Monday, May 11, 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
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.

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.

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.