Functional Independence of Children With Arthrogryposis.

BACKGROUND: Arthrogryposis (AMC) is a descriptive term to characterize a child born with multiple joint contractures. Treatment aims to improve functional independence, yet the literature objectively describing functional independence in this population is scarce. This study aimed to describe the functional independence of children with AMC through the lens of the Pediatric Evaluation of Disability Inventory-Computer Adaptive Test (PEDI-CAT) and observational activities of daily living (ADL) tasks. METHODS: Patients with AMC between the ages of 3 and 12 years participated in this prospective study. Parents completed the PEDI-CAT while a trained occupational therapist observed children as they completed a checklist of functional ADL tasks. Patients were grouped according to developmental age groups: "preschoolers" (3 to 5 y), "early school-age" (6 to 9 y), and "late school-age" (10 to 12 y). Patient's PEDI-CAT normative scores were described, comparing the study population to typically developing children, and differences in each domain were examined between developmental age groups. The observed ADL tasks completed were also described, and differences in scores were examined between developmental age groups. RESULTS: Forty-four patients (mean age of 7±2.86 y) were enrolled. The distribution between age groups was nearly even. Mean daily activities T -score for patients with AMC was 25.80±11.98 and the mean mobility T -score was 17.39±9.77. Late school-age children scored significantly lower than preschool-age children in both of these domains ( P <0.01). Observed ADL tasks demonstrated a high level of required assistance for patients (range: 27.3% to 61.4%), although older school-age children did show greater independence with tested activities than preschool-age children ( P =0.05). CONCLUSION: Children with AMC are significantly limited in functional independence, particularly regarding age-appropriate daily activities and mobility. Outcomes from this study provide a reference to help gauge the results of nonoperative and surgical treatment toward improving functional independence in this population. LEVEL OF EVIDENCE: Level III: prognostic study.

Great Toe Interphalangeal Fusion for Hallux Valgus Interphalangeus Deformity in Young Patients.

OBJECTIVES: The purposes of this study were to examine indications, radiographic outcomes, and clinical complications for primary arthrodesis of the great toe interphalangeal (IP) joint in young patients. BACKGROUND: Hallux valgus interphalangeus deformity of the great toe is uncommon in the pediatric population and often requires fusion of the IP joint. METHODS: A retrospective review of patients, ages 8 to 19 years, who underwent operative fusion of the great toe IP joint, and had >1 year radiographic follow-up was performed. Medical records and radiographs were reviewed to determine indications for surgery, concomitant procedures, success of fusion, complications, and need for revision procedures or elective hardware removal. Differences between pre and postradiographic outcomes for the hallux valgus angle, IP angle, and intermetatarsal angle were analyzed. RESULTS: Twenty-seven patients (31 feet) were included in the analyses. The average age at fusion surgery was 14.9 years (SD ± 2.3) with a mean follow-up visit of 35.2 months. The most common indications for fusion of the great toe IP joint were pain and deformity. Kirschner wire fixation was utilized in 7 cases, with the remaining 24 cases fixed with a single retrograde cannulated screw. Fifty-five concomitant toe and foot procedures were performed in 21 feet (68%) to address additional foot and toe deformity. Successful fusion occurred in 30 of 31 toes after the primary fusion. Patient satisfaction with the toe position and diminished pain were high (94.1%). Significant improvement was noted in the measure of the IP angle ( P < 0.001), with minimal clinical change in the hallux valgus angle ( P = 0.24) or the intermetatarsal angle ( P = 0.03). CONCLUSIONS: Hallux valgus interphalangeus of the great toe was successfully managed with the fusion of the IP joint. Single screw fixation or the use of Kirschner wires led to similar outcomes. Hardware-related issues with the use of screws were the most common adverse outcome. Patients should be made aware of the possible need for hardware removal after fusion utilizing screw fixation. LEVEL OF EVIDENCE: Level IV-a retrospective case series.

Is peak hamstrings muscle-tendon length criterion a sufficient indicator to recommend against surgical lengthening of hamstrings?

