Neurodevelopmental Implications on Urological Self-management Among People Living With Spina Bifida: A Practical Guide for Urology Providers.

Although folic acid fortification and advances in prenatal repair have reduced Spina Bifida (SB) prevalence and the severity of comorbidities, individuals with SB remain at elevated risk for neurocognitive impairments that studies have shown can negatively impact, among other things, urological self-care. Identifying and addressing these impairments with practical interventions can meaningfully improve long-term outcomes for individuals with SB. We review neurocognitive impairments associated with SB and provide practical solutions to support improvement of long-term urological outcomes.

Team Approach: The Management of Adolescents and Adults with Scoliosis and Spina Bifida.

¼: Globally, the prevalence of myelomeningocele, the most common subtype of spina bifida, is 0.2 to 6.5 per 1,000 live births. In the U.S., adults account for >67% of the overall population with spina bifida. ¼: With an estimated prevalence of up to 50%, scoliosis is one of the most common and severe orthopaedic conditions in patients with myelomeningocele. ¼: The variable effects that scoliosis can have on an individual, the comorbidities associated with progressive scoliosis, and the risks associated with spine surgery call for a strong partnership and care coordination between medical and surgical teams to deliver a patient-centered approach. ¼: A coordinated, structured, planned, and incremental team approach can help individuals achieve the overall goals of functionality and independence, as well as successful transition to adulthood. ¼: Teams should consider a patient's social determinants of health (e.g., poverty or language barriers) and the effect of scoliosis on quality of life before proceeding with spinal deformity correction.

What is the Role of Scoliosis Surgery in Adolescents and Adults with Myelomeningocele? A Systematic Review.

BACKGROUND: Life expectancy of individuals with spina bifida has continued to improve over the past several decades. However, little is known about the longitudinal course of scoliosis in individuals with myelomeningocele (MMC), a spina bifida subtype, across their lifespan. Specifically, it is not known whether management during or after the transition years from adolescence to adulthood is associated with comorbidities in adulthood nor if these individuals benefit from scoliosis treatment later in life. QUESTIONS/PURPOSES: In this systematic review, we asked: (1) Is the risk of secondary impairments (such as bladder or bowel incontinence, decreased ambulation, and skin pressure injuries) higher among adolescents and adults with MMC and scoliosis than among those with MMC without scoliosis? (2) Is there evidence that surgical management of scoliosis is associated with improved functional outcomes in adolescents and adults with MMC? (3) Is surgical management of scoliosis associated with improved quality of life in adolescents and adults with MMC? METHODS: We performed a systematic review of articles in Medline and Embase from 2000 until February 5, 2021. Search terms such as "spinal dysraphism," "spina bifida," "meningomyelocele," and "scoliosis" were applied in diverse combinations. A total of 1429 publications were identified, and 13 were eligible for inclusion. We included original studies reporting on scoliosis among individuals older than 15 years with MMC. When available, we extracted the prevalence of MMC and scoliosis, studied population age, percentage of patients experiencing complications, functional outcomes, and overall physical function. We excluded non-English articles and those with fewer than 10 individuals with scoliosis and MMC. We used the Preferred Reporting Items for Systematic Reviews and Meta-analyses, and registered the review before data collection (PROSPERO: CRD42021236357). We conducted a quality assessment using the Methodologic Index for Nonrandomized Studies (MINORS) tool. In 13 included studies, there were 556 individuals with MMC and scoliosis. Most were retrospective case series, although a minority were retrospective/comparative studies. The mean MINORS score was 12.3 ± 1.65 (a MINORS score over 12 generally is considered good reporting quality, scores below 12 are considered at high risk of bias). RESULTS: In general, studies found that individuals with MMC and scoliosis were more likely to have secondary impairments such as bladder/bowel incontinence, decreased ambulation, and pressure injuries than were patients with MMC without scoliosis. These secondary impairments were associated with hydrocephalus and high-level MMC lesions. However, when one study evaluated mortality, the results showed that although most deceased individuals who had spina bifida had scoliosis, no association was found between the age of death and scoliosis. Among the studies evaluating functional outcomes, none supported strong functional improvement in individuals with MMC after surgery for scoliosis. No correlation between the Cobb angle and sitting balance was noted; however, the degree of pelvic obliquity and the level of motor dysfunction showed a strong correlation with scoliosis severity. There was no change in sitting pressure distributions after spinal surgery. The lesion level and scoliosis degree independently contributed to the degree of lung function impairment. Although studies reported success in correcting coronal deformity and stopping curve progression, they found no clear benefit of surgery on health-related quality of life and long-term outcomes. These studies demonstrated that the level of neurologic function, severity of hydrocephalus, and brainstem dysfunction are greater determinants of quality of life than spinal deformity. CONCLUSION: This systematic review found that adolescents and adults with MMC and scoliosis are more likely to have secondary impairments than their peers with MMC only. The best-available evidence does not support strong functional improvement or health-related quality of life enhancement after scoliosis surgery in adolescents and adults with MMC. The level of neurologic dysfunction, hydrocephalus, and brainstem dysfunction are greater determinants of quality of life. Future prospective studies should be designed to answer which individuals with MMC and scoliosis would benefit from spinal surgery. Our findings suggest that the very modest apparent benefits of surgery should cause surgeons to approach surgical recommendations in this patient population with great caution, and surgeons should counsel patients and their families that the risk of complications is high and the benefits may be small. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Pediatric osteoarticular infection: trend in surgically treated patients and association of methicillin-resistant Staphylococcus aureus with requirement of secondary procedures.

