Myelomeningocele: neglected aspects
The commonest cause of neurogenic bladder in children is myelomeningocele. Survival of children is much improved in the Western world, but by 35 years old, about 50% will have died. In adults, the commonest causes of death are lung and heart diseases. All physical aspects deteriorate with age, especially in those with thoracic lesions. Those who walk in childhood have a 20 50% chance of becoming wheelchair dependent as adults. Immobility, poor respiratory reserve, obesity, latex allergy and worsening kyphoscoliosis contribute to the increased risks of surgery. It is essential that safe and manageable urine drainage is established in childhood: the bladder never improves with time, and surgical reconstruction becomes progressively more difficult. Independence in adult life will only be possible with intense preparation in childhood. Children must be allowed to join in with family chores and events. Education, both academic and practical, must be encouraged. Skills such as driving, shopping and birth control must be taught. However, even with the best support, less than 40% will have gainful employment. Children who are continent and have lesions below L2 are likely to have normal sexual function. Sexual activity in adolescents, especially in those with hydrocephalus, is limited (but not absent). However, by adult life, about two thirds will have established a regular partnership. All females and those males who are naturally potent are likely to be fertile. There is a high risk of neural tube defects in their offspring unless the female partner takes prophylactic folic acid for 3 months before pregnancy and for first trimester.
DOI 10.1007/s00467 007 0663 3
The definition and classification of hydrocephalus: a personal recommendation to stimulate debate
Currently there is no recognised definition of hydrocephalus. The failure to agree on a working definition impedes progress in understanding the pathophysiology and treatment of hydrocephalus. Neurosurgeon, Harold Rekate, recommends the following definition as a starting point to develop a consensus statement. “Hydrocephalus is an active distension of the ventricular system of the brain resulting from inadequate passage of cerebrospinal fluid from its point of production within the cerebral ventricles to its point of absorption into the systemic circulation.”
CSF Res 2008;5:2
Investigation of the hydrodynamic properties of a new MRI-resistant programmable hydrocephalus shunt
The Polaris valve is a reliable, adjustable valve which unlike other adjustable valves (except the Miethke ProGAV valve), cannot be accidentally re-adjusted by an external magnetic field.
Cerebrospinal Fluid Research 2008;5:8
freely available on www.cerebrospinalfluidresearch.com
Clinical diagnosis of ventriculoperitoneal shunt failure among children with hydrocephalus
Bulging fontanelle, fluid collection along the shunt, depressed level of consciousness, irritability, abdominal pain, nausea and vomiting, abnormal shunt pump test, accelerated head growth, and headache were strongly associated with shunt failure. Fever was strongly associated with shunt infection. Gross signs of wound infection were associated with shunt infection but weren’t seen often.
Fever and time since initial surgery were powerful predictors of shunt infection. Irritability emerged as an important observation in the identification of both shunt failure and shunt infection. Among children who underwent initial shunt insertion after 2 months of age, the absence of irritability, nausea/vomiting, and headache were powerful predictors of the absence of shunt failure or infection.
Pediatr Emergency Care 2008;24(4):201-210
A unifying hypothesis of hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida
This work describes the origin and consequences of the Chiari malformation and proposes that hydrocephalus is caused by inadequate central nervous system (CNS) venous drainage. A new theory regarding the pathogenesis, anencephaly and spina bifida is described.
Cerebrospinal Fluid Research 2008;5:7
freely available on www.cerebrospinalfluidresearch.com
Managing skin health in obese children with spina bifida: an overview and case study
Care of the patient with a neural tube defect requires a multi-disciplinary approach and should begin early in the child’s life. The challenges in skin health management faced by patients, families and medical professionals are illustrated with a case study.
Prevalence and outcome of congenital heart disease in patients with neural tube defect
The overall prevalence of congenital heart disease was 27.8% (40.5% in spina bifida aperta and 18.9% in spina bifida occulta). Excluding defects that closed before 12 months, the prevalence among patients with NTD is still much higher than the general population (17.7% vs ~1%). Most of the defects were simple, but the authors suggest that there are significant implications for minor and major surgery.
J Child Neurol 2008;23(5):526-530
Antibiotic-Impregnated Shunt Catheters for the Treatment of Infantile Hydrocephalus
AIS systems can safely be used to treat hydrocephalus in paediatric patients less than 1 year old, even for those with a history of prematurity. One possible therapeutic application for such premature patients may be the incorporation of antibiotic impregnation into ventricular access devices or ventriculosubgaleal components to treat infants with hydrocephalus prior to definitive CSF shunt placement.
Ped Neurosurg 2008;44:91-96
Health related physical fitness of adolescents and young adults with myelomeningocele
Adolescents and young adults with spina bifida have poor health-related physical fitness. Gender and ambulatory status are important determinants of aerobic capacity. In addition a small but significant relationship between aerobic capacity and muscle strength was found.
