Validity and Reliability of the German Version of the CP QOL-Child and CP QOL-Teen Questionnaire.

The aim of the study was to determine the psychometric properties of the German version of the Cerebral Palsy Quality of Life Questionnaire for Children and Adolescents (CP QOL-Child and -Teen). It is a condition-specific questionnaire with a self-report version, measuring well-being rather than ill-being, which differs from existing measurement methods. Fourteen children (9-12 years) and 64 caregivers (4-12 years) answered the child questionnaire. Twenty-one adolescents and 26 caregivers (all adolescents 13-18 years) replied to the teen version. Functioning was categorized by the Gross Motor Function Classification System. For CP QOL-child internal consistency (Cronbach's α) ranged from 0.58 to 0.88 and for CP QOL-Teen from 0.68 to 0.95. Test-retest reliability after 2 to 4 weeks ranged between 0.75 and 0.94 in children's version and 0.89 and 0.96 in teen's version. Correlation with well-established generic KIDSCREEN-10 questionnaire was moderate to strong. The German versions of CP QOL-Child and -Teen are appropriate tools for assessing the quality of life of children and adolescents with cerebral palsy in the German-speaking population.

Positive effect of the combination of multilevel contracture release and glucocorticoid treatment in Duchenne muscular dystrophy.

PURPOSE: In the 1980s the first results of an early multilevel contracture release (MLCR) in patients suffering from progressive Duchenne muscular dystrophy (DMD) showed a positive effect on ambulation. Despite the demonstrated positive effects of prolongation of walking this treatment is not part of current guidelines. The aim of our study was to evaluate the effect of MLCR as well as its combination with glucocorticoid (GC) treatment on ambulation. METHODS: Data of all boys (n = 86) with DMD treated in our outpatient department were analyzed regarding the treatment and loss of independent ambulation. In all, 23 were treated with GC only, ten were operated on, 21 received GC and underwent MLCR and 32 received neither of the two treatments. RESULTS: The analysis of the loss of independent ambulation in our cohort showed a comparable extension of the ambulatory period between the GC-treated and MLCR-treated boys (p = 0.008 and p = 0.005, respectively). Furthermore, an additive effect of both therapies was found; patients with DMD who had both treatments were able to walk two years longer than those with only one of the two treatment options (p<0.001). CONCLUSION: Standard GC treatment and early MLCR in lower limbs have an independent positive effect on prolongation of ambulation in patients with DMD. In our cohort, the combination of both therapies is significantly more effective than each therapy alone. We suggest both should be offered to all DMD patients eligible.Level of evidence: III.

Age-specific occurrence of pathological fractures in patients with spina bifida.

Pathological fractures (PFs) are common in patients with spina bifida. However, most previous studies refer to the overall fracture rate and largely neglecting putative age-dependent aspects. The aim of this retrospective study was to characterize patterns of fracture occurrence in childhood. In a retrospective study, we identified PF, all in the lower limbs, in 13% of 210 patients with spina bifida aperta. We further identified a bimodal frequency distribution of pathological fractures, with peaks at 1-5 and 10-12 years. We could thereby distinguish two groups of patients: (i) Children with a first fracture before an age of 6 years developed frequently multiple fractures within the following years, but fracture series typically stopped by 6 years-of-age. (ii) Children with a first fracture after the age of 6 years had fewer fractures, but these occurred also in adolescence. PF occurred rarely after the age of 13 years. The age at fracture correlated with the fracture site with 85% of the fractures occurring in the femur in the first five years of life and an increased frequency of tibia and foot fractures later in life. While, overall high lesion levels and preceding immobilizing events were risk factors for PF, femur fractures in children under 6 years-of-age occurred independent of their lesion level, and the age at verticalization did not correlate with PF rates.Conclusion: Based on these findings, standardized and effective preventive physiotherapeutic and/or pharmacological interventions to tackle PF in spina bifida need to consider age-specific differences in occurrence and reoccurrence of fractures.What is Known:• Pathological fractures are common in patients with spina bifida aperta, and associated risk factors include high lesion level, immobilization and low bone density.What is New:• We first report a bimodal frequency distribution of pathological fractures in childhood (first peak 1-5 years, second peak 10-12 years) and link early-onset fracture occurrence with the risk of multiple fractures arise in a short time period but a the chance of self-limitation of fracture series within a few years.• We show that femur fractures in children under 6 years-of-age occurred independent of their lesion level, and the age at verticalization did not correlate with PF rates.• We further link the age-dependent occurrence pattern with the risk of further fractures and with the chance of self-limitation of fracture series. The earlier a first fracture occurs, the more probable multiple fractures arise in a short time period. Nevertheless, early fracture series are often self-limiting within a few years.• Femur fractures in children under 6 years-of-age occurred independent of their lesion level, and the age at verticalization did not correlate with PF rates.• Based on these findings, physiotherapeutic and/or pharmaceutical concepts need to be developed in an age-adapted manner and in consideration of the potential self-limiting nature of fracture series.

