Aims

The health-related quality of life (HRQoL) of paediatric patients with orthopaedic conditions and spinal deformity is important, but existing generic tools have their shortcomings. We aim to evaluate the use of Paediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales in the paediatric population with specific comparisons between those with spinal and limb pathologies, and to explore the feasibility of using PedsQL for studying scoliosis patients’ HRQoL.

Aims

Although atlantoaxial rotatory fixation (AARF) is a common cause of torticollis in children, the diagnosis may be delayed. The condition is characterised by a lack of rotation at the atlantoaxial joint which becomes fixed in a rotated and subluxed position. The management of children with a delayed presentation of this condition is controversial. This is a retrospective study of a group of such children.

Aims

There is increasing evidence that flexible flatfoot (FF) can lead to symptoms and impairment in health-related quality of life. As such we undertook an observational study investigating the aetiology of this condition, to help inform management. The hypothesis was that as well as increased body mass index (BMI) and increased flexibility of the lower limb, an absent anterior subtalar articulation would be associated with a flatter foot posture.

Aims

Single event multilevel surgery (SEMLS) has been shown to improve gait in children with cerebral palsy (CP). However, there is limited evidence regarding long-term outcomes and factors influencing them.

The traditional techniques involving an oblique tunnel or triangular wedge resection to approach a central or mixed-type physeal bar are hindered by poor visualisation of the bar. This may be overcome by a complete transverse osteotomy at the metaphysis near the growth plate or a direct vertical approach to the bar. Ilizarov external fixation using small wires allows firm fixation of the short physis-bearing fragment, and can also correct an associated angular deformity and permit limb lengthening.

We accurately approached and successfully excised ten central- or mixed-type bars; six in the distal femur, two in the proximal tibia and two in the distal tibia, without damaging the uninvolved physis, and corrected the associated angular deformity and leg-length discrepancy. Callus formation was slightly delayed because of periosteal elevation and stretching during resection of the bar. The resultant resection of the bar was satisfactory in seven patients and fair in three as assessed using a by a modified Williamson–Staheli classification.

Cite this article: Bone Joint J 2015;97-B:1726–31.

We report the results of Vulpius transverse gastrocsoleus recession for equinus gait in 26 children with cerebral palsy (CP), using the Gait Profile Score (GPS), Gait Variable Scores (GVS) and movement analysis profile. All children had an equinus deformity on physical examination and equinus gait on three-dimensional gait analysis prior to surgery. The pre-operative and post-operative GPS and GVS were statistically analysed. There were 20 boys and 6 girls in the study cohort with a mean age at surgery of 9.2 years (5.1 to 17.7) and 11.5 years (7.3 to 20.8) at follow-up. Of the 26 children, 14 had spastic diplegia and 12 spastic hemiplegia. Gait function improved for the cohort, confirmed by a decrease in mean GPS from 13.4° pre-operatively to 9.0° final review (p < 0.001). The change was 2.8 times the minimal clinically important difference (MCID). Thus the improvements in gait were both clinically and statistically significant. The transverse gastrocsoleus recession described by Vulpius is an effective procedure for equinus gait in selected children with CP, when there is a fixed contracture of the gastrocnemius and soleus muscles. Cite this article: Bone Joint J 2015;97-B:564–71

Cubitus varus is the most frequent complication following the treatment of supracondylar humeral fractures in children. We investigated data from publications reporting on the surgical management of cubitus varus found in electronic searches of Ovid/MEDLINE and Cochrane Library databases. In 894 children from 40 included studies, the mean age at initial injury was 5.7 years (3 to 8.6) and 9.8 years (4 to 15.7) at the time of secondary correction. The four osteotomy techniques were classified as lateral closing wedge, dome, complex (multiplanar) and distraction osteogenesis. A mean angular correction of 27.6º (18.5° to 37.0°) was achieved across all classes of osteotomy. The meta-analytical summary estimate for overall rate of good to excellent results was 87.8% (95% CI 84.4 to 91.2). No technique was shown to significantly affect the surgical outcome, and the risk of complications across all osteotomy classes was 14.5% (95% CI 10.6 to 18.5). Nerve palsies occurred in 2.53% of cases (95% CI 1.4 to 3.6), although 78.4% were transient. No one technique was found to be statistically safer or more effective than any other.

