Update on Urological Management of Spina Bifida from Prenatal Diagnosis to Adulthood.

Snow-Lisy DC, Yerkes EB, Cheng EY. (2015) Update on Urological Management of Spina Bifida from Prenatal Diagnosis to Adulthood.

J Urol. 2015 Apr 1. pii: S0022-5347(15)03647-2. doi: 10.1016/j.juro.2015.03.107

Abstract

PURPOSE:

We review the current literature regarding urological management of spina bifida from prenatal diagnosis to adulthood.

MATERIALS AND METHODS:

We searched MEDLINE, EMBASE and PubMed for English articles published through December 2014 using search terms "spina bifida," "spinal dysraphism" and "bladder." Based on review of titles and abstracts, 437 of 1,869 articles were identified as addressing topics related to open spina bifida in pediatric patients, or long-term or quality of life outcomes in adults with spina bifida. We summarize this literature to inform clinical guidelines and create a framework for disease management.

RESULTS:

The birth prevalence of spina bifida in the United States has recently plateaued at approximately 30 per 100,000. With improved management more individuals are surviving to adulthood, with an economic impact of $319,000 during the lifetime of an individual with spina bifida. Recent advances in prenatal surgery have demonstrated that prenatal closure of spina bifida is possible. To assess safety and efficacy, the National Institutes of Health sponsored MOMS (Management of Myelomeningocele Study) was undertaken, in which subjects were randomized to prenatal or postnatal closure. Until the urological results of this trial are published, the impact of prenatal intervention on future bladder function remains unclear. Controversy continues regarding the optimal use and timing of urodynamic studies, and the indications for initiation of clean intermittent catheterization and anticholinergics in infants and children. Many favor expectant management, while others argue for a more proactive approach. Based on the current literature, both approaches appear to protect the child from renal injury, although delayed intervention may increase rates of bladder augmentation. The current literature regarding this topic is difficult to interpret and compare due to heterogeneity of patient populations, variable outcome measures and lack of reporting of quality of life outcomes. Surgical intervention is indicated for those at risk for renal deterioration and/or is considered for children who fail to achieve satisfactory continence with medical management. Traditionally surgery concentrates on 3 areas, ie bladder and bladder neck, and creation of catheterizable channels. For those with a hostile bladder enterocystoplasty remains the gold standard for bladder augmentation, although use of bowel for augmentation remains suboptimal due to secondary complications, including increased risk of infections, metabolic abnormalities, neoplastic transformation and risk of life threatening perforation. Recent advances in tissue engineering technology may provide an alternative to traditional augmentation. However, recent results from phase II trials using current techniques to augment the bladder with engineered bladder tissue are disappointing. Catheterizable channels to the bladder and ascending colon further facilitate continence measures and promote independent care. While surgical reconstruction is clearly successful in improving continence, recent outcome studies have questioned the true impact of this type of surgery on quality of life. With improved survival transitional care issues, including health related independence, sexual health needs and development of a support system, are increasingly important. Transitional care remains a significant issue for which few public health measures are being quantitatively evaluated.

CONCLUSIONS:

Despite consensus regarding early urological involvement in the care of patients with spina bifida, controversy remains regarding optimal management. Major reconstructive urological surgeries still have a major role in the management of these cases to protect the upper urinary tract and to achieve continence. However, future studies are needed to better clarify the true impact on quality of life that these interventions have on patients and their families. Transition of urological care to adulthood remains a major avenue for improvement in disease management.
Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

KEYWORDS:

disease management; meningomyelocele; spinal dysraphism; urinary bladder

Lennon JM, Klages KL, Amaro CM, Murray CB, Holmbeck GN. (2015) Longitudinal study of neuropsychological functioning and internalizing symptoms in youth with spina bifida: social competence as a mediator.

J Pediatr Psychol. 2015 Apr;40(3):336-48. doi: 10.1093/jpepsy/jsu075.

Abstract

OBJECTIVE:

To examine the longitudinal relationship between neuropsychological functioning and internalizing symptoms, as mediated by social competence in youth with spina bifida (SB).

METHODS:

A total of 111 youth (aged 8-15 years, M = 11.37) with SB, their parents, and teachers completed questionnaires regarding attention, social competence, and internalizing symptoms. Youth also completed a battery of neuropsychological tests.

RESULTS:

An indirect-only mediation model revealed that social competence mediated the relation between neuropsychological functioning and subsequent levels of teacher-reported internalizing symptoms, but not parent or youth report of internalizing symptoms. Specifically, better neuropsychological functioning was associated with better social competence, which, in turn, predicted fewer internalizing symptoms 2 years later.

CONCLUSIONS:

Youth with SB with lower levels of neuropsychological functioning may be at risk for poorer social competence and, as a result, greater internalizing symptoms. Interventions that promote social competence, while being sensitive to cognitive capacities, could potentially alleviate or prevent internalizing symptoms in these youth.
© The Author 2014. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

KEYWORDS:

internalizing symptoms; neuropsychological functioning; social competence; spina bifida

Observed differences in social behaviors exhibited in peer interactions between youth with spina bifida and their peers: neuropsychological correlates.

