Research from the Medical Journals - 2012

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Behavioral outcome in congenital shunted hydrocephalus without spina bifida
Results of this study show that 30% of children with hydrocephalus have clinically-significant behavioural and emotional difficulties compared to 11% of children without hydrocephalus. Internalising problems was particularly more common.

The observations also show that children with spina bifida and hydrocephalus have similar emotional difficulties as children with hydrocephalus but not spina bifida. The implication is that it is hydrocephalus itself and not the other brain anomalies associated with spina bifida (e.g. Chiari II) that affect emotional outcome.

The authors note that there is a growing body of evidence that pathology of the brain’s white matter is directly associated with the occurrence of these neuropsychiatric states.
J Ped Neurol;2011;9(1):41-47

Interrelationships of sex, level of lesion, and transition outcomes among young adults with myelomeningocoele 
This study of young adults (18 – 25 years old) from the US indicated that young adults with spina bifida have a lot of difficulty in achieving the milestones of emerging adulthood. 58% were unemployed and 74% were in supervised living environments.  The results might be conservative given that people who could not understand the study instruments were excluded.

One of the findings reinforced those of earlier studies which suggested that individuals with a milder disability have worse psychosocial outcomes because fitting in with peers can be difficult for them and lead to a reduced quality of life.
Dev Med Child Neurol 2011;53(7):647-652

The prevalence of hypertension in children with spina bifida
Children with spina bifida have a significantly higher prevalence of hypertension than children in the general population. In this study 41.5% of children were hypertensive (had a systolic or diastolic blood pressure greater than the 95th percentile for age, gender and height on 3 or more occasions). This compared with a national study of children in the general community which showed a prevalence of 3%.
Acta Paed 2011;100:e80-e83

Effects of latex avoidance on latex sensitization, atopy and allergic diseases in patients with spina bifida
This study shows a dramatic reduction in prevalence of latex sensitisation in children with spina bifida over the last decade – from 55% down to 5%. This suggests that implementing a latex free environment for children with spina bifida is effective in reducing the prevalence.

The study also indicates that avoidance of latex in children with spina bifida seems to prevent sensitisation to other allergens and allergic diseases.
Allergy 2010;65:1585-1593

Long-term outcome after treatment of hydrocephalus in children
In this meta-analysis (analysis of data combined from several studies) the authors found that the frequency of shunt failure is the same today as it was 40 years ago. This is despite the introduction of endoscopic placement techniques and improved shunt hardware. The main risk factors were prematurity, concomitant procedure and long duration of surgery, all things largely beyond the surgeon’s control.
The analysis also highlighted the fact that endoscopic third ventriculostomies had only a slightly lower failure rate than VP shunts in the long term.
Pediatr Neurosurg 2010;46:221-226

Norwegian study suggests that folic acid supplementation may have beneficial effects on neurodevelopment apart from lessening the risk of spina bifida
This study showed that maternal use of folic acid supplements in early pregnancy was associated with a reduced risk of severe language delay.

A severe language delay was defined as having 1 or fewer words of expressive language at 3 years of age. For mothers who took a supplement either containing folic acid or folic acid on its own were half as likely to hav ea child with a severe language delay as those who took no folic acid. Bread or cereals are not fortified with folic acid in Norway.
JAMA 2011;306(14):1566-1573

Quality of life and behavioural adjustment in childhood hydrocephalus
This study describes parent and teacher reports on behavioural outcomes and quality of life in childhood hydrocephalus in order to consider the implications for future service planning. A sample of 235 school-aged children with hydrocephalus were identified via a database of service users, held by the Scottish Spina Bifida Association.

Parent and teacher reports of behaviour on the Strengths and Difficulties Questionnaire (SDQ), and parent reports of quality of life on the Paediatric Quality-of-Life Generic Core (PedsQL Core) and Paediatric Quality-of-Life Fatigue (PedsQL Fatigue) were obtained, as were reports of service use and satisfaction.

In total, 35% of parents and 86% of teachers who were contacted participated in the study. Parents reported behavioural difficulties in 57% and teachers in 33% of children. Quality of life was significantly reduced in comparison to published norms. Children whose parents reported unmet needs had poorer psychosocial outcomes, but families rarely accessed appropriate specialist services.

The authors conclude that hydrocephalus is associated with high rates of behaviour problems and markedly reduced quality of life. They suggest that it is important to increase professional awareness of psychological need in this chronic neurological condition, and to increase access to appropriate psychosocial services.
Scott Med J 2012; 57(1):18-25

Friendships of Children and Adolescents with Spina Bifida: Social Adjustment, Social Performance, and Social Skills
The aim of this study was to look at the quality of two way and general friendships of youth with spina bifida.

Families of youth with SB recruited a friend of the person with SB to participate; 106 youth and friend dyads participated. Youth with SB and their peers were similar in many ways. However, youth with SB rated the friendship as closer and were more likely to see peers as best friends rather than the reverse.

Regarding general friendships, youth with SB spent fewer days with friends, reported lower levels of companionship, security, and closeness in their friendships, and reported lower levels of emotional support from peers and family. 
J. Pediatr. Psychol. 2012;37 (2): 220-231.

