Functional vision and quality of life in children with microphthalmia/anophthalmia/coloboma—a cross-sectional study

doi:10.1016/j.jaapos.2018.01.015

Journal of American Association for Pediatric Ophthalmology and Strabismus

Volume 22, Issue 4, August 2018, Pages 281-285.e1

https://doi.org/10.1016/j.jaapos.2018.01.015 Get rights and content

Purpose

To determine the child's and parental perception of functional visual ability (FVA), vision-related and health-related quality of life (VR-QoL, HR-QoL) in children with microphthalmia/anophthalmia/coloboma (MAC).

Methods

Between June 25, 2014, and June 3, 2015, we carried out a cross-sectional observational study at Moorfields Eye Hospital, London, UK, enrolling 45 children 2-16 years of age with MAC attending our clinics, and their parents. To assess FVA, VR-QoL, and HR-QoL we asked participants to complete three validated tools, the Cardiff Visual Ability Questionnaire for Children (CVAQC), the Impact of Vision Impairment for Children (IVI-C) instrument, and the PedsQL V 4.0. The main outcome measures were the FVA, VR-QoL, and HR-QoL scores, reported by children and parents.

Results

In children with MAC, FVA is moderately reduced, with a median CVAQC score of −1.4 (IQR, −2.4 to 0.4; range, −3.0 [higher FVA] to +2.8 [lower FVA]). VR-QoL and HR-QoL are greatly reduced, with an IVI-C median score of 63 (IQR, 52-66; normal VR-QoL, 96), a median self-reported PedsQL score of 77 (IQR, 71-90; normal HR-QoL, 100) and parental score of 79 (IQR, 61-93), and a family impact score of 81 (67-93). Psychosocial well-being scores are lower than physical well-being scores. Parents and children have a different perception of the impact of the condition on the child's HR-QoL.

Conclusions

MAC has a significant impact on a child's FVA and QoL, similar to that described by children with acute lymphoblastic leukaemia and chronic systemic conditions. Children and families may benefit from psychosocial support.

Section snippets

Subjects and Methods

This study was approved by the National Research Ethics Committee South Central–Oxford A (14/SC/1052) and adhered to the tenets of the Declaration of Helsinki. Between June 25, 2014, and June 3, 2015, children 2-16 years of age attending Moorfields Eye Hospital were prospectively enrolled. Exclusion criteria were inability to communicate in English and surgical intervention within 1 month (before or after) of completing questionnaires. We screened the medical records of all children attending

Results

We approached 62 families of children with MAC who met the inclusion criteria. Sixteen declined to take part because of perceived lack of time. We enrolled 46 children and removed one dataset, because the child did not have MAC, resulting in the analysis of 45 datasets.

The proportion of missing data was low. No data were missing for age, sex, diagnoses, laterality, and best-corrected visual acuity. Ethnicity was unknown in 13.3%. Questionnaire response rates were high (eTable 1).

The median IQR

Discussion

This is the first report of FVA, VR-QoL, and HR-QoL in children with MAC. Previous studies reported increased anxiety and feelings of shame, shyness, sadness, and fear in adults with MAC, but these studies included also nondevelopmental MAC, such as post-traumatic or post-infectious forms of anophthalmia.6, 7 The reduction in HR-QoL in children with MAC we report here is similar to levels reported by children with acute lymphoblastic leukemia and chronic diseases.14, 15 In addition, VR-QoL is

Acknowledgments

The authors thank Miss Anneka Tailor for supporting data collection and entry and Miss Konstantina Prapa for facilitating enrollment of study participants. The Quality of Life study described in this paper was carried out using the PedsQL, which was developed by Dr. James W. Varni.

