Distal femoral osteotomy and patellar tendon advancement for the treatment of crouch gait in patients with bilateral spastic cerebral palsy.

BACKGROUND: Crouch gait, or flexed knee gait, represents a common gait pattern in patients with spastic bilateral cerebral palsy (CP). Distal femoral extension and/or shortening osteotomy (DFEO/DFSO) and patellar tendon advancement (PTA) can be considered as viable options when knee flexion contractures are involved. Better outcomes have been reported after a combination of both, independently of the presence of knee extensor lag. In this study, we evaluated the clinical and kinematic outcomes of these procedures. PATIENTS AND METHODS: We reviewed a cohort of 52 limbs (28 patients) who were treated for crouch gait by DFEO/DFSO alone (group 1, n = 15) or DFEO/DFSO + PTA (group 2, n = 37) as a part of single event multilevel surgery (SEMLS). The mean age at surgery was 14 years, and the mean follow-up time was 18 months. The physical examination data and three-dimensional standardized gait analysis were collected and analyzed before the surgery and postoperatively. RESULTS: Overall knee range of motion improved in all limbs. The knee flexion decreased significantly in both groups at initial, mid, and terminal stance. Hip flexion significantly decreased in mid-stance for limbs in group 2. Both clinical and gait parameters were most improved in limbs who underwent DFEO/DFSO + PTA. Increased pelvic tilt was observed in both groups after surgery. CONCLUSION: Although DFEO/DFSO alone was successful in correcting knee flexion contractures, PTA has helped to improve knee extensor lag and knee extension during gait. LEVEL OF EVIDENCE: Therapeutic level IV.

Excessive mechanotransduction in sensory neurons causes joint contractures.

Distal arthrogryposis (DA) is a collection of rare disorders that are characterized by congenital joint contractures. Most DA mutations are in muscle- and joint-related genes, and the anatomical defects originate cell-autonomously within the musculoskeletal system. However, gain-of-function mutations in PIEZO2, a principal mechanosensor in somatosensation, cause DA subtype 5 (DA5) through unknown mechanisms. We show that expression of a gain-of-function PIEZO2 mutation in proprioceptive sensory neurons that mainly innervate muscle spindles and tendons is sufficient to induce DA5-like phenotypes in mice. Overactive PIEZO2 causes anatomical defects through increased activity within the peripheral nervous system during postnatal development. Furthermore, botulinum toxin (Botox) and a dietary fatty acid that modulates PIEZO2 activity reduce DA5-like deficits. This reveals a role for somatosensory neurons: Excessive mechanosensation within these neurons disrupts musculoskeletal development.

Ankle contractures and functional motor decline in Duchenne muscular dystrophy.

INTRODUCTION: This prospective, correlational pilot study investigated the relationship between ankle plantar flexion contractures and motor function in boys with Duchenne muscular dystrophy in British Columbia (BC), Canada. PARTICIPANTS: Ambulatory boys with Duchenne muscular dystrophy were recruited from BC Children's Hospital, which follows everyone with Duchenne muscular dystrophy in BC ≤ 18 years of age (n = 14). METHODS: Spearman and Pearson correlation coefficients were estimated to examine the association between the degree of ankle dorsiflexion range of motion and North Star Ambulatory Assessment scores and the degree of ankle dorsiflexion range and six-minute walk test distances. RESULTS: Our analysis showed a moderate correlation between the degree of ankle dorsiflexion range and North Star Ambulatory Assessment scores [rho (14) = 0.50; p = 0.070] and a weak correlation between ankle dorsiflexion range of motion and six-minute walk test distances [rho (13) = 0.08; p = 0.747], however neither result was statistically significant. DISCUSSION: Although a significant relationship between ankle dorsiflexion range of motion and motor function was not found, the variability of ankle dorsiflexion range suggests challenges with preventing ankle contracture. This reinforces the importance of assessing ankle range of motion in boys with Duchenne muscular dystrophy with sufficient frequency to identify a need for additional interventions.

3D-printed models improve surgical planning for correction of severe postburn ankle contracture with an external fixator.

