Neuromuscular Dysfunction in Clubfeet Associated With Constriction Band Syndrome.

INTRODUCTION: Constriction band syndrome (CBS) is a congenital limb anomaly frequently associated with clubfoot. Clubfeet in CBS patients may be associated with peroneal nerve dysfunction in the involved lower extremity; however, the etiology of this neuromuscular dysfunction is not clear. We sought to characterize the distribution of constriction bands on lower extremities with clubfoot and determine if neuromuscular deficit (NMD), defined here as having absent ankle dorsiflexion, was associated with ipsilateral proximal bands. Our secondary aim was to compare the treatment and outcomes of clubfeet with NMD to those without NMD. METHODS: We performed a retrospective review of all patients with CBS and clubfoot presenting to our facility between January 1, 1998 and December 31, 2018. Treatment with the Ponseti method, at least 1 year of follow-up at this facility, and a detailed physical exam describing lower extremity neuromuscular function and the presence and location of constriction bands were required for inclusion in the study cohort. RESULTS: Twenty children with 26 clubfeet were included. Forty-six percent (12/26) of the clubfeet had NMD. Clubfeet with and without NMD had ipsilateral thigh or leg constriction bands at similar rates [42% (5/12) vs. 43% (6/14), P =0.106], and the majority (7/12) of clubfeet with NMD did not have an ipsilateral thigh or leg band. While children with an NMD clubfoot tended toward more casts, relapses, and surgical procedures, these differences did not reach statistical significance. The use of a daytime AFO beyond age four was higher in the NMD clubfeet [58% (7/12) vs. 14% (2/14), P =0.04]. CONCLUSION: Clubfeet with neuromuscular deficits may occur in the absence of proximal ipsilateral constriction bands, suggesting they may be caused by mechanisms other than direct damage from visible constriction bands to underlying nerves. They can also coexist with arthrogrypotic conditions. Clubfeet with an NMD tended toward more casts, relapses, and surgeries than those without NMD, but these differences did not reach statistical significance. These patients often elect long-term use of a daytime AFO.

Predicting Deformity Correction of Growth Modulation in Late-onset Tibia Vara.

BACKGROUND: Growth modulation in late-onset tibia vara (LOTV) has been reported to yield variable results. We hypothesized that parameters of deformity severity, skeletal maturity, and body weight could predict the odds of a successful outcome. METHODS: A retrospective review of tension band growth modulation for LOTV (onset ≥8 y) was performed at 7 centers. Tibial/overall limb deformity and hip/knee physeal maturity were assessed on preoperative anteroposterior standing lower-extremity digital radiographs. Tibial deformity change with first-time lateral tibial tension band plating (first LTTBP) was assessed by medial proximal tibia angle (MPTA). Effects of a growth modulation series (GMS) on overall limb alignment were assessed by mechanical tibiofemoral angle (mTFA) and included changes from implant removal, revision, reimplantation, subsequent growth, and femoral procedures during the study period. The successful outcome was defined as radiographic resolution of varus deformity or valgus overcorrection. Patient demographics, characteristics, maturity, deformity, and implant selections were assessed as outcome predictors using multiple logistic regression. RESULTS: Fifty-four patients (76 limbs) had 84 LTTBP procedures and 29 femoral tension band procedures. For each 1-degree decrease in preoperative MPTA or 1-degree increase in preoperative mTFA the odds of their successful correction decreased by 26% in the first LTTBP and 6% by GMS, respectively, controlling for maturity. The change in odds of success for GMS assessed by mTFA was similar when controlling for weight. Closure of a proximal femoral physis decreased the odds of success for postoperative-MPTA by 91% with first LTTBP and for final-mTFA by 90% with GMS, controlling for preoperative deformity. Preoperative weight ≥100 kg decreased the odds of success for final-mTFA with GMS by 82%, controlling for preoperative mTFA. Age, sex, race/ethnicity, type of implant, and knee center peak value adjusted age (a method for bone age) were not predictive of outcome. CONCLUSIONS: Resolution of varus alignment in LOTV using first LTTBP and GMS, as quantified by MPTA and mTFA, respectively, is negatively impacted by deformity magnitude, hip physeal closure, and/or body weight ≥100 kg. The presented table, utilizing these variables, is helpful in the prediction of the outcome of the first LTTBP and GMS. Even if complete correction is not predicted, growth modulation may still be appropriate to reduce deformity in high-risk patients. LEVEL OF EVIDENCE: Level III.

Predicting Success of Deformity Correction With Tension Band Plating in Early-Onset Tibia Vara.