Holbein CE, Lennon JM, Kolbuck VD, Zebracki K, Roache CR, Holmbeck GN. (2015) Observed differences in social behaviors exhibited in peer interactions between youth with spina bifida and their peers: neuropsychological correlates.

J Pediatr Psychol. 2015 Apr;40(3):320-35. doi: 10.1093/jpepsy/jsu101.

Abstract

OBJECTIVE:

To identify differences in social behaviors in observed peer interactions between children with spina bifida (SB) and peers, and to examine neuropsychological correlates of these differences.

METHOD:

A total of 100 youth (aged 8-15 years) with SB and peers participated in video-recorded interaction tasks, which were coded for interaction style, affect, and collaboration. Children with SB also completed a neuropsychological test battery.

RESULTS:

Children with SB demonstrated less adaptive social behaviors in peer interactions, particularly within the interaction style domain. Observational items found to be different between children with SB and their peers were best predicted by social language and attention abilities.

CONCLUSIONS:

Children with SB exhibit a less adaptive interaction style and lower levels of social dominance but are comparable with typically developing peers on other social behaviors. The observed group differences may have a neuropsychological basis.
© The Author 2014. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

KEYWORDS:

neuropsychological functioning; observational methods; peer relationships; social competence; spina bifida

Postshunt lateral ventricular volume, white matter integrity, and intellectual outcomes in spina bifida and hydrocephalus.

Williams VJ, Juranek J, Stuebing KK, Cirino PT, Dennis M, Bowman RM, Blaser S, Kramer LA, Fletcher JM. (2015) Postshunt lateral ventricular volume, white matter integrity, and intellectual outcomes in spina bifida and hydrocephalus.

J Neurosurg Pediatr. 2015 Apr;15(4):410-9. doi: 10.3171/2014.10.PEDS13644

Abstract

OBJECT No previous reports exist that have evaluated the relationships of white matter (WM) integrity with the number of shunt revisions, ventricular volume after shunting, and cognition in medically stable children who have spina bifida and hydrocephalus (SBH). The authors hypothesized that enlarged ventricles and a greater number of shunt revisions decrease WM integrity in children. METHODS In total, 80 children (mean age 13.7 years) who had SBH underwent MRI and IQ testing. Probabilistic diffusion tractography was performed to determine mean diffusion tensor imaging (DTI) metrics along the frontal and parietal tectocortical pathways. The DTI metrics were evaluated for significant correlation with a composite IQ measure and with the total number of shunt revisions and the total lateral ventricular volume obtained through semiautomated parcellation of T1-weighted MRI scans. RESULTS An enlargement in total lateral ventricle volume and an increase in the number of shunt revisions were both associated with higher fractional anisotropy (FA) and with lower radial diffusivity (RD) along both frontal and parietal tectocortical pathways. Children who had not undergone a shunt revision had on average a greater lateral ventricle volume and higher FA and lower RD along frontal and parietal pathways than those who had undergone multiple shunt revisions. The mean DTI metrics along parietal pathways predicted IQ scores, but intellectual ability was not significantly correlated with ventricular volume or with the number of lifetime shunt revisions. CONCLUSIONS Significant changes in DTI metrics were observed as a function of ventricular volume. An increased lateral ventricle volume was associated with elevated FA and decreased RD. Given that the participants were medically stable at the time of the MRI examination, the results suggested that those who have enlarged ventricles show a DTI pattern consistent with axonal compression due to increased intracranial pressure (ICP) in attenuated hydrocephalus. Although limited by a cross-sectional design, the study's findings suggest that DTI metrics may serve as sensitive indicators for chronic, mild hydrocephalus in the absence of overt clinical symptoms due to increased ICP. Having enlarged ventricles and undergoing multiple shunt revisions did not affect intellectual ability in children with SBH.

Follow-up of the neuro-urological patient: a systematic review.

Averbeck MA, Madersbacher H. (2015) Follow-up of the neuro-urological patient: a systematic review.

BJU Int. 2015 Apr;115 Suppl 6:39-46. doi: 10.1111/bju.13084.

Abstract

OBJECTIVES:

To systematically review the long-term urological follow-up strategies for patients with neurogenic lower urinary tract dysfunction (NLUTD), focusing on three main groups of neurological diseases: (i) spinal cord injuries, (ii) spinal dysraphism, and (iii) multiple sclerosis.

PATIENTS AND METHODS:

Data acquisition comprised electronic search on the Medical Literature Analysis and Retrieval System Online (MEDLINE) database and the EMBASE database in August 2014 to retrieve English language studies. MEDLINE and EMBASE search included the following medical subject heading (MeSH) terms: (i) neurogenic bladder and (ii) neurogenic bladder dysfunction. Each of these terms was crossed with (i) long-term care and (ii) long-term surveillance. Only studies related to NLUTD and urological follow-up were included. Studies were also identified by hand search of reference lists and review articles.

