Hip Instability in Children With Spinal Muscular Atrophy: A Retrospective Study.

PURPOSE: Hip subluxation and dislocation are known problems for children with spinal muscular atrophy (SMA). Medical complexity of these children typically results in absence of monitoring and intervention for pathologic hips. Patterns of hip migration and acetabular morphology in SMA have not been described. This study examines the natural progression of hip instability across all types of SMA in a pediatric population. METHODS: Following institutional review board approval, a retrospective chart review of all children with SMA at our institution was performed. All x-rays taken before the age of 18 years containing adequate projections of the pelvis were measured for Reimer migration index (MI), acetabular depth ratio (ADR), and acetabular index (AI). Demographics and clinical data were collected including use of nusinersen, ambulatory status, contractures, and hip pain. Linear mixed effects model was fit to serial MI measures of individual hips with fixed effects consisting of SMA type, age at x-ray, and their interaction. ADR and AI measures were similarly modeled following conversion of raw values to z-scores based on the model developed by Novais et al Slope indicated rate of measure change as a function of age. RESULTS: Forty-five children (22 males) with SMA types 1 to 3 were included in this retrospective study. Six children were classified as type 1, 25 were type 2, and 14 were type 3. The interaction of age by SMA type was statistically significant (P=0.01), indicating a difference in the rate of hip subluxation between the 3 SMA types as measured by MI. By age 4, MI values were different from one another across all 3 groups (P<0.01). ADR decreased with age across all SMA types. The slopes of ADR regression lines were negative and statistically significant between the 3 groups (P=0.002). AI values were higher for all types of SMA, which is the opposite of expected in normal hips. CONCLUSIONS: Hip subluxation occurs across all SMA types, most rapidly in SMA type 1. Regression lines of ADR and AI compared with those seen in unaffected populations suggest hips in children with SMA demonstrate a difference in morphology of the acetabulum and do not follow normal adaptive remodeling. As treatments advance and the population of SMA patients becomes more mobile, there is an increased need to monitor hip instability in children with SMA, thus making orthopaedic management an important consideration.

Retrograde Insertion Approach for Anterior Distal Femoral Hemiepiphysiodesis Procedure: A Case Report.

CASE: A 13-year-old adolescent boy with spastic diplegic cerebral palsy (CP) presented with crouch gait and bilateral knee flexion contractures (KFCs). After failure of conservative treatments, the patient was referred to orthopaedics for surgical intervention. Anterior distal femoral hemiepiphysiodesis (ADFH), using a retrograde approach, and Strayer gastrocnemius recession were performed bilaterally. No complications were reported during surgery/follow-up. Full-active knee extension and improved gait were achieved. CONCLUSION: KFC is prevalent in ambulatory patients with CP, reducing gait function. Retrograde ADFH is an effective surgical intervention to reduce KFC and improve gait, and offers simpler insertion/removal than the traditional antegrade technique.

Two aspects of feed-forward control of action stability: effects of action speed and unexpected events.

We explored two types of anticipatory synergy adjustments (ASA) during accurate four-finger total force production task. The first type is a change in the index of force-stabilizing synergy during a steady state when a person is expecting a signal to produce a quick force change, which is seen even when the signal does not come (steady-state ASA). The other type is the drop in in the synergy index prior to a planned force change starting at a known time (transient ASA). The subjects performed a task of steady force production at 10% of maximal voluntary contraction (MVC) followed by a ramp to 20% MVC over 1 s, 3 s, and as a step function (0 s). In another task, in 50% of the trials during the steady-state phase, an unexpected signal could come requiring a quick force pulse to 20% MVC (0-surprise). Inter-trial variance in the finger force space was used to quantify the index of force-stabilizing synergy within the uncontrolled manifold hypothesis. We observed significantly lower synergy index values during the steady state in the 0-ramp trials compared to the 1-ramp and 3-ramp trials. There was also larger transient ASA during the 0-ramp trials. In the 0-surprise condition, the synergy index was significantly higher compared to the 0-ramp condition whereas the transient ASA was significantly larger. The finding of transient ASA scaling is of importance for clinical studies, which commonly involve populations with slower actions, which can by itself be associated with smaller ASAs. The participants varied the sharing pattern of total force across the fingers more in the task with "surprises". This was coupled to more attention to precision of performance, i.e., inter-trial deviations from the target as reflected in smaller variance affecting total force, possibly reflecting higher concentration on the task, which the participants perceived as more challenging compared to a similar task without surprise targets.