BACKGROUND: Angular deformity correction with tension band plating has not been as successful in early-onset tibia vara (EOTV) as it has been in other conditions. Our hypothesis is that perioperative factors can predict the success of lateral tibial tension band plating (LTTBP) in patients with EOTV. METHODS: A retrospective review was performed at 7 centers evaluating radiographic outcomes of LTTBP in patients with EOTV (onset <7 y of age). Single-event tibial LTTBP outcome was assessed through medial proximal tibial angle (MPTA). The final limb alignment following comprehensive limb growth modulation (CLGM), which could include multiple procedures, was assessed by mechanical axis zone (MAZone), mechanical tibio-femoral angle (mTFA), and mechanical axis deviation (MAD). Preoperative age, weight, deformity severity, medial physeal slope, and Langenskiöld classification +/- modification were investigated as predictors of outcome. Success was defined as the correction or overcorrection to normal age-adjusted alignment. The minimum follow-up was 2 years except when deformity correction, skeletal maturity, or additional surgery occurred. RESULTS: Fifty-two patients with 80 limbs underwent 115 tibial LTTBP procedures at a mean age of 5.3 y, including 78 primary, 21 implant revisions, and 15 reimplantations for recurrence. Tibial LTTBP resulted in a mean change of +8.6 o in MPTA and corrected 53% of tibias. CLGM resulted in MAD correction for 54% of limbs.Univariate analysis showed that success was best predicted by preoperative age, weight, MPTA, and MAD. Multivariate analysis identified that preoperative-MPTA/MAD and preoperative-weight<70 kg were predictive of MPTA and MAD correction, respectively. The probability of success tables are presented for reference. CONCLUSION: Successful correction of MPTA to age-adjusted norms following a single-event LTTBP occurred in 53% of tibias and was best predicted by preoperative-MPTA and preoperative body weight <70 kg. Comprehensive growth modulation corrected limbs in 54%. The probability of correction to age-adjusted MAD is best estimated by preoperative-MAZone 1 or 2 (MAD ≤40 mm). Limbs with preoperative-MAD>80 mm improved, but ultimately all failed to correct completely with CLGM. Osteotomy may need to be considered with these severe deformities. While modified Langenskiöld classification and medial physeal slope have been shown to predict the outcome of osteotomy, they were not predictive for LTTBP. Change in MPTA was common after physeal untethering. LEVEL OF EVIDENCE: Level-III.

Femoral Deformity in Tibia Vara and Its Response to Growth Modulation.

BACKGROUND: While tibia vara is a disorder of the proximal tibial physis, femoral deformity frequently contributes to the overall limb malalignment. Our purpose was to determine how femoral varus deformity in tibia vara responds to growth modulation, with/without lateral tension band plating (LTBP) to the femur. METHODS: One-hundred twenty-seven limbs undergoing LTBP for tibia vara were reviewed. All had tibial LTBP and 35 limbs also had femoral LTBP for varus. Radiographs were measured for correction of the mechanical lateral distal femoral angle (mLDFA) and mechanical axis deviation (MAD). Preoperative-femoral varus was defined with an age-adjusted guide: mLDFA >95 degrees for 2 to below 4 years and mLDFA >90 degrees for 4 to 18 years. The 35 limbs having femoral LTBP were compared with 50 limbs with femoral varus and no femoral LTBP. In addition, 42 limbs that did not have preoperative-femoral varus were followed. Patients with early-onset (below 7 y) tibia vara were compared with those with late-onset (≥8 y). Outcome success was based on published age-adjusted mLDFA and MAD norms. RESULTS: Following femoral LTBP, the mean mLDFA decreased from 98.0 to 87.1 degrees. All femurs had some improvement, with 28/35 femurs (80%) achieving complete correction. One limb, with late follow-up, overcorrected, requiring reverse (medial) femoral tension band plating.For the 50 limbs with femoral varus and only tibial LTBP, 16/22 limbs (73%) with early-onset and 11/28 limbs (39%) with late-onset completely corrected their femoral deformities. If the limb had preoperative-femoral varus, femoral LTBP statistically correlated with successful mLDFA correction and improvement of MAD, only in the late-onset group.Forty-two limbs, without preoperative-femoral varus, had no change in their mean mLDFA of 87 degrees. However, 4 femurs (10%) ended with posttreatment varus. CONCLUSIONS: Femoral LTBP is effective in correcting femoral varus deformity in the tibia vara. For femoral varus associated with late-onset tibia vara, femoral LTBP should be considered. Those that had femoral LTBP had statistically more successful femoral and overall limb varus correction. However, in early-onset tibia vara, with associated femoral varus, observation is warranted because 73% of femurs are corrected without femoral intervention. This study was underpowered to show additional improvement with femoral LTBP in the early-onset group. Even limbs with normal femoral alignment, should be observed closely for the development of femoral varus, during tibial LTBP treatment for tibia vara. LEVEL OF EVIDENCE: Level III.

