| 1. |
- Casslén, Beatrice, et al.
(author)
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Health-related quality of life and functional vision in individuals with retinoblastoma treated with ocular prothesis
- 2023
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In: Investigative Ophthalmology and Visual Science. - : Association for Research in Vision and Ophthalmology. - 0146-0404 .- 1552-5783. ; 64:8
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Journal article (other academic/artistic)abstract
- Purpose: Health-Related Quality of Life (HR-QoL) among enucleated retinoblastoma (RB) survivors is scarcely studied. Perceptual Visual Dysfunctions (PVDs) are problems concerning interpretation of visual input, that to our knowledge, previously not has been studied in RB patients. This prospective cross-sectional cohort study aim to evaluate HR-QoL and PVDs in RB individuals, treated with ocular prosthesis.Methods: Twenty-seven RB individuals were treated with ocular prosthesis at the Sahlgrenska University Hospital, Gothenburg, Sweden, between 2000-2019. All were invited to the study, 15 (10 females; mean age 15.6 years, range 6.8-27.0 years) accepted. HR-QoL was assessed using the Pediatric Quality of Life inventory (PedsQL) with patients self- and parents-proxy report. Results were compared to normative data. PVDs was examined by history taking covering five areas and results were compared with a healthy, age- and sex- matched control group. Best corrected visual acuity (BCVA) was measured.Results: No differences were found in HR-QoL of individuals with RB compared with healthy controls, between parent proxy compared with parents of healthy children or between individuals with RB and their corresponding parents. More individuals with RB (9/15) reported PVDs in one or more areas (median 1; range 1-4) compared with 1/15 healthy controls; p=0.005 (Fig. 1). Depth perception was the most frequent reported PVD area (n=6), followed by simultaneous perception (n=5), movement (n=2), recognition (n=1) and orientation (n=1). Better HR-QoL correlated with better BCVA (r = -0.68; p=0.01) and fewer affected PVD areas (r = -0.63; p=0.01).Conclusions: The results showed no difference in HR-QoL of the RB individuals or parent-proxy compared with healthy controls. However, enucleated RB survivors were more affected by PVDs than healthy individuals. Their HR-QoL can be negatively affected by having problems within several PVD areas, and BCVA comprise an important role in QoL. It is necessary to identify PVDs to promote the best health care in individuals with RB treated with ocular prothesis. Further research is needed to better understand the impact on QoL and the role of PVDs among these individuals.
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| 2. |
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| 4. |
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| 5. |
- Dahl, S., et al.
(author)
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Children with optic nerve hypoplasia face a high risk of neurodevelopmental disorders
- 2018
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In: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 107:3, s. 484-489
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Journal article (peer-reviewed)abstract
- Aim: Optic nerve hypoplasia (ONH) is a congenital ocular malformation that has been associated with neurodevelopmental disorders, but the prevalence in unilateral disease and less severe visual impairment is unknown. We studied intellectual disability and autism spectrum disorders (ASDs) in patients with ONH. Methods: This was a population-based cross-sectional cohort study of 65 patients (33 female) with ONH below 20 years of age, living in Stockholm in December 2009, with data analysed in January 2016. Of these 35 were bilateral and 30 were unilateral. Neurodevelopmental disorders were diagnosed or confirmed by neurological assessments, the Five to Fifteen parent questionnaire and reviewing previous neuropsychological investigations or conducting neuropsychological tests. Results: Bilateral ONH patients had lower mean full scale intelligence quotient scores than unilateral patients (84.4 and 99.4, respectively, p = 0.049). We assessed intellectual disability in 55 eligible patients, and it was more common in patients with bilateral ONH (18 of 32, 56%) than unilateral ONH (two of 23, 9%, p < 0.001). ASDs were diagnosed in seven of 42 (17%) patients. Conclusion: Children with bilateral ONH had a high risk of neurodevelopmental disorders, especially intellectual disability. The risk was lower in unilateral ONH, but the levels of neurodevelopmental disorders warrant screening of both groups.
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| 6. |
- Dahl, S., et al.
