Swedish cohort study found that half of the girls with shunted hydrocephalus had precocious or early puberty.

AIM: We aimed to evaluate the occurrence of, and risk factors for precocious and early puberty in a retrospective cohort study of girls with shunted infantile hydrocephalus. METHODS: The study population comprised 82 girls with infantile hydrocephalus, born between 1980 and 2002, and treated with a ventriculoperitoneal shunt. Data were available for 39 girls with myelomeningocele and 34 without. Medical records were analysed regarding clinical data and timing of puberty. Precocious and early puberty was defined as the appearance of pubertal signs before 8 years and 0 months and 8 years and 9 months, respectively. RESULTS: Median age at last admission was 15.8 years (range 10.0-18.0). In total, 15 girls (21%) had precocious puberty, and another 21 (29%) had early puberty. Three or more shunt revisions had been performed in 26/36 girls with early or precocious puberty and in 3/37 girls without (p = 0.01). The number of shunt revisions correlated negatively with age at the start of puberty in the girls with myelomeningocele (Spearman's correlation coefficient = -0.512, p = 0.001). CONCLUSION: Girls with shunted infantile hydrocephalus have a high risk of precocious or early puberty. Repeated shunt revisions seemed to be associated with early puberty.

Clinical characteristics and late effects in CNS tumours of childhood: Do not forget long term follow-up of the low grade tumours.

AIM: To investigate clinical characteristics and late effects of CNS tumours in childhood with a special focus on low-grade tumours, especially low-grade astrocytoma and glioneuronal tumours. METHODS: A retrospective population based study was performed at Uppsala University Children's Hospital, a tertiary referral centre for children with CNS tumours. Patients were identified from the National Brain Tumour Registry and the National Epilepsy Surgery Registry. Hospital medical records were analysed for patients with a follow up of ≥5 years after diagnosis. A re-evaluation of the neuro-pathological diagnosis was performed. RESULTS: A total of 193 patients (age 0-17.99 years) during a twelve-year period (1995-2006) were included; 149 survived ≥5 years. Three larger subgroups could be identified: astrocytic, embryonal and glioneuronal tumours. A supratentorial location was found in 52%. Medical late effects were mainly neurological and endocrinological, affecting 81% and 26% of surviving patients. Cognitive late effects were a frequent finding in the whole group but also in low-grade astrocytoma and glioneuronal tumours (53% and 67%). Thirty per cent had some kind of pedagogic support in school. CONCLUSION: Late effects are common in long-term survivors of CNS tumours in childhood. Low-grade astrocytoma and glioneuronal tumours are no exception, and the findings support the need for long-term follow up.

Increased perinatal intracranial pressure and brainstem dysfunction predict early puberty in boys with myelomeningocele.

BACKGROUND: Children with myelomeningocele (MMC) run an increased risk of developing early or precocious puberty (E/PP). AIM: To identify risk factors for E/PP in boys with MMC. METHODS: Boys born between 1970 and 1992, treated for MMC at the University Children's Hospital, Uppsala, were identified. Thirty-eight boys were eligible to be included. Medical records were examined retrospectively. Early puberty was defined as pubertal signs before the age of 10 years and 2 months. Precocious puberty was defined as the appearance of these signs before 9 years of age. Increased intracranial pressure perinatally was defined as wide sutures, bulging fontanelles and increased/increasing head circumference at birth and/or during the first week after birth. Early brainstem dysfunction was defined as severe and persistent feeding and respiratory problems before the age of 3 months despite proper control of the hydrocephalus. RESULTS: Of the 38 boys, 8 (21%) had E/PP, which was strongly associated with increased intracranial pressure perinatally and also with early brainstem dysfunction. Multivariate regression analysis showed early brainstem dysfunction to have the highest explanatory value regarding the occurrence of early puberty. CONCLUSION: Increased intracranial pressure perinatally and brainstem dysfunction early in life are strong predictors of E/PP in boys with MMC.

Longterm oculomotor and visual function in spina bifida cystica: a population-based study.

