Effect of photobiomodulation combined with physical therapy on functional performance in children with myelomeningocele: A protocol randomized clinical blind study.

INTRODUCTION: Myelomeningocele is a severe type of spina bifida, resulting from improper closure of the neural tube. This condition drastically affects the structures of the spinal cord resulting in deficiencies. The combination of these deficiencies results in an overall decrease in mobility and functional participation amongst this population. Physiotherapy plays an essential role in rehabilitating people with MMC. The current literature shows that resources such as photobiomodulation (PBM) may support the rehabilitation of neurological conditions. The aim of the proposed study is to evaluate the effects of photobiomodulation (PBM) combined with physical therapy on functional performance in children with low lumbosacral myelomeningocele. MATERIALS AND METHODS: This is a protocol randomized clinical blind study, that will include 30 individuals of both sexes, aged between 5 to 8 years, diagnosed with low and sacral lumbar myelomeningocele and capable of performing the sit-to-stand task. The participants will be randomly assigned into two treatment groups: PBM + physiotherapeutic exercises and sham PBM + physiotherapeutic exercises. Irradiation will be carried out with light emitting diode (LED) at a wavelength of 850 nm, energy of 25 J per point, 50 seconds per point and a power of 200 mW. The same device will be used in the placebo group but will not emit light. Muscle activity will be assessed using a portable electromyograph (BTS Engineering) and the sit-to-stand task will be performed as a measure of functioning. Electrodes will be positioned on the lateral gastrocnemius, tibialis anterior and rectus femoris muscles. The Pediatric Evaluation of Disability Inventory will be used to assess functional independence. Quality of life will be assessed using the Child Health Questionnaire-Parent Form 50. Changes in participation will be assessed using the Participation and Environment Measure for Children and Youth. The data will be analyzed with the aid of GraphPad PRISM. DISCUSSION: The results of this study can contribute to a better understanding of the effectiveness of PBM on functioning and quality of life in children with myelomeningocele. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04425330.

Independence in self-care activities in children with myelomeningocele: exploring factors based on the International Classification of Function model.

PURPOSE: To investigate the relationship between disease factors, body functions and structures, activities, personal and environmental factors with independence in self-care activities in children with myelomeningocele (MMC), utilizing the International Classification of Function model. METHOD: 113 children with MMC, aged 3-18 years were assessed during an annual MMC-clinic visit. Data on the personal and environmental factors, disease factors, hand function, mobility, visual perception, cognition, and communication were assessed in relation to the Pediatric Evaluation of Disability Inventory (PEDI) self-care domain. RESULTS: The mean PEDI caregiver assistance score was 22.1 (ranged between <10 and 62) well below the norms. The most significant determinant of dependence in self-care activities was the presence of hydrocephalus, a child with hydrocephalus scored a mean of 9.2 points less on the PEDI caregiver scale (p = 0.02). By using a linear regression, the most significant factors associated with the PEDI scores among children with hydrocephalus were the age of the child, function of the non-dominant hand, ambulation, and cognitive function. CONCLUSION: In children with MMC the presence of hydrocephalus appears to be more significantly associated with functional independence than the neurological level. The International Classification of Function model provides an in-depth multidisciplinary model that facilitates in investigating independence in self-care activities in children with MMC. It is important to take personal factors and various functional impairments into account when working with these children to promote independence. Implications for Rehabilitation When working with children with myelomeningocele on independence in daily living activities, it is necessary that health professionals demonstrate a holistic view of the child and his/her disability. Health professionals should take into account information on all factors of the International Classification of Function, in order to help the children, achieve maximal independence. It is important for health professionals to consider presence or lack of hydrocephalus when working with children with myelomeningocele to achieve functional gains. Improving upper extremity function and cognitive ability in children with myelomeningocele may help promote independence in activities of daily living. It is necessary for health professionals to continue to work with children with myelomeningocele on maximizing independence as they get older, for it is possible for them to achieve independence at a later age. While this study did not explore participation, this is an essential part of the International Classification of Function and is generally related to function. Participation allows a child to be involved in life situations, contributes to life satisfaction and prepares children for critical adult roles. It is therefore important to assess and monitor this measure. Further studies should examine associations between independence in self-care activities and participation.