BACKGROUND: Excessive knee flexion during stance in children with cerebral palsy is often treated by surgical hamstrings lengthening. Pre-operative hamstrings muscle-tendon length can be estimated from kinematics and often used for decision making to rule out surgical lengthening if peak hamstrings muscle-tendon length is 'Not Short'. RESEARCH QUESTION: If peak hamstrings muscle-tendon length is within two standard deviations of typical, is that a sufficient indicator to rule out surgical hamstrings lengthening? METHODS: Three motion analysis centers retrospectively identified children with cerebral palsy, age 6-17 years, who had consecutive gait analyses with knee flexion at initial contact > 20° and popliteal angle > 35° at initial study. Three groups were considered: Medial Hamstrings Lengthening (MHL), Medial and Lateral Hamstrings Lengthening (MLHL), no surgical intervention (Control). Peak hamstrings muscle-tendon length at initial gait study was computed and categorized as 'Short' or 'Not Short'. Two outcomes variables were considered: change in peak knee extension (PKE) and change in pelvic tilt. Univariate comparisons of all variables were assessed along with a multivariate stepwise regression analysis to identify pre-operative characteristics that may predict post-operative improvement. RESULTS: 440 individuals met inclusion criteria. Percentage of individuals with improved PKE by grouping were- MHL-'Short': 60%, MHL-'Not Short': 65%, MLHL-'Short': 74%, MLHL-'Not Short': 74%, Control 'Short': 20%, Control 'Not Short': 19%. Percentage of individuals with worsened pelvic tilt were- MHL-'Short': 25%, MHL-'Not Short': 11%, MLHL-'Short': 42%, MLHL-'Not Short': 21% with significantly more individuals in MHL-'Short' subgroup compared to MHL-'Not Short'. Multivariate analysis suggested that pre-operative pelvic tilt and weak hip extensor strength have the largest effect on predicting post-operative increase in APT. Peak muscle-tendon length was not a significant predictor of post-operative knee kinematics or increase in APT. SIGNIFICANCE: This study suggests that hamstrings muscle-tendon length criteria by itself is not a sufficient indicator to recommend against hamstrings lengthening.

Growth Charts for Children With Arthrogryposis Multiplex Congenita.

Children with arthrogryposis multiplex congenita (AMC) often demonstrate growth differences compared with typically developing (TD) children. However, growth charts have not been developed for this population. The purpose of this study was to create AMC-specific growth charts and to compare these values to those of TD children. A retrospective review of height/length and weight for 206 children with AMC was performed. Growth charts were developed and stratified over seven percentiles; these were then compared with growth charts of TD children. Children with AMC tend to be smaller in stature and weight compared with TD children, particularly in the first 36 months of life. Thereafter, weight values trend toward the 50th percentile of TD children, but height/length values persist around the 5th percentile of TD children. The development of AMC-specific growth charts provides health care providers an objective tool to evaluate growth patterns of patients with AMC.

Hip reconstruction in children with cerebral palsy: does magnitude of surgery influence complications and outcomes?

Children with cerebral palsy (CP) and neuromuscular hip dysplasia (NMHD) frequently require hip reconstruction including femoral and pelvic osteotomies. Outcomes and complications in CP patients with NMHD who underwent varying degrees of hip reconstruction surgeries were examined. Ninety-nine patients with a diagnosis of CP who underwent surgical management of NMHD over a 15-year period (2005-2020) were reviewed. Three patient cohorts with varying degrees of surgery were analyzed including: (1) unilateral femoral osteotomy with pelvic osteotomy; (2) bilateral femoral osteotomy with unilateral pelvic osteotomy; and (3) simultaneous bilateral femoral osteotomy and bilateral pelvic osteotomy. Data points included operative (OR) time, length of stay (LOS), estimated blood loss (EBL), and transfusion requirement. Complications during the perioperative and postoperative periods were identified and graded by severity. Increased EBL, need for transfusion, OR time, and LOS were observed more frequently in patients undergoing bilateral reconstruction. Complication rates were high for all three cohorts, with 87% of cases in the bilateral pelvic osteotomy cohort experiencing ≥1 complication. However, 90% of these were considered grade I or II and required no or minimal intervention. The average postoperative migration index at final follow-up for treated hips was significantly less in bilateral pelvic osteotomy cases. Patients with CP and bilateral NMHD can be safely managed with bilateral simultaneous comprehensive reconstruction. The data presented in this study can help both surgeons and families anticipate a more accurate postoperative course. Level of evidence: III.

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.

Does use of ankle foot orthoses affect the dynamic motor control index during walking in cerebral palsy and idiopathic toe walking populations?