Eur J Appl Physiol DOI 10.1007/s00421-008-0684-z
Risk factors for pressure sores in adult patients with myelomeningocele – a questionnaire-based study
Patients with sensory deficit, memory problems, and Arnold Chiari malformation had a higher risk of having pressure sores. This patient group needs improved skin inspection routines and sore treatment.
Early puberty in boys with myelomeningocele. Risk factors for early puberty
Children with myelomeningocele (MMC) run an increased risk of developing early or precocious puberty (E/PP). In a previous study of girls with MMC we found increased intracranial pressure during the perinatal period to be strongly associated with E/PP. We also found the incidence of E/PP among girls to be as high as 52%. In a number of studies PP in boys has also been reported, but most studies have been based on selected groups of children. The aims of the present study were to investigate the incidence of E/PP in boys with MMC treated at a regional habilitation centre, and to identify possible risk factors associated with the development of early or precocious puberty. In addition, researchers analysed the clinical course of pubertal development.
Cerebrospinal Fluid Research 2007, 4(Suppl I):S37 doi:10.1186/1743-8454-4-SI-S37
Current prevalence of latex sensitisation in children with spina bifida with use of latex precautions
The purpose of this study is to evaluate the prevalence of latex sensitisation and allergy in a population of children with spina bifida that was born after the institution of latex avoidance measures, and to assess the risk factors for latex sensitisation.
The prevalence of latex sensitisation has decreased substantially with the use of latex avoidance measures. None of the 19 children under 10 years of age who were born in the United States had the detectable igE (immunoglobulin E – antibody responsible for the allergic response).
Cerebrospinal Fluid Research 2007, 4(Suppl l):S13 doi:10.1186/1743-8454-4-S1/S13
Severe fetal hydrocephalus with and without neural tube defect: a comparative study
This study was undertaken in Brazil where the results would be less likely to be affected by terminations; abortion is illegal in Brazil. Mortality was higher in the group without a neural tube defect, (59% either stillborn or dying in the first year) possibly due to the presence of other associated malformations than the group with a neural tube defect (36% either stillborn or dying in the first year). However the morbidity was higher in the group with a neural tube defect, possibly due to the higher number of surgical interventions in the central nervous system.
Fetal Diagn Ther 2008;23:23-29
Factors affecting renal scar development in children with spina bifida
As far as the urinary system is concerned prevention of scarring of the kidneys is the main therapeutic goal in children with spina bifida because it leads to chronic renal failure. This study found that the factors affecting the development of renal scarring were late referral, female gender, overactive detrusor (bladder muscle) and detrusor sphincter dyssynergia (the bladder muscle and sphincter not working in tune with each other).
Urologia Internationalis 2007;79:133-136
Functional gait comparison between children with myelomeningocele: shunt versus no shunt
This study shows that children with no shunt tend to walk at a significantly greater velocity and stride length compared with those with a shunt.
Dev Med Child Neurol 2007;49:764-769
Ongoing botulinum toxin helpful in bladder dysfunction
German researchers report that repeated injections of botulinum-A toxin appear to be effective in the treatment of myelodysplastic children and patients with spinal cord injuries with neurogenic bladder dysfunction.
Br J Urol Int 2007;100:639-645 reported in Medscape
Maternal metabolic syndrome may raise neural tube defect risk
Pregnant women with features of metabolic syndrome may be at increased risk of having a child with a neural tube defect, according to a recent study.
BMC Pregnancy and Childbirth 2007;7:21
Surgical treatment of myelomeningocele: year 2000 hospitalisation, outcome and cost analysis in the US
Myelomeningocele repair is mostly performed in large teaching institutions in small numbers. The majority of the patients get to go home at discharge. It is surprising to note that only 35% also required VP shunt placement during the same hospitalisation. The average length of stay was 15 days and the average cost was about US$60,000.
Childs Nerv Syst 2007;23:1125-1127
Minimally invasive approach for treatment of urinary and fecal incontinence in selected patients with spina bifida
In patients with a neurogenic bladder who do not qualify for major bladder reconstructive procedures such as bladder augmentation or bladder neck repair, social continence and independence can be achieved with minimally invasive surgery. Concomitant laparoscopic appendicovesicostomy and cecostomy tube placement may be a suitable surgical option.
Incidence, prevalence, and characteristics of fractures in children, adolescents, and adults with spina bifida
Fractures in people with spina bifida are most common during early adolescence. Environmental modifications may be more effective than pharmacological treatment in reducing the prevalence of fractures in this population.
J Spinal Cord Med2007;30:Suppl; S5-9
The pediatric Chiari I malformation: a review
Appropriate literature germane to the CIM is reviewed and discussed. There is variation in the reported anatomy, outcome and treatment for children with CIM. Based on the literature, most patients have preoperative symptoms or findings that improve no matter what surgical procedure is used. However, standardised treatment paradigms based on randomised controlled studies are still necessary to elucidate the optimal selection and treatment criteria.
Childs Nerv Syst 2007;23:1239-1250