Erich Rutz

Single-event multilevel surgery for children with cerebral palsy: a systematic review

Aim  To conduct a systematic review of single‐event multilevel surgery (SEMLS) for children with cerebral palsy, with the aim of evaluating the quality of the evidence and developing recommendations for future research.

Management of the upper limb in cerebral palsy

The management of the upper limb in cerebral palsy is often complex and challenging. Effective treatment requires a multidisciplinary approach involving paediatricians, occupational therapists, physiotherapists, orthotists and upper extremity surgeons. Interventions are generally aimed at improving function and cosmesis by spasticity management, preventing contractures and correcting established deformities. Treatment objectives vary according to each child and range from static correction of deformities to ease nursing care, to improvements in dynamic muscle balance to augment hand function. Botulinum toxin A therapy has been shown to relieve spasticity and improve function in the short term. Surgery is also effective but requires careful patient selection, as many children with cerebral palsy are not candidates for surgery. Occupational therapy and physiotherapy have small treatment effects alone but are essential adjuncts to medical and surgical management.

Stability of the Gross Motor Function Classification System after single‐event multilevel surgery in children with cerebral palsy

Aim  There are conflicting reports about the stability of the Gross Motor Function Classification System (GMFCS) in children with cerebral palsy (CP) after orthopaedic surgery. We studied the stability of the GMFCS in children with bilateral spastic CP after single‐event multilevel surgery, using the Gait Profile Score (GPS) as the primary outcome measure.

Tibialis anterior tendon shortening in combination with Achilles tendon lengthening in spastic equinus in cerebral palsy

Equinus is the commonest deformity in cerebral palsy (CP). Many different surgical procedures have been described for the treatment of spastic equinus. In long standing equinus deformities the tibialis anterior muscle becomes elongated which is one reason for muscle weakness. Surgical tendon shortening of the tibialis anterior tendon was therefore introduced to rebalance muscle strength. All patients with CP who had a tibialis anterior tendon shortening (TATS) in combination with a tendo Achilles lengthening (TAL) were included in this study. A total of 29 patients had 30 surgical interventions (21 hemiplegic patients: 14 boys/7 girls, age 9-22 years; mean 15.2 years; 5 diplegics and 3 quadriplegics; 5 boys/3 girls, age 7-37.5 years; mean 14.8 years). Fifteen patients had additional surgery (soft tissue or bony procedures). The TATS was performed at the distal insertion with transosseous tendon fixation in the medial cuneiform bone at the original place. Movement Analysis Profile (MAP) for ankle dorsi-/plantarflexion, Gait Profile Score (GPS), Gait Deviation Index (GDI), and Gillette Gait Index (GGI) improved significantly for all patients compared pre- to postoperatively. In 93% of the patients active dorsiflexion of the ankle was possible postoperatively. We conclude that TATS in combination with TAL in spastic equinus in CP is a safe procedure and improves but not completely corrects foot positioning during gait. For the treatment of spastic equinus in CP we recommend shortening of the elongated antagonist (TATS) in combination with lengthening of the short agonist (TAL) for achieving optimal postoperative function.

Explaining the variability improvements in gait quality as a result of single event multi-level surgery in cerebral palsy

PURPOSE This is a study of all children with spastic diplegic cerebral palsy (Gross Motor Classification System levels II and III) who had single event multi-level surgery (SEMLS) at a single tertiary referral hospital between 1995 and 2008 to identify factors predicting improvement in gait quality as quantified by the gait profile score (GPS). 9 factors (5 dichotomous and 4 continuous, including preoperative GPS) that might be expected to predict outcomes were identified and univariate and multivariable analysis used to explore how these affected outcomes. SCOPE Data from 121 children were included. The mean improvement in GPS of 4.3° was 2.7 times the minimal clinically important difference. Univariate analysis suggested that preoperative GPS is a very strong predictor of improvement in GPS (p<10(-5)) and when this is considered as a covariate only GMFCS level (p=10(-5)) and having had previous surgery (p=0.026) were found to be statistically significant predictors of GPS improvement (p<0.05). Children of GMFCS level II improved on average by 2° more than those of level III once differences in preoperative GPS had been accounted for. CONCLUSION Children with the most abnormal gait patterns preoperatively, and hence those with the most potential to improve are those that improve the most and surgery has clearly been beneficial. Over a quarter of children show changes in GPS which were less than the MCID. The majority of these were those with the least abnormal gait patterns preoperatively and further research is required to establish whether and how such children benefit from SEMLS.

