 124 cases: 57% convergent; 40*M) divergent; and 16% vertical. No one type of strabismus was found to be present more frequently in the over 40 age group of 60, but 18 had associated defects of refractive error and cataract. Two of the younger patients had undergone corrective surgery: one 34-yearold female for cosmetic reasons and one 16-year-old female who was attending school at the time. She had a gross alternating convergent squint, restricted abduction of both eyes and a high degree of myopic astigmatis. Provision of spectacle correction improved her visual awareness and corrective surgery to improve abduction was recommended. She had a right medial rectus recession and a right lateral rectus resection in 1981 and is now cosmetically acceptable with a small right convergent squint.
Refractive error (+3/-3 spheres, cyls)
This was the second commonest defect present, occurring in 30% of the total population and 31% of the number with eye anomalies. There was no prevalence of hypermetropia or myopia. The maiority of patients had been prescribed spectacles. In the over-40 age group of 60, there were 15 who had either not been prescribed spectacles or who refused to wear them. As with the previous number of patients with strabismus, there were associated conditions; in this case, strabismus and cataract in 32 cases, 25 over 40. Screening the patients to assess their visual acuity revealed that there were a number who were quite happily wearing the wrong spectacles, having picked up a pair at random or exchanged them. It is imponant that the frames should have an identification mark for the benefit of both patient and ward staff.
Cataract The third largest group of anomalies was cataract: llVo of total population and 19% of the number witb eye anomalies. The age of onset was mainly inconclusive. Two of the under 40s had congenital cataracts, which had been treated by needling during childhood. In the over 40s, 17 had undergone cataract extraction at the Bristol Eye Hospital. Four of these patients had been diagnosed when under 40, and had been kept under observation until surgery was indicated. There were five patients on the waiting list. Patients with unicular cataract and acceptable visual acuity of the unaffected eye are kept under observation and surgery is not normally recommended unless there is deterioration of vision in the good eye. Cases with bilateral cataracts had bilateral cataract extraction with intra-ocular lens implant.
Eye abnormalities in the mentally handicapped
45
Comeal anomalies
There were a small number in this group with kerataconus, lens opacities and comeal scarring following penetrating injury. Nystagmus
There were only 17 patients in this group, mainly associated with strabismus and/or cataract. Retinopathy
Conditions of macular degeneration, optic atrophy and retinal detachment were present in 12 patients. Glaucoma
One 37-year-old male was admitted to the Bristol Eye Hospital in 1981 for bilateral trabeculectomy and later registered blind. Two females are currently being treated witb Timoptol (Timolol meleate) drops. Blindness
Patients with congenital or acquired blindness are registered with the Royal Society for the Blind for relevant benefits. Miscellaneous
There were three patients who had undergone bilateral enucleation, a 62-yearold female who had an artificial right eye and six cases with entropic and ectropic lid anomalies. Operations
The incidence of surgical treatment has been covered, in the main, by the previous group surveillance. In addition, 12 patients have been admitted to the Bristol Eye Hospital for an examination under anaesthesia. Down's syndrome
There were 26 female and five male patients, ages ranging from 21 to 65, recorded IQ extended from
