Bowel Management and Quality of Life in Children With Spina Bifida in South Korea.

Bowel management is a concern in patients with spina bifida. We evaluated the status of bowel management in children with spina bifida (SB) and the effects on quality of life (QoL) of children and their caregivers. Data were collected from 173 children with SB between January and June 2011, whose bowel management status and QoL were assessed using a self-administered questionnaire. Of the 173 children, 38 (22.0%) reported normal defecation, 73 (42.2%) reported constipation only, and 62 (35.8%) reported fecal incontinence with/without constipation. For defecation, 59 children (34.1%) used digital stimulation or manual extraction, 28 (16.2%) used suppositories or enemas, 35 (20.3%) used laxatives, 4 (2.3%) used an antegrade continence enema, and 3 (1.7%) used transanal irrigation. There were significant differences in QoL, depending on defecation symptoms. Children with fecal incontinence and their caregivers had difficulties in travel and socialization (p < .0001), caregivers' emotions (p < .0001), family relationships (p < .0001), and finances (p < .0001). Constipation and fecal incontinence affect QoL of children with SB and their caregivers. Therefore, more attention should be paid to bowel problems and help should be provided to children and their caregivers to improve QoL.

Central Somatosensory Networks Respond to a De Novo Innervated Penis: A Proof of Concept in Three Spina Bifida Patients.

INTRODUCTION: Spina bifida (SB) causes low spinal lesions, and patients often have absent genital sensation and a highly impaired sex life. TOMAX (TO MAX-imize sensation, sexuality and quality of life) is a surgical procedure whereby the penis is newly innervated using a sensory nerve originally targeting the inguinal area. Most TOMAX-treated SB patients initially experience penile stimulation as inguinal sensation, but eventually, the perception shifts to penis sensation with erotic feelings. The brain mechanisms mediating this perceptual shift, which are completely unknown, could hold relevance for understanding the brain's role in sexual development. AIM: The aim of this study was to study how a newly perceived penis would be mapped onto the brain after a lifelong disconnection. METHODS: Three TOMAX-treated SB patients participated in a functional magnetic resonance imagery experiment while glans penis, inguinal area, and index finger were stimulated with a paint brush. MAIN OUTCOME MEASURE: Brush stimulation-induced activation of the primary somatosensory cortex (SI) and functional connectivity between SI and remote cerebral regions. RESULTS: Stimulation of the re-innervated side of the glans penis and the intact contralateral inguinal area activated a very similar location on SI. Yet, connectivity analysis identified distinct SI functional networks. In all three subjects, the middle cingulate cortex (MCC) and the parietal operculum-insular cortex (OIC) were functionally connected to SI activity during glans penis stimulation, but not to SI activity induced by inguinal stimulation. CONCLUSIONS: Investigating central somatosensory network activity to a de novo innervated penis in SB patients is feasible and informative. The consistent involvement of MCC and OIC above and beyond the brain network expected on the basis of inguinal stimulation suggests that these areas mediate the novel penis sensation in these patients. The potential role of MCC and OIC in this process is discussed, along with recommendations for further research.

Sexuality, pre-conception counseling and urological management of pregnancy for young women with spina bifida.

A great number of newborns with spina bifida now survive with a growing life expectancy. Support with regard to sexual issues is essential in the management of adolescents with spina bifida, who require specific knowledge of sexual problems related to their disability. Women with spina bifida are usually fertile and need pre-conception counseling. Furthermore, compared to healthy women they have a higher chance of conceiving a child with spina bifida, so they are treated with periconceptional folic acid supplements. In addition pregnancies in women with spina bifida require adequate management of secondary conditions, mainly urological issues, which are exacerbated during pregnancy. This article gives an overview of sexual education, sex functioning and sexual activity among adolescents with spina bifida. Moreover, we aim to support young women with spina bifida, providing pre-conception counseling and practical guidelines essential for the urological management of their pregnancy.

Neural activity generated in the neural placode and nerve roots in the neonate with spina bifida.

OBJECT: The authors conducted a study to determine the neurophysiological capacity of the neural placode in spina bifida neonates and to determine if the spinal nerve roots in these neonates had normal stimulation. METHODS: The authors present a case series of 2 neonates born with open neural tube defects who underwent neural tube closure within 24 hours of birth. Neurophysiological monitoring and electrical stimulation of the placode and nerve roots was performed before and after closure of the neural tube. RESULTS: Stimulation of nerve roots resulted in evoked electromyographic responses in distinct muscle groups, indicative of the myotome innervation pattern. Stimulation threshold did not change significantly after closure of the placode. Stimulation within the placode generated an alternating pattern of activity in the left and right legs. CONCLUSIONS: Closure of the neural tube did not affect the stimulation threshold of the nerve roots, which remained easily excitable. The viability of the nerve roots suggests that they may be candidates for neural prostheses in the future. The neural placode contains basic neural elements for generating a locomotor-like pattern in response to tonic neural inputs.

