Visual Function Key in Quality-of-Life for Patients with Unilateral Anophthalmia

UNITED STATES – JUNE 05: Julianna Reala who will be 7-years-old on June 9, plays baseball in the Challenger League of the Great Kills Little League in Staten Island. Reala was born with bilateral anophthalmia, or born without eyes. She wears prosthetic eyes. (Photo by Todd Maisel/NY Daily News Archive via Getty Images)
Researchers evaluate both children born without 1 eye and their parents, and consider the weight of perceptual visual dysfunctions.

Pediatric subjects with unilateral anophthalmia or microphthalmia who wear an ocular prosthesis have lowered health-related quality of life (HR-QoL) scores, especially when they have subnormal visual acuity (VA), researchers found in a cross-sectional observational study that was published in Acta Ophthalmologica.

The researchers wanted to assess vision-specific patient-reported outcome measures of patients with unilateral anophthalmia or microphthalmia and their parents or caretakers.

They reviewed the cases of 8 male and 7 female patients between the ages of 1.7 years to 14.1 years who had been diagnosed with unilateral anophthalmia (3 subjects) or microphthalmia (12 subjects) and were treated with an ocular prosthesis at a university hospital in Sweden between 2000 and 2012 participated in the study, along with their parents.

Each participant completed the Pediatric Quality of Life Inventory (PedsQL), the Children’s Visual Function Questionnaire (CVFQ) and the Effect of Youngsters’ Eyesight on QoL (EYE-Q). Parents or caregivers were also given a separate set of the same questionnaires. The researchers assessed the VA, perceptual visual dysfunctions, genetic assessment of each pediatric subject.

They compared the HR-QoL data with previously published scores of 401 healthy children and 717 parents, the VR-QoL with a previously published reference group of 33 pediatric subjects with no eye diseases, and the perceptual visual dysfunctions with an age- and sex-matched control group of 118 healthy children aged 4 years to 18.1 years.

They found that the mean total PedsQL score in the study group was 66.3 (SD: 21.4) while the healthy controls had a mean score of 83.0 (SD: 14.79, P =.0035). The largest difference (-21.28) between the groups’ mean scores was in psychosocial scores on the PedsQL (study group: 61.1, SD: 17.1; control group: 82.38, SD: 15.51; P =.0004).

The 4 subjects in the study group who had normal VA for their age had a higher mean score for the physical health portion of the PedsQL (78.1, SD: 16.3) than the 3 subjects in the study group who had subnormal VA for their age (22.9, SD: 32.4, P =.03). The subjects with normal VA for their age group also had higher mean school function scores (66.3, SD: 4.8) than the 3 subjects with subnormal VA (55.0, SD: 7.1, P =.05).

Excluding 4 study group participants aged 1.1 to 2.1 years, the researchers found that 7 of the remaining 11 study group participants had perceptual visual dysfunctions in a median of 2 areas while only 4 of the 118 subjects in the control group had at least 1 PVD and they each had normal VA (P <.0001, confidence interval (CI): 33.3-85.6). Depth perception (5), simultaneous perception (3), and movement perception (3) were the perceptual visual dysfunctions the patients reported most frequently. Of the 7 participants with anophthalmia or microphthalmia who had normal VA, 5 had perceptual visual dysfunctions in at least 1 other area, whereas 2 of the 4 with subnormal sight had perceptual visual dysfunctions in at least 1 area.

The 5 subjects (aged 8 years to 18 years) with anophthalmia or microphthalmia who completed the EYE-Q had a mean outcome of 3.15; the 2 subjects with subnormal VA scored 2.38, and the 3 subjects with normal VA scored 3.67.

The mean total score of the 10 parents was slightly lower than the mean total score of the parents of the control group (0.77, SD: 0.10; .85, SD: 0.12).

A limitation of the study was the small sample size, the researchers said.

Eye examinations, individual assessment and case management can benefit children with A/M, and early evaluation of HR-QoL and PVDs may help identify which patients need more treatment, they added.


Casslen B, Jugard Y, Najim RT, et al. Visual function and quality of life in children andadolescents with anophthalmia and microphthalmia treated with ocular prosthesis. Acta Ophthalmologica. 2020;98(7):662-670. doi: 10.1111/aos.14424.