European consensus on the concepts and measurement of the pathophysiological neuromuscular responses to passive muscle stretch.

BACKGROUND AND PURPOSE: To support clinical decision-making in central neurological disorders, a physical examination is used to assess responses to passive muscle stretch. However, what exactly is being assessed is expressed and interpreted in different ways. A clear diagnostic framework is lacking. Therefore, the aim was to arrive at unambiguous terminology about the concepts and measurement around pathophysiological neuromuscular response to passive muscle stretch. METHODS: During two consensus meetings, 37 experts from 12 European countries filled online questionnaires based on a Delphi approach, followed by plenary discussion after rounds. Consensus was reached for agreement ≥75%. RESULTS: The term hyper-resistance should be used to describe the phenomenon of impaired neuromuscular response during passive stretch, instead of for example 'spasticity' or 'hypertonia'. From there, it is essential to distinguish non-neural (tissue-related) from neural (central nervous system related) contributions to hyper-resistance. Tissue contributions are elasticity, viscosity and muscle shortening. Neural contributions are velocity dependent stretch hyperreflexia and non-velocity dependent involuntary background activation. The term 'spasticity' should only be used next to stretch hyperreflexia, and 'stiffness' next to passive tissue contributions. When joint angle, moment and electromyography are recorded, components of hyper-resistance within the framework can be quantitatively assessed. CONCLUSIONS: A conceptual framework of pathophysiological responses to passive muscle stretch is defined. This framework can be used in clinical assessment of hyper-resistance and will improve communication between clinicians. Components within the framework are defined by objective parameters from instrumented assessment. These parameters need experimental validation in order to develop treatment algorithms based on the aetiology of the clinical phenomena.

Botulinum neurotoxin type A responders among children with spastic cerebral palsy: Pattern-specific effects.

AIM: To identify short-term effects of botulinum neurotoxin type A (BoNT) injections on gait and clinical impairments, in children with spastic cerebral palsy (CP), based on baseline gait pattern-specific subgroups. METHOD: Short-term effects of BoNT injections in the medial hamstrings and gastrocnemius were defined in a retrospective convenience sample of 117 children with CP (median age: 6 years 4 months; GMFCS I/II/III: 70/31/16; unilateral/bilateral: 56/61) who had received gait analyses before and 2 months post-BoNT. First, baseline gait patterns were classified. Statistical and meaningful changes were calculated between pre- and post-BoNT lower limb sagittal plane kinematic waveforms, the gait profile score, and non-dimensional spatiotemporal parameters for the entire sample and for pattern-specific subgroups. These gait waveforms per CP subgroup at pre- and post-BoNT were also compared to typically developing gait and composite scores for spasticity, weakness, and selectivity were compared between the two conditions. RESULTS: Kinematic improvements post-BoNT were identified at the ankle and knee for the entire sample, and for subgroups with apparent equinus and jump gait. Limbs with baseline patterns of dropfoot and to a lesser extent true equinus showed clear improvements only at the ankle. In apparent equinus, jump gait, and dropfoot, spasticity improved post-BoNT, without leading to increased weakness or diminished selectivity. Compared to typical gait, knee and hip motion improved in the crouch gait subgroup post-BoNT. CONCLUSION: This comprehensive analysis highlighted the importance of investigating BoNT effects on gait and clinical impairments according to baseline gait patterns. These findings may help identify good treatment responders.

Botulinum toxin assessment, intervention and after-care for lower limb spasticity in children with cerebral palsy: international consensus statement.

Botulinum neurotoxin type-A (BoNT-A) has been used in association with other interventions in the management of spasticity in children with cerebral palsy (CP) for almost two decades. This consensus statement is based on an extensive review of the literature by an invited international committee. The use of BoNT-A in the lower limbs of children with spasticity caused by CP is reported using the American Academy of Neurology Classification of Evidence for therapeutic intervention. Randomized clinical trials have been grouped into five areas of management, and the outcomes are presented as treatment recommendations. The assessment of children with CP and evaluation of outcomes following injection of BoNT-A are complex, and therefore, a range of measures and the involvement of a multidisciplinary team is recommended. The committee concludes that injection of BoNT-A in children with CP is generally safe although systemic adverse events may occur, especially in children with more physical limitations (GMFCS V). The recommended dose levels are intermediate between previous consensus statements. The committee further concludes that injection of BoNT-A is effective in the management of lower limb spasticity in children with CP, and when combined with physiotherapy and the use of orthoses, these interventions may improve gait and goal attainment.

