Bladder and bowel function effects on emotional functioning in youth with spinal cord injury: a serial multiple mediator analysis.

STUDY DESIGN: Preliminary explanatory or mechanistic cross-sectional study. OBJECTIVES: This preliminary cross-sectional study investigates the hypothesized serial mediating effects of bladder/bowel worry, social worry, and social participation in the relationship between bladder function or bowel function and emotional functioning in youth with spinal cord injury (SCI) from their perspective. METHODS: The Bladder Function, Bowel Function, Worry Bladder Bowel, Worry Social, and Social Participation Scales from the PedsQL™ Spinal Cord Injury Module and the Emotional Functioning Scale from the PedsQL™ 4.0 Generic Core Scales Short Form SF15 were completed by 127 youth with SCI ages 8-24. Serial multiple mediator model analyses were conducted to test the hypothesized sequential mediating effects of bladder/bowel worry, social worry, and social participation as intervening variables separately for the cross-sectional association between bladder function or bowel function and emotional functioning. RESULTS: The separate cross-sectional negative association of bladder function and bowel function with emotional functioning were serially mediated by bladder/bowel worry, social worry and social participation, accounting for 28% and 31%, respectively, of the variance in youth-reported emotional functioning (p < 0.001), representing large effect sizes. CONCLUSIONS: In this preliminary study, bladder/bowel worry, social worry, and social participation explain in part the cross-sectional negative association of bladder function and bowel function with emotional functioning in youth with SCI from the youth perspective. Identifying the hypothesized associations of bladder function and bowel function, bladder/bowel worry, social worry, and social participation with emotional functioning may help inform future clinical research and practice for youth with SCI.

A Pilot Feasibility Trial of an Upper Extremity Assistive System.

Objective: To develop and clinically evaluate a customizable active upper extremity (UE) assistive system with integrated functional electrical stimulation (FES) that improves function and independence of individuals during activities of daily living (ADLs). Design: Single-arm, prospective, open-label cohort feasibility trial. Setting: An academic research institution. Participants: Subjects were 5 adults with a medical history of stroke resulting in distal UE impairment (N=5). The subjects volunteered from recruitment materials that detailed information about the study. Interventions: A novel, wearable, lightweight, low-profile, and patient-tailored UE assistive system. It comprises a splint component and FES unit that may each be controlled by electromyography (EMG) signals, inertial measurement units (IMUs), manual control source (joystick), and/or voice control. Main Outcome Measures: Several occupational therapy outcome measures were used, including the Canadian Occupational Performance Measure (COPM), Action Research Arm Test (ARAT), The Box and Blocks Test (BBT), the ABILHAND-Manual Ability Measure, and Patient Reported Outcomes Measurement Information System (PROMIS) UE Short Form. Results: All participants learned to use our UE assistive system to perform ADLs and were able to use it independently at home. Most participants experienced a clinically meaningful improvement in both performance and satisfaction for the majority of their COPM goals while using the system. All participants experienced improvement in hand grip and release as shown by their baseline and post assessment scores for hand function (BBT, ARAT) and patient-reported outcomes (ABILHAND, PROMIS). Conclusions: The clinical outcomes suggest that our UE assistive system improves functional performance in patients with UE impairment, allowing them to engage more actively in ADLs. Further innovation including elbow and shoulder components will allow users to have more degrees of freedom during tasks.

Assessment of the relationship between Brachial Plexus Profile activity short form scores and modified Mallet scores.

