Visual defects in children with intellectual disability in Kathmandu Valley

Sudan Puri Bachelor of Optometry 11th Batch

INTRODUCTION:
Intellectual disability is the most common developmental disability1. According to the World Health Organization, up to three percent or almost 200 million people of the Worlds population have intellectual disabilities this is the largest disability population in the world. According to the definition by the American Association of Intellectual and Developmental Disabilities (AAIDD), Intellectual disability is a disability characterized by significant limitations both in intellectual functioning and in adaptive behaviour, which covers many everyday social and practical skills. This disability originates before the age of 18. It occurs in 2.5-3% of the general population. It refers to intellectual functioning level well below average and significant limitations in two or more adaptive skill areas. Adaptive skills are the skills needed for daily life. Such skills include the ability to produce and understand language (communication); home-living skills; use of community resources; health, safety, leisure, self-care, and social skills; self-direction; functional academic skills (reading, writing, and arithmetic); and work skills. Intellectual disability includes y Mental retardation y Specific learning disability y Acquired brain injuries y Neurodegenerative diseases Etiology: Intellectual disability begins in childhood or adolescence before the age of 18. Time of onset depends on the suspected cause of the disability. If retardation is caused by chromosomal or other genetic disorders, it is often apparent from infancy.


Genetic conditions. Sometimes an intellectual disability is caused by abnormal genes inherited from parents, errors when genes combine, or other reasons. Examples of genetic conditions are Down syndrome, fragile X syndrome, and phenylketonuria

Problems during pregnancy. An intellectual disability can result when the baby does not develop inside the mother properly. A woman who drinks alcohol or gets an infection like rubella during pregnancy may also have intellectually disabled child.

Problems at birth. If a baby has problems during labor and birth, such as not getting enough oxygen, he or she may have an intellectual disability. Health problems. Diseases like whooping cough, the measles, or meningitis can cause intellectual disabilities. They can also be caused by extreme malnutrition (not eating right), not getting enough medical care, or by being exposed to poisons like lead or mercury

Marked incidence of ocular abnormalities in children with intellectual disability is evident in the various literatures. Most of the children with intellectual disability (45.3-86%) have ocular disorders such as blepharitis, congenital nasolacrimal duct obstruction, blepharoconjunctivitis, chalazion and lenticular opacities; 27.3-78% had an uncorrected refractive error, followed by strabismus in 15.8-36%, nystagmus in 6.8-28%, optic atrophy in 6.5%, and congenital anomalies in 2.5% and 30% children with more than one abnormality2-5.

RATIONALE :
Visual impairment is a major debilitating condition. If visual pronlems are not corrected in normal individuals, cognitive and academic activities will decline. Unrecognized visual impairment not only represents a missed chance for adequate treatment but may also adversely affect intellectual development and social behavior. The health care of intellectually disabled people has not been perceived as an important topic, often regarded of secondary significance. Visual and ocular defects are more common in people with IDs than in the general population. A higher risk of visual impairment, even blindness, is observed in people with IDs. The most common abnormalities that cause hindrance to the normal development are refractive errors, nystagmus, strabismus and also the posterior segment abnormalities. These abnormalities make a child visually handicapped, affect learning environment and if not detected early may lead to amblyopia which ultimately leads to poor quality of life. Visual symptoms may be masked by other disabilities and often go unrecognized. Hence, early detection and intervention of ocular abnormalities (e.g. refractive error correction, prescribing bifocal for near clarity, patching to avoid diplopia) and integrated educational efforts will have a positive influence on the child's learning, academic and perceptual development. Though similar studies have been in Nepal, the previous studies have focused on single disorder rather than cohort of intellectually disabled children in general. This study is expected to assess visual and ocular defects in children diagnosed with intellectual disability and compare within different groups of disorders. This study is also expected dto determine the visual functioning of these children with the help of electrophysiological tests. Thus this study is expected to assess the visual and hence developmental problems among the children with intellectual disability so that early intervention could improve their quality of life.

