Mummareddy N, Dewan MC, Mercier MR, Naftel RP, Wellons JC 3rd, Bonfield CM. (2017) Scoliosis in myelomeningocele: epidemiology, management, and functional outcome.

Journal of Neurosurgery. Pediatrics. 2017 Apr 28:1-10. doi: 10.3171/2017.2.PEDS16641. [Epub ahead of print] PMID: 28452655

 

Abstract

OBJECTIVE The authors aimed to provide an updated and consolidated report on the epidemiology, management, and functional outcome of cases of myelomeningocele (MMC) in patients with scoliosis. METHODS A comprehensive literature search was performed using MEDLINE, Embase, Google Scholar, and the Cochrane Database of Systematic Reviews on cases of MMC in patients with scoliosis between 1980 and 2016. The initial search yielded 670 reports. After removing duplicates and applying inclusion criteria, we included 32 full-text original articles in this study. RESULTS Pooled statistical analysis of the included articles revealed the prevalence of scoliosis in MMC patients to be 53% (95% CI 0.42-0.64). Slightly more females (56%) are affected with both MMC and scoliosis than males. Motor level appears to be a significant predictor of prevalence, but not severity, of scoliosis in MMC patients. Treatment options for these patients include tethered cord release (TCR) and fusion surgeries. Curvature improvement and stabilization after TCR may be limited to patients with milder (< 50°) curves. Meanwhile, more aggressive fusion procedures such as a combined anterior-posterior approach may result in more favorable long-term scoliosis correction, albeit with greater complication rates. Quality of life metrics including ambulatory status and sitting stability are influenced by motor level of the lesion as well as the degree of the scoliosis curvature. CONCLUSIONS Scoliosis is among the most common and challenging comorbidities from which patients with MMC suffer. Although important epidemiological and management trends are evident, larger, prospective studies are needed to discover ways to more accurately counsel and more optimally treat these patients.

Polfuss M, Bandini LG, Sawin KJ. (2017) Obesity Prevention for Individuals with Spina Bifida.

Current Obesity Reports. 2017 Apr 28. doi: 10.1007/s13679-017-0254-y. [Epub ahead of print] Review. PMID: 28455677

 

Abstract

PURPOSE OF REVIEW:

Obesity is a common comorbidity in individuals with spina bifida. Carrying excess weight exacerbates the inherent health challenges associated with spina bifida, impedes the individual's ability to self-manage their condition, and creates further challenges for family members and caregivers. This manuscript provides a narrative review of key issues for understanding and prevention of obesity in persons with spina bifida within the context of the social ecological model.

RECENT FINDINGS:

Specific variables related to obesity and spina bifida include individual factors (i.e., body composition and measurement issues, energy needs, eating patterns, physical activity, and sedentary activity) family factors (i.e., parenting/family, peers), community factors (i.e., culture, built environment, healthcare and healthcare providers, and school), and societal factors (i.e., policy issues). Due to the complex etiology of obesity and its increased prevalence in individuals with spina bifida, it is critical to initiate prevention efforts early with a multifactorial approach for this at-risk population. Increased research is warranted to support these efforts.

Ware AL, Kulesz PA, Juranek J, Cirino PT, Fletcher JM. (2017) Cognitive control and associated neural correlates in adults with spina bifida myelomeningocele. Neuropsychology. 2017 May;31(4):411-423. doi: 10.1037/neu0000350. Epub 2017 Feb 16. PMID: 28206781

 

Abstract

OBJECTIVE:

Accelerated aging can occur in adult survivors of neurodevelopmental disorders, but has been narrowly studied in spina bifida myelomeningocele (SBM). Since discrete aspects of cognitive control and related neural network macrostructure deteriorate in normal aging, the specificity and trajectory of cognition and neuropathology incurred across adulthood in SBM were examined.

METHOD:

Adults (N = 120) with and without SBM completed working memory span and manipulation tasks, and an inhibitory control task. A subset (n = 53) underwent structural MRI. Effects of group, age, and their interaction on performance and select gray matter volumes were examined.

RESULTS:

Adults with SBM had significantly poorer working memory accuracy and overall inhibitory control performance than typical peers. Age negatively predicted inhibitory control. Group × Age significantly interacted on span accuracy; advanced age related to diminished performance in typical adults, but not in adults with SBM. SBM related to disproportionately enlarged cortical and putamen and reduced hippocampus volumes. Group × Age significantly interacted on cortical, but not subcortical gray matter volumes. Dorsolateral prefrontal, hippocampus, and putamen volumes negatively correlated with cognitive performance.

CONCLUSIONS:

Supporting previous literature, current findings elucidated a profile of executive impairment in SBM that was maintained in a parallel maturational trajectory to typical aging. Accelerated aging in cognitive control or subcortical gray matter was not supported in SBM. However, reductions in anterior and posterior cortical regions were exacerbated in older adults with SBM compared with typical peers. Overall results supported persistent anomalous neurodevelopmental maturation across the life span in SBM that related to diminished cognitive control. (PsycINFO Database Record

(c) 2017 APA, all rights reserved).

Chan YY, Sandlin SK, Kurzrock EA. (2017) Urological Outcomes of Myelomeningocele and Lipomeningocele. Current Urological Reports. 2017 May;18(5):35. doi: 10.1007/s11934-017-0684-9. Review.

 

Abstract

PURPOSE OF REVIEW:

Spina bifida is caused by incomplete neural tube closure during the first trimester. This condition may lead to bowel and bladder dysfunction as well as truncal weakness and motor anomalies. Presentations vary between myelomeningoceles and lipomeningoceles and may result in different outcomes. This review seeks to explore our current understanding of the variations in outcomes between individuals with myelomeningocele and lipomeningocele.

RECENT FINDINGS:

Prenatal intervention has become a standard of care for prenatal diagnoses of myelomeningocele and has been shown to reduce shunt placement and improve motor skills. However, urological benefit from early intervention remains to be seen. Early surgical repair, however, may be beneficial for patients with lipomeningocele. Literature on the urological outcomes of patients with myelomeningocele and lipomeningocele is lacking. Further research is needed to better elucidate differences in long-term urological outcomes between these two pathologies.

De la Torre GG, Martin A, Cervantes E, Guil R, Mestre JM. (2017)  Attention lapses in children with spina bifida and hydrocephalus and children with attention-deficit/hyperactivity disorder.  Journal of Clinical and Experimental Neuropsychology. 2017 Aug;39(6):563-573. doi: 10.1080/13803395.2016.1249828.

 

Abstract

Attentional lapses are usually defined as temporary and often brief shifts of attention away from some primary task to unrelated internal information processing. This study addressed the incidence of attention lapses and differences in attentional functioning in 30 children with attention-deficit/hyperactivity disorder (ADHD), 26 healthy children, and 29 children with spina bifida myelomeningocele and hydrocephalus (SBH). Assessments were conducted using computerized tonic and phasic attention tests, the Symbol Digit Modalities Test (SDMT), and the Trail Making Test Form B (TMT-B). The group with SBH differed from normal controls on cognitive measures of attention and executive functions. The ADHD group obtained lower scores than the SBH group and healthy children. ANOVA results showed that there was an effect of shunt revisions and shunt-related infections on neuropsychological performance. Lapses of attention together with reaction time may thus represent important factors for the understanding of cognitive deficits in SBH.

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