Gradual distraction with an external fixator is a widely used treatment for severe postburn ankle contracture (SPAC). However, application of external fixators is complex, and conventional two-dimensional (2D) imaging-based surgical planning is not particularly helpful due to a lack of spatial geometry. The purpose of this study was to evaluate the surgical planning process for this procedure with patient-specific three-dimension-printed models (3DPMs). In this study, patients coming from two centers were divided into two cohorts (3DPM group vs. control group) depending on whether a 3DPM was used for preoperative surgical planning. Operation duration, improvement in metatarsal-tibial angle (MTA), range of motion (ROM), the American Orthopedic Foot and Ankle Society (AOFAS) scores, complications, and patient-reported satisfaction were compared between two groups. The 3DPM group had significantly shorter operation duration than the control group ((2.0±0.3) h vs. (3.2±0.3) h, P<0.01). MTA, ROM, and AOFAS scores between the two groups showed no significant differences pre-operation, after the removal of the external fixator, or at follow-up. Plantigrade feet were achieved and gait was substantially improved in all patients at the final follow-up. Pin-tract infections occurred in two patients (one in each group) during distraction and were treated with wound care and oral antibiotics. Patients in the 3DPM group reported higher satisfaction than those in the control group, owing to better patient-surgeon communication. Surgical planning using patient-specific 3DPMs significantly reduced operation duration and increased patient satisfaction, while providing similar improvements in ankle movement and function compared to traditional surgical planning for the correction of SPAC with external fixators.

Pes Cavus Deformity: Anatomic, Functional Considerations, and Surgical Implications.

Pes cavus is a complicated, multiplanar deformity that requires a thorough understanding in order to provide the appropriate level of care. The foot and ankle surgeon should perform a comprehensive examination, including a neurologic evaluation, in the workup of this patient population. Understanding the cause of the patient's deformity is a critical step in predicting the disease course as well as the most acceptable form of treatment. The surgical correlation with the patient's pathologic anatomy requires an in-depth clinical evaluation, in addition to the radiographic findings, as the radiographic findings do not necessarily correlate with the patient's discomfort.

Knee flexion contracture impacts functional mobility in children with cerebral palsy with various degree of involvement: a cross-sectional register study of 2,838 individuals.

Background and purpose - The impact of knee flexion contracture (KFC) on function in cerebral palsy (CP) is not clear. We studied KFC, functional mobility, and their association in children with CP.Subjects and methods - From the Swedish national CP register, 2,838 children were defined into 3 groups: no (≤ 4°), mild (5-14°), and severe (≥ 15°) KFC on physical examination. The Functional Mobility Scale (FMS) levels were categorized: using wheelchair (level 1), using assistive devices (level 2-4), walking independently (level 5-6). Standing and transfer ability and Gross Motor Function Classification (GMFCS) were assessed.Results - Of the 2,838 children, 73% had no, 14% mild, and 13% severe KFC. KFC increased from 7% at GMFCS level I to 71% at level V. FMS assessment (n = 2,838) revealed around 2/3 were walking independently and 1/3 used a wheelchair. With mild KFC (no KFC as reference), the odds ratio for FMS level 1 versus FMS level 5-6 at distances of 5, 50, and 500 meters, was 9, 9, and 8 respectively. Correspondingly, with severe KFC, the odds ratio was 170, 260, and 217. In no, mild, and severe KFC 14%, 47%, and 77% could stand with support and 11%, 25%, and 33% could transfer with support.Interpretation - Knee flexion contracture is common in children with CP and the severity of KFC impacts function. The proportion of children with KFC rose with increased GMFCS level, reduced functional mobility, and decreased standing and transfer ability. Therefore, early identification and adequate treatment of progressive KFC is important.

Acupotomy treatment for finger joint contracture after immobilization: Case report.

INTRODUCTION: Contractures frequently occur in the finger joints after immobilization. This report describes the effect of acupotomy treatment in patients with joint contracture due to immobilization of the finger joints. PATIENT CONCERNS AND CLINICAL FINDINGS: Case 1 was of a 39-year-old male patient who had flexion limitation of the left thumb and difficulty in grasping. Case 2 was of a 41-year-old female patient who had flexion limitation of the right index finger and difficulty in typing. Stiffness occurred after tendon repair surgery and cast immobilization in both cases. In Case 1, the patient had limited flexion movement of the first metacarpophalangeal and interphalangeal joints after 5 weeks of immobilization of the left thumb in a cast. In Case 2, the patient had limited flexion movement after 3 weeks of immobilization of the second proximal interphalangeal joint of the left hand in a cast. DIAGNOSIS, INTERVENTIONS, AND OUTCOMES: We diagnosed both patients with finger joint contracture due to immobilization. Conservative treatment for approximately 4 weeks did not lead to improvement in either patient. Acupotomy is the key treatment for improving movement in Korean Medicine. Therefore, acupotomy was performed, and joint stiffness markedly improved without adverse events. Both patients reported that the daily use of the damaged fingers became comfortable. CONCLUSION: We found that acupotomy may be effective for finger joint contracture due to improper immobilization. We suggest it as a simple and safe treatment for joint contracture.