Applying a knowledge-to-action framework for primary prevention of spina bifida in tropical Africa.

Maternal periconceptual folate supplementation reduces the incidence of neural tube defects; however, in settings where population-level food fortification is not available, it is not clear how best to promote this prevention strategy. Guided by a knowledge-to-action methodology, we used mixed quantitative and qualitative methods to define the local disease burden, then designed, implemented and evaluated a culturally tailored educational intervention in eastern Democratic Republic of Congo, where resource limitations and threats to human security contribute to restricted capacity for the prevention and management of congenital malformations. A descriptive case series of 27 patients undergoing surgery for spina bifida demonstrated a short-term mortality of 15% and long-term disability in survivors. A survey of knowledge, attitudes and practices demonstrated a low level of folate awareness (53%) among women of reproductive age. Focus group discussions revealed exotic aetiologic views, significant gender issues and several barriers to folate use. A culturally tailored radio broadcast and an educational video were designed and produced locally based on qualitative and quantitative findings. Evaluation of the video documented high levels of viewer satisfaction and unequivocal knowledge gain (P ≤ 0.001). We conclude that spina bifida poses a significant burden on affected patients and their families in the African context, but folate is underutilized as a prevention strategy. Patient education through video media results in increased awareness and understanding of spina bifida and folate, a first step in empowering women to reduce the risk of spina bifida in their children in the absence of population-wide food fortification.

Does threshold nighttime electrical stimulation benefit children with spina bifida? A pilot study.

BACKGROUND: Electrical stimulation is an established treatment for muscle weakness. However, traditional tectonic stimulation is poorly tolerated in children as a result of discomfort. Threshold electrical stimulation performed at night reportedly increases muscle strength in a variety of neuromuscular conditions and has been well tolerated in children. QUESTIONS/PURPOSES: We asked whether threshold electrical stimulation treatment at night would increase strength and function in children with myelomeningocele. METHODS: In a pilot study we prospectively followed 15 treated children who served as his or her own control. The patients were provided with a stimulator and instructed to use it on areas of muscular weakness during sleep, six nights per week. Followup phone calls at 2 weeks and then monthly were performed by a physical therapist to address patient concerns. Assessments of muscle strength, monofilament sensation, and physical function using the Functional Independence Measure for Children were to be performed at 3-month intervals up to 12 months and compared with the pretreatment assessment. RESULTS: Only seven of the 15 subjects completed 9 months of treatment and none finished the 1-year study. The most frequent complaint was the treatment schedule was too intensive for the benefits received. We found small gains in muscle strength, gait, and bowel continence but no changes in physical function. CONCLUSIONS: Although threshold electrical stimulation made small improvements in muscle strength, the currently recommended treatment schedules are not practical for patients with myelomeningocele.

Neuropathology and structural changes in hydrocephalus.

In the context of spina bifida, hydrocephalus is usually caused by crowding of the posterior fossa with obstruction to cerebrospinal fluid flow from the forth ventricle, and less often by malformation of the cerebral aqueduct. Enlargement of the cerebral ventricles causes gradual destruction of periventricular white matter axons. Motor, sensory, visual, and memory systems may be disturbed through involvement of the long projection axons, periventricular structures including the corpus callosum, and the fimbria-fornix pathway. Secondary changes occur in neuronal cell bodies and synapses, but there is minimal death of neurons. The clinical syndrome of hydrocephalic brain dysfunction is thus due to subcortical disconnection. Some of the brain dysfunction is reversible by shunting, probably through restoration of cerebral blood flow and normalization of the extracellular environment. However, destroyed axons cannot be restored.

[Psychotherapy with physically challenged children and young people: implications of a change of paradigm]. / Psychotherapie körperbehinderter Kinder und Jugendlicher: Implikationen eines Paradigmawechsels.

