Quality of life and physician-reported developmental, cognitive, and social problems in children with benign external hydrocephalus—long-term follow-up

Introduction Benign external hydrocephalus (BEH) is characterized by too rapidly increasing head circumference in infants, combined with typical neuroimaging findings. Psychomotor developmental delay is typically seen during the first few years of life; after that, the children’s development assumedly normalizes. However, little is known about the long-term effects of BEH. Methods In this retrospective population-based study, children diagnosed with BEH during the years 1994–2003 in Southern Norway were asked to participate. Included patients (age 8–18 years old) and their parents answered the PedsQL questionnaire. The patient’s family physicians contributed by giving information from medical records, with special emphasis on developmental, cognitive, and social function. Results One hundred seventy-six children were identified with BEH. One hundred three patients and 86 parents completed the PedsQL questionnaire. Supplemental medical information for 142 of the patients was received, mainly from their family physicians. Children and adolescents with BEH score themselves better than the normative mean on health-related quality of life, while the parents score their BEH children within the normative mean, except for the school functioning subgroup, where they score significantly lower. Various developmental, physical, and social problems are reported, like mental retardation, speech problems, epilepsy, motor impairment, psychiatric disorders, and cognitive difficulties. Among these patients, there is a discrepancy in some areas between the child-reported and parent-reported quality of life. Conclusions Children and adolescents who were diagnosed with BEH during infancy generally do well. However, for some patients, there appear to be various developmental, social, and cognitive problems, and they seem to struggle more in school than their healthy peers

Quality of life and physician-reported developmental, cognitive, and social problems in children with benign external hydrocephalus—long-term follow-up

Introduction: Benign external hydrocephalus (BEH) is characterized by too rapidly increasing head circumference in infants, combined with typical neuroimaging findings. Psychomotor developmental delay is typically seen during the first few years of life; after that, the children’s development assumedly normalizes. However, little is known about the long-term effects of BEH. Methods: In this retrospective population-based study, children diagnosed with BEH during the years 1994–2003 in Southern Norway were asked to participate. Included patients (age 8–18 years old) and their parents answered the PedsQL questionnaire. The patient’s family physicians contributed by giving information from medical records, with special emphasis on developmental, cognitive, and social function. Results: One hundred seventy-six children were identified with BEH. One hundred three patients and 86 parents completed the PedsQL questionnaire. Supplemental medical information for 142 of the patients was received, mainly from their family physicians. Children and adolescents with BEH score themselves better than the normative mean on health-related quality of life, while the parents score their BEH children within the normative mean, except for the school functioning subgroup, where they score significantly lower. Various developmental, physical, and social problems are reported, like mental retardation, speech problems, epilepsy, motor impairment, psychiatric disorders, and cognitive difficulties. Among these patients, there is a discrepancy in some areas between the child-reported and parent-reported quality of life. Conclusions: Children and adolescents who were diagnosed with BEH during infancy generally do well. However, for some patients, there appear to be various developmental, social, and cognitive problems, and they seem to struggle more in school than their healthy peers

Impaired Neurocognitive Performance in Children After Liver Transplantation

Objectives To assess longitudinal neurocognitive development after liver transplantation and evaluate factors associated with neurocognitive performance. Study design Data from neurocognitive testing of 65 children (aged <18 years) who underwent liver transplantation at Oslo University Hospital between 1995 and 2018 were collected from the testing program after transplantation. The parent-reported version of the Behavior Rating Inventory of Executive Function was used to assess executive function. Results A total of 104 neurocognitive tests were conducted on 65 patients. At the first test, conducted at a median of 4.1 years (IQR, 1.5-5.3 years) after transplantation and at a median age of 6.7 years (IQR, 5.4-10.5 years), the mean full-scale IQ (FSIQ) was 91.7 ± 14, and the mean verbal comprehension index was 92.0 ± 14.5. In the 30 patients tested more than once, there was no significant difference in FSIQ between the first test at a median age of 5.8 years (IQR, 5.2-8.5 years) and the last test at a median age of 10.8 years (IQR, 9.8-12.9 years) (87.4 ± 12.9 vs 88.5 ± 13.2; P = .58). Compared with the patients who underwent transplantation a age >1 year (n = 35), those who did so at age 80 mL/kg (P = .004; adjusted for age at transplantation: P = .046) were associated with FSIQ. Conclusions Young age at transplantation and large blood transfusions during transplantation are risk factors for poor neurocognitive performance later in life. Children who undergo transplantation before 1 year of age have significantly lower neurocognitive performance compared with those who do so later in childhood. Cognitive performance did not improve over time after transplantation

Social attainment in physically well-functioning long-term survivors of pediatric brain tumour; the role of executive dysfunction, fatigue, and psychological and emotional symptoms

The purpose of this cross-sectional study was to investigate long-term social attainment in physically well-functioning adult survivors of pediatric brain tumour (PBT) and identify demographic, medical, and psychological factors related to poor social outcomes, with a special focus on the significance of executive dysfunction. One hundred and fourteen PBT survivors and a healthy control group provided personal data on social outcomes, i.e., education, work, and government benefits, and completed questionnaires on executive function (EF), psychological and emotional difficulties, and fatigue. A significantly higher number of survivors compared to healthy controls reported having received educational adjustments and substantial government benefits, and significantly more survivors than controls were currently not engaged in regular employment/training. PBT survivors and healthy controls did not differ on educational level or living situation. The factors most strongly associated with poor social outcomes were self-reported executive dysfunction, difficulties with adaptive functioning, and fatigue. The findings show that physically well-functioning PBT survivors are at risk of poorer social outcomes and financial dependence in adulthood compared to their healthy peers, and underline the importance of investigating EF in short- and long-term follow-ups. Future rehabilitation efforts should focus more on compensatory strategies for executive dysfunction and improving EF skills

Executive Function and Psychosocial Adjustment in Adolescent Survivors of Pediatric Brain Tumor

Adolescent survivors of pediatric brain tumor (PBT) are a sparsely studied subset of childhood cancer survivors. Sustaining a PBT may complicate the development of executive functions (EFs), which play a vital role in long-term psychosocial adjustment. In this study, 48 adolescent survivors and their parents completed questionnaires assessing EF, psychological symptoms, fatigue, and adaptive functioning, and 26 survivors underwent neuropsychological assessment. Survivors reported significantly more problems with adaptive functioning than a healthy control group, and this was most strongly associated to executive dysfunction, compared to psychological symptoms and fatigue. The findings have important implications for long-term follow-ups