Outcomes of incontinent ileovesicostomy in the pediatric patient.

PURPOSE: Ileovesicostomy is a reconstructive option in complex urological cases but pediatric specific outcomes are lacking. We report our results with pediatric ileovesicostomy. MATERIALS AND METHODS: We retrospectively evaluated patients younger than 18 years undergoing incontinent ileovesicostomy at Vanderbilt University. History, urinary tract management and operative course were reviewed in the electronic medical record. Particular attention was given to immediate and long-term postoperative complications. RESULTS: Nine patients underwent incontinent ileovesicostomy between 2000 and 2013 at a mean age of 10.3 years (range 1.4 to 15.5). Surgical indication was sequelae of neurogenic or nonneurogenic neurogenic bladder (such as infection or worsening hydronephrosis) in 5 patients, reversal of vesicostomy in 3 and closure of cloacal exstrophy in 1. All 9 patients were thought incapable of reliable clean intermittent catheterization due to family unwillingness, poor social support or patient refusal. Median followup was 11.5 months (mean 48.2, range 1.3 to 144.8). Immediate postoperative complications included ileus requiring total parenteral nutrition and a wound infection in 1 patient. Long-term complications included urinary tract infection in 2 patients (febrile in 1 and positive culture for foul smelling urine in 1), stomal issues in 2 and temporary urethral leakage in 1. Constipation affected 3 children in long-term followup (all with neurogenic bowel preoperatively). Postoperative creatinine was stable or improved in all patients. CONCLUSIONS: Ileovesicostomy is a viable approach in children left with few other options, particularly those who are noncompliant or physically/socially unable to handle catheterization. This operation can help keep such patients out of diapers.

Vestibular evoked myogenic potentials in children affected by myelomeningocele.

PURPOSE: The aim of the present study is to establish if the vestibular evoked myogenic potentials (VEMPs) could be used as a clinical test for the evaluation of vestibular function in children affected by myelomeningocele (MMC). MATERIALS AND METHODS: Fifteen children, aged between 3 and 17 years, who had been affected by MMC were investigated. Data obtained from these children were compared with normal data from healthy children of the same age. Electromyographic activity of sternocleidomastoid muscle was recorded, while children were laid supine and asked to raise their head off the bed in order to activate their neck flexors bilaterally. The saccular receptors were acoustically stimulated with a logon of 500 Hz at an intensity of 130 dB peSPL presented monaurally through earphones. In each recording, we analyzed latencies and amplitudes of the p13-and n23 waves and the amplitude ratio between the two ears. RESULTS: VEMPs were detected to be normal in 13 patients. In particular, the mean p13 and mean n23 latencies were 15.7 (±1.4) and 21.7 (±1.1) ms, respectively; the mean amplitude value was 84.7 (±36.6), while the mean amplitude ratio was 17.4 (±12). A comparison of latencies and amplitude ratios between the children and healthy control group did not reveal any significant difference. On the contrary, a comparison of amplitude values between the two groups showed significant differences. CONCLUSION: In conclusion, vestibulocollic reflex is normal in patients affected by MMC, and VEMPs could represent a valid and noninvasive technique eligible to investigate the vestibular functions in these children.

Transcutaneous interferential electrical stimulation for management of neurogenic bowel dysfunction in children with myelomeningocele.

