Quality of Life and Bladder Symptoms in Adolescents and Young Adults With Spina Bifida Who Catheterize via Urethra vs Catheterizable Channel.

PURPOSE: We sought to assess associations between health-related quality of life (QOL), bladder-related QOL, bladder symptoms, and bladder catheterization route among adolescents and young adults with spina bifida. MATERIALS AND METHODS: Clinical questionnaires administered to individuals ≥ 12 years old requiring catheterization between June 2019 to March 2020 in a spina bifida center were retrospectively analyzed. Questionnaires were completed in English or Spanish independently or with caregiver assistance. Medical records were reviewed for demographic and clinical characteristics. Primary exposure was catheterization route (urethra or channel). Primary outcome was health-related QOL, measured by Patient-Reported Outcomes Measurement Information System Pediatric Global Health 7 (PGH-7). Secondary outcomes were bladder-related QOL and bladder symptoms, measured by Neurogenic Bladder Symptom Score (NBSS). Nested, multivariable linear regression models assessed associations between catheterization route and questionnaire scores. RESULTS: Of 162 patients requiring catheterization, 146 completed both the PGH-7 and NBSS and were included. Seventy-three percent were catheterized via urethra and 27% via channel. Median age was 17.5 years (range 12-31), 58% of patients were female, and 80% had myelomeningocele. Urinary incontinence was more common among those who catheterized via urethra (60%) compared to channel (33%). On adjusted analyses, catheterization route was not significantly associated with PGH-7 or NBSS bladder-related QOL scores. More bladder symptoms were associated with worse bladder-related QOL. Patients who catheterized via channel had fewer bladder symptoms than those who catheterized via urethra. CONCLUSIONS: Catheterization route was not significantly associated with QOL. Though catheterization via channel was associated with fewer bladder symptoms, only degree of current bladder symptoms was significantly associated with bladder-related QOL.

Association Between Quality of Life and Neurogenic Bowel Symptoms by Bowel Management Program in Spina Bifida.

OBJECTIVE: To compare differences in bowel-specific quality of life (QOL), overall qQOL, and neurogenic bowel dysfunction (NBD) severity by bowel management program in patients with spina bifida (SB). METHODS: We performed a retrospective cross-sectional study of patients ≥12 years old at our multidisciplinary SB center who completed both a modified Peristeen NBD questionnaire (assessing bowel symptom severity and bowel-specific QOL) and the Patient-Reported Outcomes Measurement Information System Pediatric Global Health questionnaire (assessing overall QOL). Nested, multivariable models were fit for associations between outcomes and bowel management program (enemas, conservative management, and none). RESULTS: A total of 173 patients, 56.1% female and 64.6% with myelomeningocele, were included in our analysis. Median age was 18.2 years old. Patients reported using enemas (n = 42), conservative management (n = 63), and no bowel program (n = 68). When adjusting for covariates, there was no significant association between bowel-specific QOL nor overall QOL across bowel management programs. However, the use of conservative management compared to enemas was associated with worse bowel symptoms severity (adjusted beta=2.58, 95%CI=[0.09,5.06]). Additionally, greater bowel symptom severity was significantly associated with lower overall QOL (adjusted beta=-0.33, 95%CI=[-0.57,-0.10]). CONCLUSION: NBD symptom severity in SB is more strongly associated with QOL than the individual bowel program being utilized. Our findings suggest that different degrees of NBD require different invasiveness of bowel programs, but it is the outcome of the bowel management program and not the specific program itself that is most associated with QOL.

Resident muscle stem cell myogenic characteristics in postnatal muscle growth impairments in children with cerebral palsy.