RESULTS:

Initial records identified through database searching included 265 articles. In all, 23 articles were included in the quantitative synthesis. The proposed time schedule of investigations as well as the amount and type of investigation were different according to specific neurological lesions. They depend on the dysfunctional pattern of the lower urinary tract (LUT) and its risk profile. However, there is a lack of high-evidence level studies to support an optimal long-term follow-up protocol.

CONCLUSIONS:

The goal of neuro-urological management is the best possible preservation of upper urinary tract (UUT) and LUT function in relation to the individual neurological disorder. Regular and risk adapted controls ('urochecks') allow detection of risk-factors in time before irreversible changes of the LUT and UUT have occurred. With risk- and patient-oriented lifelong regular urological care an optimised quality of life and life-expectancy can be achieved, although there is a complete lack of high-evidence level studies on this topic.
© 2015 The Authors. BJU International © 2015 BJU International.

Syrinx location and size according to etiology: identification of Chiari-associated syrinx.

Strahle J, Muraszko KM, Garton HJ, Smith BW, Starr J, Kapurch JR 2nd, Maher CO. (2015) Syrinx location and size according to etiology: identification of Chiari-associated syrinx.

J Neurosurg Pediatr. 2015 Apr 3:1-9.

Abstract

OBJECT Syrinx size and location within the spinal cord may differ based on etiology or associated conditions of the brain and spine. These differences have not been clearly defined. METHODS All patients with a syrinx were identified from 14,118 patients undergoing brain or cervical spine imaging at a single institution over an 11-year interval. Syrinx width, length, and location in the spinal cord were recorded. Patients were grouped according to associated brain and spine conditions including Chiari malformation Type I (CM-I), secondary CM (2°CM), Chiari malformation Type 0 (CM-0), tethered cord, other closed dysraphism, and spinal tumors. Syringes not associated with any known brain or spinal cord condition were considered idiopathic. Syrinx characteristics were compared between groups. RESULTS A total of 271 patients with a syrinx were identified. The most common associated condition was CM-I (occurring in 117 patients [43.2%]), followed by spinal dysraphism (20 [7.4%]), tumor (15 [5.5%]), and tethered cord (13 [4.8%]). Eighty-three patients (30.6%) did not have any associated condition of the brain or spinal cord and their syringes were considered idiopathic. Syringes in patients with CM-I were wide (7.8 ± 3.9 mm) compared with idiopathic syringes (3.9 ± 1.0, p < 0.0001) and those associated with tethered cord (4.2 ± 0.9, p < 0.01). When considering CM-I-associated and idiopathic syringes, the authors found that CM-I-associated syringes were more likely to have their cranial extent in the cervical spine (88%), compared with idiopathic syringes (43%; p < 0.0001). The combination of syrinx width greater than 5 mm and cranial extent in the cervical spine had 99% specificity (95% CI 0.92-0.99) for CM-I-associated syrinx. CONCLUSIONS Syrinx morphology differs according to syrinx etiology. The combination of width greater than 5 mm and cranial extent in the cervical spine is highly specific for CM-I-associated syringes. This may have relevance when determining the clinical significance of syringes in patients with low cerebellar tonsil position.

Sleep-disordered breathing in patients with myelomeningocele.

Patel DM, Rocque BG, Hopson B, Arynchyna A, Bishop ER, Lozano D, Blount JP.  (2015) Sleep-disordered breathing in patients with myelomeningocele.

J Neurosurg Pediatr. 2015 Apr 3:1-6.

Abstract

OBJECT A paucity of literature examines sleep apnea in patients with myelomeningocele, Chiari malformation Type II (CM-II), and related hydrocephalus. Even less is known about the effect of hydrocephalus treatment or CM-II decompression on sleep hygiene. This study is an exploratory analysis of sleep-disordered breathing in patients with myelomeningocele and the effects of neurosurgical treatments, in particular CM-II decompression and hydrocephalus management, on sleep organization. METHODS The authors performed a retrospective review of all patients seen in their multidisciplinary spina bifida clinic (approximately 435 patients with myelomeningocele) to evaluate polysomnographs obtained between March 1999 and July 2013. They analyzed symptoms prompting evaluation, results, and recommended interventions by using descriptive statistics. They also conducted a subset analysis of 9 children who had undergone polysomnography both before and after neurosurgical intervention. RESULTS Fifty-two patients had polysomnographs available for review. Sleep apnea was diagnosed in 81% of these patients. The most common presenting symptom was "breathing difficulties" (18 cases [43%]). Mild sleep apnea was present in 26 cases (50%), moderate in 10 (19%), and severe in 6 (12%). Among the 42 patients with abnormal sleep architecture, 30 had predominantly obstructive apneas and 12 had predominantly central apneas. The most common pulmonology-recommended intervention was adjustment of peripheral oxygen supplementation (24 cases [57%]), followed by initiation of peripheral oxygen (10 cases [24%]). In a subset analysis of 9 patients who had sleep studies before and after neurosurgical intervention, there was a trend toward a decrease in the mean number of respiratory events (from 34.8 to 15.9, p = 0.098), obstructive events (from 14.7 to 13.9, p = 0.85), and central events (from 20.1 to 2.25, p = 0.15) and in the apnea-hypopnea index (from 5.05 to 2.03, p = 0.038, not significant when corrected for multiple measures). CONCLUSIONS A large proportion of patients with myelomeningocele who had undergone polysomnography showed evidence of disordered sleep on an initial study. Furthermore, 31% of patients had moderate or severe obstructive sleep apnea. Myelomeningocele patients with an abnormal sleep structure who had undergone nonoperative treatment with peripheral oxygen supplementation showed improvement in the apnea-hypopnea index. Results in this study suggested that polysomnography in patients with myelomeningocele may present an opportunity to detect and classify sleep apnea, identify low-risk interventions, and prevent future implications of sleep-disordered breathing.

