Appendix 24 – Ophthalmology pathway
Many hospitals routinely refer to ophthalmology after the baby is born, either following an admission to the neonatal unit or at the postnatal baby check by the paediatrician or midwife. Either way, there MUST be clear documentation that the eyes have been checked formally for cataracts, squints, nystagmus and visual behaviour. Any concerns identified refer immediately.
If the baby has not been referred routinely after birth, in Barnet we have decided to refer routinely to ophthalmology at the 12 month review in order for the child to be seen at 18 months by the Paediatric Ophthalmology team at Barnet hospital. DSMIG guidelines say to refer around age 2-3 years, but in discussion with the team they would like to see these infants within the 2nd year of life and not wait till 2yrs+. Referral by letter to Consultant Ophthalmologist at Barnet hospital (discuss with the team directly if you have urgent concerns or want the child to be seen urgently, do not just send a letter as it may not be triaged as urgent without contacting the consultant ophthalmologist).
Community paediatrician to check vision, visual behaviour and development at every review including checking for cataracts, nystagmus, squints and refer earlier if any concerns.
Children should have a full eye examination by 2 years of age.
Distance and near functioning vision should be checked at every review whenever developmentally possible and a prescription for near correction or bifocals considered at all ages Bifocals should be considered for all children with a focusing deficit
Parents and teachers must be informed that vision is below normal, and classroom modifications will be needed.
Vision is below normal even in children who do not need spectacles, or when correct spectacles are worn.
Barnet Specialist teachers/Teams:
Barnet Education and Learning Service (BELS), 3rd Floor, 2 Bristol Avenue, Colindale, London NW9 4EW
SSTV (Specialist Teaching Team, Vision): VI.Team@barnet.gov.uk
PD&CN (Physical Disability & Complex Needs Support Team): pd.team@barnet.gov.uk
D&HS (Deaf & Hearing Support Team): D&HS.Team@barnet.gov.uk
Education:
Parents and teachers must be informed that vision is below normal, and classroom modifications will be needed.
Ensure vision information is in the EHCP
Look at the type of pens, fonts, paper, print and spacing being used at school.
Children with DS have poorer acuity and contrast sensitivity than their classroom peers.
Shopping glasses frames:
Specsavers
Eye conditions:
Squint
Much more common in children with DS (25- 30%) than in the general population (2-4%)
Managed / treated in the same way – Spectacles – Surgery – Patching
Nystagmus
More common in children with DS (15%) than in the general population (0.02%)
It is NOT part of the syndrome
Nystagmus is a visually impairing condition
Children with nystagmus should be referred to the Visual Impairment support service of the LEA on diagnosis
Keratoconus
Distortion of the cornea causing poor vision
Cannot be corrected with spectacles • Prevalence in Down’s syndrome reported as 10-30%
Many people with Down’s syndrome successfully wear contact lenses - The barriers to contact lenses usually lie with family and carers
Early diagnosis of keratoconus is now critical, but early diagnosis of keratoconus is difficult
Adolescents should be encouraged to have regular eye examinations with a view to detecting keratoconus
Cerebral Visual Impairment (CVI) and Down’s syndrome:
Current research shows that Cerebral/Cortical Visual Impairment (CVI) is more common in neurodevelopmental conditions, including Down syndrome. In one small study, over 38% of kids with Down syndrome showed signs of CVI. Another genetic study suggested that the chromosomal abnormality in Down syndrome could contribute to the development of CVI. DS-CVI is an area of emerging research.
Signs of CVI are often missed in kids with Down syndrome, and the condition is not usually screened for during clinical visits. The challenges associated with CVI are often attributed to behavioural issues, cognitive delay, or other conditions such as autism and attention deficit disorder. Visual challenges might also be associated with other eye problems that commonly occur in Down syndrome, such as keratoconus, nystagmus, and strabismus.
Because of this diagnostic overshadowing, it is crucial to recognize CVI behaviours in Down syndrome to ensure that the individual receives the appropriate services and support to fully access their learning and their world. Because it’s brain-based, CVI has diverse manifestations, and it’s important to understand what access looks like for each person with CVI. Some with CVI use supports that allow them to use their vision without fatigue, some rely on other sensory modalities (e.g., touch, hearing, movement), and many with CVI use both their available functional visual skills and other senses.
Research on Bifocals:
There is some discussion about bifocals from the age of 2 in children with Down Syndrome.
"So now we prescribe bifocals routinely for all children with Down Syndrome who have the focusing defect once their long or short sight is corrected. Almost all of the children take to bifocals very readily, and most make their own choice to wear their bifocals all of the time. The children are clearly demonstrating to us that they benefit from bifocals. Our criterion is that, if a child is old enough to sit at a table and do near tasks (whether work or play), he or she is old enough for bifocals. Once we started to fit bifocals routinely, we found something quite unexpected. Some of the children began to focus accurately over the top of their bifocal (using just the part of the lens that corrects long or short sight). After, on average, two years of wear, they were able to come out of bifocals and return to ordinary lenses. Quite what is happening, we don’t yet know, but it is clear that the bifocals are ‘teaching’ the children to use their own focusing. So far, 40% have stopped needing bifocals, and none have ‘relapsed’ into needing them again. What the above outcomes of our research tell us is this: it was never the case that the children couldn’t focus properly; it was the case that they didn’t. When they wore their bifocals they learned how to do it by themselves. Why 25% of children with Down Syndrome do it anyway, and how the bifocals teach others to do so, we simply don’t know."
Maggie Woodhouse, Cardiff University.
Useful Videos:
Vision in people who have Down's syndrome: https://www.youtube.com/watch?v=uY5njg8Mfx8
Visual Acuity - what is it and why does it matter to people who have Down's syndrome? https://www.youtube.com/watch?v=OONI69GlbrQ
Eye care in young people with Down Syndrome: https://www.youtube.com/watch?v=I2YnwIGgYNM
Down Syndrome and CVI explained: https://www.perkins.org/down-syndrome-and-cvi-explained/
Research and further information:
https://www.dsmig.org.uk/wp-content/uploads/2015/09/Guideline-vision-revision-2012.pdf
https://www.downs-syndrome.org.uk/wp-content/uploads/2022/04/Vision_kp.pdf
Behavioural Features of Cerebral Visual Impairment Are Common in Children With Down Syndrome:
Front Hum Neurosci
. 2021 Jun 14;15:673342. doi:10.3389/fnhum.2021.673342