INTRODUCTION: This study aims to assess the relationship between the modified Mallet classification and the Brachial Plexus Profile activity short form (BP-PRO activity SF). The therapist or surgeon classifies upper extremity movement for the modified Mallet classification, while the BP-PRO assesses parents' perceptions of difficulty performing activities. PURPOSE: To provide a deeper understanding of the relationship of functional and perceived outcome measurements. STUDY DESIGN: Prospective, correlational design. METHODS: Eighty children with brachial plexus birth injuries were evaluated using the modified Mallet classification, while parents simultaneously answered the BP-PRO activity SF questions. All patients had undergone one of three surgical interventions to improve shoulder function. The relationship between the two measures, patient injury levels, and surgical histories were assessed. RESULTS: The average modified Mallet scores and BP-PRO activity SF scores weakly correlated (r = 0.312, P = .005) and both measures differentiated between C5-6 and C5-7 injury levels (P = .03 and P = .02, respectively). Conversely, the modified Mallet scores could differentiate between the three surgical groups (F = 8.2, P < .001), while the BP-PRO activity SF could not (P = .54). CONCLUSION: The results suggest that these tools measure different aspects of patient outcomes. The Mallet classification may be more focused on shoulder motion than the BP-PRO activity SF. Additional questions that specifically require shoulder function could be incorporated into the BP-PRO activity SF to improve understanding of patient/parent perceptions of shoulder function for children with brachial plexus injuries. Clinicians should be aware of the strengths, weaknesses, and limitations of each outcome assessment tool for appropriate use and interpretation of results.

Development of the Pediatric Quality of Life Inventory™ Spinal Cord Injury (PedsQL™ SCI) module: qualitative methods.

STUDY DESIGN: It is a qualitative study. OBJECTIVES: To develop the items and support content validity for the Pediatric Quality of Life Inventory™ (PedsQL™) Spinal Cord Injury Module for youth and emerging adults with spinal cord injury (SCI). SETTING: Community and children's hospital. METHODS: A comprehensive literature review was conducted in pediatric and SCI-specific health-related quality-of-life (HRQoL) measures to create an item bank. A multidisciplinary panel of experts convened to discuss the conceptual framework of the module, reviewed the item bank, and generated a core set of items through a process of "binning-and-winnowing". International clinicians and researchers participated in iterative rounds of a Modified Delphi survey, until 90% agreement was reached on all items of the core set, resulting in the construction of a preliminary module. Cognitive interviews were conducted on individuals with SCI and their parents to determine the relevance of items and age-appropriate wording of the preliminary module. The research team regularly reviewed transcriptions of the interviews, and incorporated participant feedback to modify the modules. This process was repeated until content saturation was achieved. RESULTS: A total of 43 participants completed the cognitive interviews. Following five iterations of cognitive interviews and modifications based on participant input, the PedsQL™ SCI Module was generated. It comprised 67 items in the 12 domains of Daily Activities, Mobility, Bladder Function, Bowel Function, Muscle Spasms, Pressure Injury, Pain, Orthostatic Hypotension, Autonomic Dysreflexia, Participation, Worry, and Emotions. CONCLUSIONS: The PedsQL™ SCI Module was developed using well-established qualitative methods. Internet-based field testing is underway to finalize its development and validation.

Progressive Neuromuscular Scoliosis Secondary to Spinal Cord Injury in a Young Patient Treated With Nonfusion Anterior Scoliosis Correction.

Ninety-eight percent of skeletally immature patients with spinal cord injury (SCI) suffer from progressive neuromuscular scoliosis (NMS). Operative treatment has typically been limited to posterior spinal fusion (PSF), but a newer technique as described may be less invasive and preserve more function. A PSF of the entire spine to the pelvis is standard of care. However, maintenance of spinal flexibility, motion, and potential growth is desirable. We present a case for proof-of-concept of utilizing a surgical motion-preserving technique to treat progressive NMS in an 11year-old girl with T10 level (AIS B) paraplegia with a progressive 60° NMS of the lumbar spine. She had anterior scoliosis correction (ASC) from T11-L5 without fusion. Over 24 months, the curve growth-modulated to a residual of 12° with continued modulation to 7° at 3-year follow-up (skeletal maturity).

Diffusion Tensor Imaging Assessment of Regional White Matter Changes in the Cervical and Thoracic Spinal Cord in Pediatric Subjects.