LITERATURE REVIEW :
In the research done by Parikshit Gogate, Freya Rao Soneji, Jitesh Kharat, Hemant Dulera, Madan Deshpande, and Clare Gilbert at the HV Desai Eye Hospital , Pune, Maharastra, India, A total of 664 students with learning disability were examined, 526 of whom were <16 years of age; 323 (61.4%) were male. A total of 326 (60%) had moderate-to-severe learning disabilities (IQs <50), and the mean IQ was 45.4. Two hundred and thirty-eight (45.3%) had ocular disorder; 143 (27.3%) had an uncorrected refractive error, followed by strabismus in 83 (15.8%), nystagmus in 36 (6.8%), optic atrophy in 34 (6.5%), and congenital anomalies in 13 (2.5%), 103 children had more than one abnormality. Only 12 of the 143 students with refractive errors were using spectacles. A total of 132 (48.7%) children with a history of perinatal insult had ocular problems. Ocular disorders were also common in those with a history of epilepsy, Down's syndrome, and cerebral palsy.

Another research by Nabin Paudel, Susan J Leat, Prakash Adhikari, J Margaret Woodhouse and Jyoti Baba Shrestha, revealed that 80 per cent of the children(36 children with Down Syndrome (19 boys and 17 girls) from the Kathmandu valley, aged from 4 months to 18 years had significant refractive error. Most of them had hyperopia (55 per cent), followed by astigmatism (44 per cent), myopia (25 per cent) and anisometropia (19 per cent). Only two (5.6 per cent) children were strabismic and both of them were alternating esotropes. Nystagmus was present in 10 (28 per cent). Other ocular findings were upward slanting palpebral fissures, blepharitis, congenital nasolacrimal duct obstruction, blepharoconjunctivitis, chalazion and lenticular opacities. In the research by Sanjay Marasini, Nabin Paudel, Prakash Adhikari, Jyoti Baba Shrestha and Merrill D. Bowan, ocular abnormalities were present in 86% of total 36 children with cerebral palsy with mean age 5.48 + 4.75 years. Refractive error was the most common abnormality, and was present in 78% of the children. Strabismus was the second most common ocular abnormality and was present in 36% of the children. 14% of the children had Nystagmus and central cortical visual impairment was suspected in 11%. In the research done by Remzi Karadag, MD; Ramazan Yagci, MD; Mesut Erdurmus, MD; Ugur Can Keskin, MD at the Department of Ophthalmology, Faith university Medical School, Ankara, Turkey, refractive error was the leading ocular diagnosis, found in 56 of 166 patients ranging from 9 to 50 (mean age 24.5years). Strabismus was the second most frequent ocular abnormality. Eyelid abnormalities were detected in 30 patients. Cataract was the fourth most frequent ocular pathology and also most frequent intraocular one. Overall, 28 patients (16.9%) had anterior segment findings, of whom 18 had congenital lens opacity. Posterior segment findings were detected in 23 of 166 patients.

OBJECTIVES:
General Objective:  To evaluate various ocular/visual defects in children with intellectual disability

Specific Objective:        To determine visual function in children with intellectual disability with age appropriate visual acuity tests To determine the prevalence of refractive error in children with intellectual disability To assess the visual function in children with intellectual disability with the help of electrophysiological tests To determine the prevalence of anterior/posterior segment disorders in children with intellectual disability To determine the accommodative status in children with intellectual disability. To determine contrast sensitivity in children with intellectual disability. To determine prevalence of low vision in children with intellectual disability.

METHODOLOGY
Study design: Descriptive, cross-sectional
.

Place of study: B P Koirala Lions Center for Ophthalmic studies Study period : 1 year Target Population: All the children diagnosed to have intellectual disability
Inclusion Criteria: All children diagnosed to have intellectual disability - attending or admitted to Kanti Children hospital - attending pediatric outpatient department of BPKLCOS - enrolled in special schools/ day care centers Exclusion criteria: - Children whose examination cannot be carried out - Children who need anesthesia for ocular examination.