Guided Growth for Varus Deformity Following Early Tibial Osteotomy in Infantile Tibia Vara-A Multi-Center Study.

BACKGROUND: Despite early osteotomy, many patients with infantile tibia vara (ITV) have persistent or recurrent varus deformity and disordered growth at the medial proximal tibial physis. Our hypothesis was that lateral tibial tension band plating (LTTBP) could guide correction. METHODS: A retrospective review at 6 centers of 15 patients (16 extremities) was performed of LTTBP for varus deformity following early osteotomy in ITV, diagnosed≤4years of age. Correction of deformity parameters on digital standing anteroposterior lower extremity radiographs determined outcome. RESULTS: Twenty-two LTTBP procedures were performed at mean age of 7.5 years, including 4 revisions for implant failure and 2 reimplantations for recurrence. Single event LLTBP, improved the medial proximal tibial angle with a mean change of 13.4 degrees (0.39 degrees/month). Eleven limbs had preoperative mechanical lateral distal femoral angle (mLDFA)>90 degrees. While n degree femoral procedures were performed, at study end, 11 femurs had mechanical lateral distal femoral angle≤90°. Pretreatment, 13 extremities had mechanical axis zone (MAZone) III varus (81%) and 3 had MAZone II varus (19%). LTTBP's were able to initially correct 13 limbs to MAZone I or valgus but 4 limbs rebounded to MAZone II varus after implant removal. Final limb alignment, after all surgeries and rebound, included 9 in MAZone I, 5 in MAZone II varus and 2 in MAZone III varus. Average follow-up was 3.0 years at mean 10.7 years of age. Fifteen procedures resulted in improvement in MAZone and 7 had no change. On average, those that improved were younger (7.3 vs. 8.0 y), weighed less (45.5 kg with body mass index 26.5 kg/m 2 vs. 67.8 kg and body mass index 35.7 kg/m 2 ), had lower mechanical axis deviation (37.1 mm vs. 43.9 mm), lower medial physeal slope (61.7 vs. 68.7 degrees) and had a higher percentage of open triradiate phases (87 vs. 57%). CONCLUSIONS: LTTBP for residual varus, after initial osteotomy in ITV, resulted in 81% of limbs initially achieving MAZone I or valgus with implant failure revisions and femoral remodeling. Rebound after implant removal reduced the corrected rate to 56%. Ninety-four percent avoided osteotomy during the study period. LEVEL OF EVIDENCE: IV.

Guided Growth Procedures: Broken Tension Band Implants in Patients With Blount Disease.

BACKGROUND: Tension band plate and screw implants (TBI) are frequently used for temporary hemiepiphyseodeses to manage angular deformity in growing children. The reported implant breakage rate, when TBI is used for deformities in patients with Blount disease, is much higher than when used in other diagnoses. Our hypothesis is that perioperative factors can identify risks for TBI breakage. METHODS: A retrospective case-control study was performed of 246 TBI procedures in 113 patients with Blount disease at 8 tertiary pediatric orthopaedic centers from 2008 to 2018. Patient demographics, age at diagnosis, weight, body mass index (BMI), radiographic deformity severity measures, location, and types of implants were studied. The outcome of implant breakage was compared with these perioperative factors using univariate logistic regression with Bonferroni correction for multiplicity to significance tests. RESULTS: There were 30 broken implants (12%), failing at mean 1.6 years following implantation. Most failures involved the metaphyseal screws. Increased BMI was associated with increased implant breakage. Increased varus deformity was directly associated with greater implant breakage and may be a more important factor in failure for those below 7 years compared with those 8 years or above at diagnosis. There was a 50% breakage rate for TBI with solid 3.5 mm screws in Blount disease with onset 8 years or above of age. No demographic or implant factors were found to be significant. CONCLUSIONS: Breakage of TBI was associated with increased BMI and varus deformity in patients with Blount disease. Larger studies are required to determine the relative contribution and limits of each parameter. Solid 3.5 mm screws should be used with caution in TBI for late-onset Blount disease. LEVEL OF EVIDENCE: Level III.

Use of PROMIS in Assessment of Children With Ponseti-treated Idiopathic Clubfoot: Better Scores With Greater Than 3 Years of Brace Use.