There are no studies to date,describing changes in the diffusion tensor imaging (DTI) metrics of the white matter (WM) regions of the entire cervical and thoracic spinal cord (SC) remote from the lesion in pediatric spinal cord injury (SCI) subjects. The purpose of this study was to determine whether DTI at sites cephalad and caudal to a lesion provides measures of cord abnormalities in children with chronic SCI. A retrospective study included 10 typically developing subjects (TD) and 10 subjects with chronic SCI who underwent SC imaging in 2014-2017. Axial diffusion tensor images using an inner field of view DTI sequence were acquired to cover the entire cervical and thoracic SC. Regions of interest were drawn on the SC WM: right and left lateral (motor), ventral (motor), and dorsal (sensory) tracts. To detect differences in DTI metrics between TD and SCI of the cord, a one way analysis of variance with pooled t test was performed. A stepwise regression analysis was performed to assess the correlation between DTI metrics and clinical scores. In motor and sensory tracts, fractional anisotropy (FA) and axial diffusivity (AD) were significantly decreased in the proximal segments of the caudal cord. In motor tracts cephalad to the lesion, FA was significantly decreased whereas AD was significantly increased in the proximal segment; however, AD was decreased in the distal and middle segments. International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) total score was significantly correlated with FA and AD of the motor and sensory tracts cephalad to the lesion. This study demonstrates that FA and AD have the potential to be sensitive biomarkers of the full extent of cord injury and might be useful in detecting remote injuries to the SC and in guiding new treatments.

Considerations and recommendations for selection and utilization of upper extremity clinical outcome assessments in human spinal cord injury trials.

STUDY DESIGN: This is a focused review article. OBJECTIVES: This review presents important features of clinical outcomes assessments (COAs) in human spinal cord injury research. Considerations for COAs by trial phase and International Classification of Functioning, Disability and Health are presented as well as strengths and recommendations for upper extremity COAs for research. Clinical trial tools and designs to address recruitment challenges are identified. METHODS: The methods include a summary of topics discussed during a two-day workshop, conceptual discussion of upper extremity COAs and additional focused literature review. RESULTS: COAs must be appropriate to trial phase and particularly in mid-late-phase trials, should reflect recovery vs. compensation, as well as being clinically meaningful. The impact and extent of upper vs. lower motoneuron disease should be considered, as this may affect how an individual may respond to a given therapeutic. For trials with broad inclusion criteria, the content of COAs should cover all severities and levels of SCI. Specific measures to assess upper extremity function as well as more comprehensive COAs are under development. In addition to appropriate use of COAs, methods to increase recruitment, such as adaptive trial designs and prognostic modeling to prospectively stratify heterogeneous populations into appropriate cohorts should be considered. CONCLUSIONS: With an increasing number of clinical trials focusing on improving upper extremity function, it is essential to consider a range of factors when choosing a COA. SPONSORS: Craig H. Neilsen Foundation, Spinal Cord Outcomes Partnership Endeavor.

Development and Initial Validation of the Pediatric Neuromuscular Recovery Scale.

PURPOSE: The Neuromuscular Recovery Scale (NRS) was developed to assess the capacity of adults' post-spinal cord injury (SCI) to perform functional tasks without compensation. Application of the NRS to children has been challenging. The purpose of this study was to develop and complete the initial validation of a pediatric version of the NRS. METHODS: First, the investigative team developed a draft Pediatric NRS. Next, a Delphi method was used to amend the draft by 12 pediatric experts. Finally, the revised Pediatric NRS was field-tested on a sample of children with SCI (n = 5) and without (n = 7). RESULTS: After the Delphi process and field testing, the Pediatric NRS consists of 13 items scored on a 12-point scale. All items, except 1, achieved 80% agreement by experts. CONCLUSIONS: This is the first step in development and validation of a pediatric SCI scale that evaluates neuromuscular capacity, in the context of pediatric function, without compensation.

Differential item functioning in the Patient Reported Outcomes Measurement Information System Pediatric Short Forms in a sample of children and adolescents with cerebral palsy.