BACKGROUND: The dynamic motor control (walk-DMC) index during walking is a measure of the complexity of muscle activation pattern. Ankle Foot Orthoses (AFO) are frequently used to improve the gait of children with Cerebral Palsy (CP) and Idiopathic Toe Walking (ITW). The purpose of this study was to assess the change in walk-DMC index secondary to AFO use. RESEARCH QUESTION: Does the change in walk-DMC reflect the change in walking kinematics with the use of AFO. METHODS: Individuals with diagnosis of CP or ITW with gait analysis data available for barefoot and AFO condition were retrospectively identified. For each individual, the walk-DMC index, Gait Deviation Index (GDI) and Gait Variable Scores (GVS) of knee and ankle kinematics were computed for BF and AFO conditions. Paired t-tests were used to compare key variables between BF and AFO conditions. Multi-variate stepwise regression analysis was performed to identify variables that may predict the increase in walk-DMC between BF and AFO condition. RESULTS: 253 individuals were included in the study. For CP individuals (n = 208), statistically significant but quantitatively minimal improvement was observed in walk-DMC (1 ± 9), GDI (2 ± 9) and ankle GVS (2 ± 7). For ITW individuals (n = 45), larger improvements were observed in walk-DMC (11 ± 13), GDI (9 ± 11) and ankle GVS (6 ± 7). Diagnosis of ITW, use of Solid-AFO and Posterior Leaf Spring-AFO were the significant predictor of increase in walk-DMC with AFO. Higher ankle GVS at BF condition (larger deviation from TD) led to larger increase in walk-DMC. Higher knee GVS (larger deviation from TD) led to smaller increase in walk-DMC. SIGNIFICANCE: Use of AFO can lead to improvement in walking kinematics that is reflected in increase in walk-DMC with AFO compared to BF for ITW individuals. The change in kinematics and walk-DMC with use of AFO was minimal for CP individuals.

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.

Surgical Management of Severe Equinus Deformity in Ambulatory Children With Cerebral Palsy.

BACKGROUND: Tendo Achilles lengthening (TAL) for the management of equinus contractures in ambulatory children with cerebral palsy (CP) is generally not recommended due to concerns of over-lengthening, resulting in weakness and plantar flexor insufficiency. However, in some cases, surgical correction of severe equinus deformities can only be achieved by TAL. The goal of this study is to assess the outcomes following TAL in these cases. METHODS: A retrospective cohort study of children with CP with severe equinus contractures (ankle dorsiflexion with the knee extended of -20 degrees or worse) who underwent TAL as part of a single event multilevel surgery, with preoperative and postoperative gait analysis studies. Continuous data were analyzed by paired t test, and categorical data by McNemar Test. RESULTS: There were 60 subjects: 42 unilateral, 18 bilateral CP; 41 GMFCS II, 17 GMFCS I; mean age at surgery was 10.6 years, mean follow-up was 1.3 years. Ankle dorsiflexion with the knee extended improved from -28 to 5 degrees (P<0.001). The ankle Gait Variable Score improved from 34.4 to 8.6 (P<0.001). The ankle moment in terminal stance improved from 0.43 to 0.97 Nm/kg (P<0.001). Significant improvements (P<0.001) were seen in radiographic measures of foot alignment following surgery. There were few significant differences in the outcome parameters between subjects with unilateral versus bilateral CP (eg, only the bilateral group showed improved but persistent increased knee flexion in mid-stance). CONCLUSIONS: The outcomes following TAL for the management of severe equinus deformity in ambulatory children with CP were favorable 1 year after surgery, with significant improvements in all domains measured. SIGNIFICANCE: This study does not advocate for the widespread use of TAL to correct equinus deformity in children with CP. However, it does show that good short-term outcomes following TAL are possible in properly selected subjects with severe contractures when the dosing of the surgery is optimal (correction of contracture to between 0 and 5 degrees of dorsiflexion with the knee extended) and the procedure is performed in the setting of single event multilevel surgery with subsequent proper orthotic management and rehabilitation.

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.