Cite this article: Bone Joint J 2014;96-B:691–700.

Matrix metalloproteinases (MMPs), responsible for extracellular matrix remodelling and angiogenesis, might play a major role in the response of the growth plate to detrimental loads that lead to overuse injuries in young athletes. In order to test this hypothesis, human growth plate chondrocytes were subjected to mechanical forces equal to either physiological loads, near detrimental or detrimental loads for two hours. In addition, these cells were exposed to physiological loads for up to 24 hours. Changes in the expression of MMPs -2, -3 and -13 were investigated.

We found that expression of MMPs in cultured human growth plate chondrocytes increases in a linear manner with increased duration and intensity of loading. We also showed for the first time that physiological loads have the same effect on growth plate chondrocytes over a long period of time as detrimental loads applied for a short period.

These findings confirm the involvement of MMPs in overuse injuries in children. We suggest that training programmes for immature athletes should be reconsidered in order to avoid detrimental stresses and over-expression of MMPs in the growth plate, and especially to avoid physiological loads becoming detrimental.

Cite this article: Bone Joint J 2013;95-B:568–73.

Although equinus gait is the most common abnormality in children with spastic cerebral palsy (CP) there is no consistency in recommendations for treatment, and evidence for best practice is lacking. The Baumann procedure allows selective fractional lengthening of the gastrocnemii and soleus muscles but the long-term outcome is not known. We followed a group of 18 children (21 limbs) with diplegic CP for ten years using three-dimensional instrumented gait analysis. The kinematic parameters of the ankle joint improved significantly following this procedure and were maintained until the end of follow-up. We observed a normalisation of the timing of the key kinematic and kinetic parameters, and an increase in the maximum generation of power of the ankle. There was a low rate of overcorrection (9.5%, n = 2), and a rate of recurrent equinus similar to that found with other techniques (23.8%, n = 5). As the procedure does not impair the muscle architecture, and allows for selective correction of the contracted gastrocnemii and soleus, it may be recommended as the preferred method for correction of a mild fixed equinus deformity

The outcome of 56 children (61 shoulders) treated surgically at the Rizzoli Institute between April 1975 and June 2010 for congenital elevation of the scapula is reported. There were 31 girls and 25 boys with a mean age at surgery of 6.4 years (2 to 15). The deformity involved the right shoulder in 20 cases, the left in 31 and was bilateral in five. The degree of the deformity was graded clinically and radiologically according to the classifications of Cavendish and Rigault, respectively. All patients underwent a modified Green procedure combined, in selected cases, with resection of the superomedial portion of the scapula and excision of any omovertebral connection. After a mean follow-up of 10.9 years (1 to 29.3), there was cosmetic improvement by at least one Cavendish grade in 54 shoulders (88.5%). The mean abduction of the shoulder improved from 92° (50° to 155°) to 112° (90° to 170°) and the mean flexion improved from 121° (80° to 160°) to 155° (120° to 175°). The unsatisfactory cosmetic result in seven shoulders was due to coexistent scoliosis in two cases and insufficient reduction of the scapular elevation in the other five. An incomplete upper brachial plexus palsy occurred post-operatively in three patients but resolved within seven months.

We suggest that a modified Green procedure combined with resection of the superomedial portion of the scapula provides good cosmetic and functional results in patients with Sprengel’s shoulder.

Congenital pseudarthrosis of the tibia is an uncommon manifestation of neurofibromatosis type 1 (NF1), but one that remains difficult to treat due to anabolic deficiency and catabolic excess. Bone grafting and more recently recombinant human bone morphogenetic proteins (rhBMPs) have been identified as pro-anabolic stimuli with the potential to improve the outcome after surgery. As an additional pharmaceutical intervention, we describe the combined use of rhBMP-2 and the bisphosphonate zoledronic acid in a mouse model of NF1-deficient fracture repair.

Fractures were generated in the distal tibiae of neurofibromatosis type 1-deficient (Nf1^+/−) mice and control mice. Fractures were open and featured periosteal stripping. All mice received 10 μg rhBMP-2 delivered in a carboxymethylcellulose carrier around the fracture as an anabolic stimulus. Bisphosphonate-treated mice also received five doses of 0.02 mg/kg zoledronic acid given by intraperitoneal injection.

