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- Teär Fahnehjelm, Kristina, et al.
(author)
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Children with mucopolysaccharidosis risk progressive visual dysfunction despite haematopoietic stem cell transplants
- 2018
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In: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 107:11, s. 1995-2003
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Journal article (peer-reviewed)abstract
- Aim: This prospective study assessed the long-term ocular and visual outcomes of children with mucopolysaccharidoses type I Hurler syndrome (MPS IH) who were treated with haematopoietic stem cell transplants (HSCT). Methods: Clinical ophthalmological assessments were performed on eight patients at the St Erik Eye Hospital, Huddinge, Stockholm, Sweden, from 2001-2018: The median age at diagnosis and HSCT were 12.2 (range 5.0-16.4) and 16.7 (8.0-20.4) months. The last eye examination was at a median of 13.4 (6.3-19.0) years and follow-up lasted a median of 12.0 (5.0-17.4) years. Results: Poor visual acuity, poor night vision and, or, photophobia were reported by six children. The best corrected visual acuity at the last visit was a median of 0.4 and 0.5 in the right and left eye and had declined significantly in two patients. Corneal opacities had increased despite HSCT in five patients. High hyperopia, at a median of +6 Dioptres, occurred in all patients and stiff corneas in all four patients that were measured. The patients' corrected intraocular pressures were normal. Retinal degeneration was identified in two patients. Conclusion: Despite HSCT, the long-term follow-up of patients with MPS IH showed reduced visual acuity due to corneal opacities or retinal degeneration.
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- Teär Fahnehjelm, Kristina, et al.
(author)
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Visual perceptual skills and visual motor integration in children and adolescents after allogeneic hematopoietic stem cell transplantation
- 2018
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In: Pediatric Transplantation. - : Wiley. - 1397-3142 .- 1399-3046. ; 22:2
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Journal article (peer-reviewed)abstract
- The aim of the study was to study visual acuity, visual perceptual, and VMI skills in patients after HSCT in childhood. Tests of visual perceptual skills, VMI, and visual acuity were performed in 102 children/adolescents (age range 4.3-20.9years). Mean time from HSCT to testing was 6.0years (0.9-17.5years). Visual acuity was median 1.0 decimal (range 0.16-1.6). Visual perceptual skills (memory, form constancy, visual sequential memory) and VMI were low compared to age-equivalent normative data with, respectively, 36%, 45%, 60%, and 46% of all patients performing below the 25 percentile. All patients performed significantly lower than the 50 percentile in the reference material in visual sequential memory, P<.001 (boys P<.001 and girls P<.05). All patients also performed significantly lower than the 50 percentile in VMI (P<.01) (boys P<.05). Pretransplant conditioning regimen did not affect outcome if the results were corrected for age at HSCT. Visual perceptual skill problems and VMI problems frequently occur in patients after HSCT in childhood. Age at HSCT and original diagnosis influence the outcome. Neuropsychological assessment including visual perception is recommended in children after HSCT.
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