(author)
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High prevalence of pituitary hormone deficiency in both unilateral and bilateral optic nerve hypoplasia
- 2019
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In: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 108:9, s. 1677-1685
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Journal article (peer-reviewed)abstract
- Aim This study examined the prevalence of neurological impairment and pituitary hormone deficiency (PHD) in patients with unilateral and bilateral optic nerve hypoplasia (ONH). Methods A population-based cross-sectional cohort study of 65 patients (51% female) with ONH was conducted in Stockholm. Of these were 35 bilateral and 30 unilateral. The patients were below 20 years of age, living in Stockholm in December 2009 and found through database searching. The median age at the analysis of the results in January 2018 was 16.1 years (range 8.1-27.5 years). Neurological assessments and blood sampling were conducted, neuroradiology was reviewed and growth curves were analysed. Diagnoses of PHDs were based on clinical and biochemical evidence of hormone deficiency. Results Neurological impairments were identified in 47% of the patients and impairments in gross and fine motor function were more prevalent in bilateral ONH (p < 0.001). In addition, 9% had cerebral palsy and 14% had epilepsy. The prevalence of PHD was 29 and 19% had multiple PHD. Conclusion Children with ONH had a high risk of neurological impairment, especially in bilateral disease. Both unilateral and bilateral ONH signified an increased prevalence of PHD and all these children should be endocrinologically followed up until completed puberty.
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| 7. |
- Fahnehjelm, Kristina Teär, et al.
(author)
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Ocular characteristics in 10 children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency : a cross-sectional study with long-term follow-up.
- 2008
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In: Acta Ophthalmologica Scandinavica. - : Wiley. - 1395-3907 .- 1600-0420 .- 1755-375X .- 1755-3768. ; 86:3, s. 329-337
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Journal article (peer-reviewed)abstract
- PURPOSE To present long-term ocular complications and electroretinographic (ERG) findings in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency - a life-threatening metabolic disease - and the relation to age at diagnosis, treatment and other clinical parameters. METHODS Ten children with LCHAD deficiency underwent repeated ophthalmological evaluations including ERG. RESULTS All 10 children developed chorioretinal pathology. Regardless of age at diagnosis, initiation of treatment and age at examination, inter-individual differences were present. Profound chorioretinal atrophy, severe visual impairment and progressive myopia had developed in two teenagers. Milder chorioretinopathy with or without subnormal visual acuity was present in all other children. ERG was pathological in seven children. The chorioretinopathy often started in the peripapillary or perimacular areas. In one patient, unilateral visual impairment was associated with fibrosis. CONCLUSION Early diagnosis and adequate therapy might delay but not prevent the progression of retinal complications. Late diagnosis with severe symptoms at diagnosis, neonatal hypoglycaemia and frequent decompensations may increase the progression rate of the chorioretinopathy. LCHAD deficiency, a potentially lethal disease, is sometimes difficult to diagnose. Unusual chorioretinal findings should alert the ophthalmologist to the long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, especially if there is a history of neonatal hypoglycaemia or failure to thrive.
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| 8. |
- Fahnehjelm, Kristina Teär, et al.
(author)
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Visual function, ocular motility and ocular characteristics in patients with mitochondrial complex I deficiency
- 2012
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In: Acta ophthalmologica. - : Wiley. - 1755-3768 .- 1755-375X. ; 90:1, s. 32-43
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Journal article (peer-reviewed)abstract
- Abstract. Purpose: The aims of the present study were to investigate visual function, ocular motility and ocular characteristics in children and young adults with complex I deficiency. Material and Methods: In a prospective study with longitudinal follow-up, the visual and ocular outcome in 13 patients with deficiency in complex I [nicotine-amide adenine dinucleotide (NADH) dehydrogenase] in the mitochondrial respiratory chain is presented. The patients were diagnosed during 1995-2007 and assessed during 1997-2009 at a median age of 12.8 years (range 3.1-23.4). Results: Twelve of 13 patients had visual impairment and/or ocular pathology. Four of 10 patients who co-operated in visual assessment had a best corrected decimal visual acuity of A mutation in mitochondrial DNA. Only one patient had normal visual acuity and ocular outcome including refraction and visual fields. Follow-up time was median 3.0 years (range 0-11). Conclusion: Visual impairment, ocular motility problems and OA are common in children and young adults with complex I deficiency and should prompt the paediatric ophthalmologist to consider mitochondrial disorders.