A systematic review of arm activity measures for children with hemiplegic cerebral palsy.

OBJECTIVE: To identify psychometrically sound and clinically feasible assessments of arm activities in children with hemiplegic cerebral palsy for implementation in research and clinical practice. DATA SOURCES: PubMed, CINAHL, Cochrane Library, Web of Science and reference lists of relevant articles were searched. REVIEW METHODS: A systematic search was performed based on the following inclusion criteria: (1) evaluative tools at the activity level according to the International Classification of Functioning, Disability and Health; (2) previously used in studies including children with hemiplegic cerebral palsy aged 2-18 years; (3) at least one aspect of reliability and validity in children with cerebral palsy should be established. Descriptive information, psychometric properties and clinical utility were reviewed. RESULTS: Eighteen assessments were identified of which 11 met the inclusion criteria: eight functional tests and three questionnaires. Five functional tests were condition-specific, three were generic. All functional tests measure different aspects of activity, including unimanual capacity and performance during bimanual tasks. The questionnaires obtain information about the child's abilities at home or school. The reliability and validity have been established, though further use in clinical trials is necessary to determine the responsiveness. CONCLUSIONS: To obtain a complete view of what the child can do and what the child actually does, we advise a capacity-based test (Melbourne Assessment of Unilateral Upper Limb Function), a performance-based test (Assisting Hand Assessment) and a questionnaire (Abilhand-Kids). This will allow outcome differentiation and treatment guidance for the arm in children with cerebral palsy.

Single-event multilevel surgery, but not botulinum toxin injections normalize joint loading in cerebral palsy patients.

BACKGROUND: Many patients with cerebral palsy present a pathologic gait pattern, which presumably induces aberrant musculoskeletal loading that interferes with natural bone growth, causing bone deformations on the long term. Botulinum toxin interventions and single-event multilevel surgeries are used to restore the gait pattern, assuming that a normal gait pattern restores musculoskeletal loading and thus prevents further bone deformation. However, it is unknown if these interventions are able to restore musculoskeletal loading. Hence, we investigated the impact of botulinum toxin injections and single-event multilevel surgery on musculoskeletal loading. METHODS: Gait data collected in 93 children with bilateral cerebral palsy, which included pre- and post multi-level botulinum toxin (49 children) and single-event multilevel surgery (44 children) assessments, and 15 typically developing children were retrospectively processed using a musculoskeletal modelling workflow to calculate joint angles, moments, muscle and joint contact force magnitudes and orientations. Differences from the typically developing waveform were expressed by a root-mean square difference were compared using paired t-tests for each intervention separately (alpha <0.05). FINDINGS: Botulinum toxin induced significant changes in the joint angles, but did not improve the muscle and joint contact forces. Single-event multilevel surgery induced significant kinematic and kinetic changes, which were associated with improved muscle and joint contact forces. INTERPRETATION: The present results indicate that botulinum toxin injections were not able to restore normal gait kinematics nor musculoskeletal loading, whereas single-event multilevel surgery did successfully restore both. Therefore, single-event multilevel surgery might be protective against the re-occurrence of bone deformation on the longer term.

Comparison of the Melbourne Assessment of Unilateral Upper Limb Function and the Quality of Upper Extremity Skills Test in hemiplegic CP.

This study investigated interrater reliability and measurement error of the Melbourne Assessment of Unilateral Upper Limb Function (Melbourne Assessment) and the Quality of Upper Extremity Skills Test (QUEST), and assessed the relationship between both scales in 21 children (15 females, six males; mean age 6y 4mo [SD 1y 3mo], range 5-8y) with hemiplegic CP. Two raters scored the videotapes of the assessments independently in a randomized order. According to the House Classification, three participants were classified as level 1, one participant as level 3, eight as level 4, three as level 5, one participant as level 6, and five as level 7. The Melbourne Assessment and the QUEST showed high interrater reliability (intraclass correlation 0.97 for Melbourne Assessment; 0.96 for QUEST total score; 0.96 for QUEST hemiplegic side). The standard error of measurement and the smallest detectable difference was 3.2% and 8.9% for the Melbourne Assessment and 5.0% and 13.8% for the QUEST score on the hemiplegic side. Correlation analysis indicated that different dimensions of upper limb function are addressed in both scales.