PURPOSE: To document and describe the development from birth of visual and oculomotor functions in a group of children with spina bifida cystica (myelomeningocele and myeloschisis [MMC]). The emphasis in this study is on findings at 12-14 year follow-up. METHODS: Twenty children aged 12-14 years with myelomeningocele and Chiari-related malformations were examined by an orthoptist and a paediatric ophthalmologist. A further child who did not wish to participate actively in the study is also reported. Visual acuity for near and distance, refractometer readings in cycloplegia, the presence of ocular motility disorders and nystagmus were recorded. Accommodation, convergence, colour vision and stereo acuity were assessed and the fundus and media were examined. RESULTS: Six children (29%) in the study group had subnormal vision, although no child was visually impaired. Eleven (52%) showed manifest strabismus and 17 (81%) had a significant refractive error. Near visual acuity was normal in nearly all the children, but accommodation was defective in 10. Nine children had nystagmus and two had optic atrophy. No visual field defects were found. CONCLUSIONS: The high incidence of ocular disturbances in children with spina bifida highlights the importance of regular ophthalmological investigation and follow-up.

Children with early refusal to eat: follow-up in adolescence.

AIM: To describe the adolescent outcome of infant refusal to eat (RTE) and to investigate early predictors of adolescent outcomes. METHODS: 19 of 25 children with RTE and 35 of 42 controls were assessed at age 16. The participants rated eating attitudes and self-perceptions with a Swedish version of the Children's Eating Attitudes Test (ChEAT) and a Swedish self-perception measure. Weight and height were assessed through self-reports, and body mass index (BMI) was computed. Child and family variables measured from infancy through age 4 were used as predictors. RESULTS: RTE and controls did not differ with regard to disturbed eating attitudes, BMI or self-perceptions. There were no relations between early predictors and adolescent disturbed eating for the whole sample, but breastfeeding problems and social adversity in the early years predicted negative self-perceptions, and weight at 4 y predicted adolescent BMI. For the RTE group, low birthweight predicted higher levels of disturbed eating attitudes and less positive self-perceptions. For the control group, social adversity predicted high BMI. CONCLUSION: RTE does not seem to constitute a risk factor for adolescent disturbed eating. Further, there may be different risk factors for adolescent disturbed eating for children with and without RTE.

Control of reaching movements in children and young adults with myelomeningocele.

The aim of the present study was to analyze the ability to programme and execute reaching movements in individuals with myelomeningocele (MMC) and in a control group. Thirty-one participants (18 males, 13 females; mean age 12 years 11 months, SD 2 years 7 months, range 9 to 19 years) with MMC and 31 participants (matched for age and sex) without disabilities were investigated. Reaching was performed with and without visual feedback toward three targets displayed on a computer screen and data were collected using a digitizing tablet linked to a computer. The kinematics of reaching were analyzed and analysis of variance was used for statistical analysis. Results showed that both groups were able to programme reaching movements under both visual conditions. Although the execution of reaching was poor in the MMC group compared with the control individuals, as indicated by larger end-point errors (p=0.002), less straight movements (p=0.018), and shorter deceleration phases (p=0.004), movement time was not prolonged in the MMC group. Those with shunt treatment (n=21) had more difficulties when visual feedback was provided. Those with symptoms of early brainstem dysfunction (n=5) had shorter deceleration phases under both visual conditions.

Force measurements of postural sway and rapid arm lift in seated children with and without MMC.

OBJECTIVE: The aim was to investigate the horizontal ground reaction forces of seated postural sway and rapid arm lift in children with and without myelomeningocele. BACKGROUND; It is unclear whether children with myelomeningocele have limited control of body posture entirely caused by the impairment in the legs or also by other dysfunction. METHODS: 11 children with myelomeningocele, 10-13 years, and 20 children without physical impairment were investigated. Data were collected by force plate measurements during quiet sitting and during rapid arm lift. The forces were expressed as the corresponding acceleration of the centre of mass. The amplitude and the frequency of the centre of mass acceleration quantified the sway. Movement time, onset and anteroposterior peak acceleration were analysed during arm lift. RESULTS: The children with myelomeningocele had a low sway frequency under both conditions: eyes open and eyes closed. The movement time was longer for these children compared to the controls. The onset of initial anteroposterior centre of mass acceleration preceded the arm lift and was directed forward in both groups. The peak centre of mass acceleration was usually directed backward. CONCLUSIONS: The control of postural sway was different in children with myelomeningocele compared to children without disabilities and this could not be explained by the cele level. The children with myelomeningocele had a slow motor performance of the seated sway and during arm lift. RELEVANCE: Slow motor performance involves functional limitations in the individual child and is important for the therapy program.