In the light of newer scientific developments the biopsychosocial approach in psychotherapy appears to be in need of a revision. In neurosciences a paradigm change from a linear to a dynamic outlook on development as a self-organizing process guided by interactions with the environment took place under the heading of Neural Plasticity during the last ten years. This implies that the conditions of development for challenged children are not comparable with those of healthy children as the case-examples of children with Spina Bifida indicate. On this background, a pilot project was launched with the goal of determining which forms of psychotherapy are helpful for challenged children. A practically oriented, eclectic approach was developed applying empiric-regulative cycles which promotes the dynamics of self-organizing psychic and physical processes as shown in a presented example of a child experiencing post-lesional plasticity. Thus, psychotherapy is understood as a co-constructive process of reciprocal shaping of relationship; it fosters beneficial organization processes of psychic and physical impact.

Treatment of the neurogenic bladder in spina bifida.

Renal damage and renal failure are among the most severe complications of spina bifida. Over the past decades, a comprehensive treatment strategy has been applied that results in minimal renal scaring. In addition, the majority of patients can be dry for urine by the time they go to primary school. To obtain such results, it is mandatory to treat detrusor overactivity from birth onward, as upper urinary tract changes predominantly start in the first months of life. This means that new patients with spina bifida should be treated from birth by clean intermittent catheterization and pharmacological suppression of detrusor overactivity. Urinary tract infections, when present, need aggressive treatment, and in many patients, permanent prophylaxis is indicated. Later in life, therapy can be tailored to urodynamic findings. Children with paralyzed pelvic floor and hence urinary incontinence are routinely offered surgery around the age of 5 years to become dry. Rectus abdominis sling suspension of the bladder neck is the first-choice procedure, with good to excellent results in both male and female patients. In children with detrusor hyperactivity, detrusorectomy can be performed as an alternative for ileocystoplasty provided there is adequate bladder capacity. Wheelchair-bound patients can manage their bladder more easily with a continent catheterizable stoma on top of the bladder. This stoma provides them extra privacy and diminishes parental burden. Bowel management is done by retrograde or antegrade enema therapy. Concerning sexuality, special attention is needed to address expectations of adolescent patients. Sensibility of the glans penis can be restored by surgery in the majority of patients.

The use of complementary and alternative medicine in children with chronic medical conditions.

OBJECTIVES: The objectives of this study were to examine whether progressive medical conditions lead to greater use of complementary and alternative medicine (CAM) as compared with more stable conditions, to see whether disease severity influences CAM use, and to identify the main motivations behind CAM use. METHODS: Subjects were selected from outpatient clinics at Hotel Dieu Hospital. Surveys were conducted by mail and telephone. Medical diagnosis and severity were obtained from medical files. Statistical tests included chi, Kruskal-Wallis, and correlations. RESULTS: One hundred ninety-four children were surveyed. The "progressive" group included 15 patients with Duchenne muscular dystrophy and 22 patients with cystic fibrosis. The "nonprogressive" group included 85 patients with cerebral palsy (CP), 49 with diabetes mellitus, and 23 with spina bifida. Twenty-three percent were using CAM. CP had the highest use; diabetes mellitus had the lowest. Popular therapies included massage and dietary/herbal remedies. Progressiveness had no impact on CAM use. Within the CP group, greater disease severity was associated with higher use (P < 0.001). The main reason for CAM use was to complement conventional medicine. CONCLUSIONS: Disease progressiveness had no impact on CAM use, but severity within the CP group did. Complementing conventional medicine was the main motive. Understanding the reasons and patterns of use of CAM is beneficial in efforts to improve the care of children with chronic medical conditions.

A multicentre study of the management of disorders of defecation in patients with spina bifida.

Patients with spinal dysraphism may have severe constipation and faecal incontinence. The impact of antegrade colonic enema (ACE) in the management of patients with spina bifida (SB) is analysed. In a multicentre cross-sectional study, constipation, faecal incontinence and faecal management were described. Cases surgically treated were identified. Data were collected from 423 patients, of whom 230 did not use any manoeuvre or laxatives to assist evacuation. Conventional treatment was used in 193 patients, including digital extraction in 39%, retrograde enema in 21% and oral laxatives in 52%. For intractable constipation and overflow of faecal incontinence, 47 patients were treated with ACE, of whom 41 used the method at a mean time of interview of 4.1 +/- 1.9 years after ACE operation; six abandoned ACE for conventional management. With ACE, faecal continence was significantly improved compared with conventional management, and neither retrograde rectal enema nor digital extraction were required. The conduit was fashioned to the right colon in 32 cases and to the left colon in nine cases. This study provides information on a multicentre experience in bowel management in SB patients. Whatever the technique used, ACE has improved faecal status compared with conventional therapy.