Clinical value of botulinum toxin in neurological indications.

Botulinum toxin type-A (BoNT-A) prevents the release of acetylcholine at cholinergic junctions, thereby causing temporary muscle weakness lasting 3-4 months. It is now widely used to treat a broad range of clinical disorders characterized by muscle hyperactivity. BoNT-A has proved effective in the management of several neurological conditions and, in particular, in the management of movement disorders (e.g. blepharospasm, cervical dystonia, laryngeal dystonia, limb dystonia, hemifacial spasm, focal tics, tremor and other hyperkinetic disorders). As a treatment of spasticity, BoNT-A can improve mobility and dexterity as well as preventing the development of distressing and costly secondary complications. In cerebral palsy, BoNT-A is of value, being able to delay or even avoid surgery until motion patterns have become established.

Safety and efficacy of botulinum toxin type A following long-term use.

Botulinum toxin serotype A (BoNT-A) has long heritage of use leading to confidence in its safety and efficacy. The application of BoNT-A does not lead to persistent histological changes in the nerve terminal or the target muscle. Clinical trials defined the safety and tolerability profile of BoNT-A across common therapeutic indications and showed an incidence of adverse events of approximately 25% in the BoNT-A-treated group compared with 15% in the control group. Focal weakness was the only adverse event to occur more often following BoNT-A treatment. Long-term BoNT-A administration has been assessed in various treatment settings, with the level and duration of BoNT-A efficacy response being maintained over repeated rounds of injection with no major safety concerns. The treatment of children with cerebral palsy often require long-term, repeated, multimuscle BoNT-A injections that lead to the administration of comparably higher toxin doses. Despite the high total body doses used, their distribution over multiple muscles and injection sites means that systemic side effects are rare. Recent formulation changes have reduced the incidence of antibody development following treatment with BOTOX. These findings show long-term BoNT-A treatment to be both safe and efficacious for a wide variety of indications.

An evaluator-blinded randomized controlled trial evaluating therapy effects and prognostic factors for a general and an individually defined physical therapy program in ambulant children with bilateral spastic cerebral palsy.

BACKGROUND: Cerebral palsy (CP) is characterized by a heterogeneous nature with a variety of problems. Therefore, individualized physical therapy might be more appropriate to address the needs for these children. AIM: The first aim was to compare the effectiveness of an individually-defined therapy program (IT) and a general therapy program (GT) on gait and gross motor function in children with CP. The second aim was to evaluate interaction-effects, time-effects, treatment with botulinum toxin A, age, gross Motor Function Classification Scale (GMFCS), treatment frequency and quality as factors influencing outcome. DESIGN: An evaluator-blinded, randomized controlled trial. SETTING: Outpatient rehabilitation unit. POPULATION: Forty ambulant children with spastic bilateral CP (mean age 6 years 1 month). METHODS: All children were randomly assigned to receive either IT or GT over a 10 week period. Nineteen of these children were enrolled into a second and/or third program, resulting in 60 interventions. Primary outcome was assessed with the Goal Attainment Scale (GAS) for gross motor function goals and z-scores for goals based on specific 3D gait parameters. Secondary outcome included the Gross Motor Function Measure-88 (GMFM-88) scores, time and distance gait parameters, Gait Profile Score, Movement Analysis Profiles and time needed to complete Timed-Up-and-Go and Five-Times-Sit-To-Stand tests. RESULTS: There were higher, but non-significant GAS and z-score changes following the IT program compared to the GT program (GAS: 46.2 for the IT versus 42.2 for the GT group, P=0.332, ES 0.15; z-score: 0.135 for the IT compared to 0.072 for the GT group, P=0.669, ES 0.05). Significant time-effects could be found on the GAS (P<0.001) and the GMFM-88 total score (P<0.001). Age was identified as a predictor for GAS and GMFM-88 improvement (P=0.023 and P=0.044). CONCLUSION: No significant differences could be registered between the effects of the IT and the GT. The favorable outcome after the IT program was only a trend and needs to be confirmed on larger groups and with programs of longer duration. CLINICAL REHABILITATION IMPACT: Both programs had a positive impact on the children's motor functioning. It is useful to involve older children more actively in the process of goal setting.