Barriers to Community Participation: Teens and young adults with spina bifida
Due to advancements in medical management and improved community access the number of adults with spina bifida is increasing. However, participation and integration in society for many people with spina bifida still remains low and this study aimed to discover what the main barriers to community participation are. 
A clinic transition coordinator worked with 101 teenagers and young adults for 6 – 12 months in Chicago to increase community participation. Participation rates in a variety of domains: school, employment, community participation, social and physical activity and barriers to community participation were assessed at the beginning and end of the program.

Initially, only 30% participated in community activities at least once a week; the most common barriers were motivation, lack of information, and time. At the end of the intervention period, 17% had a positive change, 35% had no change, 22% had no contact, and 24% did not need intervention, because they were already participating in the community.

The largest percentage of change was in teenagers aged 15-17 years (32%), and then teenagers aged 10-14 (17%). Older people aged 21-31 who were not in school, not working, and had no community activities were unlikely to change.

Participation only increased for a small group after working with the coordinator. It appeared to the coordinator that internal barriers, such as motivation, were the main barrier.

In general, positive change was limited to short term events or programs, which gave teens an opportunity to explore their interests.
Barriers that were addressed during the study included motivation, lack of information, transportation and language.
J Pediatr Rehabil Med 2008;1(4):303-310

Risk Factors of Sudden Death in Young Adult Spina Bifida Patients
Although survival for patients with myelomeningocele has dramatically improved over the last decades, many young adult patients continue to experience sudden death. This study was undertaken to determine risk factors for sudden death in the young adult myelomeningocele population.

The relationship between sudden death and patient demographics, midbrain length, presence of cerebrospinal fluid shunt and history of shunt revisions,severity of hindbrain malformation, seizures, pulmonary and ventilatory dysfunction, scoliosis, renal dysfunction, cardiac disease was evaluated. 

Of 106 patients, 6 experienced sudden death. These 6 patients were all female, mostly with sleep apnoea and an elongated midbrain. No other comorbidities were found to significantly increase the risk of sudden death.

The authors recommend that further investigation be carried out to determine if routine sleep apnoea testing and brain imaging may help mitigate the sudden death in at-risk patients who might benefit from closer cardiopulmonary monitoring and treatment.
J Neurosurg Pediatr 2012;9(2):149-155

Effects of latex avoidance on latex sensitisation, atopy and allergic diseases in patients with spina bifida
Latex avoidance in children with spina bifida even when they don’t have latex allergy, reduces the likelihood of it being acquired in the future. Additionally, the avoidance of latex prevents sensitisation to other allergic diseases, including from air borne and food borne allergens.
Allergy 2010;65;1585-1593

Adolescent predictors of emerging adulthood milestones in youth with spina bifida
This study compares whether factors present during adolescence such as illness status, socioeconomic status, motivation, cognitive functioning, and parental intrusiveness can predict the achievement of adult milestones. When compared to typically developing young adults those with spina bifida, were less likely to leave home, less likely to attend college, less likely to be employed, less likely to have had a romantic relationship and less likely to be currently in a romantic relationship.

Youth with greater executive functioning and intrinsic motivation are more likely to be successful in achieving various milestones. The authors suggest that parents, teachers and schools can promote development by fostering intrinsic motivation, addressing executive difficulties and helping youth build positive social relationships. 
JPedPsychol 2011;36(3):265-276

Behavioral outcome in congenital shunted hydrocephalus without spina bifida
Research has shown that emotional and behavioural problems are prevalent in children with hydrocephalus associated with spina bifida. It has been less clear whether children with congenital hydrocephalus but without spina bifida experience similar emotional and behavioural problems. This study shows that children with congenital hydrocephalus (without spina bifida) have almost 3 times the incidence (30%) of clinically significant behavioural and emotional difficulties compared to typical children (11%).
J PedNeurol  2011;9:41-47

Newer generation antiepileptic drugs and the risk of major birth defects
Some of the older drugs used to treat epilepsy have been shown to increase the risk of pregnant women having a child with a neural tube defect such as spina bifida. This Danish study found no increased risk of birth defects associated with the latest anti-epileptic drugs.
JAMA 2011;305(19):1996-2002

Cognitive and functional outcome in spina bifida –Chiari II malformation
Despite adequate medical intervention in childhood and adequate cerebrospinal fluid diversion, the prognosis for independent living into adulthood remains poor. This study confirms recent research which indicates that anomalies in brain structure may be more important determinants of cognitive outcome than shunt malfunction. 
ChildsNervSyst  201127:967-974

Interrelationships of sex, level of lesion, and transition outcomes among young adults with myelomeningocoele
The low rates of employment and independent living suggest that people with myelomeningocoele are experiencing great difficulty in achieving milestones of emerging adulthood. However employment is more likely to be achieved by males and independent living more likely by females. The level of lesion is significantly associated with self-management, community integration and quality of life.

The authors add that young adults with myelomeningocoele require ongoing assistance with management activities specific to spina bifida as well as general activities of daily living.
DevMedChildNeurol  2011;53(7):647-652