A do-it-yourself membrane-activated auditory feedback device for weight bearing and gait training: a case report.

An augmented auditory feedback device comprised of a thin membrane switch mini-buzzer, and battery is described as a modification of a previously described feedback device. The membrane switch can be customized for the patient and is designed to fit inside a patient's shoe without altering the heel height. Its appeal lies in its simplicity of construction, low cost, and ease of implementation during a patient's training for weight bearing and gait. An ever-present source of information, it provides performance-relevant cues to both patient and clinician about the occurrence, duration, and location of a force component of motor performance. The report includes suggested applications of the device, instructions to construct it, and a case report in which the device was used to improve weight bearing and gait in a cognitively healthy person with spina bifida.

The influence of colonic enema irrigation on urodynamic findings in patients with neurogenic bladder dysfunction.

OBJECTIVE: To evaluate whether colonic enema irrigation influences the urodynamic characteristics of patients with spina bifida, an overactive bladder and detrusor sphincter dyssynergia (DSD). PATIENTS AND METHODS: Since 1991, 83 patients with spina bifida at our institution have treated their bowel dysfunction by colonic irrigation every 24-48 h. In 12 patients (seven boys and five girls, mean age 7.7 years, range 0.7-13.8) with an overactive bladder and DSD, urodynamic studies of the bladder before and after enema treatment were available with no intercurrent changes in urological therapy. RESULTS: There were no significant changes overall in bladder capacity, leak-point pressure, bladder compliance and bladder instability in the selected group of children. CONCLUSION: Although enema therapy for bowel treatment in patients with spina bifida gave good results for faecal incontinence, with good patient compliance, no favourable effect on bladder function should be expected in most patients with a high-risk urinary tract dysfunction. Further study is needed to determine factors in patients who will benefit urologically from enema treatment.

Primary care for children with spina bifida.

Primary care providers are recognizing the need to be able to establish protocols for children with complex conditions, such as spina bifida. Best practices are to form partnerships with families to address the complex health care needs for their children who require comprehensive care, coordination of community services and resources, regular communication with interdisciplinary and specialty teams, and preparation for transition into young adulthood. Health status and quality of life for children with spina bifida depends in part on preventing complications or further disability and maximizing functional independence and inclusion in society. The content in this article presents special considerations through guidelines for medical and psychosocial aspects of primary health care for children with spina bifida.

Altered bladder and bowel function following cutaneous electrical field stimulation in children with spina bifida--interim results of a randomized double-blind placebo-controlled trial.

Bladder and bowel dysfunction in spina bifida are the result of abnormal electrical input, secondary to the neurological lesion of the spinal cord. Experimental attempts to correct this deficit with invasive electrical stimulation have demonstrated promising effects, as has a recent preliminary study of transcutaneous electro-stimulation in children with myelomeningocoele. A randomized controlled trial of non-invasive electrical stimulation in children with neuropathic bladder and bowel has been established. Interim results of 50 patients are presented. Treatment was performed at home for one hour daily for a mean period of 45 days. The only statistically significant difference between the active and placebo-groups was a 32% relative decrease in night-time urinary incontinence, favoring the placebo group. However there were non-significant trends of preferential improvement in the active group for the relative increases in maximum and average bladder content and episodes of spontaneous normal defecation. It is anticipated that a continued increase in patient numbers will overcome the large placebo effect observed and yield more significant results.

The effect of electrical stimulation on quadriceps femoris muscle torque in children with spina bifida.

The effects of neuromuscular electrical stimulation (NMES) on the torque production of the quadriceps femoris muscles were examined in five children with spina bifida. Two male subjects, aged 5 and 12 years, and three female subjects, aged 5, 12, and 21 years, participated in the study. Surface stimulation was applied to the quadriceps femoris muscles of one lower extremity for 30 minutes each day over an 8-week period. At 0, 4, and 8 weeks, maximum isometric voluntary knee extension torques were measured for both control and stimulated lower extremities with a dynamometer at 0, 15, 30, 45, and 60 degrees of knee flexion. The three oldest subjects had torque measurements of acceptable reliability (intraclass correlation coefficient greater than .72). Two of these three subjects also had significant increases in the torque produced by the stimulated limb relative to the torque produced by the control limb. The data were unreliable from the two youngest subjects. Completion times for functional tasks (walking and step ascension/descension) were also recorded before and after the 8 weeks of stimulation. The completion times were lower following stimulation for four subjects.