Perception of secondary conditions in adults with spina bifida and impact on daily life.

Wagner R, Linroth R, Gangl C, Mitchell N, Hall M, Cady R, Christenson M. (2015) Perception of secondary conditions in adults with spina bifida and impact on daily life.

Disabil Health J. 2015 Apr 4. pii: S1936-6574(15)00048-5. doi: 10.1016/j.dhjo.2015.03.012.

Abstract

BACKGROUND:

Spina bifida is a congenital defect of the neural tube resulting in motor and sensory disruption. Persons with spina bifida can also experience executive function impairments. Secondary conditions are physical, medical, cognitive, emotional, or psychosocial consequences to which persons with disabilities are more susceptible. Our experience suggested clinicians underappreciate the presence and impact of secondary conditions in adults with spina bifida because they do not specifically ask for this information.

OBJECTIVE:

Describe the presence and impact of secondary conditions on daily life, as perceived by adults with spina bifida.

METHODS:

A clinic-based sample was recruited from the active patient population of an adult specialty center for spina bifida-related care. All subjects were verbally administered a survey developed through literature review and clinical experience of the researchers. The survey measured the presence and perceived impact of secondary conditions. Recruitment and survey data collection occurred over a 6-month period to maximize age representation. Survey data were stratified by age, gender and lesion level for analysis.

RESULTS:

Seventy-two respondents completed the survey. Pain was commonly reported, along with pressure ulcers, bowel & bladder concerns, depression, sleep disturbance, and limited social and community participation. No significant relationships were found between the presence or perceived impact of secondary conditions and age, gender or level of lesion.

CONCLUSIONS:

Secondary conditions in spina bifida are present by early adulthood. Identifying these conditions during clinical encounters requires specific rather than general questions. Future study should evaluate earlier initiation of preventative measures by pediatric providers.
Copyright © 2015 Elsevier Inc. All rights reserved.

Descriptive study on neural tube defects in Argentina.

Sargiotto C, Bidondo MP, Liascovich R, Barbero P, Groisman B. (2015) Descriptive study on neural tube defects in Argentina.

Birth Defects Res A Clin Mol Teratol. 2015 Apr 8. doi: 10.1002/bdra.23372.

Abstract

BACKGROUND:

Neural tube defects (NTDs) are the most common congenital anomalies of the central nervous system. Prevalence of NTDs varies depending on geographic region and folic acid fortification. The main objectives of this study are to analyze the prevalences of NTDs reported to the National Registry of Congenital Anomalies of Argentina (RENAC) during the period 2009 to 2013, to compare them with data from other selected surveillance systems of the Americas, and to evaluate the impact of folic acid fortification on the prevalence of NTDs in Argentina.

METHODS:

We analyzed changes in prevalence between 2009 and 2013 for the RENAC, and differences in prevalence between RENAC and other surveillance systems. We evaluated changes for the pre and postfortification time periods in Argentina.

RESULTS:

In Argentina, anencephaly and spina bifida showed no statistically significant variation between 2009 and 2013, and encephalocele showed a statistically significant decline. The RENAC showed a statistically significant higher prevalence for all three defects when compared with surveillance systems from Chile, Colombia, and Costa Rica, and a statistically significant lower birth prevalence than Cuba for anencephaly. No differences were observed when comparing it to the Atlanta-USA and Mexico systems. A significant decrease in prevalence was observed for all three anomalies for the postfortification period in Argentina.

CONCLUSION:

The characteristics of cases reported to the RENAC are similar to those already reported in the literature. The differences in prevalence between the RENAC and other surveillance systems could be artifactual or real. We confirmed the decrease of prevalence of NTDs after folic acid fortification. Birth Defects Research (Part A), 2015. © 2015 Wiley Periodicals, Inc.
© 2015 Wiley Periodicals, Inc.

Maternal consumption of non-staple food in the first trimester and risk of neural tube defects in offspring.

Wang M, Wang ZP, Gao LJ, Yang H, Zhao ZT. (2015) Maternal consumption of non-staple food in the first trimester and risk of neural tube defects in offspring.  Nutrients. 2015 Apr 24;7(5):3067-77. doi: 10.3390/nu7053067.