[Clubfoot Therapy in Accordance with Ponseti - Current Standard]. / Klumpfußtherapie nach Ponseti ­ aktueller Standard.

This manuscript evaluates the recent standard concept for clubfoot treatment. With regard to the history of clubfoot therapy and the return to conservative methods, the focus is laid on Ponseti's treatment concept. Due to its development according to the precise analysis of the pathoanatomy, the practical principle is simple and easy to learn and consists basically of two redression maneuvers, percutaneous achillotenotomy, and boots and bar abduction treatment. Therefore, about 60 years after its implementation in Iowa it can be said to be the worldwide golden standard. It is known that Ponseti treated feet are better with regard to function and pain when compared to surgically treated clubfeet. The best results can be achieved when one sticks exactly to the method. Hence, plaster of Paris above the knee casts yield better results than fibreglass materials or short-leg casts. The brace should be worn 23 hours a day for 3 months and during sleep until the fourth birthday of the child. For reasons including the structured concept of treating relapses, the method is applicable in high and low income countries. Before transferring the tibialis anterior tendon, it is mandatory to correct the relapse of the heel varus. The Ponseti method can also correct clubfeet of non-idiopathic origin. Although a higher rate of relapses must be expected in these cases, initial Ponseti treatment lowers the extent of the necessary surgery. Emphasis is put on the importance of counselling prenatally as well as during the boots and bar period. To yield the best results, it is necessary to train and counsel physicians as well as parents. There is no need to fear significant delay in reaching motor milestones when clubfeet are treated conservatively. Other conservative methods - such as the French physiotherapy method - are able to correct the deformity, but usually do not consist of a concept as structured as the Ponseti method. They are also often more time consuming for the families when compared to Ponseti's technique and are not available ubiquitously. While the diagnosis of the clubfoot deformity is still a clinical one and scores are the main tools for grading the severity today followed by X-rays and to some extent sonography, in experimental settings MRI may be helpful in finding abnormalities in muscles, blood vessels, and cartilage structures. The study of genetic associations of pathway abnormalities and single nucleotide polymorphisms with regard to the development of clubfeet enhances our knowledge concerning the origin of the deformity during limb development. In the future, this may enable us to provide not only a better prognosis for the outcome but also a more individualised therapy for each child born with a clubfoot.

Correction: The Effects of Selective Dorsal Rhizotomy on Balance and Symmetry of Gait in Children with Cerebral Palsy.

[This corrects the article DOI: 10.1371/journal.pone.0152930.].

The Effects of Selective Dorsal Rhizotomy on Balance and Symmetry of Gait in Children with Cerebral Palsy.