Children with cerebral palsy (CP), a perinatal brain alteration, have impaired postnatal muscle growth, with some muscles developing contractures. Functionally, children are either able to walk or primarily use wheelchairs. Satellite cells are muscle stem cells (MuSCs) required for postnatal development and source of myonuclei. Only MuSC abundance has been previously reported in contractured muscles, with myogenic characteristics assessed only in vitro. We investigated whether MuSC myogenic, myonuclear, and myofiber characteristics in situ differ between contractured and noncontractured muscles, across functional levels, and compared with typically developing (TD) children with musculoskeletal injury. Open muscle biopsies were obtained from 36 children (30 CP, 6 TD) during surgery; contracture correction for adductors or gastrocnemius, or from vastus lateralis [bony surgery in CP, anterior cruciate ligament (ACL) repair in TD]. Muscle cross sections were immunohistochemically labeled for MuSC abundance, activation, proliferation, nuclei, myofiber borders, type-1 fibers, and collagen content in serial sections. Although MuSC abundance was greater in contractured muscles, primarily in type-1 fibers, their myogenic characteristics (activation, proliferation) were lower compared with noncontractured muscles. Overall, MuSC abundance, activation, and proliferation appear to be associated with collagen content. Myonuclear number was similar between all muscles, but only in contractured muscles were there associations between myonuclear number, MuSC abundance, and fiber cross-sectional area. Puzzlingly, MuSC characteristics were similar between ambulatory and nonambulatory children. Noncontractured muscles in children with CP had a lower MuSC abundance compared with TD-ACL injured children, but similar myogenic characteristics. Contractured muscles may have an intrinsic deficiency in developmental progression for postnatal MuSC pool establishment, needed for lifelong efficient growth and repair.

The Association of Health Literacy with Health-Related Quality of Life in Youth and Young Adults with Spina Bifida: A Cross-Sectional Study.

OBJECTIVE: The objective of the study was to determine if health literacy is associated with health-related quality of life (HRQOL) in adolescents and young adults (AYAs) with spina bifida. STUDY DESIGN: Between June 2019 and March 2020, the Patient-Reported Outcome Measurement Information System Pediatric Global Health-7 (PGH-7), a measure of HRQOL, and the Brief Health Literacy Screening Tool (BRIEF) were administered to patients ≥12 years old with a diagnosis of spina bifida seen in our multidisciplinary spina bifida center. Questionnaires were completed at scheduled clinic visits. The primary outcome was the PGH-7 normalized T-score. The primary exposure was the BRIEF score. Demographic and clinical characteristics were obtained from the medical record. Nested, multivariable linear regression models assessed the association between health literacy and the PGH-7 score. RESULTS: Of 232 eligible patients who presented to clinic, 226 (97.4%) met inclusion criteria for this study. The median age was 17.0 years (range: 12-31). Most individuals were female (54.0%) and had myelomeningocele (61.5%). Inadequate, marginal, and adequate health literacy levels were reported by 35.0%, 28.3%, and 36.7% of individuals. In univariable analysis, higher health literacy levels were associated with higher PGH-7 scores. In nested, sequentially adjusted multivariable linear regression models, a higher health literacy level was associated with a stepwise increase in the PGH-7 score. In the fully adjusted model, adequate health literacy and marginal health literacy, compared with inadequate health literacy, were associated with increases in a PGH-7 score of 3.3 (95% CI: 0.2-6.3) and 1.1 (95% CI: -2.0 to 4.2), respectively. CONCLUSIONS: Health literacy was associated with HRQOL after adjusting for demographic and clinical factors. Strategies incorporating health literacy are needed to improve HRQOL in AYAs with spina bifida.

Assessment of Health Literacy and Self-reported Readiness for Transition to Adult Care Among Adolescents and Young Adults With Spina Bifida.