Three Levels of Neural Control Contributing to Performance-stabilizing Synergies in Multi-finger Tasks.

We tested a hypothesis on force-stabilizing synergies during four-finger accurate force production at three levels: (1) The level of the reciprocal and coactivation commands, estimated as the referent coordinate and apparent stiffness of all four fingers combined; (2) The level of individual finger forces; and (3) The level of firing of individual motor units (MU). Young, healthy participants performed accurate four-finger force production at a comfortable, non-fatiguing level under visual feedback on the total force magnitude. Mechanical reflections of the reciprocal and coactivation commands were estimated using small, smooth finger perturbations applied by the "inverse piano" device. Firing frequencies of motor units in the flexor digitorum superficialis (FDS) and extensor digitorum communis (EDC) were estimated using surface recording. Principal component analysis was used to identify robust MU groups (MU-modes) with parallel changes in the firing frequency. The framework of the uncontrolled manifold hypothesis was used to compute synergy indices in the spaces of referent coordinate and apparent stiffness, finger forces, and MU-mode magnitudes. Force-stabilizing synergies were seen at all three levels. They were present in the MU-mode spaces defined for MUs in FDS, in EDC, and pooled over both muscles. No effects of hand dominance were seen. The synergy indices defined at different levels of analysis showed no correlations across the participants. The findings are interpreted within the theory of control with spatial referent coordinates for the effectors. We conclude that force stabilization gets contributions from three levels of neural control, likely associated with cortical, subcortical, and spinal circuitry.

Force matching: motor effects that are not reported by the actor.

We explored unintentional drifts of finger forces during force production and matching task. Based on earlier studies, we predicted that force matching with the other hand would reduce or stop the force drift in instructed fingers while uninstructed (enslaved) fingers remain unaffected. Twelve young, healthy, right-handed participants performed two types of tasks with both hands (task hand and match hand). The task hand produced constant force at 20% of MVC level with the Index and Ring fingers pressing in parallel on strain gauge force sensors. The Middle finger force wasn't instructed, and its enslaved force was recorded. Visual feedback on the total force by the instructed fingers was either present throughout the trial or only during the first 5 s (no-feedback condition). The other hand matched the perceived force level of the task hand starting at either 4, 8, or 15 s from the trial initiation. No feedback was ever provided for the match hand force. After the visual feedback was removed, the task hand showed a consistent drift to lower magnitudes of total force. Contrary to our prediction, over all conditions, force matching caused a brief acceleration of force drift in the task hand, which then reached a plateau. There was no effect of matching on drifts in enslaved finger force. We interpret the force drifts within the theory of control with spatial referent coordinates as consequences of drifts in the command (referent coordinate) to the antagonist muscles. This command is not adequately incorporated into force perception.

Two classes of action-stabilizing synergies reflecting spinal and supraspinal circuitry.

We explored force-stabilizing synergies during accurate four-finger constant force production tasks in spaces of finger modes (commands to fingers computed to account for the finger interdependence) and of motor unit (MU) firing frequencies. The main specific hypothesis was that the multifinger synergies would disappear during unintentional force drifts without visual feedback on the force magnitude, whereas MU-based synergies would be robust to such drifts. Healthy participants performed four-finger accurate cyclical force production trials followed by trials of constant force production. Individual MUs were identified in the flexor digitorum superficialis (FDS) and extensor digitorum communis (EDC). Principal component analysis was applied to motor unit frequencies to identify robust MU groups (MU-modes) with parallel scaling of the firing frequencies in FDS, in EDC, and the combined MUs of FDS + EDC. The framework of the uncontrolled manifold hypothesis was used to quantify force-stabilizing synergies when visual feedback on the force magnitude was available and 15 s after turning the visual feedback off. Removing visual feedback led to a force drift toward lower magnitudes, accompanied by the disappearance of multifinger synergies. In contrast, MU-mode synergies were minimally affected by removing visual feedback off and continued to be robust for the FDS and for the EDC, while being absent for the (FDS + EDC) analysis. We interpret the findings within the theory of hierarchical control of action with spatial referent coordinates. The qualitatively different behavior of the multifinger and MU-mode-based synergies likely reflects the difference in the involved neural circuitry, supraspinal for the former and spinal for the latter.NEW & NOTEWORTHY Two types of synergies, in the space of commands to individual fingers and in the space of motor unit groups, show qualitatively different behaviors during accurate multifinger force-production tasks. After removing visual feedback, finger force synergies disappear, whereas motor unit-based synergies persist. These results point at different neural circuitry involved in these two basic classes of synergies: supraspinal for multieffector synergies, and spinal for motor unit-based synergies.