AIM: The present study examined the Patient Reported Outcomes Measurement Information System (PROMIS) Mobility, Fatigue, and Pain Interference Short Forms (SFs) in children and adolescents with cerebral palsy (CP) for the presence of differential item functioning (DIF) relative to the original calibration sample. METHOD: Using the Graded Response Model we compared item parameter estimates generated from a sample of 303 children and adolescents with CP (175 males, 128 females; mean age 15y 5mo) to parameter estimates from the PROMIS calibration sample, which served as the reference group. DIF was assessed in a two-step process using the item response theory-likelihood ratio-differential item functioning detection procedure. RESULTS: Significant DIF was identified for four of eight items in the PROMIS Mobility SF, for two of eight items in the Pain Interference Scale, and for one item out of 10 on the Fatigue Scale. Impact of DIF on total score estimation was notable for Mobility and Pain Interference, but not for Fatigue. INTERPRETATION: Results suggest differences in the responses of adolescents with CP to some items on the PROMIS Mobility and Pain Interference SFs. Cognitive interviews about the PROMIS items with adolescents with varying degrees of mobility limitations would provide better understanding of how they are interpreting and selecting responses to the PROMIS items and thus help guide selection of the most appropriate way to address this issue.

Evaluation of the box and blocks test, stereognosis and item banks of activity and upper extremity function in youths with brachial plexus birth palsy.

BACKGROUND: One of the greatest limitations to measuring outcomes in pediatric orthopaedics is the lack of effective instruments. Computer adaptive testing, which uses large item banks, select only items that are relevant to a child's function based on a previous response and filters items that are too easy or too hard or simply not relevant to the child. In this way, computer adaptive testing provides for a meaningful, efficient, and precise method to evaluate patient-reported outcomes. Banks of items that assess activity and upper extremity (UE) function have been developed for children with cerebral palsy and have enabled computer adaptive tests that showed strong reliability, strong validity, and broader content range when compared with traditional instruments. Because of the void in instruments for children with brachial plexus birth palsy (BPBP) and the importance of having an UE and activity scale, we were interested in how well these items worked in this population. METHODS: Cross-sectional, multicenter study involving 200 children with BPBP was conducted. The box and block test (BBT) and Stereognosis tests were administered and patient reports of UE function and activity were obtained with the cerebral palsy item banks. Differential item functioning (DIF) was examined. Predictive ability of the BBT and stereognosis was evaluated with proportional odds logistic regression model. Spearman correlations coefficients (rs) were calculated to examine correlation between stereognosis and the BBT and between individual stereognosis items and the total stereognosis score. RESULTS: Six of the 86 items showed DIF, indicating that the activity and UE item banks may be useful for computer adaptive tests for children with BPBP. The penny and the button were strongest predictors of impairment level (odds ratio=0.34 to 0.40]. There was a good positive relationship between total stereognosis and BBT scores (rs=0.60). The BBT had a good negative (rs=-0.55) and good positive (rs=0.55) relationship with the clinical category of BPBP and Raimondi classification, respectively. There was a moderate negative (rs=-0.36) and moderate positive (rs=0.47) relationship between total stereognosis and clinical category of BPBP and Raimondi, respectively. Individual stereognosis items had moderate (rs=0.34 to 0.45) to good (rs=0.52 to 0.74) correlation with total stereognosis score. CONCLUSIONS: In children with BPBP, there was little to no DIF with item banks of activity and UE functioning. The BBT is a good predictor of degree of impairment. Stereognosis with 2 items may provide comparable information as 12 items. LEVELS OF EVIDENCE: Level II.

Coping and participation in youth with spinal cord injury.