PURPOSE: We describe the efficacy of transcutaneous interferential (IF) electrical stimulation on constipation symptoms in children with myelomeningocele (MMC). METHODS: MMC children (30, comprising of 17 girls and 13 boys), mean age 6.7 ± 2.9, with moderate to severe intractable constipation were enrolled in this study. They were divided into treatment (IF stimulation, 15 children) and control (sham stimulation, 15 children) groups. All children underwent anorectal manometry before and 6 months after IF therapy considering the rectoanal inhibitory reflex and sphincter pressure. Parents were instructed to complete a bowel habit diary by providing data on the number of defecations per week, form of stool and episodes of pain during defecation. According to parents' report a total neurogenic bowel dysfunction score questionnaire was filled before and 6 months after treatment. A 15-course abdominal area IF electrical stimulation was performed for 20 min and three times per week, with low-frequency current in a duration of 250 µs every 6 s. Children were followed up for a minimum period of 6 months. RESULTS: In the treatment group, sphincter pressure and rectoanal inhibitory reflex significantly improved compared with sham stimulation and pretreatment measures (P < .05). In 73% of patients, the characteristics of constipation decreased immediately after IF therapy, while in 53% patients, they persisted for 6 months. Frequency of defecation increased statistically significant from 2.5 ± 1.1 per week before treatment to 4.7 ± 2.3 per week after treatment (P < .001). CONCLUSIONS: This pilot study showed that IF therapy is safe, noninvasive, and effective modality to improve constipation symptoms and anorectal manometry parameters in children with history of myelomeningocele.

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.

Early spontaneous leg movements in infants born with and without myelomeningocele.

PURPOSE: To compare quantity and quality of spontaneous leg movements during early infancy in babies with myelomeningocele (MMC) and babies with typical development (TD). METHODS: Nine infants with MMC and 12 with TD moved spontaneously while supine for 5 minutes at ages 1, 3, and 6 months. We used a 6-camera system to monitor leg movements. Resultant leg displacement and velocity were used to determine movement frequency and, for each movement, duration, distance, peak velocity, jerk, and number of acceleration peaks. RESULTS: Movements of infants with MMC were shorter in duration with fewer acceleration peaks than their peers. Asymmetrical interlimb frequencies in infants with TD resulted in one leg moving more than the other, which was similar to the lower symmetrical interlimb frequencies of infants with MMC. CONCLUSIONS: Infants with MMC show depressed movement activity. Further research is needed to determine if therapy can facilitate spontaneous activity and leg control.

Movement analysis in neonates with spina bifida aperta.

INTRODUCTION: In neonates with spina bifida aperta (SBA), leg movements by myotomes caudal to the meningomyelocele (MMC) are transiently observed. It is unclear whether these leg movements relate to functional neural conduction through the MMC. For optimal therapeutical intervention, pathophysiological insight in these transient leg movements seems relevant. If leg movements by myotomes caudal to the MMC concur with the execution of general movements (GMs), functional neural conduction through the MMC is implicated. OBJECTIVE: In neonates with SBA, we aimed to determine whether the transiently present leg movements caudal to the MMC indicate functional neural conduction through the MMC. METHODS: During the perinatal period, fetuses and neonates with SBA (n = 7 and n = 13, respectively) were longitudinally analysed for concurrency between leg movements caudal to the MMC and GMs. To address the integrity of the reflex arc in spinal segments (at, or) caudal to the MMC, tendon leg reflexes were assessed during the first postnatal week. RESULTS: At postnatal day 1, leg movements caudal to the MMC concurred with GMs in 12 of 13 infants. Isolated leg movements were observed in only 3 of these 12 infants (isolated vs. concurrent; p < 0.005). Leg movements concurring with GMs lasted longer than isolated leg movements (median duration = 11 s vs. 2 s; p < 0.05). Between days 1 and 7, tendon leg reflexes (at, or) caudal to the MMC had disappeared in all but 1 neonate. However, leg movements caudal to the MMC remained concurrently present with GMs in all five neonates available for follow-up after day 7. Comparing these leg movements between days 1 and 7 indicated a decreased duration (-44%, p < 0.05). CONCLUSIONS: In neonates with SBA, leg movements caudal to the MMC concur with GMs, indicative of functional neural conduction through the MMC. The disappearance of these leg movements is caused by lower motor neuron dysfunction at the reflex arc, whereas neural conduction through the MMC is still functional.

Initial experience with home therapeutic electrical stimulation for continence in the myelomeningocele population.