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| 9. |
- Inamoto, Y., et al.
(author)
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Non-Graft-versus-Host Disease Ocular Complications after Hematopoietic Cell Transplantation: Expert Review from the Late Effects and Quality of Life Working Committee of the Center for International Blood and Marrow Transplant Research and the Transplant Complications Working Party of the European Society for Blood and Marrow Transplantation
- 2019
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In: Biology of Blood and Marrow Transplantation. - : Elsevier BV. - 1083-8791. ; 25:5, s. E145-E154
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Journal article (peer-reviewed)abstract
- Non-graft-versus-host disease (GVHD) ocular complications are generally uncommon after hematopoietic cell transplantation (HCT) but can cause prolonged morbidity affecting activities of daily living and quality of life. Here we provide an expert review of non-GVHD ocular complications in a collaboration between transplantation physicians and ophthalmologists through the Late Effects and Quality of Life Working Committee of the Center for International Blood and Marrow Transplant Research and the Transplant Complications Working Party of the European Society of Blood and Marrow Transplantation. Complications discussed in this review include cataracts, glaucoma, ocular infections, ocular involvement with malignancy, ischemic microvascular retinopathy, central retinal vein occlusion, retinal hemorrhage, retinal detachment and ocular toxicities associated with medications. We summarize the incidence, risk factors, screening, prevention, and treatment of individual complications and generate evidence-based recommendations. Baseline ocular evaluation before HCT should be considered in all patients who undergo HCT. Follow-up evaluations should be considered according to clinical signs and symptoms and risk factors. Better preventive strategies and treatments remain to be investigated for individual ocular complications after HCT. Both transplantation physicians and ophthalmologists should be knowledgeable about nonGVHD ocular complications and provide comprehensive collaborative team care. (C) 2018 American Society for Blood and Marrow Transplantation.
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| 10. |
- Inamoto, Y., et al.
(author)
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Non-GVHD ocular complications after hematopoietic cell transplantation: expert review from the Late Effects and Quality of Life Working Committee of the CIBMTR and Transplant Complications Working Party of the EBMT
- 2019
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In: Bone Marrow Transplantation. - : Springer Science and Business Media LLC. - 0268-3369 .- 1476-5365. ; 54:5, s. 648-661
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Journal article (peer-reviewed)abstract
- Non-graft-vs.-host disease (non-GVHD) ocular complications are generally uncommon after hematopoietic cell transplantation (HCT), but can cause prolonged morbidity affecting activities of daily living and quality of life. Here we provide an expert review of non-GVHD ocular complications in a collaboration between transplant physicians and ophthalmologists through the Late Effects and Quality of Life Working Committee of the Center for International Blood and Marrow Transplant Research and the Transplant Complications Working Party of the European Society of Blood and Marrow Transplantation. Complications discussed in this review include cataracts, glaucoma, ocular infections, ocular involvement with malignancy, ischemic microvascular retinopathy, central retinal vein occlusion, retinal hemorrhage, retinal detachment, and ocular toxicities associated with medications. We have summarized incidence, risk factors, screening, prevention and treatment of individual complicastions and generated evidence-based recommendations. Baseline ocular evaluation before HCT should be considered in all patients who undergo HCT. Follow-up evaluations should be considered according to clinical symptoms, signs and risk factors. Better preventive strategies and treatments remain to be investigated for individual ocular complications after HCT. Both transplant physicians and ophthalmologists should be knowledgeable of non-GVHD ocular complications and provide comprehensive collaborative team care.
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| 11. |
- Ohden, K. L., et al.
