Research from the Medical Journals-2009

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Long-term outcomes in patients with treated childhood hydrocephalus
The cumulative risk of shunt complications, which is probably a function of shunt revision rates, is high, and should be included in any discussion with the parent of an infant or child receiving a new CSF shunt. The results clearly indicate that the individual and social impacts of hydrocephalus need to be better defined. This is necessary before the long-term disease burden of childhood hydrocephalus can be accurately measured.
J Neurosurg Pediatrics 2007;106:334-339

Self-concept in children with spina bifida compared with typically developing children
Children with spina bifida scored significantly lower than children with typical development for the domains of global self-worth; physical appearance; athletic competence; social acceptance and scholastic competence. There was no difference between the groups for the behavioural conduct domain.
Dev Med Child Neurol 2008;50:733-743

Sports participation in adolescents and young adults with myelomeningocele and its role in total physical activity behaviour and fitness
Sports participation seems to be due to personal preferences rather than physical ability; it could benefit from improving social support and perceived competence, and is associated with higher self-reported physical activity.
J Rehabil Med 2008;40:702-708

Obesity across the lifespan among persons with spina bifida
Obesity rates for children and adolescents with spina bifida are similar to the general population (in the US); however obesity rates are higher among adults, particularly women. Risk factors are similar to those observed in the general population.
Disabil Rehab DOI: 10.1080/09638280802356476

Idiopathic normal pressure hydrocephalus: the benefits and problems of shunting
CSF shunting in INPH produces long-lasting, excellent outcomes in many patients. The potential for abuse of the shunting procedure however, looms large and demands firm adherence to high quality standards in the selection process and honest appraisal of the limitations of the procedure.
Nat Clin Prac Neurol 2009;5(2):80-81

Evaluating outcomes of enterocystoplasty in patients with spina bifida: a review of the literature
Medical management is the mainstay of neurogenic bladder therapy in the spina bifida population. Enterocystoplasty remains an important option to prevent or reverse upper tract deterioration, and/or improve or cure socially unacceptable incontinence despite poorly defined outcome measures. The development of appropriate and validated outcome measures may enable more uniform, effective and safe urological care of patients with spina bifida.
J Urol 2008;180:2323-2329

Maternal vitamin B12 status and risk of neural tube defects in a population with high neural tube defect prevalence and no folic acid fortification
This study confirms that low maternal vitamin B12 status is an independent risk factor for having an NTD-affected pregnancy and is the first to address the public health question of what B12 level might be protective for women entering pregnancy.
Pediatrics 2009;123(3):917-923

Neural tube defects and maternal folate intake among pregnancies conceived after folic acid fortification in the United States
In this study conducted among pregnancies conceived after mandatory folic acid fortification, the authors found little of an association between neural tube defects and maternal folic acid intake. A possible explanation is that folic acid fortification reduced the occurrence of folic acid-sensitive neural tube defects. Further investigation is warranted to possibly identify women who remain at increased risk of preventable neural tube defects.
Am J Epidemiol 2009;169:9-17

Risk factors of congenital hydrocephalus: a 10 year retrospective study
A number of key risk factors have been identified to be strongly associated with the development of congenital hydrocephalus in an infant. The prevalence of familial patterns of inheritance for congenital hydrocephalus suggests a broader role for genetic factors in the pathogenesis of congenital hydrocephalus
J Neurol Neurosurg Psychiatry 2009;80:213-217

The Changing Incidence of myelomeningocele and its impact on pediatric neurosurgery: a review from the Children’s Memorial Hospital
The declining prevalence of myelomeningocele, the most common NTD, is secondary to several factors including folic acid fortification, prenatal diagnosis with termination of affected foetuses, and unknown factors. Survival during infancy and preschool years has improved over the last 25 years. Of a cohort born in 1975 – 1979 and treated at a single US institution, 74% have survived into young adulthood. One of the greatest challenges facing these young adults is the transitioning of their medical care into an adult medical community.
Childs Nerv Syst DOI 10.1007/s00381-009-0865-z

Implications of family environment and language development: comparing typically developing children to those with spina bifida
The relationship between language performance and family environment appears statistically and intuitively sound. The positive link between family focus on intellectually and culturally enhancing activities and language performance among children with myelomeningocele and shunted hydrocephalus remains robust. Knowledge of this relationship should assist parents and professionals in supporting language development through activities within the natural learning environment.
Child: care, health & development DOI:10.1111/j.1365-2214.2009.00966.x