PURPOSE: In an attempt to enhance bladder and bowel continence 29 patients underwent long duration, low intensity transcutaneous therapeutic electrical stimulation. This technology has previously been shown to be effective for inducing regenerative hypertrophy of disuse atrophied skeletal muscle, and it has been associated with improved continence in the myelomeningocele population. Our objectives were to enhance urinary and fecal continence, and evaluate the mechanisms by which therapeutic electrical stimulation influences continence. MATERIALS AND METHODS: A total of 29 children with lumbar or sacral myelomeningocele underwent therapeutic electrical stimulation at home during sleep using a commercially available stimulator with a safety sensing circuit and 2 sets of electrodes placed on the skin at bedtime. Left and right side electrode placement was alternated on 6 of 7 consecutive nights. In 9 boys and 2 girls with more than 9 months of followup initial and subsequent cystometrography, urethral pressure profile, electromyography, voiding cystourethrography and renal ultrasound studies were evaluated. RESULTS: In the 11 children there was no radiographic evidence of urinary tract or musculoskeletal deterioration during treatment. Mean actual bladder capacity increased from 133 to 196 ml. (p < 0.05). Mean bladder capacity, as a percent of maximum predicted bladder capacity for a normal age matched child without myelomeningocele, also significantly increased from 59 before to 76% after 9 months of therapeutic electrical stimulation (p < 0.05). No significant change was noted in urethral pressure profile. A subjective improvement in the sensation of pelvic fullness was also observed. CONCLUSIONS: When administered at home by parents while the child sleeps, therapeutic electrical stimulation is safe for bladder and bowel continence in the myelomeningocele population. It seems to increase significantly bladder capacity, does not appear to change urethral pressure profile and results in a subjectively improved sensation of pelvic fullness, enhancing urinary and fecal continence. The most beneficial effect of therapeutic electrical stimulation seems to be on the bladder wall and less so on the striated pelvic floor musculature with subjective enhancement of pelvic fullness also contributing positively.

Stooling problems in patients with myelomeningocele.

In children with myelomeningocele fecal and urinary incontinence lowers self-esteem and decreases social interaction. The defects in the lumbosacral spine disturb the sensory and motor nerves supplying the skin and muscles of the perianal region, including the puborectalis muscle, and the external anal sphincter. The sensations in the region, as well as the motor functions of the striated muscles suffer, compromising the dynamics of continence and the normal process of stooling and leading to incontinence and constipation. Constipation has been treated by disimpaction of stools from the colon and rectum, administration of stool softeners, and a healthy diet containing adequate bulk-forming items. Incontinence has usually been managed by behavior modification of self-initiating stooling after meals and positive reinforcement of this process. This method has helped up to 75% of patients to become socially continent. Biofeedback training has been helpful in patients who have preservation of some sensorimotor functions in the perianal region and who understand and cooperate in the process of biofeedback training. The enema continence catheter has been used to empty the rectosigmoid every 48 hours; most children treated in this manner have achieved social continence. Electric stimulation of the pudendal nerves using a neuroprosthetic device has been used in some patients. The pudendal nerve is stimulated continuously to achieve continence; stimulation is discontinued only for stooling and/or urination.

Biofeedback training for patients with myelomeningocele and fecal incontinence.

This study evaluated the efficacy of biofeedback training for fecal in continence in patients with myelomeningocele. 12 patients were randomized to receive conventional treatment alone, or in conjunction with biofeedback. Anorectal manometric functions were evaluated before and after treatment, six and 12 months later. 16 control children were also studied. Three of eight patients in the biofeedback group and three of the four given conventional treatment alone reported greater than or equal to 75 per cent improvement in frequency of soiling 12 months later. Biofeedback training did not improve anal squeeze, rectal sensation or continence of rectal infused saline. The number of patients who improved in both treatment groups was not different.

Electrical stimulation for incontinence in myelomeningocele.

Urinary incontinence is the most obvious urinary tract complication in children with myelomeningocele. Incontinence is owing to a hyperreflexic (spastic) bladder and/or pelvic floor weakness, which responds to functional electrical stimulation. Of 33 incontinent children the criteria for functional electrical stimulation were absent in 21 because of denervation of the pelvic floor muscles. Of 6 children who used functional electrical stimulation 5 had a successful result. However, incontinence recurred in 3 of the 5 patients between 6 and 18 months after functional electrical stimulation treatment was completed and they are awaiting repeat treatment.