Journal of Pediatric Rehabilitation Medicine - Volume 10, issue 3-4
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The Journal of Pediatric Rehabilitation Medicine (JPRM): An Interdisciplinary Approach Throughout the Lifespan is designed to parallel the multidisciplinary teams caring for children, adolescents and adults with childhood-onset physical disabilities and complex care needs worldwide. Published quarterly, topics include, and are not limited to, cerebral palsy, traumatic brain injury, spinal cord injury, spina bifida, limb deficiency, muscular dystrophy, stroke, cancer, developmental delays, and rare disorders. Furthermore, the journal welcomes papers dedicated to pediatric rehabilitation from a global health perspective.
The aim of JPRM is to engage a diverse group of international experts with the goal of providing readers with comprehensive information regarding children and adolescents requiring rehabilitation. JPRM brings together specialists from medicine, nursing, psychology, social work, nutrition, child life, family centered care, and occupational, physical, and speech therapy. For manuscript submissions, authorship involving at least two different specialties is encouraged, although not required, to facilitate a transdisciplinary and collaborative approach. Manuscripts are blinded and peer reviewed including biostatistical analysis. Authors are invited to submit original research, systematic and scoping reviews, guidelines, protocols, care pathways, case reports, book reviews, commentaries, editorials, and dates for future conferences.
Abstract: PURPOSE: Endoscopic Third Ventriculostomy (ETV) and Choroid Plexus Cautery (CPC) are low-cost, safe, and promising interventions for spina bifida-associated hydrocephalus (SBHCP). The purpose of this review was to explore and describe these efforts in Africa in order to upscale surgical training and rehabilitation services. METHODS: A PubMed search for articles on ETV and CPC as management of SBHCP in Africa was performed. Two authors appraised the results for key themes in content: indications, technique, outcomes, complications, education, and rehabilitation. RESULTS: Twenty of 47 articles identified were included for appraisal. Twelve described indications, ten…and seven outlined technique and complications, respectively, and four described predictors of operative success. Fourteen studies describe outcomes, including operative and neurodevelopmental outcomes. Only two outlined educational efforts. Half of the literature stems from a single site in Uganda; in total, only six countries were represented. No articles described significant post-operative rehabilitation services or related training. CONCLUSION: The experience of ETV and CPC in Africa is promising, however, efforts to train and empower local staff in surgical technique and methods to upscale post-operative community-based rehabilitation services remain as a key to long-term success.
Keywords: Myelomeningocele, hydrocephalus, global health, neurosurgery, rehabilitation, Africa
Abstract: PURPOSE: Recent studies have revealed that the lack of continuity in preparing patients with spina bifida to transition into adult-centered care may have detrimental health consequences. We sought to describe current practices of transitional care services offered at spina bifida clinics in the US. METHODS: Survey design followed the validated transitional care survey by the National Cystic Fibrosis center. Survey was amended for spina bifida. Face validity was completed. Survey was distributed to registered clinics via the Spina Bifida Association. Results were analyzed via descriptive means. RESULTS: Total of 34 clinics responded. Over…90 characteristics were analyzed per clinic. The concept of transition is discussed with most patients. Most clinics discuss mobility, bowel and bladder management, weight, and education plans consistently. Most do not routinely evaluate their process or discuss insurance coverage changes with patients. Only 30% communicate with the adult providers. Sexuality, pregnancy and reproductive issues are not readily discussed in most clinics. Overall clinics self-rate themselves as a 5/10 in their ability to provide services for their patients during transition. CONCLUSIONS: Characteristics of current transitional care services and formal transitional care programs at US clinics show wide variances in what is offered to patients and families.
Keywords: Spina bifida, transitional care, myelomeningocele
Abstract: PURPOSE: Atypical skeletal development is common in youth with myelomeningocele (MM), though the underlying reasons have not been fully elucidated. This study assessed skeletal maturity in children and adolescents with MM and examined the effects of sex, age, sexual development, ethnicity, anthropometrics and shunt status. METHODS: Forty-three males and 35 females with MM, 6–16 years old, underwent hand radiographs for bone age determination. The difference between bone age and chronological age was evaluated using Wilcoxon sign rank tests. Relationships between age discrepancy (skeletal-chronological) and participant characteristics were assessed using multiple linear regression with forward selection.…RESULTS: Overall, forty percent (31/78) of MM participants had an advanced bone age of 1 year or greater (median: 2.5 years), while 47% (37/78) were within 1 year above or below their chronological age (- 0.001 years) and 13% (10/78) were delayed by more than 1 year (- 1.4 years). Bone age was advanced compared to chronologic age in both males and females (p ⩽ 0.024). Advanced bone age was observed in early to late puberty and after maturation (p ⩽ 0.07), as well as in Hispanic participants (p = 0.003) and in those with a shunt (p = 0.0004). Advanced bone age was positively correlated with height, weight and body mass index (BMI) percentiles (p = 0.004). In multiple linear regression analysis, advanced bone age was most strongly associated with higher Tanner stage of sexual development, and higher weight, height or BMI percentile. CONCLUSIONS: Advanced skeletal maturity is common in children/adolescents with MM over 8 years of age who have reached puberty (65%), particularly those who are overweight (80%). Hormonal effects associated with adiposity and sexual maturity likely influence skeletal maturation. Clinicians may use Tanner stage and weight or BMI to gain insight into skeletal maturity.
