Ligamentum teres tenodesis in medial approach open reduction for developmental dislocation of the hip.

BACKGROUND: When closed reduction of a developmental dislocation of the hip fails, some form of open reduction is required. In recent years, the many advantages of the medial approach open reduction have been emphasized. However, there have been suggestions that the rate of growth disturbance in the proximal femur and the requirement for secondary surgical procedures may be higher with this route than with others. The purpose of this study was to investigate the efficacy and safety of a modified medial approach open reduction, in which the stability of the reduction is enhanced by resection of the redundant ligamentum teres and suturing of the stump of the tendon to the anteromedial capsule. METHODS: The hospital records and radiographs of 92 infants and children with 109 dislocated hips were reviewed retrospectively. Key demographic and pretreatment data were collected by the first author and compared with the outcome at the most recent follow-up. In addition, 69 children returned for a clinical evaluation by the first author. The outcome at the most recent follow-up was graded according to Severin, and associations were sought between pretreatment grade of dislocation according to Tonnis, the presence of ossific nucleus, changes in the acetabular index, the requirements for secondary surgery, associations with previous treatment, and the position of abduction in the postoperative cast. RESULTS: At a mean follow-up of 9 years, 89% of hips were classified as Severin grade 1 or 2. Avascular necrosis (AVN) was classified according to the system of Kalamchi and MacEwen. The incidence of AVN was 41%, but two thirds of these were grade 1 (temporary irregular ossification), and the Severin grading in these hips was not compromised. The presence of ossification in the capital epiphysis and a range of abduction of less than 60 degrees in the hip spica were noted to be protective against the development of AVN. Three hips redislocated and required additional treatment. Thirty-eight hips required a total of 44 additional surgical procedures. CONCLUSIONS: We have demonstrated that it is possible to use a medial approach for open reduction of the congenitally dislocated hip in combination with tenodesis of the ligamentum teres to the anteromedial joint capsule. The incidence of growth disturbance in the proximal femur is high and cumulative with long-term follow-up. However, in this large series, the rate of hip stability, growth disturbance, and need for secondary surgery are comparable to other series. We conclude that the many advantages of open reduction by the medial approach outweigh the disadvantages.

Does botulinum toxin a combined with bracing prevent hip displacement in children with cerebral palsy and "hips at risk"? A randomized, controlled trial.

BACKGROUND: Cerebral palsy is the most common cause of childhood physical disability in developed countries, affecting two children per 1000 live births. Hip displacement affects about one-third of children with cerebral palsy and may result in pain, deformity, and impaired function. The prevention of hip displacement has not been studied in a randomized trial as far as we know. METHODS: A randomized, controlled trial was conducted to examine the effect of intramuscular injections of botulinum toxin A combined with use of a variable hip abduction brace on the progression of hip displacement in children with cerebral palsy. The patients in the treatment group received injections of botulinum toxin A to the adductor and hamstring muscles every six months for three years and were prescribed a hip abduction brace to be worn for six hours per day. In the control group, no hip bracing was used nor were injections performed. The primary outcome measure was hip displacement from the acetabulum as determined by serial measurements of the migration percentage. RESULTS: Ninety children with bilateral cerebral palsy and so-called hips at risk (a migration percentage of >10% but <40%) were entered into the study. Fifty-nine patients were boys, and the mean age was three years. Progressive hip displacement, as determined by serial measurements of the migration percentage, was found in both the treatment and control groups. The rate of hip displacement was reduced in the treatment group by 1.4% per year (95% confidence interval, -0.6% to 3.4%; p = 0.16) when weighted for the uncertainty in rates due to the differing numbers of migration percentage measurements per subject. CONCLUSIONS: There may be a small treatment benefit for the combined intervention of intramuscular injection of botulinum toxin A and abduction hip bracing in the management of spastic hip displacement in children with cerebral palsy. However, progressive hip displacement continued to occur in the treatment group, and our data do not support recommending this treatment.

Tibiotalocalcaneal arthrodesis for severe calcaneovalgus deformity in cerebral palsy.