When only rhBMP but no zoledronic acid was used to promote repair, 75% of fractures in Nf1^+/− mice remained ununited at three weeks compared with 7% of controls (p < 0.001). Systemic post-operative administration of zoledronic acid halved the rate of ununited fractures to 37.5% (p < 0.07).

These data support the concept that preventing bone loss in combination with anabolic stimulation may improve the outcome following surgical treatment for children with congenital pseudarthoris of the tibia and NF1.

Progressive angular deformity of an extremity due to differential physeal arrest is the most common late orthopaedic sequela following meningococcal septicaemia in childhood. A total of ten patients (14 ankles) with distal tibial physeal arrest as a consequence of meningococcal septicaemia have been reviewed. Radiological analysis of their ankles has demonstrated a distinct pattern of deformity. In 13 of 14 cases the distal fibular physis was unaffected and continued distal fibular growth contributed to a varus deformity. We recommend that surgical management should take account of this consistent finding during the correction of these deformities.

This study compares the initial outcomes of minimally invasive techniques for single-event multi-level surgery with conventional single-event multi-level surgery. The minimally invasive techniques included derotation osteotomies using closed corticotomy and fixation with titanium elastic nails and percutaneous lengthening of muscles where possible. A prospective cohort study of two matched groups was undertaken. Ten children with diplegic cerebral palsy with a mean age of ten years six months (7.11 to 13.9) had multi-level minimally invasive surgery and were matched for ambulatory level and compared with ten children with a mean age of 11 years four months (7.9 to 14.4) who had conventional single-event multi-level surgery. Gait kinematics, the Gillette Gait Index, isometric muscle strength and gross motor function were assessed before and 12 months after operation.

The minimally invasive group had significantly reduced operation time and blood loss with a significantly improved time to mobilisation. There were no complications intra-operatively or during hospitalisation in either group. There was significant improvement in gait kinematics and the Gillette Gait Index in both groups with no difference between them. There was a trend to improved muscle strength in the multi-level group. There was no significant difference in gross motor function between the groups.

We consider that minimally invasive single-event multi-level surgery can be achieved safely and effectively with significant advantages over conventional techniques in children with diplegic cerebral palsy.

We report the outcome of 28 patients with spina bifida who between 1989 and 2006 underwent 43 lower extremity deformity corrections using the Ilizarov technique. The indications were a flexion deformity of the knee in 13 limbs, tibial rotational deformity in 11 and foot deformity in 19. The mean age at operation was 12.3 years (5.2 to 20.6). Patients had a mean of 1.6 previous operations (0 to 5) on the affected limb. The mean duration of treatment with a frame was 9.4 weeks (3 to 26) and the mean follow-up was 4.4 years (1 to 9). There were 12 problems (27.9%), five obstacles (11.6%) and 13 complications (30.2%) in the 43 procedures. Further operations were needed in seven patients. Three knees had significant recurrence of deformity. Two tibiae required further surgery for recurrence. All feet were plantigrade and braceable.

We conclude that the Ilizarov technique offers a refreshing approach to the complex lower-limb deformity in spina bifida.

We have tested the reliability of a recently reported classification system of hip morphology in adolescents with cerebral palsy in whom the triradiate cartilage was closed. The classification is a six-grade ordinal scale, based on the measurement of the migration percentage and an assessment of Shenton’s arch, deformity of the femoral head, acetabular deformity and pelvic obliquity.

Four paediatric orthopaedic surgeons and four physiotherapists received training in the use of the classification which they applied to the assessment of 42 hip radiographs, read on two separate occasions. The inter- and intra-observer reliability was assessed using the intraclass correlation coefficient and found to be excellent, with it ranging from 0.88 to 0.94. The classification in our study was shown to be valid (based on migration percentage), and reliable. As a result we believe that it can now be used in studies describing the natural history of hip displacement in cerebral palsy, in outcome studies and in communication between clinicians.

There is much debate about the nature and extent of deformities in the proximal femur in children with cerebral palsy. Most authorities accept that increased femoral anteversion is common, but its incidence, severity and clinical significance are less clear. Coxa valga is more controversial and many authorities state that it is a radiological artefact rather than a true deformity.