BACKGROUND: Patient-Reported Outcomes Measurement Information System (PROMIS) is a well-validated tool used to measure health-related quality of life for children and adolescents with chronic medical conditions. The current study evaluates PROMIS scores in 3 domains for children with Ponseti-treated idiopathic clubfoot. METHODS: This is a retrospective cohort study of 77 children, ages 5 to 16 years, treated by Ponseti protocol for idiopathic clubfoot. Three pediatric PROMIS domains (Mobility, Pain Interference, and Peer Relationships) were administered between April 2017 and June 2018. One-way analysis of variance with Bonferroni post hoc and independent sample t tests were performed to explore differences across PROMIS domain scores by sex, age, initial Dimeglio score, laterality, bracing duration, and whether the child underwent tibialis anterior transfer. RESULTS: In the self-reported group (ages 8 to 16), mean T-scores for all 3 domains in both unilaterally and bilaterally affected groups were within the normal range, with respect to the general reference pediatric population. However, children with unilateral clubfoot had a significantly higher mean Mobility T-score (54.77) than children with bilateral clubfoot (47.81, P=0.005). Children with unilateral clubfoot also had significantly lower mean pain scores (39.16) than their bilateral counterparts (46.56, P=0.005). Children who had braced >36 months had a significantly higher mean Mobility T-score (53.68) than children who braced ≤36 months (46.28, P=0.004).In the proxy group (ages 5 to 7), mean T-scores for all 3 domains in both laterality groups were within the normal range, with respect to the reference population. Children who had braced >36 months had a significantly higher mean Mobility T-score (52.75 vs. 49.15, P=0.014) and lower Pain Interference score (43.04 vs. 49.15, P=0.020) than children who braced ≤36 months. CONCLUSIONS: Children treated by Ponseti protocol for idiopathic clubfoot yielded PROMIS scores for Mobility, Pain Interference, and Peer Relationships domains similar to the reference population. Bracing duration >36 months and unilaterality were associated with less mobility impairment than their counterparts. These findings may help guide parent recommendations. LEVEL OF EVIDENCE: Level III.

Design and descriptive data of the randomized Clubfoot Foot Abduction Brace Length of Treatment Study (FAB24).

The aim of this study was to describe the design and baseline characteristics of participants enrolled in the prospective randomized-controlled Clubfoot Foot Abduction Brace Length of Treatment Study (FAB24). Foot abduction bracing is currently the standard of care for preventing clubfoot relapse. Current recommendations include full-time bracing for the first 3 months and then 8-12 h a day for 4 years; however, the optimal length of bracing is not known. The FAB24 trial is a clinical randomized study to determine the effectiveness of 2- versus 4-year foot abduction bracing. Participant enrollment for FAB24 was conducted at eight sites in North America and included enrollment and randomization of 139 participants with isolated clubfoot. This clinical trial will generate evidence-based data that will inform and improve patient care.

Reliability of radiographic parameters in neuromuscular scoliosis.

STUDY DESIGN: Retrospective review of radiographic data. OBJECTIVES: This study sought to define interobserver and intraobserver variability to further delineate reliable means by which radiographs of patients with neuromuscular scoliosis can be examined. SUMMARY OF BACKGROUND DATA: Previous studies analyzed the use of Cobb angles in the measurement of idiopathic and congenital scoliosis, but no study until now describes a critical analysis of measurement in evaluating neuromuscular scoliosis. METHODS: Forty-eight patients with neuromuscular scoliosis radiographs were reviewed. These were evaluated for Cobb angle, end vertebrae selection, Ferguson angle, apex of the curve, C7 balance, pelvic obliquity, Risser sign, status of the triradiate cartilage, kyphosis Cobb angle, endplate selection for kyphosis, and kyphotic index. Interclass and intraclass variability was examined with statistical analysis. RESULTS: Cobb angle had an intraobserver variability was 5.7 degrees and the interobserver variability was 14.8 degrees . The intraobserver and interobserver variability for Ferguson angle was 6.8 degrees and 20.6 degrees, respectively. The kyphotic Cobb angle intraobserver variability was found to be 17.4 degrees, and the interobserver variability was 24.01 degrees . CONCLUSIONS: Neuromuscular scoliosis radiographs can be reliably analyzed with the use of Cobb angle. Other forms of analysis, such as Ferguson angle, are not as reliable. Pelvic obliquity should be measured from the horizontal, as other methods are not as reliable. Kyphosis is best evaluated with the use of the kyphotic Cobb angle. Finally, it is felt that a separate anteroposterior pelvis radiograph should be used to assess skeletal maturity, as scoliosis films often truncate the vital anatomy necessary to determine skeletal maturity.

Assessment of children with brachial plexus birth palsy using the Pediatric Outcomes Data Collection Instrument.

