Quality of life and functional vision in adolescents with surgically treated hydrocephalus in infancy.

AIM: To evaluate health- and vision-related quality of life (HR- and VR-QoL) and perceptual visual dysfunction (PVD) in adolescents with hydrocephalus surgically treated in infancy. METHODS: In total, 23 adolescents (15 males and 8 females; median age 14.9 years) with hydrocephalus and 31 controls were evaluated using validated instruments to measure HR-QoL and VR-QoL. PVDs were reported by history taking in five areas: recognition, orientation, depth, movement and simultaneous perception. RESULTS: Adolescents with hydrocephalus and the parent proxy reports showed lower mean total Paediatric Quality of Life Inventory 4.0 scores (75.8 and 63.7, respectively) compared with controls (87.6 and 91.5), p = 0.016 and p < 0.0001. Parent-reported scores were lower than self-reported scores (p = 0.001). Adolescents with myelomeningocele (n = 10) showed lower physical health scores (p = 0.001). No VR-QoL difference was found between groups. PVDs were reported in ≥1 area by 14/23 hydrocephalus participants and 2/31 controls (p < 0.0001). Associations were found in the hydrocephalus group between VR-QoL and HR-QoL (rs = 0.47, p = 0.026) and number of PVD areas (rs = -0.6, p = 0.003). CONCLUSION: Adolescents with hydrocephalus and their parents reported lower HR-QoL and more PVDs. These problems indicate the need for not only ophthalmological follow-ups but also evaluation of QoL and PVDs in individuals with infantile hydrocephalus.

Temporal trends in paediatric hydrocephalus - Rising prematurity and persistent ophthalmological challenges.

AIM: To study changes in aetiology, prematurity, comorbidity and ophthalmological outcomes in children with surgically treated hydrocephalus to provide information needed to maintain the best possible healthcare for a fragile and changing population. METHODS: Two population-based cohorts, born two decades apart in Region Västra Götaland Sweden, surgically treated for hydrocephalus at Sahlgrenska University Hospital in Gothenburg were recruited at approximately 10 years of age. The participants were examined according to an ophthalmological protocol, including history taking regarding perceptual visual dysfunction (PVD). Gestational age, aetiology and comorbidities were registered. RESULTS: The 1989-1993 group, comprised 52 children (48% girls; mean age 10, range 7.7-12.8 years), was compared with 24 children, born in 2007-2012 (29% girls; mean age 10, range 7.0-13.8 years). Extreme prematurity (gestational age ≤ 28 weeks) increased over time (p = 0.001). The vast majority of the children showed ophthalmological abnormalities, although motility defects and nystagmus decreased in the latter population. Subnormal visual acuity was associated with extreme prematurity (RR = 4.69; p = 0.030), and PVD with learning disability (RR = 2.44; p = 0.032). CONCLUSION: Paediatric hydrocephalus populations may change with improved healthcare. Since a high percentage shows ophthalmological abnormalities and more children are born extremely preterm, the entirety needs consideration both neurologically and ophthalmologically.

Thinner peripapillary retinal nerve fibre layer and macular retinal thickness in adolescents with surgically treated hydrocephalus in infancy.

PURPOSE: To map the morphology of the retina and optic disc in adolescents with surgically treated hydrocephalus (HC) in infancy and to compare the results with healthy controls. METHODS: The study comprised 26 adolescents (16 male, mean age 15 years) with HC and 31 sex- and aged-matched controls. The following optical coherence tomography (OCT) parameters were obtained: macular retinal thickness (MRT) and volume (MRV), thickness of the macular and peripapillary retinal nerve fibre layer (ppRNFL), and area of the optic disc, cup and rim. RESULTS: The MRT was thinner amongst those with HC compared with controls (right eye (RE) 262.2 ± 15.3 and 275.8 ± 15.1 µm; p = 0.0051), and the MRV was smaller (RE 7.37 ± 0.36 and 7.83 ± 0.35 mm3 ; p = 0.0002). The HC group showed a thinner ppRNFL (RE 88.3 ± 14.9 and 103.5 ± 8.1 µm; p = 0.0002) but a thicker central macular RNFL (RE 11.6 ± 10.4 and 2.07 ± 3.00 µm; p = <0.0001) and foveal minimum (RE 211.1 ± 32.0 and 186.3 ± 15.9 µm; p = 0.0013). Optic disc variables showed no difference between groups. Correlations were found in the HC group between best corrected visual acuity (expressed in logMAR) and ppRNFL (RE r = -0.56, p = 0.018), and disc area (RE r = -0.52, p = 0.033). CONCLUSION: Thinner ppRNFL and MRT and smaller MRV were found in adolescents with surgically treated HC in infancy compared with controls. In contrast, the central macular RNFL and foveal minimum were thicker. Further studies are required to evaluate the diagnostic value of OCT to indicate increased intracranial pressure timely and follow-up in individuals with surgically treated HC.