Abstract

To study the associations between maternal consumption of non-staple food in the first trimester and risk of neural tube defects (NTDs) in offspring. Data collected from a hospital-based case-control study conducted between 2006 and 2008 in Shandong/Shanxi provinces including 459 mothers with NTDs-affected births and 459 mothers without NTDs-affected births. Logistic regression models were used to examine the associations between maternal consumption of non-staple food in the first trimester and risk of NTDs in offspring. The effects were evaluated by odds ratio (OR) and 95% confidence intervals (95% CIs) with SAS9.1.3.software. Maternal consumption of milk, fresh fruits and nuts in the first trimester were protective factors for total NTDs. Compared with consumption frequency of ˂1 meal/week, the ORs for milk consumption frequency of 1-2, 3-6, ≥7 meals/week were 0.50 (95% CI: 0.28-0.88), 0.56 (0.32-0.99), and 0.59 (0.38-0.90), respectively; the ORs for fresh fruits consumption frequency of 1-2, 3-6, ≥7 meals/week were 0.29 (95% CI: 0.12-0.72), 0.22 (0.09-0.53), and 0.32 (0.14-0.71), respectively; the ORs for nuts consumption frequency of 1-2, 3-6, ≥7 meals/week were 0.60 (95% CI: 0.38-0.94), 0.49 (0.31-0.79), and 0.63 (0.36-1.08), respectively. Different effects of above factors on NTDs were found for subtypes of anencephaly and spina bifida. Maternal non-staple food consumption of milk, fresh fruits and nuts in the first trimester was associated with reducing NTDs risk in offspring.

Resilience and Disruption in Observed Family Interactions in Youth With and Without Spina Bifida: An Eight-Year, Five-Wave Longitudinal Study.

Lennon JM, Murray CB, Bechtel CF, Holmbeck GN. (2015) Resilience and Disruption in Observed Family Interactions in Youth With and Without Spina Bifida: An Eight-Year, Five-Wave Longitudinal Study.

J Pediatr Psychol. 2015 Apr 24. pii: jsv033.

Abstract

OBJECTIVE:

To examine differences between families of youth with spina bifida (SB) and families of typically developing (TD) youth on family-, parent-, and youth-level variables across preadolescence and adolescence.  METHODS:  Participants were 68 families of youth with SB and 68 families of TD youth. Ratings of observed family interactions were collected every 2 years at 5 time points (Time 1: ages 8-9 years; Time 5: ages 16-17 years).  RESULTS:  For families of youth with SB: families displayed less cohesion and more maternal psychological control during preadolescence (ages 8-9 years); parents presented as more united and displayed less dyadic conflict, and youth displayed less conflict behavior during the transition to adolescence (ages 10-13 years); mothers displayed more behavioral control during middle (ages 14-15 years) and late (ages 16-17 years) adolescence; youth displayed less engagement and more dependent behavior at every time point.  CONCLUSIONS:  Findings highlight areas of resilience and disruption in families of youth with SB across adolescence.
© The Author 2015. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Arm cranking versus wheelchair propulsion for testing aerobic fitness in children with spina bifida who are wheelchair dependent.

Bloemen MA, de Groot JF, Backx FJ, Westerveld RA, Takken T. (2015) Arm cranking versus wheelchair propulsion for testing aerobic fitness in children with spina bifida who are wheelchair dependent.

J Rehabil Med. 2015 Apr 28;47(5):432-7. doi: 10.2340/16501977-1944.

Abstract

OBJECTIVE:

To determine the best test performance and feasibility using a Graded Arm Cranking Test vs a Graded Wheelchair Propulsion Test in young people with spina bifida who use a wheelchair, and to determine the reliability of the best test.

DESIGN:

Validity and reliability study.

SUBJECTS:

Young people with spina bifida who use a wheelchair.

METHODS:

Physiological responses were measured during a Graded Arm Cranking Test and a Graded Wheelchair Propulsion Test using a heart rate monitor and calibrated mobile gas analysis system (Cortex Metamax). For validity, peak oxygen uptake (VO2peak) and peak heart rate (HRpeak) were compared using paired t-tests. For reliability, the intra-class correlation coefficients, standard error of measurement, and standard detectable change were calculated.

RESULTS:

VO2peak and HRpeak were higher during wheelchair propulsion compared with arm cranking (23.1 vs 19.5 ml/kg/min, p = 0.11; 165 vs 150 beats/min, p < 0.05). Reliability of wheelchair propulsion showed high intra-class correlation coefficients (ICCs) for both VO2peak (ICC = 0.93) and HRpeak (ICC = 0.90).

CONCLUSION:

This pilot study shows higher HRpeak and a tendency to higher VO2peak in young people with spina bifida who are using a wheelchair when tested during wheelchair propulsion compared with arm cranking. Wheelchair propulsion showed good reliability. We recommend performing a wheelchair propulsion test for aerobic fitness testing in this population.