The authors studied the outcome of a consecutive series of nine tibiotalocalcaneal fusions for severe calcaneovalgus deformity in five adolescents with severe cerebral palsy. The indications for surgery were severe deformity, pain, brace intolerance, difficulties with shoe wear, and progressive loss of transfer, standing, or walking ability. The goals of surgery were deformity correction, reduced bracing, and ability to wear regular shoes and to maintain function. Successful correction of deformity was achieved in all patients. One patient had a stable fibrous ankylosis despite revision surgery. Functional goals were fully achieved in three patients and partially achieved in two patients. The authors conclude that tibiotalocalcaneal fusion is useful as a salvage procedure in a small group of adolescents to correct severe deformity and to maintain limited function. Isolated lengthening of the tendo Achillis had been performed in four of these patients and was a contributing factor to the development of this deformity.

Accuracy of intramuscular injection of botulinum toxin A in juvenile cerebral palsy: a comparison between manual needle placement and placement guided by electrical stimulation.

Most clinicians who perform botulinum toxin A injections for children with cerebral palsy do so using the "free-hand" or manual technique without using radiologic or electrophysiologic guidance to aid needle placement. The objective of this study was to investigate the accuracy of manual needle placement compared with needle placement guided by electrical stimulation. A total of 1,372 separate injections for upper and lower limb spasticity were evaluated in 226 children with cerebral palsy. The accuracy of manual needle placement compared with electrical stimulation was acceptable only for gastroc-soleus (>75%); it was unacceptable for the hip adductors (67%), medial hamstrings (46%), tibialis posterior (11%), biceps brachii (62%), and forearm and hand muscles (13% to 35%). The authors recommend using electrical stimulation or other guidance techniques to aid accurate needle placement in all muscles except the gastroc-soleus. Further study is needed to determine whether more accurate injecting will lead to better functional outcomes and more efficient use of botulinum toxin A.

Treatments for paediatric femoral fractures: a randomised trial.

BACKGROUND: Treatments for femoral fractures in children vary widely and have been investigated only in case series. We did a multicentre randomised trial to compare malunion rates after external fixation and after early application of a hip spica cast for paediatric femoral shaft fractures. METHODS: All children aged 4-10 years with femoral fractures, admitted to four paediatric hospitals, were randomly assigned early application of hip spica or external fixation. The primary outcome was malunion at 2 years after the fracture. Secondary outcomes were scores on the RAND physical function child health questionnaire and the post-hospitalisation behavioural questionnaire, and parents' and children's ratings of overall satisfaction with treatment. Analysis was by intention to treat based on children who reached the 2 year evaluation. FINDINGS: Of 60 children assigned to the hip-spica group, 56 reached the 2-year assessment; of them, six (11%) required other forms of treatment because of unacceptable loss of reduction. Of 48 children assigned external fixation, 45 reached the 2-year assessment; two (4%) had refractures and five (11%) required operative adjustment of the fixator. The rate of malunion was significantly higher in the hip-spica group than in the external-fixator group (25/56 [45%] vs 7/45 [16%]; 95% CI for difference 12-46%; p=0.002). The two groups had similar mean scores for the RAND physical function health questionnaire (0.34 vs 0.45; 95% CI for difference, -0.57 to 0.34; p=0.61), for the post-hospitalisation questionnaire (106.8 vs 106.3; -4.9 to 5.9; p=0.86), and for parents' satisfaction (4.3 vs 4.2; -0.3 to 0.6; p=0.5) and children's ratings of happiness with treatment (6.9 vs 7.7; -2.2 to 0.5; p=0.21). INTERPRETATION: Early application of hip spica has a small role in the treatment of paediatric femoral fractures. Future trials need to compare external fixation with flexible intramedullary nails.

Gait patterns after fracture of the femoral shaft in children, managed by external fixation or early hip spica cast.

The authors prospectively studied three-dimensional kinematics and kinetics of gait in children recovering from a closed, isolated, nonpathologic fracture of the femoral diaphysis, who had been randomly assigned to management by monolateral external fixation or early hip spica casting. The aims were to investigate the gait patterns soon after injury and at 2 years after injury. Children treated with external fixation, walking with the external fixator in situ, demonstrated asymmetric gait abnormalities in all three anatomic planes affecting the trunk, pelvis, hip, knee, and ankle. This appears to be a strategy to minimize movement and pain at the fixator pin sites. The gait pattern normalized rapidly after removal of the external fixator with few kinematic or kinetic abnormalities and no clinically significant disturbances of gait at 2 years after injury. In contrast, children in the early hip spica cast group developed a "crouch gait" pattern in the sagittal plane, most likely due to weakness. They also had abnormal coronal plane kinematics related to shortening of the injured side. Gait patterns improved, but at 2 years there were some persistent gait deviations, probably related to residual limb length discrepancy.