Ophthalmological characteristics in children with Leigh syndrome - A long-term follow-up.

PURPOSE: To describe ophthalmological characteristics in children with Leigh syndrome (LS), an inherited, progressive, mitochondrial encephalomyopathy, at diagnosis and over time, and relate the results to causative genetic mutations. METHODS: Forty-four children with LS (19 females), with a median age of 2.4 years (range: 0.6-14.2 years) at diagnosis, were studied at the Queen Silvia Children's Hospital, Gothenburg, Sweden. Twenty-eight children had known genetic defects. The children underwent an ophthalmological examination, including visual acuity (VA), eye motility, refraction, slit lamp examination, ophthalmoscopy and a full-field electroretinogram (ff-ERG). Seventeen children were available for follow-up over a mean time of 5.4 years (range: 0.3-14.8 years). The results of these children were compared with an age- and sex-matched reference group of healthy children (n = 119). RESULTS: Altogether 36/44 of the children (82%) had ophthalmological abnormalities. The most common findings were refractive errors (n = 16/25), low VA (n = 9/36), strabismus (n = 8/42), reduced eye motility (n = 8/40), optic atrophy (n = 7/41), retinal pigmentation (n = 6/40) and nystagmus (n = 6/42). Several ophthalmological manifestations appeared over time. In 5/22 children, ff-ERG showed retinal dystrophy. No significant correlation between phenotype and genotype was found. The children with LS had significantly lower VA (p < 0.0001, Mantel-Haenszel chi-square exact test), more astigmatism (p = 0.012, Fisher's exact test) and higher incidence of strabismus (p = 0.0002) compared to controls at follow-up. CONCLUSIONS: In this unique cohort of children with LS, the vast majority showed ophthalmological findings at diagnosis, which increased over time. Therefore, we recommend that all children diagnosed with LS should be followed up with regular ophthalmological examinations.

Ocular dimensions in relation to auxological data in a sample of Swedish children aged 4-15 years.

PURPOSE: The purpose was to characterize normal growth patterns of ocular and optical components and to relate them to auxological data in a sample of Swedish children aged 4-15 years. METHODS: A prospective cross-sectional study was carried out in 143 Swedish children with a mean age of 9.8 years. Variables including gestational age (GA), weight, length and head circumference (HCF) at birth and at the time of assessment were registered. Visual acuity (VA), cycloplegic refraction and biometric measures were obtained. Palpebral fissure length and inner canthal distance were measured. Optic disc morphology as seen on fundus photographs was analysed. RESULTS: Children born more mature, with male predilection, were found to have deeper anterior and vitreous chamber depths, longer axial lengths and thinner crystalline lens thickness. No correlations were found between ocular biometric measurements and VA or refraction after adjustment for confounding variables. Inner canthal distance was significantly correlated with birth length (p = 0.03), height, weight, BMI and HCF (p = 0.0008, p = 0.0007, p = 0.037, and p = 0.04, respectively) at time of assessment. Total axial length was found to be significantly correlated with GA (p = 0.0226) and length at assessment in girls (p = 0.0084). Right optic disc and rim areas decreased with increasing age (p = 0.0078 and p = 0.0107, respectively); however, optic disc parameters were not dependent on any other variable. CONCLUSION: These normative values may serve as a basis for the ocular findings and their relationship to auxological data in Caucasian children aged 4-15 years, as well as for future comparison in patients with paediatric ocular pathologies.

Strabismus, binocular functions and ocular motility in children with hydrocephalus.

PURPOSE: To investigate heterotropia, heterophoria, head posture, nystagmus, stereo acuity, ocular motility and near point of convergence (NPC) in children with hydrocephalus treated surgically before 1 year of age. In addition, the effects of being born with hydrocephalus, the effect of the etiology of hydrocephalus, number of shunt revisions and the size of the ventricles on these variables were studied. METHODS: A population-based study was performed in 75 children and the results were compared with the results of an age- and sex-matched group (comp group) (n = 140). RESULTS: Heterotropia 68.9% (comp group 3.6%; p < 0.001), abnormal head posture 41.3% (comp group 0; p < 0.001), nystagmus 44.0% (comp group 0; p < 0.001), stereo acuity < or =60'' 33.8% (comp group 97.1%; p < 0.001) and ocular motility defects 69.7% (comp group 0.7%; p < 0.001) were more common among children with hydrocephalus than in the comparison group. Children with overt hydrocephalus at birth had significantly more heterotropia (p = 0.0006), esotropia (p = 0.002), abnormal head posture (p = 0.02) and motility defects (p = 0.003) compared to those with hydrocephalus developing during the first year of life. The etiology, number of shunt revisions and the size of the ventricles had no significant effect on any of the investigated variables. CONCLUSIONS: Children with hydrocephalus surgically treated before the age of one year commonly present orthoptic abnormalities. The etiology of hydrocephalus, number of shunt revisions and ventricle size seem to be of minor importance compared with the age of onset of hydrocephalus with regard to the risk for orthoptic abnormalities.