Urinary incontinence in spina bifida: Initial instrument validation.

Hubert KC, Sideridis G, Sherlock R, Rosoklija I, Kringle G, Johnson K, Bauer SB, Nelson CP.

(2015)  Urinary incontinence in spina bifida: Initial instrument validation.  Res Dev Disabil. 2015 May;40:42-50. doi: 10.1016/j.ridd.2015.01.008.

Abstract

The purpose of this study was to perform a psychometric assessment of the Incontinence Symptom Index-Pediatric (ISI-P) in a cohort of adolescents with spina bifida (SB) and neuropathic urinary incontinence (UI) to test its validity and reliability. The ISI-P, an 11-item instrument with domains for symptom severity and impairment, was self-administered by subjects 11-17 years old with SB and UI. Controls were 11-17 years old, with nephrolithiasis and no history of UI. Formal psychometric assessment included an evaluation of internal consistency, test re-test reliability and factor analysis. Of 78 study-eligible subjects we attempted to contact, 33 (66.7% female) with a median age of 13.1 years completed the ISI-P (42.3% response rate). 21 control patients also completed the ISI-P. Cronbach's alpha was 0.936 and 0.792 for the severity and bother factors respectively. The delta Chi-square test for the two-factor (vs. one-factor) model was significantly [χ(2)(89)=107.823, p<0 .05="" adolescents="" all="" analysis="" and="" associated="" being="" categories="" category="" comparative="" conclusion="" contributed="" descriptive="" desirable="" different="" e.g.="" each="" excellent="" favor="" fit="" for="" former="" furthermore="" has="" impairment="" in="" index="0.969)." indices="" information="" isi-p="" latent="" measurement="" model="" namely="" of="" p="" properties="" psychometric="" sb.="" severity="" showed="" symptom="" that="" the="" threshold="" trait.="" ui="" unique="" values="" were="" with="">Copyright © 2015 Elsevier Ltd. All rights reserved.

An increase in spina bifida cases in Tunisia, 2008-2011.

Nasri K, Ben Fradj MK, Aloui M, Ben Jemaa N, Masmoudi A, Elmay MV, Marrakchi R, Siala Gaigi S. (2015) An increase in spina bifida cases in Tunisia, 2008-2011.

Pathol Res Pract. 2015 May;211(5):369-73. doi: 10.1016/j.prp.2014.12.011

Abstract

BACKGROUND:

The term spina bifida refers to a group of neural tube defects that result in malformations of the spinal cord and the surrounding vertebrae. Though the etiologies of spina bifida remain largely unknown, several risk factors have been identified, including feto-maternal characteristics.

AIM OF THE STUDY:

To discover possible underlying reasons for the increase of spina bifida and identify intervention targets, an investigation was undertaken comparing spina bifida-affected pregnancy notifications in 2008-2011 with notifications in the period 1991-1994.

METHODS:

Characteristics and outcomes of births with spina bifida and pregnancy characteristics of mothers were recorded in the medical chart. Comparisons of pregnancies affected by a spina bifida in 2008-2011 were made with pregnancies affected by a spina bifida in the period 1991-1994. Statistical analysis was undertaken using Poisson regression and Chi-squared tests.

RESULTS:

From 1991 through 1994, the prevalence of identified spina bifida cases was equal to 0.3/10,000 births compared to 1.6/10,000 births in 2008-2011. This increase was statistically significant (P<0 .001="" 0.16="" 0.45="" 1.88="" 10="" 1991-1994="" 2008-2011.="" 30="" a="" age="" as="" between="" births="" both="" class="highlight" compared="" difference="" during="" emergence="" equal="" females="" for="" genders.="" had="" impact="" in="" males="" mother="" of="" on="" over="" per="" period="" prevalence="" s="" significant="" span="" statistically="" the="" to="" was="" years="">spina bifida

(P=0.02, OR=3.93, CI=1.23-12.47). As well as, maternal blood type was a significant risk factor for the appearance of spina bifida (P=0.008). Results also had shown that fetal weight and term, gestity and parity were significant risk factors for the occurrence of spina bifida (P<0 .05="" adjusted.="" analyses="" been="" being="" caution="" due="" have="" interpreted="" n="" not="" p="" results="" study="" this="" to="" with="">

CONCLUSION:

This analysis highlighted areas where prevention efforts should be strengthened and surveillance data improved.
Copyright © 2014 Elsevier GmbH. All rights reserved.

A longitudinal examination of health-related quality of life in children and adolescents with spina bifida.

Murray CB, Holmbeck GN, Ros AM, Flores DM, Mir SA, Varni JW. (2015) A longitudinal examination of health-related quality of life in children and adolescents with spina bifida.

J Pediatr Psychol. 2015 May;40(4):419-30. doi: 10.1093/jpepsy/jsu098

Abstract

OBJECTIVE:

The current study examined (1) spina bifida (SB) youths' health-related quality of life (HRQOL) compared with nonclinical and chronic health condition (CHC) samples, (2) parent-child agreement regarding HRQOL, and (3) prospective changes in HRQOL. 