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.

Neurophysiological validation of simultaneous intrinsic and reflexive joint impedance estimates.

BACKGROUND: People with brain or neural injuries, such as cerebral palsy or spinal cord injury, commonly have joint hyper-resistance. Diagnosis and treatment of joint hyper-resistance is challenging due to a mix of tonic and phasic contributions. The parallel-cascade (PC) system identification technique offers a potential solution to disentangle the intrinsic (tonic) and reflexive (phasic) contributions to joint impedance, i.e. resistance. However, a simultaneous neurophysiological validation of both intrinsic and reflexive joint impedances is lacking. This simultaneous validation is important given the mix of tonic and phasic contributions to joint hyper-resistance. Therefore, the main goal of this paper is to perform a group-level neurophysiological validation of the PC system identification technique using electromyography (EMG) measurements. METHODS: Ten healthy people participated in the study. Perturbations were applied to the ankle joint to elicit reflexes and allow for system identification. Participants completed 20 hold periods of 60 seconds, assumed to have constant joint impedance, with varying magnitudes of intrinsic and reflexive joint impedances across periods. Each hold period provided a paired data point between the PC-based estimates and neurophysiological measures, i.e. between intrinsic stiffness and background EMG, and between reflexive gain and reflex EMG. RESULTS: The intrinsic paired data points, with all subjects combined, were strongly correlated, with a range of [Formula: see text] in both ankle plantarflexors and dorsiflexors. The reflexive paired data points were moderately correlated, with [Formula: see text] in the ankle plantarflexors only. CONCLUSION: An agreement with the neurophysiological basis on which PC algorithms are built is necessary to support its clinical application in people with joint hyper-resistance. Our results show this agreement for the PC system identification technique on group-level. Consequently, these results show the validity of the use of the technique for the integrated assessment and training of people with joint hyper-resistance in clinical practice.

Wrist Contracture Caused by Adhesion of the Extensor Carpi Radialis Tendon after Distal Radius Fracture: A Case Report.

Although distal radius fractures are common, wrist contracture caused by an extra-articular lesion after a distal radius fracture is seldom reported. We report a rare case of wrist contracture caused by adhesion of extensor carpi radialis brevis (ECRB) tendon after distal radius fracture. The patient was successfully treated with tenolysis of the ECRB tendon.

The effect of hamstring tightness on intraoperative extension gap in posterior stabilized total knee arthroplasty.

This study aimed to determine the factors related to intraoperative extension gap (EG) in patients who underwent posterior-stabilized total knee arthroplasty (TKA). A total of 106 TKAs in 84 patients were retrospectively reviewed. Only patients who underwent the same method of bone resection were included consecutively. Bilateral popliteal angle (BPA) was used as an indicator of hamstring tightness. EG and extension space angle were measured using an offset type tensor. The associations between patient variables and EG were analyzed using multivariable linear regression and Pearson's correlation coefficients. The average EG was 12.9 ± 2.1 mm, and the average extension space angle was 2.8° ± 3.2°. BPA was greater than flexion contracture in most cases (94.3%), and no difference was found in only six cases (5.7%). According to multivariable linear regression analysis which was conducted after modifying the BPA into a categorical variable by 5°, EG was correlated with BPA (p < 0.001). Pearson's correlation coefficient between EG and BPA was - 0.674 (p < 0.001). No other factors were significantly correlated with intraoperative EG. The present study found that popliteal angle is a different entity from flexion contracture, and that it is a predictable factor for EG in osteoarthritis patients. Smaller BPAs led to larger EG in patients who underwent the same degree of bone resection.

NRG/ErbB signaling regulates neonatal muscle growth but not neuromuscular contractures in neonatal brachial plexus injury.