Patient-Reported Outcome Measurement Information System (PROMIS) Scores in Pediatric Patients With Arthrogryposis.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a clinical term that is used to describe congenital contractures that lead to childhood deformities. Treatment aims are to maximize function while minimizing pain and disability. Few studies have explored patient-reported outcomes in the pediatric arthrogrypotic population, particularly concerning mobility. The aim of this study was to report scores for the Patient-Reported Outcome Measurement Information System (PROMIS) questionnaire for pediatric patients with arthrogryposis with regards to mobility, upper extremity (UE) function, pain interference, and peer relationships. METHODS: A retrospective chart review of 76 patients with AMC aged 5 to 17 who completed the PROMIS questionnaire between January 1, 2017 to March 24, 2020 was performed. Results were collected for four domains: mobility, UE function, pain interference, and peer relationships. Outcomes were stratified by type of arthrogryposis: Amyoplasia (Am), Distal Arthrogryposis (DA), and Other Diagnoses (OD). Results of subjects with isolated upper or lower extremity involvement were compared with subjects with involvement of upper and/or lower extremities. Outcomes were correlated with history of surgical intervention. RESULTS: Children with Am and OD demonstrated moderate impairment of mobility (average: 35.2 and 35.9, respectively), while those with DA reported only mild impairment (average: 44.9). UE function was severely impaired for children with Am (average: 23.0), moderately impaired for OD (average: 33.0), and mildly impaired for DA (average: 43.4). All patient groups reported normal ranges of pain interference, as well as good peer relationships. A moderate negative correlation between number of surgical interventions and mobility scores, and a weak negative correlation between number of surgeries and pain interference scores were found. CONCLUSIONS: Children with AMC experience limited mobility and UE function, but normal levels of pain interference and good peer relationships. The average values provided in this study will serve as a baseline from which to evaluate the efficacy of both nonoperative and surgical interventions. LEVEL OF EVIDENCE: Level II-prognostic study; retrospective study.

Terminal Syme Amputation of the Great Toe in the Pediatric Population.

BACKGROUND: In the pediatric population, chronic ingrown toenails (onychocryptosis) can cause infection (paronychia), debilitating pain, and may be unresponsive to conservative treatments. Following multiple failed interventions, a terminal Syme amputation is one option for definitive treatment of chronic onychocryptosis. This procedure involves amputation of the distal aspect of the distal phalanx of the great toe with complete removal of the nail bed and germinal center, preventing further nail growth and recurrence. METHODS: A retrospective review was performed to determine outcomes of a terminal Syme amputation in the pediatric population. Inclusion criteria included treatment of onychocryptosis involving terminal Syme amputation with a minimum follow-up of 1 year. The medical record was reviewed to assess previous failed treatment efforts, perioperative complications, radiographic outcomes, and the need for additional procedures. RESULTS: From 1984 to 2017, 11 patients (13 halluces) with onychocryptosis were treated with a terminal Syme amputation. There were no intraoperative complications. One hallux had a postoperative infection requiring antibiotics as well as partial nail regrowth following the terminal Syme procedure that required subsequent removal of the residual nail. Following partial nail ablation, the patient had no further nail growth. An additional patient also developed a postoperative infection requiring oral antibiotic treatment. All patients returned to full weight-bearing physical activities within 6 weeks of surgery. CONCLUSIONS: Terminal Syme amputation was successful in treating pediatric patients who have recalcitrant onychocryptosis and paronychia. There was little functional consequence following terminal Syme amputation of the great toe in this patient population, making it an effective salvage procedure. LEVEL OF EVIDENCE: Level IV-retrospective comparative study.

Outcomes of Patellar Tendon Imbrication With Distal Femoral Extension Osteotomy for Treatment of Crouch Gait.

BACKGROUND: Crouch gait is a frequent gait abnormality observed in children with cerebral palsy. Distal femoral extension osteotomy (DFEO) with the tightening of the extensor mechanism is a common treatment strategy to address the pathologic knee flexion contracture and patella alta. The goal of this study was to review the results of a patellar tendon imbrication (PTI) strategy to address quadriceps insufficiency in the setting of children undergoing DFEO. METHODS: After institutional review board approval, all patients with crouch gait treated at a single institution with DFEO and PTI were identified. Clinical, radiographic, and instrumented gait analysis data were analyzed preoperatively and at 1 year following surgery. RESULTS: Twenty-eight patients (54 extremities) with a diagnosis of cerebral palsy and crouch gait were included. Significant improvements were appreciated in the degree of knee flexion contracture, quadriceps strength, knee extensor lag, and popliteal angle (P<0.01). Knee flexion at initial contact and during mid-stance improved significantly (P<0.0001), and knee moments in late stance were significantly reduced (P<0.01). The anterior pelvic tilt, however, significantly increased postoperatively (P<0.0001). Radiographic improvements were seen in the knee flexion angle and patellar station as assessed by the Koshino Sugimoto Index (P<0.0001). Four patients (14.2%) developed a recurrence of knee flexion contracture requiring further intervention. CONCLUSIONS: PTI is a simplified and safe technique to address quadriceps insufficiency when performing DFEO. The short-term results of patients who underwent DFEO with PTI demonstrated improvements in clinical, radiographic, and gait analysis variables of the knee. Investigating long-term outcomes, comparing techniques, and assessing quality of life measures are important next steps in research. LEVEL OF EVIDENCE: Level IV-case series.