METHODS:

Child and parent-proxy reports of Pediatric Quality of Life were collected at two time waves (Time 1: N = 134, ages 8-15 years; Time 2: N = 109, ages 10-17 years) as part of a larger longitudinal study. 

RESULTS:

SB youth had statistically and clinically reduced physical HRQOL compared with the nonclinical and CHC samples at both time points. There were significant discrepancies between youth and parent-proxy reports of HRQOL; youth reported higher levels of physical and social HRQOL than parents. The majority of parent- and child-reported HRQOL domains remained stable, yet youth-reported social HRQOL increased over time. 

CONCLUSIONS:

Youth with SB are at risk for poor HRQOL. Examining modifiable condition and social-environmental predictors of youth HRQOL will be important in informing future interventions.
© The Author 2014. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Early outcome of combined endoscopic third ventriculostomy and choroid plexus cauterization in childhood hydrocephalus.

Bankole OB, Ojo OA, Nnadi MN, Kanu OO, Olatosi JO. (2015) Early outcome of combined endoscopic third ventriculostomy and choroid plexus cauterization in childhood hydrocephalus.

J Neurosurg Pediatr. 2015 May;15(5):524-8. doi: 10.3171/2014.10.PEDS14228

Abstract

OBJECT Although shunts have been the mainstay in treating hydrocephalus over the past 5 decades, the use of endoscopic techniques in addressing this disorder in children offers both the neurosurgeon and the patient a unique opportunity to avoid shunting and its attendant complications. The combination of endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) remains uncommon in most centers despite its potential promise. The authors sought to investigate the efficacy of combining ETV and CPC (ETV+CPC) in treating childhood hydrocephalus in Nigeria. Infection and spina bifida contribute a high percentage of the cases of hydrocephalus in Nigeria. METHODS Over a 2-year period, all children 0-18 years of age who had endoscopic treatment for hydrocephalus were prospectively evaluated to determine the need for subsequent treatment. Children who had the combination of ETV+CPC were identified as a subcategory and form the basis of this retrospective study. RESULTS Twenty-two of 38 endoscopically treated children had undergone the combination of ETV+CPC for hydrocephalus of varied etiology. There was a male preponderance (2.5:1), and 90% of the patients were infants. The overall success rate was 75%, with the best outcome in children with spina bifida. One child required a repeat ETV. CONCLUSIONS The combination of ETV+CPC is useful in treating children with hydrocephalus of varied etiology. The complication profile is acceptable, and the overall success rate is comparable to that associated with shunt insertion.

Risk of fracture prevention in spina bifida patients: correlation between bone mineral density, vitamin D, and electrolyte values.

Risk of fracture prevention in spina bifida patients: correlation between bone mineral density, vitamin D, and electrolyte values.  (2015) Martinelli V, Dell'Atti C, Ausili E, Federici E, Magarelli N, Leone A, Massimi L, Di Rocco C, Bonomo L, Rendeli C.

Childs Nerv Syst. 2015 May 1.

Abstract

PURPOSE:

The aim of our study was to investigate the relationship between bone mineral density (BMD), vitamin D, and electrolyte blood values in patients with spina bifida, to find a possible therapeutic regimen and an intervention to reduce the risk of fractures in this population.

METHODS:

BMD values were measured in 49 patients (32 females, 17 males; aged 14.1 ± 3.86 years; range 5-20 years) using dual-energy X-ray absorptiometry (DEXA) and were analyzed based on sex, the level of spinal involvement, vitamin D, and electrolyte values, physical activity, body mass index (BMI), and ambulatory status [patients were divided into three subgroups: full-time wheelchair (FTWC), limited ambulator (LA), and full-time ambulator (FTA)]. These data were analyzed considering sex-, age-, and BMD-matched values and compared with those of normal population.

RESULTS:

BMD was significantly lower in these patients compared with that in the general healthy population (Z-score: -1.2 ± 1.8); in particular, females had Z-score values significantly lower that of the males (Z-score: -2.43 ± 2.02; P < 0.0004). In FTWC subgroup, Z-score was lower than that of the other two subgroups (P < 0.009). Vitamin D values were significantly lower compared with those in the general healthy population (vitamin D spina bifida group: 14.6 ± 8.7 mg/dL; normal subjects: 35 ± 9.8 mg/dL; P < 0.001). Subjects with spina bifida showed hypophosphatemia (<3 0.001="" 0.9="" because="" d="" dl="" levels="" lower="" mg="" nbsp="" of="" p="" the="" vitamin="">

CONCLUSIONS:

Spina bifida patients showed lower BMD, vitamin D, and electrolyte values than the healthy population; hence, they have an increase risk of developing pathological fractures. Vitamin D supplementation for a longer time period could reduce this risk.

Past and current use of walking measures for children with spina bifida: a systematic review.