Effects of fatigue on intramuscle force-stabilizing synergies.

We applied the recently introduced concept of intramuscle synergies in spaces of motor units (MUs) to quantify indexes of such synergies in the tibialis anterior during ankle dorsiflexion force production tasks and their changes with fatigue. We hypothesized that MUs would be organized into robust groups (MU modes), which would covary across trials to stabilize force magnitude, and the indexes of such synergies would drop under fatigue. Healthy, young subjects (n = 15; 8 females) produced cyclical, isometric dorsiflexion forces while surface electromyography was used to identify action potentials of individual MUs. Principal component analysis was used to define MU modes. The framework of the uncontrolled manifold (UCM) was used to analyze intercycle variance and compute the synergy index, ΔVZ. Cyclical force production tasks were repeated after a nonfatiguing exercise (control) and a fatiguing exercise. Across subjects, fatigue led, on average, to a 43% drop in maximal force and fewer identified MUs per subject (29.6 ± 2.1 vs. 32.4 ± 2.1). The first two MU modes accounted for 81.2 ± 0.08% of variance across conditions. Force-stabilizing synergies were present across all conditions and were diminished after fatiguing exercise (1.49 ± 0.40) but not control exercise (1.76 ± 0.75). Decreased stability after fatigue was caused by an increase in the amount of variance orthogonal to the UCM. These findings contrast with earlier studies of multieffector synergies demonstrating increased synergy index under fatigue. We interpret the results as reflections of a drop in the gain of spinal reflex loops under fatigue. The findings corroborate an earlier hypothesis on the spinal nature of intramuscle synergies.NEW & NOTEWORTHY Across multielement force production tasks, fatigue of an element leads to increased indexes of force stability (synergy indexes). Here, however, we show that groups of motor units in the tibialis anterior show decreased indexes of force-stabilizing synergies after fatiguing exercise. These findings align intramuscle synergies with spinal mechanisms, in contrast to the supraspinal control of multimuscle synergies.

Motor unit-based synergies in a non-compartmentalized muscle.

The concept of synergies has been used to address the grouping of motor elements contributing to a task with the covariation of these elements reflecting task stability. This concept has recently been extended to groups of motor units with parallel scaling of the firing frequencies with possible contributions of intermittent recruitment (MU-modes) in compartmentalized flexor and extensor muscles of the forearm stabilizing force magnitude in finger pressing tasks. Here, we directly test for the presence and behavior of MU-modes in the tibialis anterior, a non-compartmentalized muscle. Ten participants performed an isometric cyclical dorsiflexion force production task at 1 Hz between 20 and 40% of maximal voluntary contraction and electromyographic (EMG) data were collected from two high-density wireless sensors placed on the skin over the right tibialis anterior. EMG data were decomposed into individual motor unit frequencies and resolved into sets of MU-modes. Inter-cycle analysis of MU-mode magnitudes within the framework of the uncontrolled manifold (UCM) hypothesis was used to quantify force-stabilizing synergies. Two or three MU-modes were identified in all participants and trials accounting, on average, for 69% of variance and were robust to cross-validation measurements. Strong dorsiflexion force-stabilizing synergies in the space of MU-modes were present in all participants and for both electrode locations as reflected in variance within the UCM (median 954, IQR 511-1924) exceeding variance orthogonal to the UCM (median 5.82, IQR 2.9-17.4) by two orders of magnitude. In contrast, MU-mode-stabilizing synergies in the space of motor unit frequencies were not present. This study offers strong evidence for the existence of synergic control mechanisms at the level of motor units independent of muscle compartmentalization, likely organized within spinal cord circuitry.