Surgical Outcomes for Severe Idiopathic Toe Walkers.

BACKGROUND: Idiopathic toe walking (ITW) is a diagnosis of exclusion and represents a spectrum of severity. Treatment for ITW includes observation and a variety of conservative treatment methods, with surgical intervention often reserved for severe cases. Previous studies reviewing treatment outcomes are often difficult to interpret secondary to a mixture of case severity. The goal of this study was to review surgical outcomes in patients with severe ITW who had failed prior conservative treatment, as well as determine differences in outcomes based on the type of surgery performed. METHODS: After IRB approval, all patients with surgical management of severe ITW at a single institution were identified. Zone II or zone III plantar flexor lengthenings were performed in all subjects. Clinical, radiographic, and motion analysis data were collected preoperatively and at 1 year following surgery. RESULTS: Twenty-six patients (46 extremities) with a diagnosis of severe ITW from 2002 to 2017 were included. Zone II lengthenings were performed in 25 extremities (mean age=9.9 y) and zone III lengthenings were performed in 21 extremities (mean age=8.6 y). At the most recent follow-up, 100% of zone III lengthening extremities and 88% of zone II lengthening demonstrated decreased severity of ITW. Six extremities required additional treatment, all of which were initially managed with zone II lengthenings. CONCLUSIONS: Severe ITW or ITW that has not responded to conservative treatment may benefit from surgical intervention. More successful outcomes, including continued resolution of toe walking, were observed in subjects treated with zone III lengthenings. LEVEL OF EVIDENCE: Level III-case series.

Outcomes of Naviculectomy for Severe Recurrent Clubfoot Deformity.

BACKGROUND: Naviculectomy was originally described for resistant congenital vertical talus deformity but was later expanded to use in rigid cavus deformity. This study reviews the operative outcomes of complete excision of the navicular for recurrent deformity in the talipes equinovarus (TEV) population. METHODS: After institutional review board approval, all patients undergoing naviculectomy at a single institution were identified. Clinical, radiographic, and pedobarographic data (minimum 2 years' follow-up) were reviewed. RESULTS: Twelve patients (14 feet) with TEV from 1984 to 2019 were included. All feet had minimum 1 prior operative intervention on the affected foot (mean age = 4.0 years, range 0.2-14.5), with 8/14 having at least 3 prior operative procedures. Complete navicular excision with concomitant procedures was performed in all patients (mean age = 11.7 years, range 5.5-16.1). Mean clinical follow-up from naviculectomy was 5.1 years (range, 2.2-11.2). During follow-up, 6 patients required subsequent surgery, most often secondary to pain and progressive deformity. One patient underwent elective below-knee amputation of the affected extremity. Of the remaining 11 patients, 7 of 11 reported continued pain and 8 of 11 maintained adequate range of motion at the ankle at the most recent follow-up. CONCLUSION: Clinical follow-up demonstrated deteriorating results in a large percentage of patients. The high rate of additional procedures and continued pain in the current series suggests that even as a salvage procedure, naviculectomy may not provide adequate results for patients. LEVEL OF EVIDENCE: Level IV, case series.

Outcomes of knee arthrodesis in proximal femoral focal deficiency.