The pediatric LCP hip plate for fixation of proximal femoral osteotomy in cerebral palsy and severe osteoporosis.

Background Hip dislocation or subluxation together with poor nutrition, reduced weight bearing, and osteoporosis is a frequent condition in severe cerebral palsy (CP). Severe osteoporosis may cause difficulties in fixing a proximal femoral osteotomy with a conventional blade plate. The Pediatric locking compression plate (LCP) Hip Plate system offers better grip and more stable fixation. Methods Fifty-three proximal femoral osteotomies, alone or as part of a more complex surgical intervention, were performed in 28 patients (17 boys and 11 girls, age at surgery average 10.4 y, 3 to 19 y). All children suffered from CP (24 quadriplegics, 2 diplegics, and 2 hemiplegics) with Gross Motor Function Classification System levels: 3× III, 3× IV, and 22× V. This cohort was compared with a historical (conventional AO blade plate) group (38 patients with 53 operative interventions, 24 girls and 14 boys, age at surgery average 9.8 y, 3 to 18.5 y, Gross Motor Function Classification System levels: 4× III, 5× IV, and 29× V; 34 quadriplegics, 3 diplegics, and 1 hemiplegic). Results The operative interventions of both cohorts that are performed are comparable considering the average amount of varisation and derotation. Operations with the conventional AO blade plate were 17.2 minutes shorter on average and the blood loss was 45.6 mL less on average. Radiologically, 19.6% of the patients had signs of complete consolidation with the LCP Hip Plate 6 weeks after surgery (vs. 91.1% of the patients of the historical cohort, P <0.001) but all osteotomies in both groups were completely consolidated by 12 weeks. In the LCP cohort in 3 patients (10.7%) full weight bearing was allowed immediately after the operation. Conclusions Both implants, the Pediatric LCP Hip Plate and the conventional AO blade plate, produce similar results regarding fixation and correction of the neck-shaft angle. The consolidation rate 6 weeks postoperatively using the LCP plate is lower than with the conventional blade plate, whereas equivalent healing at 3 months was found. Hence, LCP plate removal is recommended not earlier than 6 months after surgery despite good callus formation on x-ray. Level of Evidence Level III.

Transverse plane rotation of the foot and transverse hip and pelvic kinematics in diplegic cerebral palsy

External rotation of the foot associated with mid-foot break is a commonly observed gait abnormality in diplegic CP patients. Previous studies have shown a correlation between equinus and internal hip rotation in hemiplegic patients. This study aimed to determine if there was a correlation between the amount of transverse plane rotation in diplegic CP patients using kinematic data from standardised gait analysis. Lower limb data of 134 ambulant children with diplegic CP was analysed retrospectively determining the maximum change in foot, hip and pelvis rotation during loading response. Highly significant negative correlations (P=<0.001) were found between foot and hip movements and foot and pelvic movements. Equinus at initial contact diminished the foot:hip correlation while it enhanced the foot:pelvic correlation. There was less external rotation of the foot in equinus patients (P=0.012) and more external rotation of the pelvis in the equinus group (P=<0.001). This data reveal a correlation between transverse plane rotation at foot level to that at the hip and pelvis. The likely biomechanical explanation is relatively excessive transverse external rotation of the foot due to abnormalities such as mid-foot break. When under load, where the foot is fixed to the floor, internal rotation of the entire leg occurs. This is due to lever arm disease as a result of the relatively shortened foot and inefficiency of the plantar-flexion knee-extension couple. Equinus modulates the effect. When treating such patients, lever arm deformities at all levels must be considered to result in the best outcome and prevent recurrences.