(author)
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Outcomes of keratoplasty in the mucopolysaccharidoses: an international perspective
- 2017
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In: Br J Ophthalmol. - : BMJ. - 0007-1161 .- 1468-2079. ; 101:7, s. 909-912
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Journal article (peer-reviewed)abstract
- OBJECTIVE: To describe visual outcomes after penetrating keratoplasty and deep anterior lamellar keratoplasty in patients with mucopolysaccharidoses. METHODS: This is a retrospective review of keratoplasty in consecutive patients from Brazil, England, Finland, Germany, Portugal, Sweden and the USA. All patients had corneal clouding due to mucopolysaccharidoses. Preoperative and postoperative visual outcome and ocular comorbidities were identified. Success was arbitrarily defined as any improvement in visual acuity or best-corrected visual acuity better than logarithm of the minimum angle of resolution 0.30 (20/40). Statistical analysis included only data from first operated eyes in the 16 patients who underwent bilateral keratoplasty. RESULTS: Forty-eight eyes from 32 patients with mucopolysaccharidoses I, IV or VI are reported. Mean follow-up was 70 months (range: 5-186). Penetrating keratoplasty was performed in 45 eyes and deep anterior lamellar keratoplasty in 3 eyes. At last follow-up, a successful visual outcome for penetrating keratoplasty in first operated/only operated eyes was found in 63%. Rejection episodes occurred in 23% of grafts; however, a clear graft was recorded at last follow-up in 94%. Ocular pathway comorbidities were identified in 63% of eyes transplanted. CONCLUSIONS: Clear corneal grafts can be obtained for patients with corneal clouding due to mucopolysaccharidosis with improvement in visual acuity in the majority.
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| 12. |
- Skriapa Manta, Athanasia, et al.
(author)
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Optic Disc Coloboma in children - prevalence, clinical characteristics and associated morbidity
- 2019
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In: Acta Ophthalmologica. - : Wiley. - 1755-375X .- 1755-3768. ; 97:5, s. 478-485
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Journal article (peer-reviewed)abstract
- Purpose: The aim of the study was to report prevalence, ocular outcome, neurological characteristics, cognitive and behavioural problems in children with optic disc coloboma (ODC).Methods: This was a population-based, cross-sectional study of 31 children between 2 and 18 years of age diagnosed with ODC. The children were part of a larger cohort of 184 children with congenital optic disc malformations. Clinical ophthalmological examinations, neurological assessments, behavioural and developmental screening were performed.Results: The prevalence of ODC was 8.9/100 000 children. Of the 31 patients, 18 had unilateral ODC (p = 0.21). The best-corrected visual acuity (BCVA) in the ODC eye ranged from blindness to 1.3 (median 0.3). BCVA was 0.82 in eyes with an isolated ODC (range 0.4-1.3) and 0.15 (range 0-0.5) in eyes with concurrent macular involvement (p < 0.0001). Nystagmus was observed more often in patients with bilateral ODC (9/13 versus 3/17, p = 0.004). Two patients had retinal detachment. Behavioural/psychological screening was performed in 21 patients with severe deficits identified in six cases. Intellectual disability was present in seven patients. Neurological dysfunction was diagnosed in 8/22 cases. All of the above children had already systemic diagnoses before the ODC diagnosis was made.Conclusions: ODC was the second most common optic disc malformation in this cohort after optic nerve hypoplasia. The children had a wide range of ocular comorbidity. An isolated ODC without macular involvement was not associated with profound vision loss. The ability of screening in the regular child care centres to diagnose extraocular comorbidities was very good and referral to a paediatrician appears redundant in cases of normal development.
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| 13. |
- Teär Fahnehjelm, Kristina, et al.
(author)
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Cataract after allogeneic hematopoietic stem cell transplantation in childhood
- 2016
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In: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 105:1, s. 82-89
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Journal article (peer-reviewed)abstract
- AimThe aim of this study was to study long-term visual outcome and cataract development in children and adolescents after hematopoietic stem cell transplantation (HSCT) in childhood. MethodsBest corrected visual acuity (BCVA), refraction and lens status were examined in a prospective study of 139 children and adolescents. ResultsIn total, 139 patients (58 female), median age 6.6years at HSCT (range 0.4-17.5years), were followed up for a median of 8.0years (1-19.4years). Median BCVA in the better eye was 1.0 decimal. Altogether 19 of 131 patients developed cataract requiring surgery, while 46 developed less prominent lens opacities and 66 had clear lenses at time of latest follow-up. Patients conditioned with total body irradiation had a higher risk of developing lens opacities or cataract (p<0.0001) as did patients with malignant disease, irrespective of irradiation treatment (p<0.0001). Cumulative analysis showed that 50% of all patients had developed lens opacities/cataract after 10.2years. Patients who ultimately needed cataract surgery developed cataract earlier than others (p=0.006). ConclusionLens opacities or cataract were more common in children or adolescents with malignant disease and after conditioning with irradiation. Regular ophthalmological follow-up is important after HSCT for early intervention to avoid amblyopia.