Treatment options for patients with severe forms of proximal femoral focal deficiency include limb salvage procedures or foot ablation and use of a prosthesis. In patients with amputation, an ipsilateral knee arthrodesis can provide an efficient lever arm that is easy to contain in a prosthetic socket. We performed a retrospective review of proximal femoral focal deficiency patients treated at a single institution with knee arthrodesis. From 1986 to 2018, 26 patients (18 males, eight females) met study inclusion criteria (mean follow-up 13.2 years). Aitken C or D deformities were present in 65% of extremities. Ten patients had concomitant knee fusion with foot ablation (mean age 4.3 years). Fifteen patients had staged procedures with initial foot ablation (mean age 1.3 years) followed by a knee arthrodesis (mean age 4.7 years). One patient was managed with knee arthrodesis and retention of a three-ray foot. Subsequent realignment osteotomies were required in six patients to correct progressive malalignment with growth, often due to retained physis or incomplete physeal resection during the arthrodesis. In severe forms of proximal femoral focal deficiency, a straight residual limb can be achieved with foot ablation and knee arthrodesis to improve weight-bearing alignment in an above knee prosthesis. Progressive deformity, which may interfere with prosthetic fitting and require repeat osteotomy, may occur if complete excision of the physis is not achieved.

Calcaneocuboid distraction arthrodesis for children with symptomatic pes planovalgus: does fixation improve outcomes?

Lateral column lengthening is a common surgical procedure for addressing symptomatic pes planovalgus foot deformity. For more severe cases, the use of a calcaneocuboid distraction arthrodesis (CCDA) can allow for more powerful correction. Previous reports have cited an increased risk of graft collapse with loss of correction when this procedure is performed without supplemental hardware fixation. The purpose of this study was to assess the outcomes of CCDA in children with and without supplemental locking fixation. A retrospective review from 2008 to 2016 of CCDA procedures with and without supplemental fixation was performed. The primary endpoint was graft collapse. Secondary objectives included evaluation of radiographic loss of correction, hardware failure, pain at 1-year follow-up, reoperations, and changes in the foot loading pattern foot per pedobarography. Twenty-nine feet in 24 patients were eligible for review. Supplemental locked fixation was used in 18 feet [hardware (HW)], with the remaining 11 feet managed without fixation [no hardware (NoHW)]. The overall failure rate on the basis of graft collapse and loss of correction was 55% (56% for the HW group, 55% for the NoHW group). Eleven patients (61%) in the HW group experienced hardware failure, with six (33%) of these requiring hardware removal. Fifty-six percent of the HW group and 45% of the NoHW group reported continued pain at 1-year follow-up. One patient from each group underwent revision arthrodesis. Supplemental locked fixation did not provide additional benefit in preventing graft collapse and loss of correction in this cohort. Alternative strategies should be considered to improve the outcomes for this procedure.

Patella Alta in Ambulatory Children With Cerebral Palsy: Prevalence and Functional Significance.

BACKGROUND: Gait dysfunction associated with patella alta (PA) in subjects with cerebral palsy (CP) has been presumed but not objectively established clinically or through biomechanical modeling. It is hypothesized that PA is associated with increasing level of motor impairment, increasing age, obesity, and worse stance phase knee kinematics and kinetics in children with CP. METHODS: Retrospective case series of 297 subjects with CP studied in our Motion Analysis Center. Data analyzed included patient demographics (age, body mass index, CP classification), patella height (Koshino-Sugimoto Index), and knee kinematics and kinetics. RESULTS: PA was present in 180 of 297 subjects (61%), in 68 of the 146 (47%) with unilateral CP, and 112 of 151 subjects (74%) with bilateral CP. For unilateral CP, the prevalence of PA was not significantly different between Gross Motor Function Classification System (GMFCS) I and II (P=0.357). For bilateral CP, the prevalence of PA in GMFCS III was significantly greater than in GMFCS I and II (P=0.02). Regression analysis showed a significant trend between increasing age and PA in unilateral and bilateral groups (P<0.001 and 0.001, respectively). The prevalence of PA was not significantly different across body mass index categories for either unilateral or bilateral groups. There were only 2 of 10 significant correlations between PA and gait parameters for subjects with unilateral CP functioning at the GMFCS I and II levels. There were 8 of 12 significant correlations between PA and gait parameters for subjects with bilateral CP functioning at the GMFCS I, II, and III levels. CONCLUSIONS: PA is common in ambulatory children with CP across topographic types and motor functional levels. PA is well tolerated with respect to gait dysfunction in unilateral CP, but may contribute to crouch gait in bilateral CP. Gait dysfunction cannot be inferred from the radiographic assessment of patellar height, and radiographic evidence of PA by itself does not justify surgical correction with patellar tendon advancement or shortening. LEVEL OF EVIDENCE: Level III-prognostic, retrospective series.