Importance: Health literacy has been shown to play an important role in transitions of care in adult populations, with low health literacy associated with adverse health outcomes. The role of health literacy in the transition from pediatric to adult care has been less well studied. Among adolescents and young adults with spina bifida, high rates of unsuccessful transition have been shown, but how patient health literacy affects transition readiness remains unknown. Objective: To determine whether health literacy is associated with transition readiness in adolescents and young adults with spina bifida. Design, Setting, and Participants: This cross-sectional study involved collection of patient-reported questionnaires between June 2019 and March 2020 at a multidisciplinary spina bifida center at a single, free-standing children's hospital. Patient demographic and clinical characteristics were obtained from medical record review. Patients were aged 12 years or older with a diagnosis of spina bifida (myelomeningocele and nonmyelomeningocele) whose primary language was English or Spanish. Data analysis was performed from October 2020 to March 2021. Exposures: Health literacy as assessed by the Brief Health Literacy Screening Tool. Main Outcomes and Measures: The primary outcome was total Transition Readiness Assessment Questionnaire (TRAQ) score, normalized into units of SD. Nested, multivariable linear regression models assessed the association between health literacy and TRAQ scores. Results: The TRAQ and Brief Health Literacy Screening Tool were completed by 200 individuals (median [range] age, 17.0 [12.0-31.0] years; 104 female participants [52.0%]). Most of the patients were younger than 18 years (110 participants [55.0%]) and White (136 participants [68.0%]) and had myelomeningocele (125 participants [62.5%]). The mean (SD) TRAQ score was 3.3 (1.1). Sixty-six participants (33.0%) reported inadequate health literacy, 60 participants (30.0%) reported marginal health literacy, and 74 participants (37.0%) reported adequate health literacy. In univariable analysis, health literacy, age, type of spina bifida, level of education, self-administration vs completion of the questionnaires with assistance, ambulatory status, and urinary incontinence were associated with total TRAQ score. In all nested, sequentially adjusted, multivariable models, higher health literacy remained a significant, stepwise, independent variable associated with higher TRAQ score. In the fully adjusted model, having adequate compared with inadequate health literacy was associated with an increase in normalized TRAQ score of 0.49 SD (95% CI, 0.19-0.79). Conclusions and Relevance: Patient-reported transition readiness is associated with health literacy, even after adjustment for education level and other demographic and clinical factors. Developing and implementing health literacy-sensitive care programs during the transition process may improve patient transition readiness.

Myelomeningocele: a new functional classification.

PURPOSE: In myelomeningocele, several classifications have been used. The present manuscript proposes a new functional classification to better assess the prognosis and management of these patients. METHODS: The manual muscle test is what defines the actual group in which the patient should be included. Furthermore, this new classification brings information about the bracing and external supports recommended to each functional level. We also recommend that the patient's Functional Mobility Scale should always be mentioned together with their functional level. RESULTS: The four levels in this classification are MMFC1, MMFC2, MMFC3 and MMFC4. The MMFC1 group includes patients with significant muscle weakness. They need to use high braces crossing the hip joint with a walker to achieve some ambulation. The MMFC2 group includes patients who have functional hip flexors, knee extensors and knee flexors. However, the hip abductors are quite weak. These patients usually need to use a walker - or crutches - and Ankle-Foot Orthosis (AFOs). The MMFC3 group includes patients with functional hip flexors, knee extensors, knee flexors and hip abductors. However, the ankle plantar flexion function is absent. Most of them are able to walk independently, only using AFOs without any external support. The MMFC4 group includes patients who have preserved function in the entire lower limb musculature. These patients don't need any assistive devices to achieve an adequate ambulation pattern. CONCLUSIONS: We hope that this new classification is a system that is simple to understand, serves as a gait prognosis guide and facilitates communication among healthcare professionals. LEVEL OF EVIDENCE: V.

Cast Saw Burn Prevention: An Evidence-Based Review.

Cast saw burns are an avoidable complication of cast removal and cast splitting. These iatrogenic injuries often lead to unacceptable clinical sequalae with significant financial and legal consequences. Therefore, a considerable body of research has been directed toward cast saw burn prevention. This review of currently published data provides clinicians with a summary of the literature to guide practice based on the best available evidence, with the goal of preventing iatrogenic cast saw burns. The PubMed database was queried for articles published from 1980 until present with the following key words: cast saw burns, cast saw blades, cast saws, orthopaedic education or surgical simulation. Relevant articles were reviewed and summarized. The prevention of cast saw burns involves awareness of clinical risk factors, maintenance of equipment, use of the proper technique, and the education of novice providers. By implementing evidence-based methods, orthopaedic surgeons and associated healthcare providers can aim to eliminate these preventable complications from their practice.

IS (Idiopathic Scoliosis) etiology: Multifactorial genetic research continues. A systematic review 1950 to 2017.