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| 14. |
- 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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| 15. |
- Teär Fahnehjelm, Kristina, et al.
(author)
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Most patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency develop pathological or subnormal retinal function
- 2016
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In: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 105:12, s. 1451-1460
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Journal article (peer-reviewed)abstract
- Aim: There have been few studies on long-term electroretinographic findings in patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). This study correlated long-term electroretinographic findings with age, metabolic control and clinical symptoms. Methods: We examined 12 Swedish patients with LCHADD. Visual acuity testing, fundus examinations, optical coherence tomography and electroretinography were performed. The results were correlated to age, the levels of 3-hydroxyacylcarnitine and acylcarnitine and clinical metabolic control. Results: Blindness or moderate visual impairment was found in two patients. Retinal pigmentation, atrophy and fibrosis were present in 11, seven and one of the patients, respectively, and optical coherence tomography showed retinal thinning in three of the six patients examined. Electroretinography was performed on 11 of the 12 patients. It was pathological, with reduced rod and cone responses, in five patients, subnormal in four and was related to poor clinical metabolic control and severe neonatal symptoms. Repeated electroretinographies revealed reduced function with increasing age. Conclusion: More than 80% of the LCHADD patients developed pathological or subnormal retinal function. This was more pronounced in patients with neonatal symptoms, but ameliorated by strict dietary treatment. Annual ophthalmological follow-ups, with electroretinography every second or third year, are recommended.
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| 16. |
- Teär Fahnehjelm, Kristina
(author)
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Posterior ocular malformations in children : teratological aspects
- 2003
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Doctoral thesis (other academic/artistic)abstract
- Posterior ocular malformations are common causes of visual impairment in Swedish children. The most frequent diagnoses within this group are optic nerve hypoplasia and optic nerve/chorioretinal coloboma. Genetic factors, teratogens like medical/addictive drugs or congenital infections and unknown factors may cause these malformations. The general aims of this work were to study the impact of various teratogens on ocular structures and visual function in children and to evaluate associated somatic disorders in children with posterior ocular malformations. Paper I describes 43 children exposed to anti-epileptic drugs during pregnancy, compared to 47 control children. Children exposed to low amounts of single therapy anti-epileptic drugs (predominately phenytoin or carbamazepine), where the mothers were carefully monitored during pregnancy did not develop severe ocular malformations. Papers II-IV are based on Swedish data from a prospective European multi-centre study on infections with Toxoplasma gondii. Among 40 978 newborn children screened neonatally, three had congenital toxoplasmosis. Two were asymptomatic at birth and would have escaped early detection and therapy without neonatal screening. They were treated for 12 months with anti-parasitic therapy. Seroprevalence in adults, incidence of primary infection during pregnancy with Toxoplasma gondii and prevalence of congenital toxoplasmosis were low in Sweden. In Papers V and VI, 40 visually impaired children with posterior ocular malformations from the whole of Sweden, referred to Tomteboda Resource Centre were assessed on a multi-disciplinary basis. Children with optic nerve hypoplasia more often had severe visual impairment, were diagnosed later and suffered to a greater extent from neonatal and endocrinological insufficiencies than children with coloboma/microphthalmus. Neuroradiologically, an abnormal pituitary region indicated endocrinological insufficiencies. Neonatal hypoglycaemia predicted for neurological, endocrinological and/or cognitive dysfunction. Exposure to Vitamin A, alcohol or anti-depressive drugs was found in five children. Dried blood spot cards, collected for routine metabolic screening, were retrospectively analysed with polymerase chain reaction to detect congenital cytomegalo- or herpes simplex virus infection. Herpes simplex virus type 1 DNA was identified in one child with optic nerve hypoplasia whereas cytomegalovirus DNA was not found in any child. In Paper VII, four children with hepatic disease presenting as neonatal cholestasis and visual impairment due to optic nerve hypoplasia are described. Common aetiological mechanisms are discussed and it is suggested that endocrine insufficiencies and cholestasis occur in variable clinical combinations in infants with optic nerve hypoplasia. In conclusion a multi-disciplinary approach is of great importance in children with posterior ocular malformations in order to accurately describe associated endocrinological insufficiencies, CNS abnormalities, neurological or neuro-psychiatric dysfunction and, with reference to young infants, accompanying cholestasis. Further evaluation is needed of the role of congenital infections and anti-depressive drugs and their association to posterior ocular malformations.