Acute pediatric osteoarticular infection demonstrates variability in both presentation and response to treatment. Many respond to antibiotics ± single operation, while some require multiple surgeries. Currently, it is difficult to predict who may require additional procedures. Infections due to methicillin-resistant Staphylococcus aureus (MRSA) have been associated with more complications. The purpose of this study is to determine MRSA trends and degree of association with the occurrence of multiple procedures. We performed a retrospective analysis of surgically treated pediatric (1 month-18 years) patients for acute osteomyelitis and septic arthritis at a tertiary children's hospital from 2003-2017. The cohort was divided into single-procedure and multiple-procedure groups. A total of 753 patients were studied with a mean age of 7.05 years (2.4 months-17.9 years). We identified 645/753 (85.6%) patients who were treated with a single-procedure and 108/753 (14.4%) patients who required multiple- procedures. The lower extremity (hip, knee, tibia, and femur) was most commonly involved. The epidemiologic trend runs almost parallel between two groups with a peak in 2009. The odds ratio for multiple-procedures was 2.0 [95% confidence interval (CI), 1.2-3.1; P = 0.002] with dual infection (osteomyelitis + septic arthritis), 2.6 (95% CI, 1.6-4.4; P = 0.001) with high-risk conditions and 4.6 (95% CI, 3.0-7.1; P < 0.001) if MRSA was present. MRSA significantly predicts the requirement of additional operative procedures for the treatment of osteoarticular infections in children. Besides clinical deterioration and other markers, the presence of MRSA can be a considerable indicator for a planned secondary-procedure. Level III retrospective cohort study.

Neurosurgical training and global health education: systematic review of challenges and benefits of in-country programs in the care of neural tube defects.

OBJECTIVE: The recognition that neurosurgeons harbor great potential to advocate for the care of individuals with neural tube defects (NTDs) globally has sounded as a clear call to action; however, neurosurgical care and training in low- and middle-income countries (LMICs) present unique challenges that must be considered. The objective of this study was to systematically review publications that describe the challenges and benefits of participating in neurosurgery-related training programs in LMICs in the service of individuals with NTDs. METHODS: Using MEDLINE (PubMed), the authors conducted a systematic review of English- and Spanish-language articles published from 1974 to 2019 that describe the experiences of in-country neurosurgery-related training programs in LMICs. The inclusion criteria were as follows-1) population/exposure: US residents, US neurosurgeons, and local in-country medical staff participating in neurosurgical training programs aimed at improving healthcare for individuals with NTDs; 2) comparison: qualitative studies; and 3) outcome: description of the challenges and benefits of neurosurgical training programs. Articles meeting these criteria were assessed within a global health education conceptual framework. RESULTS: Nine articles met the inclusion criteria, with the majority of the in-country neurosurgical training programs being seen in subregions of Africa (8/9 [89%]) and one in South/Central America. US-based residents and neurosurgeons who participated in global health neurosurgical training had increased exposure to rare diseases not common in the US, were given the opportunity to work with a collaborative team to educate local healthcare professionals, and had increased exposure to neurosurgical procedures involved in treating NTDs. US neurosurgeons agreed that participating in international training improved their own clinical practices but also recognized that identifying international partners, travel expenses, and interference with their current practice are major barriers to participating in global health education. In contrast, the local medical personnel learned surgical techniques from visiting neurosurgeons, had increased exposure to intraoperative decision-making, and were given guidance to improve postoperative care. The most significant challenges identified were difficulties in local long-term retention of trained fellows and staff, deficient infrastructure, and lower compensation offered for pediatric neurosurgery in comparison to adult care. CONCLUSIONS: The challenges and benefits of international neurosurgical training programs need to be considered to effectively promote the development of neurosurgical care for individuals with NTDs in LMICs. In this global health paradigm, future work needs to investigate further the in-country professionals' perspective, as well as the related outcomes.