Bisaro DL, Bidonde J, Kane KJ, Bergsma SA, Musselman KE. (2015) Past and current use of walking measures for children with spina bifida: a systematic review.

Arch Phys Med Rehabil. 2015 May 2. pii: S0003-9993(15)00384-6. doi: 10.1016/j.apmr.2015.04.014

Abstract

OBJECTIVE:

To describe walking measurement in children with spina bifida, and to identify patterns in the use of walking measures in this population.

DATA SOURCES:

Seven medical databases were searched from inception until March 2014. Search terms encompassed three themes: 1) children, 2) spina bifida, and 3) walking.

STUDY SELECTION:

Articles were included if participants were children aged 1-17 years with spina bifida, and if walking was measured. Articles were excluded if the assessment was restricted to kinematic, kinetic or electromyographic analyses of walking. A total of 1,751 abstracts were screened by two authors independently, and 109 articles were included in this review.

DATA EXTRACTION:

Data were extracted using standardized forms. Extracted data included study and participant characteristics, and details about the walking measures used, including psychometric properties. Two authors evaluated the methodological quality of articles using a previously published framework that considers sampling method, study design, and psychometric properties of the measures used.

DATA SYNTHESIS:

Nineteen walking measures were identified. Ordinal-level rating scales (e.g., Hoffer Functional Ambulation Scale) were most commonly used (57% of articles), followed by ratio-level, spatiotemporal measures, such walking speed (18% of articles). Walking was measured for a variety of reasons relevant to multiple health care disciplines. A machine learning analysis was used to identify patterns in the use of walking measures. The learned classifier predicted whether or not a spatiotemporal measure was used with 77.1% accuracy. A trend to use spatiotemporal measures in older children and those with lumbar and sacral spinal lesions was identified. Most articles were prospective studies that used samples of convenience and unblinded assessors. Few articles evaluated or considered the psychometric properties of the walking measures.

CONCLUSIONS:

Despite a demonstrated need to measure walking in children with spina bifida, few valid, reliable and responsive measures have been established for this population.
Copyright © 2015 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

The Severity of Bowel Dysfunction in Patients with Neurogenic Bladder.

Cameron AP, Rodriguez GM, Gursky A, He C, Clemens JQ, Stoffel JT. (2015) The Severity of Bowel Dysfunction in Patients with Neurogenic Bladder.

J Urol. 2015 May 5. pii: S0022-5347(15)03905-1. doi: 10.1016/j.juro.2015.04.100

Abstract

PURPOSE:

Patients with neurological conditions often suffer from severe debilitating lower urinary and bowel dysfunction in addition to their physical disabilities. However, only the bladder has received the attention of medical providers with neurogenic bowel being poorly understood and characterized.

MATERIALS AND METHODS:

This is a cross-sectional analysis of a prospective institutional Neurogenic Bladder Database from 2010-2013.

RESULTS:

Among the 175 patients 60.6% had traumatic spinal cord injury (SCI) and 18.3% multiple sclerosis. Fecal Incontinence Severity Index (FISI) scores were a median of 18.0±1.39 (moderate). Neurogenic Bowel Dysfunction (NBD) score were a median of 11.0±0.63 (moderate). NBD Scores were worse in those patients with SCI and spina bifida compared to other diseases (P=0.020), in younger patients (p=0.020) and in the SCI group those with higher levels of injury (p=0.0046). Based on the Bristol stool scale 65% of patient had abnormal stool consistency, mostly constipation. None of the FISI, Bristol or NBD scores correlated significantly with SF-12 quality of life measures. However, both of the bladder symptom scores Michigan Incontinence Symptom Index (M-ISI)(p=0.05) and the AUA-SI (p=0.03) correlated with FISI severity and the NBD score correlated with the M-ISI (ρ=0.29, p=0.02). Those patients with abnormal stool consistency on the Bristol reported more urgency and stress incontinence on M-ISI.

CONCLUSIONS:

Bowel dysfunction is very common among patients with neurogenic bladder. Those patients with worse bladder symptoms also suffered from worse bowel dysfunction. This highlights the importance of addressing both bowel and bladder dysfunction in this often poorly understood population.
Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

Examination of the relationship between body mass index (BMI) and functional independence level in children with spina bifida.

Şimşek TT, Türkücüoğlu B, Tezcan S. (2015) Examination of the relationship between body mass index (BMI) and functional independence level in children with spina bifida.

Dev Neurorehabil. 2015 Jun;18(3):149-54. doi: 10.3109/17518423.2013.796419

Abstract

OBJECTIVE:

The aim of this study is to examine the relationship between body mass index (BMI) and functional independence level in children with spina bifida (SB).

METHODS:

The study included 116 children between 5 and 18 years. Subjects' socio-demographic characteristics, BMI values and functional independence levels were recorded.

RESULTS:

There was negative correlation between BMI and communication and social cognition in girls (p < 0.05). No correlation was found between BMI and WeeFIM in boys (p > 0.05).

CONCLUSION:

The results indicated that body weight in children with SB affected functional independency and that precautions to control weight important in these children.