Three-dimensional imaging of acetabular dysplasia: diagnostic value and impact on surgical type classification.

OBJECTIVE: To investigate the diagnostic value and the impact on surgical type classification of three-dimensional (3D) images for pre-surgical evaluation of dysplastic hips. MATERIALS AND METHODS: Three children with a different surgical type of hip dysplasia were investigated with helical computed tomography. For each patient, two-dimensional (2D) images, 3D, and a stereolithographic model of the dysplastic hip were generated. In two separate sessions, 40 medical observers independently analyzed the 2D images (session 1), the 2D and 3D images (session 2), and tried to identify the corresponding stereolithographic hip model. The influence of both image presentation (2D versus 3D images) and observer (degree of experience, radiologist versus orthopedic surgeon) were statistically analyzed. The SL model choice reflected the impact on surgical type classification. RESULTS: Image presentation was a significant factor whereas the individual observer was not. Three-dimensional images scored significantly better than 2D images (P=0.0003). Three-dimensional imaging increased the correct surgical type classification by 35%. CONCLUSION: Three-dimensional images significantly improve the pre-surgical diagnostic assessment and surgical type classification of dysplastic hips.

Recommendations for the use of botulinum toxin type A in the management of cerebral palsy.

Botulinum toxin type A (BTX-A) is increasingly being used for the treatment of childhood spasticity, particularly cerebral palsy. However, until very recently, all such use in this indication has been unapproved with no generally accepted treatment protocols, resulting in considerable uncertainty and variation in its use as a therapeutic agent. In view of the increasing awareness of, and interest in, this approach to the treatment of spasticity, and also the recent licensing in a number of countries of a BTX-A preparation for treating equinus deformity in children, it would seem timely to establish a framework of guidelines for the safe and efficacious use of BTX-A for treating spasticity in children. This paper represents an attempt, by a group of 15 experienced clinicians and scientists from a variety of disciplines, to arrive at a consensus and produce detailed recommendations as to appropriate patient selection and assessment, dosage, injection technique and outcome measurement. The importance of adjunctive physiotherapy, orthoses and casting is also stressed.

Femoral derotation for increased hip anteversion. A new surgical technique with a modified Ilizarov frame.

We describe a technique of femoral derotation osteotomy performed according to the Ilizarov principles of percutaneous corticotomy and fixation with a frame. We performed 24 femoral osteotomies in 16 patients, four with cerebral palsy and 12 with idiopathic femoral anteversion. All had rapid union and there were few complications. The advantages of the method include early ambulation, good control of rotation and axial alignment, and minimal scarring.

Carpo-tarsal osteolysis. Case report and review of the literature.

A hereditary form of multicentric osteolysis is described in an 11-year-old girl whose father is also affected. The results after corrective surgery on the foot with the Ilizarov fixator and the findings during arthroscopy of the knee are discussed. The literature is reviewed.

Ground reaction torque and pathway of point of application of ground reaction force during gait of intoeing children.

One hundred twenty-four children with intoeing and 80 age-matched controls were investigated with the Kistler force plate. Tr2, reflecting the maximum external rotational ground reaction torque, is reduced by 30% to 40% in all intoeing children. There also is an appreciably shorter pathway of the point of application of the ground reaction force in the Y-direction (from 20% to 25%) and an obvious medial shift of the point of application of the ground reaction force in the X-direction (from 105% to 290%), from heel strike to toe-off as compared with controls. In spontaneous correction of intoeing, point of application of the ground reaction force in the X-direction becomes more close to normal. The "foot progression angle," which can easily be calculated from the force plate measurements to judge the degree of intoeing, is different from the values in the literature.

Split posterior tibial tendon transfer through the interosseus membrane in spastic equinovarus deformity.