Keywords: Skeletal maturity, myelomeningocele, bone age, spina bifida, pediatrics
Abstract: PURPOSE: Spina bifida (SB) often results in a complex disability and can also cause cognitive dysfunction. No previous study has investigated the ability to adapt to time in children with SB. This ability is crucial for an individual’s possibility to develop autonomy in life. The purpose of this study was to investigate whether children aged 10–17 with SB have lower time-processing abilities than typically-developing children, and to describe the profile of time-processing in children with SB. METHOD: Participants comprised a consecutive sample of 21 children (drawn from a geographical cohort of 45) aged 10–17 years (mean: 14…years, SD: 2 years); 13 were boys. The instruments used were KaTid-Y, Time-S, and Time-P. RESULTS: The children with SB had lower time-processing abilities than typically-developing children (52.4% under - 2SD), particularly difficulties to orient to and to estimate objective time, to understand time perspectives and with time planning. They also self-rated low use of strategies to adapt to time. The parents rated their children as having extensive difficulties in daily time management. CONCLUSIONS: The low time-processing ability found in children with SB is likely to be an important contributing factor to low autonomy and independence.
Keywords: Myelomeningocele, time perception, time management, time orientation, executive function
Abstract: PURPOSE: Optimal management of neurogenic bowel in patients with spina bifida (SB) remains controversial. Surgical interventions may be utilized to treat constipation and provide fecal continence, but their use may vary among SB treatment centers. METHODS: We queried the National Spina Bifida Patient Registry (NSBPR) to identify patients who underwent surgical interventions for neurogenic bowel. We abstracted demographic characteristics, SB type, functional level, concurrent bladder surgery, mobility, and NSBPR clinics to determine whether any of these factors were associated with interventions for management of neurogenic bowel. Multivariable logistic regression with adjustment for selection bias was performed.…RESULTS: We identified 5,528 patients with SB enrolled in the 2009-14 NSBPR. Of these, 1,088 (19.7%) underwent procedures for neurogenic bowel, including 957 (17.3%) ACE/cecostomy tube and 155 (2.8%) ileostomy/colostomy patients. Procedures were more likely in patients who were older, white, non-ambulatory, with higher-level lesion, with myelomeningocele lesion, with private health insurance (all p < 0.001), and female (p = 0.006). On multivariable analysis, NSBPR clinic, older age (both p < 0.001), race (p = 0.002), mobility status (p = 0.011), higher lesion level (p < 0.001), private insurance (p = 0.002) and female sex (p = 0.015) were associated with increased odds of surgery. CONCLUSIONS: There is significant variation in rates of procedures to manage neurogenic bowel among NSBPR clinics. In addition to SB-related factors such as mobility status and lesion type/level, non-SB-related factors such as patient age, sex, race and treating center are also associated with the likelihood of undergoing neurogenic bowel intervention.
Keywords: Pediatrics, spina bifida, neurogenic bowel, clinical care variation
Abstract: PURPOSE: Sexuality has received little attention in spina bifida (SB) care. The aim of this study was to assess sexual identity and orientation in adults with SB. METHODS: An international online survey to adults with SB was administered over 10-months (recruitment: SB clinics, SB organizations via social media). Collected data included demographics, sexual identity and orientation. Non-parametric tests were used for analysis. RESULTS: Median age of 77 men and 119 women was 35 years old (52.0% shunted, 48.5% community ambulators, 42.3% outside United States). Most commonly, men identified as male (96.1%), while 1.3%…each described themselves as female, transgender and other. All women reporting sexual identity identified as female (99.2%), 0.8% not providing an answer. Most men reported heterosexual orientation (89.6%), followed by gay (7.8%) and bisexual (2.6%). Most women reported heterosexual orientation (84.9%), followed by bisexual (10.4%), gay/lesbian (2.5%), asexual (0.8%) and other (1.7%). CONCLUSION: As in the general population, sexual identity typically coincides with biological gender. Sexual orientation of adults with SB mirrors the general population. Due to self-selection, these findings likely do not reflect exact prevalence in the SB population.