Neonatal brachial plexus injury (NBPI) causes disabling and incurable muscle contractures that are driven by impaired growth of denervated muscles. A rare form of NBPI, which maintains afferent muscle innervation despite motor denervation, does not cause contractures. As afferent innervation regulates various aspects of skeletal muscle homeostasis through NRG/ErbB signaling, our current study investigated the role of this pathway in modulating contracture development. Through pharmacologic modification with an ErbB antagonist and NRG1 isoforms, we discovered that NRG/ErbB signaling does not modulate the development of contractures in neonatal mice. Instead, ErbB inhibition impeded growth in nondenervated skeletal muscles, whereas increased ErbB activation exacerbated denervation-induced skeletal muscle atrophy. This potential regulatory effect of NRG/ErbB signaling on neonatal muscle growth warrants deeper investigation.

Diverse manifestations of a sickle cell crisis.

We describe the case of a 21-year-old man with a background of sickle cell disease (SCD) who was on acute presentation in a sickle cell crisis required immediate intensive care admission with red blood cell exchange and ventilatory support. He had right frontal lobe infarcts and extensive bilateral deep white matter lesions most likely secondary to fat embolism. Inpatient investigations demonstrated a patent foramen ovale, explaining the route of spread of the fat embolus. He then had a transcatheter closure of the atrial defect. The patient needed prolonged inpatient rehabilitation. He was discharged from hospital in a wheelchair secondary to severe lower limb neurology and bilateral knee heterotopic ossification. He lives with the possibility of early onset dementia and cognitive decline, requiring constant care. The case highlights the multiple manifestations of SCD and their diverse and debilitating consequences.

Contracture Severity at Hospital Discharge in Children: A Burn Model System Database Study.

Contractures can complicate burn recovery. There are limited studies examining the prevalence of contractures following burns in pediatrics. This study investigates contracture outcomes by location, injury, severity, length of stay, and developmental stage. Data were obtained from the Burn Model System between 1994 and 2003. All patients younger than the age of 18 with at least one joint contracture at hospital discharge were included. Sixteen areas of impaired movement from the shoulder, elbow, wrist, hand, hip, knee, and ankle joints were examined. Analysis of variance was used to assess the association between contracture severity, burn size, and length of stay. Age groupings were evaluated for developmental patterns. A P value of less than .05 was considered statistically significant. Data from 225 patients yielded 1597 contractures (758 in the hand) with a mean of 7.1 contractures (median 4) per patient. Mean contracture severity ranged from 17% (elbow extension) to 41% (ankle plantarflexion) loss of movement. Statistically significant associations were found between active range of motion loss and burn size, length of stay, and age groupings. The data illustrate quantitative assessment of burn contractures in pediatric patients at discharge in a multicenter database. Size of injury correlates with range of motion loss for many joint motions, reflecting the anticipated morbidity of contracture for pediatric burn survivors. These results serve as a potential reference for range of motion outcomes in the pediatric burn population, which could serve as a comparison for local practices, quality improvement measures, and future research.

Knee and foot contracture occur earliest in children with cerebral palsy: a longitudinal analysis of 2,693 children.

Background and purpose - Joint contracture is a common problem among children with cerebral palsy (CP). To prevent severe contracture and its effects on adjacent joints, it is crucial to identify children with a reduced range of motion (ROM) early. We examined whether significant hip, knee, or foot contracture occurs earliest in children with CP.Patients and methods - This was a longitudinal study involving 27,230 measurements obtained for 2,693 children (59% boys, 41% girls) with CP born 1990 to 2018 and registered before 5 years of age in the Swedish surveillance program for CP. The analysis was based on 4,751 legs followed up for an average of 5.0 years. Separate Kaplan-Meier (KM) curves were drawn for each ROM to illustrate the proportions of contracture-free legs at a given time during the follow-up. Using a clustered bootstrap method and considering the child as the unit of clustering, 95% pointwise confidence intervals were generated for equally spaced time points every 2.5 years for each KM curve.Results - Contracture developed in 34% of all legs, and the median time to the first contracture was 10 years from the first examination. Contracture was most common in children with a higher Gross Motor Function Classification System (GMFCS) level. The first contracture was a flexion contracture preventing dorsiflexion in children with GMFCS level I or II and preventing knee extension in children with GMFCS level III to V.Interpretation - Early interventions to prevent knee and foot contractures in children with CP should be considered.

[Stiff skin syndrome in a pediatric patient: a therapeutic challenge. Clinical case]. / Síndrome de piel indurada en un paciente pediátrico: un desafío terapéutico. Presentación de un caso.