BACKGROUND: Coping and participation are important adjustment outcomes of youth with spinal cord injury (SCI). Research addressing how these outcomes are related is limited. OBJECTIVE: This cross-sectional study examined relationships between coping and participation in youth with SCI. METHOD: Youth ages 7 to 18 years were recruited from 3 hospitals specializing in rehabilitation of youth with SCI. The Kidcope assessed coping strategies, and the Children's Assessment of Participation and Enjoyment (CAPE) examined participation patterns. Point biserial and Pearson correlations assessed relationships among variables, and hierarchical multiple regression analyses examined whether coping significantly contributed to participation above and beyond significant demographic and injury-related factors. RESULTS: The sample included 294 participants: 45% female, 65% Caucasian, 67% with paraplegia. Mean age was 13.71 years (SD = 3.46), and mean duration of injury was 5.39 years (SD = 4.49). Results indicated that higher levels of social support and lower levels of self-criticism predicted higher participation in informal activities, lower levels of social withdrawal predicted participation in informal activities with a greater diversity of individuals, lower levels of blaming others predicted higher enjoyment of informal activities, and higher levels of cognitive restructuring predicted participation in formal activities with a greater diversity of individuals and in settings further from home. CONCLUSION: Results suggest higher levels of social support and cognitive restructuring and lower levels of self-criticism, social withdrawal, and blaming others predicted favorable participation outcomes. Interventions for youth with SCI that encourage higher levels of positive coping strategies and lower levels of negative and avoidant strategies may promote positive participation outcomes.

Psychosocial outcomes among youth with spinal cord injury and their primary caregivers.

BACKGROUND: Past research has found youth with disabilities to experience poor psychosocial outcomes, but little is known about factors related to psychosocial health among youth with spinal cord injury (SCI). OBJECTIVE: To describe psychosocial health among youth with SCI, examine relationships between psychosocial outcomes and demographic and injury-related factors, and examine relationships between aspects of psychosocial health. Youth activity, participation, quality of life (QOL), coping, anxiety and depression, and caregiver mental health were included. METHODS: Data were collected as part of a prospective study of 420 youth with SCI ages 1-18 and their primary caregivers. Activity data were also presented from a study developing a computerized adaptive testing (CAT) platform with 226 youth with SCI ages 8-21. RESULTS: Although relationships varied by factor, youth outcomes were related to youth age, sex, age at injury/injury duration, and level/extent of injury. Caregiver mental health related to child age and age at injury. Further, relationships were uncovered between aspects of psychosocial health: aspects of youth mental health were related to youth participation and QOL, youth coping was related to youth mental health, participation, and QOL, and caregiver mental health was related to child mental health and QOL. CONCLUSION: Psychosocial outcomes relate to each other and vary by child and injury-related factors and should be understood in a comprehensive, developmental context. Identifying best measures of activity and psychosocial functioning among youth with SCI and understanding factors related to their psychosocial health is critical to improving outcomes for the pediatric-onset SCI population.

Perceptions of self-image and physical appearance: conversations with typically developing youth and youth with idiopathic scoliosis.

PURPOSE: To report how youths, both with and without idiopathic scoliosis (IS), respond to questions about their self-image and perceptions of body shape. An additional purpose is to describe themes that emerged as important to youths with IS to better understand scoliosis from their perspective. METHODS: Descriptive qualitative and quantitative methods were utilized. Subject interviews were conducted, as part of a larger cognitive interviewing study on the Spinal Appearance Questionnaire, using a cross-sectional sample of 76 females between 8 and 16 years of age with IS and who were typically developing (TD), without scoliosis. RESULTS: IS and TD subjects revealed similar findings when asked what makes them look good versus their peers; self-image ratings were also positive. Predominant themes from open-ended responses include physical appearance, feelings, brace wear, and discomfort. CONCLUSION: Self-image and body shape did not differ significantly between groups. The identified themes warrant further exploration as they are significant and important to youth with scoliosis.

Coping in caregivers of youth with spinal cord injury.

This study examined coping among caregivers of youth with spinal cord injuries (SCI). Using a cross-sectional survey study design, 164 caregivers completed a demographics questionnaire and the Brief COPE. Their children, youth with SCI ages 7-18, completed the Kidcope. T-tests were conducted to examine differences in caregiver coping by demographic and injury-related factors. Further, logistic regression models were evaluated to examine predictive relationships between caregiver coping and youth coping. Several demographic and injury-related factors were related to caregiver coping, including caregiver gender, race, and education, as well as youth gender, age at injury, and time since injury. In the logistic regressions, two caregiver coping strategies were related to youth coping: caregiver self-blame coping was related to youth self-criticism, and caregiver behavioral disengagement coping (giving up attempts to cope) was related to youth blaming others coping. The findings suggest that caregiver coping may play a role in the coping of their children, and should be considered when addressing coping among youth with SCI.