AIM: Cerebral palsy (CP) is associated with dysfunction of the upper motor neuron and results in balance problems and asymmetry during locomotion. Selective dorsal rhizotomy (SDR) is a surgical procedure that results in reduced afferent neuromotor signals from the lower extremities with the aim of improving gait. Its influence on balance and symmetry has not been assessed. The aim of this prospective cohort study was to evaluate the impact of SDR on balance and symmetry during walking. METHODS: 18 children (10 girls, 8 boys; age 6 years (y) 3 months (m), SD 1y 8m) with bilateral spastic CP and Gross Motor Function Classification System levels I to II underwent gait analysis before and 6 to 12 months after SDR. Results were compared to 11 typically developing children (TDC; 6 girls, 5 boys; age 6y 6m, SD 1y 11m). To analyse balance, sway velocity, radial displacement and frequency were calculated. Symmetry ratios were calculated for balance measures and spatio-temporal parameters during walking. RESULTS: Most spatio-temporal parameters of gait, as well as all parameters of balance, improved significantly after SDR. Preoperative values of symmetry did not vary considerably between CP and TDC group and significant postoperative improvement did not occur. INTERPRETATION: The reduction of afferent signalling through SDR improves gait by reducing balance problems rather than enhancing movement symmetry.

Predictors for the benefit of selective dorsal rhizotomy.

Selective dorsal rhizotomy (SDR) is a spasticity reducing treatment option for children with spastic cerebral palsy. Selection criteria for this procedure are inconclusive to date. Clinical relevance of the achieved functional improvements and side effects like the negative impact on muscle strength are discussed controversially. In this prospective cohort study one and two year results of 54 children with a mean age of 6.9 (±2.9) years at the time of SDR are analyzed with regard to gross motor function and factors affecting the functional benefit. Only ambulatory children who were able to perform a gross motor function measure test (GMFM-88) were included in this study. Additionally, the modified Ashworth scale (MAS), a manual muscle strength test (MFT), and the body mass index (BMI) were evaluated as possible outcome predictors. MAS of hip adductors and hamstrings decreased significantly (p<0.001) and stayed reduced after two years, while GMFM improved significantly from 79% to 84% 12 months after SDR (p<0.001) and another 2% between 12 and 24 months (p=0.002). Muscle strength did improve significantly concerning knee extension (p=0.008) and ankle dorsiflexion (p=0.006). The improvement of function correlated moderately with age at surgery and preoperative GMFM and weakly with the standard deviation score of the BMI, the dorsiflexor and plantarflexor strength preoperatively as well as with the reduction of spasticity of the hamstrings and the preoperative spasticity of the adductors and hamstrings. Correctly indicated SDR reduces spasticity and increases motor skills sustainably in children with spastic cerebral palsy corresponding to clinically relevant changes of GMFM without compromising muscular strength. Outcome correlates to GMFM and age rather than to MAS and maximal strength testing. The data of this evaluation suggest that children who benefit the most from SDR are between 4 and 7 years old and have a preoperative GMFM between 65% and 85%.

Changes in hip geometry after selective dorsal rhizotomy in children with cerebral palsy.

PURPOSE: Hip development in children with spastic cerebral palsy (CP) may be different in comparison to that of typical developing children due to impaired motor function and altered lever arms. Selective dorsal rhizotomy (SDR) is known to reduce spasticity. It is postulated that it also improves mobility. Its influence on hip development is unclear. The aim of this study is to evaluate changes in hip geometry before and after selective dorsal rhizotomy. METHODS: Conventional radiographs (Rippstein I and II) of 33 ambulatory children aged 2.67 to 11.75 years who underwent SDR were analysed pre- and postoperatively at a mean of 18 months (range 12-29 months). Migration percentage, acetabular index, and anteversion were evaluated. The reduction of spasticity was measured with the modified Ashworth scale. A priori power analysis was performed. As data was normally distributed statistical analysis was performed applying the t-test for paired variables. RESULTS: Radiographic parameters concerning hip geometry improved significantly after SDR. The spasticity of adductors and hamstrings was significantly reduced through SDR from on average 1.7 to 0.8 on the modified Ashworth scale (p<0.001). The acetabular index decreased from 19° to 17° (p = 0.001), the migration percentage improved from 24% to 21% (p<0.001). Anteversion was also significantly reduced from 41° to 38° (p<0.001). Function improved significantly from 80% to 85% when measured with the GMFM-88 (p<0.001). CONCLUSIONS: The results confirm that SDR improves hip geometry as well as function in ambulatory CP children. Long-term studies need to show whether this radiographic improvement has clinical relevance with regard to pain and function.

Calcaneocuboid distraction arthrodesis with allogenic bone grafting for correction of pes planovalgus.