Abstract: PURPOSE: Report urologic outcomes among newborns with spinal dysraphism managed within an expectant clean intermittent catheterization (CIC) program. METHODS: Newborns were followed clinically and with serial ultrasound (US). Urodynamics (UD) and dimercaptosuccinic acid (DMSA) renal scan were obtained at 3–6 months, 1 year, 3 years, then as needed. Patients with initial evaluation after 6 months were excluded. RESULTS: Median follow-up was 3.2 years. 11/102 began catheterization for continence (median 4.0 years) and 47/102 did not start CIC. Of these, 2/58 developed a DMSA abnormality. 44/102 began CIC early, often for elevated storage pressures and febrile…urinary tract infection (UTI). Of these, 20/44 developed a DMSA abnormality including 9 who had abnormality detected prior to starting CIC. Being on CIC or starting immediately upon recognition of new hydronephrosis, reflux, elevated filling pressures, or febrile UTI was associated with lower chance of DMSA abnormalities (4/17, 24%) compared to delaying CIC (16/27, 60%) (p = 0.03). CONCLUSIONS: CIC can be deferred until continence in select infants with a low risk of significant DMSA abnormality. However, immediate initiation of CIC upon recognition of risk factors is recommended as this was associated with fewer DMSA abnormalities than delaying CIC. Recommendations for expectantly-managed patients include close follow-up, serial US and UD, and prompt initiation of CIC upon recognition of new hydronephrosis, reflux, elevated storage pressures, or febrile UTIs.
Abstract: PURPOSE: Prenatal repair of myelomeningocele (MMC) via hysterotomy has demonstrated neurosurgical and motor benefits, when compared to postnatal repairs. Urologic benefits, however, remain to be seen. The purpose of this study was to review early postnatal bladder function in patients undergoing a novel endoscopic approach for MMC repair using an exteriorized uterus. METHODS: A prospective urologic assessment of patients undergoing fetoscopic MMC repair and receiving subsequent care at our facility, was performed. Patients were managed and urodynamic studies risk-stratified according to the Centers for Disease Control and Prevention Urologic and Renal Protocol for the Newborn and…Young Child with Spina Bifida. RESULTS: Fetoscopic MMC repair was performed in 14 patients. No patients had hydronephrosis or bladder thickening at birth. Detrusor overactivity was observed in nine (64.3%) patients. Impaired compliance was seen in eight (57.1%) patients. No patients had a detrusor leak point pressure of > 40 cm H 2 O or evidence of detrusor sphincter dyssynergia. Three (21.4%) patients had vesicoureteral reflux, seven (50.0%) had an open bladder neck, and none had trabeculated bladders. CONCLUSION: In this early experience with fetoscopic MMC repair, postnatal bladder function does not appear to be any worse than that of previously reported prenatal or postnatal closures.
Abstract: INTRODUCTION: Advances in care have allowed most children with spina bifida (SB) to live to adulthood. The majority have neuropathic bowel dysfunction (NBD), resulting in constipation, incontinence, and diminished quality of life. We sought to 1) describe contemporary NBD management and continence outcomes of adults with SB; 2) describe differences from younger patients; and 3) assess for association with socio-economic factors. METHODS: We analyzed data on NBD management and continence from the National Spina Bifida Patient Registry (NSBPR). Patients were segregated into young children (5–11 years), adolescents (12–19 years), and adults (20 years and older). A strict…definition of continence was utilized. Statistical analysis compared cohorts by gender, ethnicity, SB type, lesion level, insurance status, educational attainment, employment status, and continence. RESULTS: A total of 5209 SB patients were included, of whom 1370 (26.3%) were adults. Management and continence varied by age and SB type. Oral medication use did not differ between groups (5.2–6.6%). Suppositories and rectal enemas were used only by 11.5% of adults, which was significantly less than among school-aged children. Antegrade enemas were used by 17.7% of adults which was significantly less than among adolescents (27.2%). Adults were more likely to use digital stimulation or disimpaction or have undergone a colostomy. Bowel continence was reported by 58.3% of overall adult cohort: 55.6% of adults with myelomeningocele and 74.9% with non-myelomeningocele. Bowel continence was significantly associated with employment (p = 0.0002), private insurance (p = 0.0098), non-myelomeningocele type of SB (p = 0.0216) and educational attainment (p = 0.0324) on univariate analysis but only with employment on multivariable logistic regression (p = 0.0027). CONCLUSIONS: Bowel management techniques differed between adults and younger patients with SB. Bowel continence was reported by over half of SB adults and was associated with socio-economic factors.