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“My child gets along well with our other children and friends”, “My child makes new friends easily”, “My child enjoys playing with others (sisters and brothers or friends).”). Psychosocial well-being scores are similarly reduced in children with microphthalmia (ocular malformations in newborns resulting in smaller than average eyes), anophthalmia (the eyes fail to develop prenatally) and coloboma (ocular malformation affecting the eyelid, lens, macula, optic nerve, choroid, iris, or corpus ciliare),23 as well as in children with retinoblastoma (an ocular cancer that can require removal of the eye).24 At least some of these psychosocial impairments may persist into adolescence.
Visual cues usually play a vital role in social interaction. As well as being the primary cue for identifying other people, visual cues also provide crucial non-verbal social information via both facial expressions and body language. One consequence of vision loss is the need to rely on non-visual cues during social interaction. Although verbal cues can carry a significant amount of information, this information is often not available to an untrained listener. Here, we review the current literature examining potential ways that the loss of social information due to vision loss might impact social functioning. A large number of studies suggest that low vision and blindness is a risk factor for anxiety and depression. This relationship has been attributed to multiple factors, including anxiety about disease progression, and impairments to quality of life that include difficulties reading, and a lack of access to work and social activities. However, our review suggests a potential additional contributing factor to reduced quality of life that has been hitherto overlooked: blindness may make it more difficult to effectively engage in social interactions, due to a loss of visual information. The current literature suggests it might be worth considering training in voice discrimination and/or recognition when carrying out rehabilitative training in late blind individuals.
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In a study of children with glaucoma, teens also reported better QoL than younger children with the same diagnosis.20 Similar findings were reported in a small study on microphthalmia.21 This may indicate that as children mature, they learn to cope with their visual impairment better, and it affects their QoL less.
The PedsQL 4.0 is a generic health-related quality-of-life (HRQoL) instrument that has been used across many pediatric health conditions. The purpose of this study was to investigate its use in children with vision impairment and to test its ability to distinguish between visually impaired and normally sighted children.
Children with vision impairment and age-matched controls underwent vision testing, including binocular best-corrected distance visual acuity. They completed the PedsQL 4.0 Generic Core Scale Child Report (ages 8-12) or Teen Report (ages 13-18), as appropriate. Parents completed the Parent Proxy Report in a room separate from their children. Analyses included descriptive statistics, intraclass correlations, t tests, and determination of Cronbach's α for subscales.
A total of 70 study subjects and 44 controls were included. Groups were similar with regard to age, race, and sex. PedsQL 4.0 subscales showed no floor effects; however, some scales had ceiling effects of up to 28% in certain groups. Cronbach's α was excellent (0.88) for all subscales and reports. PedsQL 4.0 total score for children with vision impairment differed significantly from that for normally sighted children for both age groups and for both parent and child reports. Total score was also significantly associated with visual acuity. Parent and child reports correlated poorly (ICC, <0.5), highlighting the importance of both perspectives.
The PedsQL 4.0 can be used to assess HRQoL in children and teens with low vision. It has internal consistency reliability, and construct validity.
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: AHDN and VT are employed by the National Institute for Health Research Biomedical Research Centre at Moorfields Eye Hospital and UCL Institute of Ophthalmology, and as such the work was supported by the NIHR. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR, or the Department of Health.

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Major ArticleFunctional vision and quality of life in children with microphthalmia/anophthalmia/coloboma—a cross-sectional study

Purpose

Methods

Results

Conclusions

Section snippets

Subjects and Methods

Results

Discussion

Acknowledgments

Microphthalmia, anophthalmia, and coloboma and associated ocular and systemic features: understanding the spectrum

JAMA Ophthalmol

The genetic architecture of microphthalmia, anophthalmia and coloboma

Eur J Med Genet

Congenital microphthalmia, anophthalmia and coloboma among live births in Denmark

Ophthalmic Epidemiol

Anophthalmos, microphthalmos, and typical coloboma in the United Kingdom: a prospective study of incidence and risk

Invest Ophthalmol Vis Sci

Health-related quality of life and emotional status of anophthalmic patients in Korea

Am J Ophthalmol

Psychosocial impact on anophthalmic patients wearing ocular prosthesis

Int J Oral Maxillofac Surg

Development of the 25-item Cardiff Visual Ability Questionnaire for Children (CVAQC)

Br J Ophthalmol

The Impact of Vision Impairment for Children (IVI_C): validation of a vision-specific pediatric quality-of-life questionnaire using Rasch analysis

Invest Ophthalmol Vis Sci

Major Article
Functional vision and quality of life in children with microphthalmia/anophthalmia/coloboma—a cross-sectional study