Vision in children with hydrocephalus.

Hydrocephalus in children has many aetiologies, and can cause multiple ophthalmic and visual disorders. This study sets out to detect and quantify visual and visuoperceptual dysfunction in children who have received surgical treatment for hydrocephalus with and without myelomeningocele, and to relate the results to the associated diagnoses and results from a comparison group. Seventy-five school-aged children (41 males, 34 females) with surgically-treated hydrocephalus and 140 comparison children (76 males, 64 females) matched for age and sex underwent comprehensive ophthalmologic examination. Median age at examination was 9 years and 4 months (range 7 y 4 mo-12 y 10 mo). Visual function deficits were identified in 83% (62/75) of the children with hydrocephalus. Visual impairment (binocular visual acuity <0.3) was found in 15% (11/73; comparison group 0%) but in none with myelomeningocele. Strabismus was found in 69% (51/74; comparison group 4% [5/140], p < 0.001), and refractive errors were found in 67% (47/70; comparison group 20% [28/140], p < 0.001). Cognitive visual dysfunction was identified in 59% (38/64; comparison group 3% [4/140], p < 0.001). These disorders were identified in various combinations and comprised impaired ability to plan movement through depth (e.g. going down a stair), impaired simultaneous perception, impaired perception of movement, impaired orientation, and (least frequently) impaired recognition. In this study, children with hydrocephalus associated with myelomeningocele were least commonly affected. Visual disorders were most frequent in those with epilepsy, cerebral palsy, and/or cognitive disability.

Ophthalmological findings in a sample of Swedish children aged 4-15 years.

AIMS: To characterize ophthalmological findings in a sample of Swedish children aged 4-15 years. METHODS: A prospective cross-sectional comprehensive ophthalmological investigation was performed on a sample of 143 children (67 girls, 76 boys) aged 4-15 years. RESULTS: Visual acuity (VA) in the better eye >or=1.0 (0.3 logMAR). Amblyopia was found in 0.7% of subjects. A total of 68% of the children had no refractive errors. Hyperopia (>or=2.0 dioptres [D] in spherical equivalent [SE]) was found in 9% and myopia (>or=0.5 D SE) in 6% of children. Astigmatism (>or=0.75 D) was recorded in 22% and anisometropia (>or=1.0 D SE) in 3%. A total of 8% were optically corrected. Strabismus was recorded in 3.5%. Signs of visuoperceptual problems were reported in 3% of the children. CONCLUSION: This sample of Swedish children may serve as a comparison group regarding ophthalmological findings in children aged 4-15 years.

Strabismus and binocular functions in a sample of Swedish children aged 4-15 years.

PURPOSE: To investigate strabismus, head posture, nystagmus, stereoacuity, ocular motility, near point of convergence (NPC) and accommodative convergence to accommodation ratio (AC/A) in a sample of Swedish children. METHODS: A prospective cross-sectional study was carried out on 143 children, 4-15 years of age. RESULTS: Heterotropia was found in five children (3.5%), four with esotropia and one with exotropia. One child with esotropia had a slight overaction of both inferior oblique muscles. Heterophoria was found in 37 children (26%) at near and/or distance fixation and it was four times more common at near than at distance. In 29 children, heterophoria was found at one distance only and orthophoria at the other. Orthophoria at both near and distance fixation was noted in 101 children (70.5%). The near point of convergence was < or =6 cm in 97% of the children and 97% had stereoacuity of 60" or better. In the whole group, the median AC/A ratio calculated with the heterophoria method was 5.6/1 prism diopters/diopters (PD/D) and with the gradient method, 1.3/1 PD/D. No anomalous head postures or nystagmus were observed and all children had normal versions. CONCLUSION: In this study, 143 well-defined children were investigated with a battery of accurately described tests, commonly used in clinical practice. These results are in agreement with those of other studies examining one or few variables in larger populations and the authors therefore conclude that their results may be used for comparisons with different patient groups.