Interrater reliability of the international standards for neurological classification of spinal cord injury in youths with chronic spinal cord injury.

OBJECTIVES: To evaluate the interrater reliability of the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) in children with chronic spinal cord injury (SCI), and to define the lower age limit at which the examinations have clinical utility. DESIGN: Repeated measures, multicenter reliability study. SETTING: Two U.S. pediatric specialty hospitals with recognized SCI programs. PARTICIPANTS: Children (N=236) with chronic SCI. INTERVENTIONS: Subjects underwent 4 examinations by 2 raters: sensory tests (pin prick [PP] and light touch [LT]), a motor test, and a test of anal sensation (AS) and anal contraction (AC). MAIN OUTCOME MEASURES: A 2-way general linear model analysis of variance was used for analysis. Intraclass correlation coefficients (ICCs) and 95% confidence intervals were calculated for PP, LT, motor, AS, and AC. RESULTS: No child younger than 6 years completed the examination. When examined as a function of age, interrater reliability for motor, PP, LT, AS, and AC was moderate (ICC=.89) to high (ICC=.99). There was poor reliability for AS (ICC=.49) in subjects with complete injuries but moderate reliability for all other variables. There was moderate to high reliability for classification of type (tetraplegia/paraplegia) and severity (complete/incomplete) of injury across age groups. CONCLUSIONS: The ISNCSCI does not have utility for children younger than 6 years. For children older than 6 years, interrater reliability of PP, LT, and motor examinations is high.

Cognitive testing of the spinal appearance questionnaire with typically developing youth and youth with idiopathic scoliosis.

BACKGROUND: The Spinal Appearance Questionnaire (SAQ) underwent initial psychometric studies, which suggested good reliability and discriminative ability. Although the SAQ is used as a self report of appearance, our center was concerned about its use with youth owing to complex words and vague questions. We conducted this cross-sectional study to evaluate the readability, comprehension, and interpretation of items on the SAQ. METHODS: Cognitive interview methodology of 76 youths (8 to 16 y; average age 13) included 31 with scoliosis and 45 typically developing. Subjects were required to read each SAQ item and think aloud to capture cognitive processes about the items and responses. Interviews were audiotaped and transcribed verbatim. Problems were categorized and frequencies for each category were calculated. RESULTS: There were reading and comprehension problems and problems understanding the illustration with every written and pictorial SAQ item, respectively. The percent of subjects who encountered at least one problem ranged from 16% to 96%. Subjects had difficulty with understanding the intent of every SAQ item and with understanding the meaning of specific words such as "prominence" and "flank." The pictorial illustrations for items 2 and 3 were problematic for 58% and 49% of subjects, respectively. The illustrations of the lungs (item 4) and hips (items 4 and 5) were problematic for 42% and 27% of subjects, respectively. These results were consistent regardless of age or diagnoses. CONCLUSION: This study does not support the use of the SAQ as currently used with youth owing to use of complex medical words, vague questions, difficult illustrations, and various interpretations of the intent of many of the items. LEVEL OF EVIDENCE: Not applicable (not an intervention study).

Description of sensory preservation in children and adolescents with incomplete spinal cord injury.