Language and Latino immigrants living with spina bifida: Social determinants of health - the missing dimension in quality of life research.

PURPOSE: Aware of the higher birth prevalence of spina bifida (SB) among Hispanics/Latinos, we aimed to appraise the literature as it relates to cultural context through a review of quality of life (QOL) studies conducted among individuals with SB in order to improve care among immigrant families. METHODS: A systematic review was conducted consistent with the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines. The inclusion criteria were: (1) children and adolescents (5-21 years of age) with SB and/or myelomeningocele; (2) quantitative studies; (3) health-related QOL outcome measured by validated instrument determinants; and (4) US-based studies. Articles meeting inclusion criteria were assessed using the focused conceptual framework informing the study (i.e., social determinants of health). RESULTS: Eighteen studies met inclusion criteria, with eight different QOL instruments represented. The majority of studies used generic assessments of QOL (72%), two reported the use of both a generic and a SB-specific QOL measure (11%), and three (17%) documented QOL utilizing a SB-specific validated instrument. Only seven (39%) of the studies stated that they included Hispanics/Latinos and only six (33%) reported including Spanish-speaking individuals. CONCLUSIONS: QOL in individuals with SB is mediated by a wide-range of interrelated factors. In order to better serve this vulnerable population as they transition across the lifespan, multilingual condition-specific QOL measures need to be further developed and implemented among Hispanic/Latino individuals with SB, especially those who are recent immigrants.

Towards Guideline-Based Management of Tethered Cord Syndrome in Spina Bifida: A Global Health Paradigm Shift in the Era of Prenatal Surgery.

An estimated 60% of the world's population lives in Asia, where the incidence of neural tube defects is high. Aware that tethered cord syndrome (TCS) is an important comorbidity, the purpose of this systematic review was to explore the treatment of TCS among individuals living with spina bifida (SB) in Asia. MEDLINE and Embase databases were searched for relevant studies published from January 2000 to June 2018. Search terms such as 'spinal dysraphism,' 'spinabifida,' 'diastematomyelia,' 'lipomeningocele,' 'lypomyelomeningocele,' 'meningomyelocele,' and 'tethered cord syndrome' were used in diverse combinations. Of the 1,290 articles that were identified in accordance with PRISMA (Preferred Items for Systematic Reviews and Meta-Analyses) guidelines, 15 Asia-based studies met the inclusion criteria. Significant differences in the diagnostic criteria and management of TCS were documented. As the surgical techniques for prenatal closure of the spinal defect continue to evolve, their adoption internationally is likely to continue. In this setting, a clear and evidence-based approach to the definition and management of TCS is essential. The recent publication by the Spina Bifida Association of America of their updated care guidelines may serve as a tool used to promote a systematized approach to diagnosing and treating TCS among individuals with SB in the region, as well as globally.

Two cases of combined patellar tendon avulsion from the tibia and patella.

Avulsion fractures of the inferior pole of the patella and proximal tibial apophysis are independently rare injuries. They occur in children due to the relative weakness of the apophyseal cartilage compared to the ligaments and tendons. The combination of these two fractures, is exceedingly rare, with only a few previously described cases in the literature. Due to the infrequent presentation of this injury, careful examination and consideration of advanced imaging is important for diagnosis and preoperative planning. Here we present two cases of combined sleeve fractures of the inferior pole of the patella and tibial apophysis, with discussion of the pathophysiology, classification, identification and management of the injury.