OBJECTIVE: IS (idiopathic scoliosis) is a common spinal condition occurring in otherwise completely healthy adolescents. The root cause of IS remains unclear. This systematic review will focus on an update of genetic factors and IS etiology. Though it is generally accepted that the condition is not due to a single gene effect, etiology studies continue looking for a root cause including genetic variants. Though susceptibility from multiple genetic components is plausible based on known family history data, the literature remains unclear regarding multifactorial genetic influences. The objective of this study was to critically evaluate the evidence behind genetic causes (not single gene) of IS through a systematic review and strength-of-study analysis of existing genetic and genome-wide association studies (GWAS). We used the protocol of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). METHODS: PubMed was searched for the terms IS, scoliotic, spinal curve, genetic, gene, etiology, polymorphisms. Articles were assessed for risk-of-bias. Level-of-evidence grading was completed via Oxford Centre for Evidence-Based Medicine criteria. The assessment scores factor strength of a study in determining a positive or negative association to a gene etiology. RESULTS: After screening of 36 eligible papers, 8 relevant studies met inclusion criteria at this time, 3 were in favor of a genetic factor for IS, whereas 5 studies were against it. CONCLUSION: Based on the literature analyzed, there is moderate evidence with a low risk-of-bias that does not clarify a genetic cause of IS. The 2 studies in favor of a genetic etiology were completed in homogeneous populations, limiting their generalizability. Relying on a genetic etiology alone for IS may over simplify its multifactorial nature and limit appreciation of other influences.

What Should I Wear to Clinic? A National Survey of Pediatric Orthopaedic Patients and Parents.

BACKGROUND: A number of factors have been shown to affect how surgeons are subjectively viewed, including their appearance in clinic. Patient preference for pediatric orthopaedic surgeon attire has not previously been studied, nor has its influence on initial perception of the surgeon. METHODS: At 5 pediatric regional sites across the United States, parents and patients presenting to the pediatric orthopaedic clinic were given anonymous surveys showing 8 photos of surgeons in different clinical dress including in business or scrub dress, each with or without white coat (WC). Subjects reported their demographics, single preferred photo, rated characteristics of the surgeons in each photo on a 5-point Likert scale, and answered specific questions on scrubs and WCs. The first consecutively completed 100 parent and 100 patient surveys were included from each site. RESULTS: One thousand surveys were collected from patients (500) and parents (500). The majority felt a surgeon's clinical dress would not affect how they are cared for (83%), and that it was ok to wear scrubs in clinic (90%). Overall, WC was preferred to those without no matter the age, but there was no difference between scrub and business choices. Of those stating a preference, woman in business and WC (24%) and woman in scrubs and WC (21%) were most selected, with the only geographic difference being the midwest's preference for man in business and WC. Females were more likely to prefer women photographs (P<0.0001). CONCLUSIONS: In general, pediatric orthopaedic patients and parents do not have a strong specific preference toward what their surgeon wears to clinic, including whether or not we are dressed in scrubs, but some initial biases exist. When asked to choose, the traditional WC worn over any attire is preferred, and female patients and parents uncover a preference for a surgeon of their own sex. LEVEL OF EVIDENCE: Level III.

Timing of Pavlik Harness Initiation: Can We Wait?