The physics of hydrocephalus
This article reviews previous work on the dynamics of the intracranial cavity and presents new clinically relevant results about hydrocephalus that can be gained from this approach. Basic laws of physics, appropriately applied to the complex physiology of hydrocephalus, have already provided insight into the mechanics of hydrocephalus. The clinical challenge is to use our understanding to produce shunting systems that can deal with the altered brain physiology and result in better outcomes for our patients.
Pediatr Neurosurg 2009;45:161-174

The scientific basis for eliminating folic acid-preventable spina bifida: a modern miracle from epidemiology
One of the most remarkable successes of epidemiology was the demonstration in the late twentieth century that spina bifida and anencephaly – two of the most common and severe birth defects – are caused primarily by folate deficiency. This article reviews the descriptive epidemiological studies that began when we did not have a clue about aetiology. The paper tells the success story of the trials that proved that folic acid would prevent folic-acid-preventable spina bifida. Finally, it will tell how difficult it is to get prevention policy implemented, even when the scientific evidence is compelling. It concludes by noting that the inaction or inappropriate actions of food regulatory bodies in so many countries means that only 10% of folic-acid-preventable spina bifida is actually being prevented – a serious failure of public health policy.
Ann Epidemiol 2009;19:226-230

Headaches in patients with shunts
Headache is one of the most common afflictions suffered by humans. Headache in patients with a shunt triggers a series of events that includes utilisation of expensive technologies and often potentially dangerous surgical intervention. The purpose of this study was to determine the incidence of headaches in patients with shunts and, hopefully, the relationship of those headache disorders to the treatment of hydrocephalus.
Semin Pediatr Neurol 2009;16:27-30

Neuropsychological assessment of attention in children with spina bifida
Assessment of attention functions in children with spina bifida myelomeningocele by traditional tests may be misleading, because this paediatric population with complex cerebral malformations has difficulty with the cognitive and visual-motor requirements. To control for these interactions, the use of both traditional and computerised attention tests is recommended.

The Malone Antegrade Continence Enema: single institution review
The long-term results of the MACE in a large cohort (256) of patients with neuropathic bowel and chronic constipation are encouraging. Of the patients, 17% will require revision surgery and patients/parents should be counselled accordingly. The faecal continence rate, defined as no stool accidents for a 12 month interval, was 94%.
J Urol 2008;180:1106-1110

Risk and protective influences in the lives of siblings of youths with spina bifida
Data reveal generally resilient siblings who, overall, have successfully integrated the challenges and opportunities generated by the experience of SB. The risk experiences described in their stories no doubt present credible threats to their psychosocial health, but by helping siblings draw out and build on their inherent strengths and protective resources found in the broader social environment, we may better support their resilience.
Health and Social Work 2008;33(3):199-209

The surgical management of paediatric hydrocephalus
This review of the last 30 years says that despite the advances in surgery and technology clinical outcomes for people with hydrocephalus have not improved much. It outlines promising future directions.
Neurosurgery 2008;62(2)(Sup):633-642

Surgical management of adult hydrocephalus
Review of the range of treatments for all types of hydrocephalus found in adult patients.
Neurosurgery 2008;62(2)(Sup):643-660

Neuropsychological effects of shunt treatment in idiopathic normal pressure hydrocephalus
Most of the wide range of neuropsychological functions that are affected by INPH are markedly improved by shunt treatment, but not completely restored.
Neurosurgery 2008;63(3):527-536

Bone mineral density in children with myelomeningocele
The present study demonstrates that osteoporosis osteopenia are a major complication in children with spina bifida. It established the significant relationship with low bone mineral density (BMD) in children who are wheelchair dependent compared with ambulatory children.  There was a strong trend toward lower BMD in children who have higher neurological levels. Children with a history of fractures did not appear to have reduced BMD.
Dev Med Child Neurol 2008;50:1-5

Use of the anal plug in the treatment of fecal incontinence in patients with myelomeningocele
The anal plug is a useful aid in achieving social continence by reducing or even abolishing faecal soiling throughout most of the day. Although accidents may still occur, this modality should be offered to young patients with myelomeningocele as part of their continence program.
J Ped Nurs 2008;23(5):395-399

Cardiovascular disease risk factors and the relationships with physical activity, aerobic fitness and body fat in adolescents and young adults with myelomeningocele
A large proportion of the sample of adolescents and young adults with MMC was at increased CVD risk as indicated by the clustering of risk factors. Fifteen percent of ambulators and 61% of nonambulators were at increased risk. Participants with higher aerobic fitness tended to be more likely to have no CVD risk. Improving aerobic fitness in persons with MMC deserves attention in health care because it may contribute in reducing cardiovascular risk.
Arch Phys Med Rehabil doi:10.1016/j.apmr.2008.04.015