Stiff skin syndrome is a chronic, rare sclerosing disorder that occurs in childhood, characterized by progressive induration of the skin that can cause thoracic restrictions and respiratory distress, limitations in joint mobility and gait difficulties, with significant deterioration of the quality of life. Because their therapeutic options are scarce and ineffective it is essential to start an early physical therapy to prevent these complications and to continue studying this condition to be able to offer patients more and better treatments. We present the case of a 9-year-old patient with indurated skin syndrome and its therapeutic challenge.

El síndrome de la piel indurada es un trastorno esclerosante crónico, infrecuente, que se presenta en la infancia, caracterizado por la induración progresiva de la piel. Esta afección puede provocar restricciones torácicas y dificultad respiratoria, limitaciones en la movilidad articular y trastornos en la marcha, con importante deterioro de la calidad de vida. Debido a que sus opciones terapéuticas son escasas y poco eficaces, es fundamental que el paciente inicie precozmente una terapia física para prevenir estas complicaciones y que se continúe estudiando esta enfermedad a fin de poder ofrecer a los pacientes más y mejores tratamientos. Se presenta el caso de una paciente de 9 años con síndrome de la piel indurada y su desafío terapéutico.

Macroscopic changes in sural nerve after gastrocnemius recession: a cadaver study.

BACKGROUND: Gastrocnemius recession is a common foot and ankle procedure and various techniques that have been utilized are mainly delineated by the anatomic position of the gastrocnemius transection; the 2 common ones are the Baumann and Strayer procedure. Both can adversely affect the sural nerve. The objective of this study was to evaluate the macroscopic changes in the sural nerve following gastrocnemius recession, and to compare the efficacy of the two procedures, regarding the improvement of maximal ankle dorsiflexion. METHODS: Ten fresh-frozen, above knee cadaveric legs were assigned to one of two gastrocnemius recession techniques: Baumann (n = 5) or Strayer (n = 5). A goniometer was used to measure degree of ankle dorsiflexion before and after the surgery. The sural nerve was meticulously dissected and marked with two suture knots, 2 cm apart. The ankle was passively dorsiflexed from 90° to maximal dorsiflexion in 5° degree increments, and the distance between two suture knots was measured at each increment. The distance between the two cut ends of gastrocnemius muscle was measured with the ankle at 90° and at maximal dorsiflexion. RESULTS: Overall, a mean increase in length between the suture knots on the sural nerve was 0.2 cm, from 90° to maximum ankle dorsiflexion (130°); both the Baumann and Strayer techniques resulted in 0.2 cm increase. The mean improvement in maximal ankle dorsiflexion in the Baumann and Strayer group was 22.6° and 22°, respectively. The mean change in distance between the two cut ends of the gastrocnemius muscle in the Baumann and Strayer group was 1.0 cm and 0.9 cm, respectively. CONCLUSION: Increased dorsiflexion of the ankle following Strayer or Baumann gastrocnemius recession resulted in similar macroscopic change in the sural nerve, which may contribute to the development of sural neuritis. Further clinical studies are warranted to assess clinical implications of these findings.

Effects of lateral opening wedge and medial closing wedge distal femoral osteotomies on axial load stability.

BACKGROUND: The stability of the current distal femoral osteotomy is insufficient for early weight bearing and bone healing. The purpose of this study was to test the stability of medial closing wedge- (MCW-) and lateral opening wedge-distal femoral osteotomies (LOW-DFO), which have improved with the recent advances in technology. METHODS: We used composite bone models with MCW-DFO, improved with biplanar osteotomy technique and a Tomofix™ MDF plate, or LOW-DFO improved with biplanar osteotomy technique, a Tomofix™ LDF plate, and a ß-TCP bone substitute, BONISH® graft. A cyclic axial loading test was employed to evaluate the strain on a plate and hinge point. The breaking axial load was measured. RESULTS: Tensile strain on the plate produced by cyclic axial loading was significantly lower for LOW-DFO (2.0 ± 0.8 MPa) than for MCW-DFO (3.9 ± 1.6 MPa, P < .05). Compressive strain on the hinge point produced by cyclic axial loading was lower for LOW-DFO (6.6 ± 2.9 MPa) than for MCW-DFO (7.7 ± 4.6 MPa,). The maximum breaking axial load was significantly higher for LOW-DFO (5511 ± 945 N) than for MCW-DFO (4303 ± 518 N, P < .05). CONCLUSIONS: LOW-DFO improved with recent advanced technology was superior to MCW-DFO improved with advanced technology in both cyclic axial loading test and breaking axial load test. This suggests that LOW-DFO facilitates earlier weight bearing and bone healing than does MCW-DFO.