The purpose of this study was to determine whether the Pediatric Outcomes Data Collection Instrument (PODCI) measures differences in function between children with brachial plexus birth palsy (BPBP) who are candidates for shoulder tendon surgery and age-matched controls. The PODCI was administered prospectively to 23 children with BPBP who were candidates for shoulder tendon surgery. Their results were compared with published PODCI data for control subjects, and factors associated with function within the BPBP cohort were determined. Children in the BPBP cohort had significantly lower PODCI scores in upper extremity function, sports, and global function than control subjects. Limited active shoulder external rotation was significantly associated with lower functional scores. The PODCI measures diminished upper extremity function in children with BPBP who are candidates for shoulder tendon surgery, thereby showing promise as a tool for measuring baseline function and postoperative functional gains for children with BPBP.

The Pediatric Outcomes Data Collection Instrument (PODCI) and functional assessment of patients with unilateral upper extremity deficiencies.

The Pediatric Outcomes Data Collection Instrument (PODCI) questionnaire was used to quantify functional abilities of a group of unilateral upper extremity deficiency (U-UED) patients and compare them with "normal" control children. Sixty-four consecutive patients with U-UED were assessed. Parents and adolescent (ages 11-21) patients responded. Underlying diagnosis, amputation level, and type of prosthesis were recorded. Scores were compared for congenital versus traumatic etiologies for patients with various amputation levels, and for patients using prostheses versus those not using prostheses. In both parent and patient responses, PODCI scores were significantly lower than "normal" for upper extremity function and sports. Scores were similar for congenital and acquired amputees. Responses from adolescent patients showed progressively decreasing scores for upper extremity, transfers, sports, and global function with progressively proximal amputation levels. Patients using prostheses with different terminal devices did not significantly differ. Parent responses for prosthesis wearers showed lower comfort/pain scores (ie, increased pain) than non-prosthesis wearers, but no significant differences in function, including upper extremity function.

Spinal arthrodesis for scoliosis in Down syndrome.

The results of operative treatment of scoliosis in Down syndrome at one institution are reviewed. Seven patients with progressive scoliosis who had undergone arthrodesis of the thoracolumbar spine were identified. Clinical and radiographic results were noted, as were any complications. Mean patient follow up was 9 years (range 2-25). All patients eventually showed radiographic evidence of solid fusion with no curve progression. One patient had undergone a revision procedure for pseudoarthrosis and failure of Dwyer instrumentation. Other complications included a lateral subluxation under the fusion area, pneumonia, and one asymptomatic hook dislodgment. Progressive deformities of the thoracolumbar spine in Down syndrome patients can be treated with fusion and instrumentation with cessation of progression. As is the case for upper cervical fusion in these patients, the complication rate is notable.

The Pediatric Outcomes Data Collection Instrument (PODCI) and functional assessment in patients with adolescent or juvenile idiopathic scoliosis and congenital scoliosis or kyphosis.

STUDY DESIGN: An initial examination of functional assessment scores in scoliosis and kyphosis populations. OBJECTIVE: Examination of scores from the Pediatric Outcomes Data Collection Instrument for patients with idiopathic scoliosis, congenital scoliosis, and congenital kyphosis, comparing scores with those of children without orthopedic disabilities. SUMMARY OF BACKGROUND DATA: Little information has been presented regarding performance of scoliosis patients on the Pediatric Outcomes Data Collection Instrument. METHODS: A total of 102 patients with adolescent idiopathic scoliosis, 47 with congenital scoliosis without kyphosis, and 9 with congenital kyphosis completed the Pediatric Outcomes Data Collection Instrument. Responses were compared with those from a "normal" population. Subgroup analyses were performed for patients with adolescent idiopathic scoliosis. A P value <0.05 was considered statistically significant. RESULTS: Scores in Transfers, Sports, Comfort, and Happiness were significantly lower than "normal" in adolescent idiopathic scoliosis. In congenital scoliosis without kyphosis, scores in all categories except Happiness were significantly lower than "normal." All category scores were significantly lower than "normal" in congenital kyphosis. In adolescent idiopathic scoliosis, age and curve location did not influence Comfort scores. Comfort scores were significantly lower than "normal" for all curve locations and for all ranges of Cobb angle. Happiness scores were significantly lower in adolescent idiopathic scoliosis patients with Cobb angles >50 degrees who had not received surgery when compared with either patients who had received surgery or patients with Cobb angles <50 degrees. CONCLUSION: These findings provide some normative values for the Pediatric Outcomes Data Collection Instrument for three specific diagnoses. Patients with adolescent idiopathic scoliosis, congenital scoliosis, and congenital kyphosis gave responses significantly different from "normal" children. Pain appeared to be a common finding with these diagnoses.