Rectus femoris transfers with and without a hamstring lengthening will not change hip kinematics in children with cerebral palsy.

BACKGROUND: A rectus femoris transfer (RFT) surgery with and without a hamstring lengthening (HSL) is used to treat stiff-knee gait in children with cerebral palsy (CP). While current literature has reported that a RFT surgery improves the kinematics at the knee, little is known about the kinematic changes at the hip. RESEARCH QUESTION: Does a RFT surgery change hip joint kinematics in children with CP? METHODS: This retrospective study included children (<18 years old) diagnosed with CP, who underwent a RFT procedure, and who were seen at our institution's accredited clinical motion laboratory. Patients with both pre- and post-operative gait analysis were identified and comparison between those analyses were performed to identify kinematic differences at the hip and knee. A total of 66 legs from 46 children (mean age: 11.1 ± 3.6) met the inclusion criteria. RESULTS: Overall results revealed that a RFT did not change kinematics at the hip [p > 0.05], however, a RFT did increase the maximum knee flexion during the swing period [Mean Difference Post - Pre: 8.3°, 95% CI: 4.9-11.8, p < 0.0001]. Additionally, it was found that changes in hip extension during the terminal stance phase were significantly different between the combined RFT and HSL compared to solely an RFT. The results of this study also revealed that children whose stiff-knee gait did not improve, tended to have increased hip external rotation during terminal stance and swing and greater hip extension during terminal stance, compared to children whose stiff-knee gait did improve. SIGNIFICANCE: Overall, a RFT with and without a HSL surgery improves hip and knee kinematics in the sagittal plane, however, improvements at the hip were not clinically significant. As a result, a RFT or a combined RFT with HSL should not be used to change hip kinematics in children with CP.

Delayed Radial Nerve Palsy after Closed Reduction of a Pediatric Humeral Shaft Fracture.

Humeral shaft fractures are common in the United States and may be associated with radial nerve injuries due to their close anatomic relationship in the spiral groove. Most radial nerve palsies are found at presentation due to the initial trauma; however, they can present secondary to orthopaedic intervention following reduction. In this case report, we present a case of delayed radial nerve palsy in a pediatric patient that was identified four days after closed reduction and splinting which required open reduction, nerve exploration, and internal fixation. Fortunately, full motor and sensory recovery was observed at 6 weeks post-op. A unique aspect of this case is that immediate postreduction exam in the emergency department showed no signs of injury or entrapment of the radial nerve.

Posterolaterally displaced and flexion-type supracondylar fractures are associated with a higher risk of open reduction.

To identify factors predictive of the risk of conversion from closed to open reduction. International Classification of Disease-9 codes were used to identify completely displaced pediatric supracondylar humerus fractures that were subjected to planned closed reduction and percutaneous pinning. Clinical and radiographic variables were retrospectively collected. Compared with posterior extension fractures, flexion (risk ratio: 34.1, 95% confidence interval: 8.1-143.6, P<0.0001) and posterolateral extension (risk ratio: 6.0, 95% confidence interval: 1.3-27.5, P=0.0221) fractures were significantly more likely to undergo conversion from closed to open reduction. The direction of displacement should be considered during the preoperative evaluation of supracondylar fractures.

Fracture of the cuboid.

Cuboid fracture accounts for a minority of all foot fractures in adults and often is indicative of a multiply injured foot. Understanding the normal anatomy and function of the cuboid and its relation to foot biomechanics is necessary for appropriate management. Clinical evaluation includes history, physical examination, and thorough assessment of the skin and soft tissues. Plain radiographs and CT are helpful in preoperative planning. Cuboid fractures may be managed either nonsurgically (splinting or casting) or surgically (closed reduction and external fixation or open reduction and internal fixation). Careful handling of the soft tissues is important, as is restoration of articular congruity, lateral column length, and a stable midfoot. Postoperative care consists of prolonged immobilization followed by 3 months of progressive weight bearing. Published reports of long-term outcomes and functional postoperative assessments are lacking.