Methods:
Informed consent will be obtained from the guardians of the children. All the relevant particulars will be noted. All the children will be called to B P Koirala Lions center for Ophthalmic studies. Detail ophthalmological examination will be carried out that will include best corrected visual acuity testing with appropriate test accordance to the cooperation of the child; CSM (central ,steady, maintained) test , catford drum, Sheridan Gardiner chart, Kay picture chart , Snellen chart, Bailey-Lovie log MAR chart, Teller Acuity cards, VEP, visual behavior and fixation preference in those children who are non verbal or severely mentally retarded. Contrast sensitivity will be tested by using Hiding Heidi Contrast test. Accommodation will be measured by dynamic retinoscopy using Notts method. Examination of the ocular adnexa, ocular motility and strabismus (Hirschberg test /cover test), slit lamp examination, cycloplegic refraction, and dilated ophthalmoscopy will be performed. Amblyopia will be considered if there is difference of 2 lines or more. Cycloplegic refraction will be done by instilling 1% cyclopentolate, 3 times in 10 minutes interval, and retinoscopy will be carried out 45 minutes after first drop. Myopia will be considered if the error is equal or more than -0.50D, hyperopia will be considered for equal or more than +1.00 and astigmatism as 0.75 DC. Full refractive correction will be prescribed. Strabismus examination will be done by evaluating the versions and ductions using a light or an object and cover tests to diagnose and measure ocular misalignment. Strabismus will be measured using prisms or the Hirschberg method. Esodeviation will be considered if the deviation is greater than +4 prism diopters and exodeviation will be considered if the deviation is >-7 prism diopters. If the child is found to have low vision he/she will be evaluated for low vision care and appropriate device will be prescribed or recommended. Posterior segment anomalies will be evaluated with the help of Indirect ophthalmoscopy. Intervention (if needed) will be recommended and referred to Ophthalmologist. Keratometry will be done with the help of Nidek Autokeratometer to detect keratoconus. Stereopsis will be measured by appropriate tests if possible. Data will be analysed with the help of SPSS computer software.

REFERENCES: 1. National Center on Birth Defects and Developmental Disabilities. (2005). Intellectual disability. Available online at: http://www.cdc.gov/ncbddd/actearly/pdf/parents_pdfs/IntellectualDisability.pdf 2. Paudel N, Leat S.J, Adhikari P, Woodhouse M.J, Shrestha J.B. Visual defects in Nepalese children with Down Syndrome. Clinical and Experimental Optometry,2010:93,83-90 3. Marasini S, Paudel N, Adhikari P, Shrestha J.B, Bowan M.D. Ocular manifestations in children with cerebral palsy. Optometry and Vision Development.2011: 42,10-14. 4. Gogate P, Rishikeshi N, Mehta R, Deshpande M, Ranade S. Vision impairment in the hearing impaired. Ind J Ophthalmol. 2009;57:451 3. 5. Ghising R, ShakyaS , Rizyal A, Shrestha R, Shrestha S, Wang-Harris S. Prevalence of refractive error in intellectually retarded students of Kathmandu valley. Nepal Med Coll J. 2007;9:262 5. 6. Woodruff, M.E., Clearly, T.E. & Bader, D. (1980). The prevalence of refractive and ocular anomalies among 1242 institutionalized mentally retarded persons. American Journal of Optometry and Physiological Optics, 57, 57-70. 7. McCulloch, D.L, Sludden, P.A., & McKeown, K., (1996). Vision care requirements among intellectually disabled adults: a residence-based pilot study.Journal of Intellectual Disability Research, 40, 140-150. 8. Levy, B. (1984). Incidence of oculo-visual anomalies in an adult population of mentally retarded persons. American Journal of Optometry and Physiological Optics, 61, 324-326. 9. Haugen, O.H., Aasved, H. & Bertelsen, T. (1995) .Refractive state and correction of refractive errors among mentally retarded adults in a mental institution. Acta Ophthalmologica Scandinavica, 66, 46-52. 10. Casta, M., Peris, E., & Snchez, E. (1995).Ocular dysfunction associated with mental handicap. Ophthalmic and Physiological Optics, 5 (15) 489-492. 6. France TD,Shapiro Mb ,Trisomy 21 Down Syndrome In Gold Dh ,Weigeist TA,eds.The eye in systemic disease.Philadelphia J.B.Lippincott 1990:35-37 7 Falls HF,ocular changes in mongolism ,Ann NY Acad Sci 1970:171:627-36 8 Black, P. (1982). Visual disorders associated with cerebral palsy. British Journal of Ophthalmology, 66, 46-52. 9 Susan Day. Normal and abnormal visual development. Section 1, chapter 2. In: Taylor D, editor. Pediatric ophthalmology. Oxford UK: Blackwell Science; 1997. pp. 13 28. 10 Warburg M. Visual impairment in adult people with intellectual disability: Literature review. J Intellect Disabil Res. 2006;50:470. 11 Van den Broek EC, Janssen CG, Van Ramshorst T, Deen L. Visual impairments in peoples with severe and profound multiple disabilities: An inventory of visual functioning. J Intellect Disabil Res. 2006;50:470 5. 12 Warburg M. Visual impairment in adult people with moderate, severe, and profound intellectual disability. Acta Ophthalmol Scand. 2001;79:450 4.