Improved Muscle Function in Duchenne Muscular Dystrophy through L-Arginine and Metformin: An Investigator-Initiated, Open-Label, Single-Center, Proof-Of-Concept-Study

Altered neuronal nitric oxide synthase function in Duchenne muscular dystrophy leads to impaired mitochondrial function which is thought to be one cause of muscle damage in this disease. The study tested if increased intramuscular nitric oxide concentration can improve mitochondrial energy metabolism in Duchenne muscular dystrophy using a novel therapeutic approach through the combination of L-arginine with metformin. Five ambulatory, genetically confirmed Duchenne muscular dystrophy patients aged between 7–10 years were treated with L-arginine (3 x 2.5 g/d) and metformin (2 x 250 mg/d) for 16 weeks. Treatment effects were assessed using mitochondrial protein expression analysis in muscular biopsies, indirect calorimetry, Dual-Energy X-Ray Absorptiometry, quantitative thigh muscle MRI, and clinical scores of muscle performance. There were no serious side effects and no patient dropped out. Muscle biopsy results showed pre-treatment a significantly reduced mitochondrial protein expression and increased oxidative stress in Duchenne muscular dystrophy patients compared to controls. Post-treatment a significant elevation of proteins of the mitochondrial electron transport chain was observed as well as a reduction in oxidative stress. Treatment also decreased resting energy expenditure rates and energy substrate use shifted from carbohydrates to fatty acids. These changes were associated with improved clinical scores. In conclusion pharmacological stimulation of the nitric oxide pathway leads to improved mitochondria function and clinically a slowing of disease progression in Duchenne muscular dystrophy. This study shall lead to further development of this novel therapeutic approach into a real alternative for Duchenne muscular dystrophy patients. Trial Registration ClinicalTrials.gov NCT02516085

Long-term results and outcome predictors in one-stage hip reconstruction in children with cerebral palsy.

BACKGROUND One-stage hip reconstruction is the gold standard for treatment of hip displacement in children with cerebral palsy. The aims of this study were (1) to report the subjective clinical, objective clinical, and radiographic outcomes; and (2) to investigate outcome predictors, including the influence of the following risk factors: femoral head shape, migration percentage, direction of migration, and age at surgery. METHODS We reviewed 168 hip reconstructions (eighty-two right, eighty-six left) in 121 patients (101 male, twenty female) at a mean follow-up of 7.3 ± 4.6 years (range, four to eighteen years). Surgical outcomes were assessed on the basis of the pain intensity and frequency (measured on 10-point visual analog scales) as well as GMFCS (Gross Motor Function Classification System) and MCPHCS (Melbourne Cerebral Palsy Hip Classification System) scores and postoperative migration percentage. The effects of femoral head shape, preoperative migration percentage, direction of migration, and age at surgery on surgical outcome were assessed by multivariate regression adjusting for potential confounders including sex, triradiate cartilage status, type of cerebral palsy, and surgical technique. RESULTS Pain intensity and frequency were reduced significantly. Preoperative femoral head shape had no significant effect on the changes in pain, MCPHCS grade, and GMFCS level. The preoperative migration percentage was the most influential risk factor with respect to postoperative outcome. Age at surgery had no effect on the changes in pain score and GMFCS level. The overall surgical complication rate was 10.5%. CONCLUSIONS Our data on 168 hip reconstructions at a mean follow-up of seven years showed significant and clinically meaningful improvements in pain intensity and frequency as well as in clinical scores and hip coverage. Analysis of potential risk factors showed only the preoperative migration percentage to have a relevant influence on outcomes.

Patellar tendon shortening for flexed knee gait in spastic diplegia.

UNLABELLED We evaluated the outcome of three different approaches to the management of flexed knee gait patients with spastic diplegia. The three surgical procedures were patellar tendon shortening (PTS), PTS combined with rotational osteotomies of the femur and/or tibia, and PTS combined with supracondylar extension osteotomy (SEO) of the distal femur. The primary outcome measure was gait kinematics. The knee gait variable score (GVS) and the gait profile score (GPS) were derived from gait kinematics. 24 patients (16 male and 8 female), mean age 16.1 years (SD 5.8 years), who had surgery between 2002 and 2008, were followed for a mean of 22 months. Knee extension during gait improved by a mean of 20° throughout the gait cycle, with an improvement in the knee GVS of 14° (p<0.001). The overall gait pattern improved with a mean decrease in GPS of 4.6°. Correction of patella alta was demonstrated by an improvement in the Koshino index from 1.34 pre-operatively to 1.10 post-operatively (p<0.001). Knee and gait kinematics, physical examination measures and Koshino Index improved in all three surgical groups, suggesting that a tailored approach to the correction of flexed knee gait in spastic diplegia is both feasible and appropriate. LEVEL OF EVIDENCE Level III.