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| 17. |
- 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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| 18. |
- Törnquist, Alba Lucia, et al.
(author)
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Visual evoked potentials after hematopoietic allogeneic stem cell transplantation in childhood
- 2017
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In: Clinical Neurophysiology Practice. - : Elsevier. - 2467-981X. ; 2, s. 67-71
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Journal article (peer-reviewed)abstract
- OBJECTIVE: To study visual pathway pathology detected by visual evoked potentials (VEPs) in patients treated with hematopoietic stem cell transplantation (HSCT) in childhood and to determine the impact of adverse ocular findings, somatic diseases, and conditioning regimens on the VEP results.METHODS: Ophthalmological assessments including pattern VEPs were performed in 47 of 79 patients at a median age of 15 years (range 3-21 years) in median 6 years (1-17 years) after HSCT. Somatic data were extracted from medical records.RESULTS: Eight patients of 47 (17%) demonstrated pathological VEPs with prolonged latencies bilaterally (n = 3) or unilaterally (n = 5) at their latest VEP test at an age of 12-18 years. A subnormal visual acuity was present in 8/11 eyes with pathological VEPs: one eye had cataract, six eyes had cataract surgery where of two had developed secondary cataracts. One eye had residual retinopathy of prematurity. Pathological VEPs were associated with decreased visual acuity (p = 0.00019) but not linked to gender, malignant diagnosis or conditioning.CONCLUSION: VEP recordings showed an association with decreased visual acuity but no relationship with irradiation or chemotherapy in the present study.SIGNIFICANCE: VEP recordings might be of clinical value for children with an unexplained subnormal visual acuity undergoing HSCT.
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| 19. |
- Törnquist, Alba Lucia, et al.
(author)
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Visual field results and optic disc morphology in patients treated with allogeneic stem-cell transplantation in childhood.
- 2010
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In: Acta ophthalmologica. - : Wiley. - 1755-3768 .- 1755-375X. ; 89:1, s. 62-69
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Journal article (peer-reviewed)abstract
- Abstract. Purpose: This study aimed to describe the Rarebit (RB) visual field and optic nerve size/morphology in patients treated with allogeneic stem-cell transplantation (SCT) in childhood, and to determine the impact of ocular status and conditioning regimens such as total body irradiation (TBI), chemotherapy and/or immunosuppressive drugs on the RB visual field. Methods: Ocular fundi were evaluated in 79 patients. Digital analyses regarding optic disc area (DA), rim area (RA) and cup area (CA) were performed in 45 of 49 patients. RB visual field testing was performed in 53 of 79 patients. The mean hit rate (MHR) was compared to corneal status, cataract, diagnosis and pre and post-SCT treatment. Two groups of healthy children and young adults (RB = 51, disc analysis = 49) were used as controls. Results: The SCT patients, examined at a median age of 15.4 years, had a significantly lower MHR [median 91% (range 45-99) right eye and 91% (range 41-91) left eye] compared to controls [median 96% (range 78-100) right eye]. SCT patients treated surgically for cataract and with intraocular lenses (IOLs) had a significantly lower MHR compared to other SCT patients. The MHR was also significantly influenced by type of conditioning. Patients conditioned with chemotherapy other than busulfan had significantly better MHR compared to those who had received single-dose TBI. SCT patients had a significantly larger CA and smaller RA than the controls. Conclusion: Patients treated with SCT in childhood have a significantly lower MHR than controls when examined with RB. Also, patients surgically treated for cataract and with IOL implantation had a significantly lower MHR.
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