Calcaneocuboid distraction arthrodesis is regarded as an excellent and reliable surgical procedure for correction of pes planovalgus. Despite a potentially high complication rate, the use of an autologous iliac crest graft is regarded as the method of choice. In a prospective trial the results in 12 feet after calcaneocuboid distraction arthrodesis for pes planovalgus correction with allogenic bone graft were investigated. The mean age was 64 (52-81) years. An angle-stable plate was generally used for fixation of the arthrodesis. Successful bone healing was diagnosed clinically if local pain was absent during weight bearing, forefoot manipulation and palpation. The AOFAS score improved from 58.9 (+/- 6) points preoperatively to 89.8 (+/- 6.3) points 12 months postoperatively and the Visual Analogue Pain Scale (VAS) from 5.1 (+/- 1.4) to 0.7 (+/- 1) 12 months postoperatively. On radiological analysis, the lateral and dorsoplantar talometatarsal axis, hindfoot axis, the navicular floor distance and talonavicular coverage angle improved considerably. Bone fusion was observed in 11 of 12 cases postoperatively. In conclusion, the use of an allogenic graft for calcaneocuboid distraction arthrodesis did not result in a higher pseudarthrosis rate compared with results reported after use of an autologous iliac crest graft. Fixation with an angle-stable plate is recommended.

Treatment of aseptic loosened MTPI prosthesis by one-stage revision with ToeFit Plus prosthesis.

INTRODUCTION: Due to the missing bony integration of the ceramic Moje prosthesis for replacing the first metatarsophalangeal joint (MTPI) in hallux rigidus, the mid-term results were bad so far. In case of revision, the distraction arthrodesis with autologous bone taken from the iliac crest as a salvage procedure is the method of choice. METHOD: In our prospective case series, the short-term results after revision of the Moje prosthesis with the ToeFit Plus prosthesis were investigated. The clinical and radiological investigations were done in six MTPI over a 24-month period using AOFAS score and visual analogue scale. RESULTS: There were no radiological signs of loosening or implant migration of the ToeFit Plus 24 months, postoperatively. There was one fissure at the first proximal phalanx necessitating a wire stabilisation. No other complications could be observed. A significant improvement of the AOFAS score and the range of motion were observed 6 weeks postoperatively. CONCLUSION: We could show good and very good short-term results after the replacement of a loosened MTPI prosthesis with a ToeFit Plus. Due to the conic screw anchorage, ToeFit Plus is excellently suited for prosthesis replacement at the MTPI. With sufficient bony anchorage prerequisites, it is possible to preserve and improve the range of motion by changing the loosened MTPI prosthesis in the ToeFit Plus, thereby avoiding the morbidity of gaining autologous bone from the iliac crest.

Influence of ion channels on the proliferation of human chondrocytes.

The goal of the study was to examine connections between ion channel activity and the proliferation of human chondrocytes. Chondrocytes were isolated form human osteoarthritic knee joint cartilage. In this study the concentration-dependent influence of the ion channel modulators tetraethylammonium (TEA), 4-aminopyridine (4-AP), 4',4' diisothiocyanato-stilbene-2,2'-disulfonic acid (DIDS), 4-acetamido-4'-isothiocyano-2,2'-disulfonic acid stilbene (SITS), verapamil (vp) and lidocaine (lido) on the membrane potential and the proliferation of human chondrocytes was investigated using flow cytometry and the measurement of (3)H-thymidine incorporation as measure for the cell proliferation. The results show an effect of the used ion channel modulators causing a change of the membrane potential of human chondrocytes. The maximal measurable effects of the membrane potential were listed with 0.25 mmol/l verapamil (-18%) and 0.1 mmol/l lidocaine (+20%). When measuring DNA distribution, it became apparent that the human chondrocytes are diploid cells with a very low proliferation tendency. After 12 days culture duration, lidocaine and 4-AP cause an increase of the DNA synthesis rate being a limited effect. These results allow the conclusion of an influence of ion channel modulators on chondrocyte proliferation. To gain knowledge of the regulation of chondrocyte proliferation via ion channel modulators could serve the research of new osteoarthritis treatment concepts.