The split posterior tibial tendon transfer procedure was first reported by Green for correction of equinovarus hindfoot deformity in patients with cerebral palsy. A modification of the split posterior tibial tendon transfer combined with an Achilles tendon lengthening is described in 17 children (21 procedures) with a minimum follow-up of 3 years. This modified technique is indicated in young children with a continuously spastic posterior tibial tendon to correct a dynamic equinovarus. It restores active dorsiflexion when the anterior tibial and extensor muscles are weak. The anterior half of the split tibialis posterior is transferred through the interosseus membrane to the dorsum of the foot. Excellent or good results and two poor results were noted after a mean follow-up of 29 months. In the patients with an excellent or good result, marked improvement of their equinovarus foot deformity in stance and swing phase of gait was seen. In two patients, the procedure failed because of technical errors.

A false aneurysm complicating a subcutaneous Achilles tendon lengthening.

Subcutaneous lengthening of the Achilles tendon is a routinely used technique in our department. We report a case with a severe complication after this minor procedure in a 13-year-old patient with cerebral palsy. Six weeks after the operation the patient had a false aneurysm caused by a lesion of the posterior tibial artery at the time of surgery. After ligating the artery and draining the false aneurysm, recovery was uneventful. Although subcutaneous lengthening is an easy and reliable operation, one has to keep in mind its possible complications.

Ultrasound, radiographs, and clinical symptoms in developmental dislocation of the hip: a study of 170 patients.

We evaluated ultrasound findings in developmental dislocation of the hip in relation to clinical signs, risk factors, and radiographic measurements. The ultrasound rating was determined according to the method of Graf. An ultrasound rating > IIa was considered abnormal. The risk factors corresponding best with an abnormal ultrasound were breech delivery (42.8%) and familial occurrence (21%). For the clinical findings, the risk factors corresponding best were abduction < 50 (42.5%) and asymmetric skinfolds in patients (26.4%). The radiographic evaluation showed more abnormal findings, but there was no statistical difference between the overall ultrasound and radiographic results.

Gait pattern in patients with spastic diplegic cerebral palsy who underwent staged operations.

Fifteen patients with spastic diplegic cerebral palsy (CP) were monitored for a mean length of 9.5 years after they underwent staged operations and were evaluated by gait analysis, including joint motion in the sagittal plane and the ground reaction force (GRF) in three dimensions. Results showed an increased hip flexion (132%) at midstance, a reduction of peak knee flexion (PKF) during swing (45%) accompanied by an augmented time of PKF during swing (50%), and an increased dorsiflexion of the ankle during swing (293%) as well as its time during the gait cycle, in comparison with normal values. Moreover, significant decreases of the vertical GRF at the terminal stance and the forward and backward GRF were present. Additionally, it was found that a bilateral popliteal angle < 20 degrees is acceptable in spastic CP. Staged operations gave unpredictable results in the correction of contracture of the hamstrings, the Achilles tendon, and the iliopsoas. The authors are convinced that gait analysis is useful in evaluating these patients and enhances the results of operative treatment, and they have since changed their approach toward multilevel simultaneous corrections.

Gait analysis in myelomeningocele: possibilities and applications.

Gait analysis with a fully integrated laboratory is a relatively new instrument in the armamentarium of the pediatric orthopedic surgeon. The introduction of it has been especially successful in neuromuscular pathology and, particularly, in cerebral palsy. In spina bifida, however, it also enhances substantially the possibilities of detailed analysis of the locomotion problem. It is, furthermore, a very useful instrument in evaluating treatment and in follow-up. With a few examples, this paper tries to show the possibilities and advantages of a gait laboratory in the evaluation of patients with ambulatory spina bifida.

Slipped capital femoral epiphysis: a long-term follow-up, with special emphasis on the capacities for remodeling.

We reviewed 59 hips in 44 children with slipped capital femoral epiphysis (SCFE), all treated by in situ pinning. The average clinical and radiographic follow-up was 11.4 years. Fifty-three hips (90%) were rated as either excellent or good. Osteonecrosis or chondrolysis developed in five patients. Postoperative remodeling was noted, not only by a process of local resorption and apposition of bone, but also by correction of the disturbed anatomic axes, in proportion to the severity of the slip, together with global thickening of the femoral neck. We believe that the good long-term results after in situ pinning are the consequence of this important remodeling process.