BACKGROUND/OBJECTIVE: This cross-sectional, multicenter cohort study describes patterns of preserved sensation in persons with American Spinal Injury Association (ASIA) Impairment Scale (AIS) B (sensory incomplete, or SI) and AIS C/D (motor incomplete, or MI). METHODS: A total of 93 subjects with incomplete spinal injuries (58 with tetraplegia and 35 with paraplegia) were included for analysis. Sensation was based on the International Standards for Neurological Classification of SCI (ISNCSCI). RESULTS: In the 44 subjects with AIS B (SI), some light touch (LT) was present in 35% of dermatomes below the neurological level and pin prick (PP) in 8%. In contrast, in the 49 subjects with AIS C/D (MI), LT was present in 77% of dermatomes and PP in 27%. AIS C/D (MI) subjects with tetraplegia had more dermatomes with preserved sensation than those with paraplegia. When reviewing areas at highest risk for pressure sores, only 4 of 22 (19%) of subjects with AIS B (SI)/tetraplegia had any preserved LT or PP sensation in the periscapular region (dermatomes T1-T6). In the buttocks region (S3 and S4-S5), sensation was preserved in fewer than 50% of patients with either tetraplegia or paraplegia. CONCLUSIONS: (1) Sensory sparing below the neurologic injury was found to be surprisingly sparse in patients classified as AIS B (SI) (35% LT and 8% PP). Sparing was considerably better in patients who were AIS C/D (MI) (77% LT and 27% PP). (2) Preserved sensation in the periscapular region was very low in subjects with tetraplegia (19%) and was also low in the buttocks, with fewer than half of those classified as AIS B (SI) with either tetraplegia or paraplegia reporting sensation.

Is the vertebral expandable prosthetic titanium rib a surgical alternative in patients with spina bifida?

BACKGROUND: Nonambulatory children with myelodysplasia are most likely to develop spinal deformity. As the deformity progresses, the overall health of the patient deteriorates. Traditional management of the deformity with fusion results in a short trunk, crankshaft deformity, and spine and lung growth inhibition. One alternative that potentially minimizes these problems is the vertebral expandable prosthetic titanium rib (VEPTR). QUESTIONS/PURPOSES: We therefore asked whether the use of the VEPTR in immature nonambulating children with myelodysplasia with spinal deformity would (1) correct deformity; (2) allow growth; and (3) allow adequate respiratory function. PATIENTS AND METHODS: We identified 20 nonambulatory patients with myelodysplasia who were part of a multicenter Investigational Device Exemption study of 214 patients treated with the VEPTR system. Demographics, standard radiographic measurements, pulmonary function parameters, and complications in 16 patients were analyzed. Average age at first surgery was 48.6 months. The minimum followup was 25 months (mean, 59 months; range, 25-164 months). RESULTS: The Cobb angle decreased postoperatively in nine patients, increased less than 10° in five patients, and increased less than 20° in two patients. The mean increase in thoracic spinal length (growth) by year after the initial procedure with lengthening was 0.48 cm. Ventilatory function improved in 11 patients and deteriorated in five patients. Intraoperative complications occurred in two patients. Complications directly related to the implant were seven infections and five implant migrations. CONCLUSIONS: Our observations suggest VEPTR is a reasonable treatment option for spinal deformity in the immature, nonambulatory myelodysplasia population correcting the spinal deformity, allowing spinal growth, and maintaining adequate respiratory function. The rate of complications is within the range reported for spinal fusion using standard approaches.

Histology of a fusion mass augmented with demineralized bone matrix for congenital scoliosis.

The pseudarthrosis rate after a posterior spinal fusion for congenital scoliosis and kyphoscoliosis is high, between 9.1 and 46.1%. Autogenous iliac crest bone graft is the current gold standard graft material, but is associated with complications that include donor site pain and infection. Demineralized bone matrix (DBM) may be a cost effective, safe, and clinically effective alternative bone graft material. The medical records of a single patient with the diagnosis of congenital scoliosis who had undergone a posterior spinal fusion were reviewed under an institutional review board approved protocol. At 11 months after posterior spinal fusion, bone biopsies were obtained upon routine planned exploration of the spinal fusion site. These samples were sectioned and reviewed by an independent pathologist to assess for bone maturity and the presence of residual allograft bone graft material. The histological specimens that were obtained from the patient showed mature bone with no residual graft material evident in any of the samples. In conclusion, in this particular case, the DBM was fully incorporated into the fusion mass. Although a large clinical trial would be necessary to make generalized conclusions about the use of DBM in the congenital scoliosis population, this histological analysis adds evidence that DBM is incorporated and may aid in obtaining a solid spinal fusion in this difficult population.