BACKGROUND: Developmental dysplasia of the hip is effectively treated with a Pavlik harness (PH) within the first 6 months of life. Over 80% of unstable hips in the newborn period will naturally stabilize by 2 months of age. If there is no difference in the effectiveness of initiating PH treatment at 1 week compared with 4 weeks of age, waiting may allow the hips to naturally stabilize and avoid treatment. The purpose of this study is to evaluate whether the timing of PH implementation influences its effectiveness in the treatment of developmental dysplasia of the hip. METHODS: A retrospective review was conducted between 2004 and 2010. Patients were included if PH therapy was prescribed for hip instability or dislocation at or before 6 months of age. PH failure was defined as requiring any operative procedure for definitive management. Groups were divided based on the age at which the PH was initiated-group1=<30 days, group 2=30 to 60 days, group 3=>60 days. RESULTS: A total of 176 children were included with 38 (21.6%) failing PH treatment. The mean age at PH initiation was 1.3 months (SD=1.3) in the successfully treated children and 1.4 months (SD=1.2) in the failures (P=0.77). There was no difference in the failure rates by age with group 1=19.1% (18/94), group 2=22.5% (9/40), and group 3=26.2% (11/42) (P=0.87). There was no statistical difference with respect to sex or breech positioning in the success or failure groups; however, there was a higher percentage of bilateral involvement in the failure group (P=0.04). CONCLUSIONS: Patients who had PH initiation before 30 days of age were no more or less likely to fail than when PH was initiated after 30 days of age. Parents can be counseled that waiting until after 30 days of age is appropriate before PH implementation. By avoiding swaddling during this period, the hips may stabilize without treatment and allow for more parental-infant bonding before implementation of PH. LEVEL OF EVIDENCE: Level III-therapeutic, case control study.

Pavlik Harness initiation on Barlow positive hips: Can we wait?

We investigated if infants with a Barlow positive hip(s) have natural hip stabilization and can thus avoid Pavlik Harness (PH) treatment. We conducted a chart review for infants who presented within two weeks of life, had a Barlow positive hip, and were deferred treatment. Of the thirty infants, eighteen were treated with PH at 4-6 weeks or 12 weeks due to persistent dysplasia. Twelve infants avoided PH entirely. There were zero cases of PH failure. Parents can be counseled that deferring treatment until at least 4-6 weeks of age might avoid treatment altogether without an increased risk of harness failure.

Late Presentation of Developmental Hip Dislocation: A Case Report.

CASE: We describe a patient who was diagnosed with developmental hip dislocation at 21 months of age despite having had normal ultrasonography findings at 5 weeks of age. CONCLUSION: This case report provides evidence that late developmental hip dislocation can occur despite normal clinical and sonographic findings early in life, and that it is difficult to know the cause of developmental hip dislocation when it presents late.

Evaluation of angle trunk rotation measurements to improve quality and safety in the management of adolescent idiopathic scoliosis.

PURPOSE: The evaluation, management and follow-up of adolescent idiopathic scoliosis (AIS) occur frequently within a pediatric orthopedic surgery practice. Curve status can be assessed with Scoliometer measurements of angle trunk rotation (ATR), which are reliable and reproducible to within 3°. This study assessed the longitudinal efficacy, safety and cost savings of integrating ATR measurements to monitor curve status and progression in AIS, and suggests a quality-based management strategy. METHODS: A retrospective review of medical records between 2004 and 2014 included patients with AIS between 10-17 years, excluding those with Cobb angle >52° at presentation. Two cohorts were analyzed based on presentation prior to menarche (PRE) or after menarche (POST). The PRE groups was further classified based on whether the curve was Stable or Unstable. The cost of a single PA thoracolumbar radiograph was defined based on the 2015 CMS fee schedule ($36.27). Safety was defined based on the effective radiation dose avoided (0.14 millisieverts/radiograph). RESULTS: A total of 59 children were included with 45 in PRE and 14 in the POST cohort. The use of ATR measurements provided a cost benefit in both the PRE Stable and Unstable cohorts, by avoiding radiographs with an average savings of $161.76 and $147.50 respectively. Similarly in POST, there was an average cost savings of $105.18 per patient. The safety benefit of using ATR measurements included avoiding an average of 0.62, 0.56 and 0.4 millisieverts of radiation in the PRE Stable, PRE Unstable and POST groups respectively. CONCLUSIONS: An evaluation strategy with ATR measurements provides for a reliable, cost-effective and safety advantage in the monitoring of curve progression in both skeletally mature and immature patients with AIS. These findings suggest that stable ATR measurements are a safe and cost effective alternative to serial radiographs in the clinical monitoring of AIS. Recent evidence from 25 years of scoliosis treatment in Denmark noted a cancer rate 17 times that of an age-matched population. Thus, reducing radiation exposure during scoliosis monitoring using ATR measurements has important clinical significance for cancer risk reduction.