The Functional Mobility Scale (FMS).

We devised a new Functional Mobility Scale (FMS) to describe functional mobility in children with cerebral palsy, as an aid to communication between orthopaedic surgeons and health professionals. The unique feature of the FMS is the freedom to score functional mobility over three distinct distances, chosen to represent mobility in the home, at school and in the wider community. We examined the construct, content, and concurrent validity of the FMS in a cohort of 310 children with cerebral palsy by comparing the FMS to existing scales and to instrumented measures of physical function. We demonstrated the scale to be both valid and reliable in a consecutive population sample of 310 children with cerebral palsy seen in our tertiary referral center. The FMS was useful for discriminating between large groups of children with varying levels of disabilities and functional mobility and sensitive to detect change after operative intervention.

Tissue-specific RNA surveillance? Nonsense-mediated mRNA decay causes collagen X haploinsufficiency in Schmid metaphyseal chondrodysplasia cartilage.

Mutations resulting in a premature termination codon (PTC) are a major cause of inherited disorders, and the majority of these mutant RNA transcripts are subjected to nonsense-mediated mRNA decay (NMD). This RNA surveillance results in reduced mutant allele expression, the extent of which can impact on the clinical severity. The molecular mechanisms of NMD in mammalian cells, its relationship to splicing and translation, downstream sequence elements and binding factors remains only partially understood. Currently there is little information on whether the extent of NMD is gene- or tissue-specific, although nonsense mutation inhibition of RNA splicing has been shown to exhibit some tissue and gene specificity in vitro. Schmid metaphyseal chondrodysplasia results from heterozygous mutations in the gene for collagen X (COL10A1), expressed by the hypertrophic chondrocytes of growth plate cartilage. In one patient a PTC mutation has been shown to result in complete NMD and collagen X haploinsufficiency in cartilage. Here we show that, in this patient, and in another with a different collagen X PTC mutation also leading to complete NMD in cartilage, the mutant mRNAs were not subjected to NMD in non-cartilage cells (lymphoblasts and bone cells). These data suggest that novel RNA surveillance mechanisms may exist in cartilage and that tissue specificity of NMD could be of importance in understanding the molecular pathology of nonsense mutations. Furthermore, the demonstration of collagen X haploinsufficiency in the second patient to be studied at the level of tissue expression, confirms that nonsense mutations leading to complete mutant collagen X mRNA degradation in cartilage is an important molecular cause of SMCD.

Hip displacement in spastic cerebral palsy: repeatability of radiologic measurement.

Radiologic measures of migration percentage (MP) and acetabular index (AI) taken from plain radiographs of the pelvis are the most commonly used tools for determining hip displacement and management options in children with cerebral palsy and spastic hip disease. This study determined interrater and intrarater reliability of MP and AI on pelvic radiographs chosen to represent a wide range of age (11 months to 8 years 5 months), MP (0%-56%), and AI (9 degrees -33 degrees ). The study demonstrated that an experienced rater would be expected to measure MP on a single radiograph to within +/-5.8% of the true value and a change in MP between two radiographs taken at different times to within +/-8.3% of the true value. Similarly for AI, the measurement error for a typical rater would be within +/-2.6 degrees on a single reading and +/-3.7 degrees if recording change between two occasions. The authors believe that the results indicating true change are acceptable in clinical practice, provided treatment decisions are based on a series of radiographs taken at 6-month intervals, methods and training are standardized, and consistent raters are used.

Magnetic resonance imaging evaluation of surgical management in developmental dysplasia of the hip in childhood.

Magnetic resonance imaging (MRI) was performed on 73 hips in 59 children aged 3 to 16 months after surgical reduction of developmental dislocation of the hip. Twenty-seven hips in 22 children had repeat MRI 6 weeks after reduction, and 20 hips in 16 patients had further MRI at least 1 year later. Only 38% of hips appeared concentrically reduced on the initial MRI scan, but this increased to 90% by 1 year later, without intervention. The authors measured coronal plane acetabular index and transverse plane anterior, posterior, and axial acetabular indices, as well as acetabular version and anteversion. Persistent difference could be shown in the coronal plane acetabular index between the dysplastic and normal sides for the cartilaginous anlage and the bony model of the acetabulum in scans performed at least 1 year after reduction. However, 40% of cartilaginous coronal plane acetabular indices fell within the "normal" range at 1 year. No other parameters could be shown to be persistently different.