13 Evenhuis HM, Theunissen M, Denkers I, Verschuure H, Kemme H. Prevalence of visual and hearing impairment in a Dutch institutionalized population with intellectual disability. J Intellect Disabil Res. 2001;45:457 64. 14 Van Splunder J, Stilma JS, Bernsen RM, Arentz TG, Evenhuis HM. Refractive errors and visual impairment in 900 adults with intellectual disabilities in the Netherlands. Acta Ophthalmol Scand. 2003;81:123 30. Van SplunderJ, Stilma JS, Bernsem RM, Evenhuis HM. Prevalence of ocular diagnosis found in screening 1539 adults with interectual disabilities. Ophthalmol. 2004;118:1457 63. 15 Van Isterdael CE, Stilma JS, Bezemer PD, Tijmes NT. 6220 institutionalized people with intellectual disability referred for visual assessment between 1993 and 2003: Overview and trends. Br J Ophthalmol. 2006;90:1297 303. 16 Katoch S, Devi A, Kulkarni P. Ocular defects in cerebral palsy. Ind J Ophthalmol. 2007;55:154 6. 17 Govind A, Lamba PA. Visual disorders in cerebral palsy. Ind J Ophthalmol. 1988;36:88 91. 18 World Health Organization. Mental Retardation: From knowledge to action. What causes Mental Retardation? Why does mental retardation occur? 19 Saigal S, Doyle LW. An overview of mortality and squeal of preterm birth from infancy to adulthood. Lancet. 2008;371:261 9. 20 Kamat V. Measuring intelligence of Indian children. Oxford: Oxford university press; 1967. 21 Kennerley Bankes JL. The ophthalmologist's role in multidisciplinary assessment of developmentally handicapped children. Br J Ophthalmol. 2006;90:1297 303. 22 Nepal BP, Koirala S, Adhikary S, Sharma AK. Ocular morbidity in school children in Kathmandu. Br J Ophthalmol. 2003;87:531 4.

Visual defects in children with intellectual disability in Kathmandu Valley

Address: .. Visual Acuity: Unaided

Aided

Pinhole (if possible)

Please specify the method of taking VA

Any ocular complains.

Ocular examination
Eyelids and adnexa
Epicanthal folds Present Blepharitis Present Nasolacrimal duct obstruction Present Absent Absent Absent

If others ,Specify

Conjunctiva :
Blepharoconjunctivitis : Present Absent

Specify, if others

Cornea :
K reading (if possible): OD .. Keratoconus: Present OS Absent

If others, specify

Pupil :
Normal Abnormal A Specify .

Anterior chamber:
Normal Abnormal Specify.

Intraocular pressure :
Normal High

Lens :
Normal Abnormal Specify, If abnormal (type of cataract).................

Vitreous :
Normal Abnormal Specify.

Fundus :
Macula : Optic disc Periphery Normal Abnormal

Normal Normal

Abnormal Abnormal

If Abnormal,specify..

Orthoptics check up:

Nystagmus :
Present Absent If present, mention type..

Strabismus :
Present Absent If present; mention type.. Magnitude of strabismus. Specify test performed to detect strabismus:...................................................................

Accommodation :
Lead Lag

If present, magnitude..

Contrast sensitivity:
OD .. OS

Gross stereopsis :
Present Absent Specify the test and magnitude of stereopsis:

Visual Evoked Potential findings. ...

Refraction
Objective refraction:
Dry(WDR) OD OS OD Wet{WDR} OS

Dynamic retinoscopy: OD OS

Subjective refraction:

Acceptance: OD: OS:..

Signature........................... Date.................................

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