We measured femoral anteversion clinically and the neck-shaft angle radiologically in 292 children with cerebral palsy. This represented 78% of a large, population-based cohort of children with cerebral palsy which included all motor types, topographical distributions and functional levels as determined by the gross motor function classification system.

The mean femoral neck anteversion was 36.5° (11° to 67.5°) and the mean neck-shaft angle 147.5° (130° to 178°). These were both increased compared with values in normally developing children. The mean femoral neck anteversion was 30.4° (11° to 50°) at gross motor function classification system level I, 35.5° (8° to 65°) at level II and then plateaued at approximately 40.0° (25° to 67.5°) at levels III, IV and V. The mean neck-shaft angle increased in a step-wise manner from 135.9° (130° to 145°) at gross motor function classification system level I to 163.0° (151° to 178°) at level V. The migration percentage increased in a similar pattern and was closely related to femoral deformity.

Based on these findings we believe that displacement of the hip in patients with cerebral palsy can be explained mainly by the abnormal shape of the proximal femur, as a result of delayed walking, limited walking or inability to walk. This has clinical implications for the management of hip displacement in children with cerebral palsy.

We reviewed the outcome in 24 children with bilateral spastic cerebral palsy aged seven years or younger for whom surgery was recommended between 1999 and 2005 following gait analysis. A total of 13 children (operative group) had surgery and the remaining 11 (control group) did not, for family or administrative reasons. The operative group had at least two post-operative gait analyses at yearly intervals, with eight children having a third and six children a fourth. The control group had a second analysis after a mean interval of 1.5 years (95% confidence interval 1.1 to 1.9). In the operative group, the Gillette gait index, the ranges of movement in the lower limb joint and knee extension in stance improved following surgery, and this was maintained overall at the second post-operative analysis. The minimum knee flexion in stance in the control group increased between analyses.

These results suggest that surgical intervention in selected children can result in improvements in gait and function in the short to medium term compared with non-operative management.

We carried out a morphometric analysis of the acetabulum following Dega osteotomy in patients with cerebral palsy using three-dimensional CT. We assessed 17 acetabula in 12 patients with instability of the hip. A Dega osteotomy and varus derotation femoral osteotomy were performed in all 17 hips. Three-dimensional CT scans were taken before and approximately one year after operation. Acetabular cover was evaluated using anterosuperior, superolateral and posterosuperior acetabular indices, and the change in the acetabular volume was calculated. Inter- and intra-observer reliability was assessed using the intraclass correlation coefficient. After the osteotomy, the anterosuperior, superolateral and posterosuperior cover had improved significantly towards the value seen in a control group. The mean acetabular volume increased by 68%

The medial rotation contracture caused by weak external rotation secondary to obstetric brachial plexus injury leads to deformation of the bones of the shoulder. Scapular hypoplasia, elevation and rotation deformity are accompanied by progressive dislocation of the humeral head. Between February and August 2005, 44 children underwent a new surgical procedure called the ‘triangle tilt’ operation to correct this bony shoulder deformity. Surgical levelling of the distal acromioclavicular triangle combined with tightening of the posterior glenohumeral capsule (capsulorrhaphy) improved shoulder function and corrected the glenohumeral axis in these patients. The posture of the arm at rest was improved and active external rotation increased by a mean of 53° (0° to 115°) in the 40 children who were followed up for more than one year.

There was a mean improvement of 4.9 points (1.7 to 8.3) of the Mallet shoulder function score after surgical correction of the bony deformity.

The deformity index is a new radiological measurement of the degree of deformity of the femoral head in unilateral Perthes’ disease. Its values represent a continuous outcome measure of deformity incorporating changes in femoral epiphyseal height and width compared with the unaffected side. The sphericity of the femoral head in 30 radiographs (ten normal and 20 from patients with Perthes’ disease) were rated blindly as normal, mild, moderate or severe by three observers. Further blinded measurements of the deformity index were made on two further occasions with intervals of one month.

There was good agreement between the deformity index score and the subjective grading of deformity. Intra- and interobserver agreement for the deformity index was high. The intraobserver intraclass correlation coefficient for each observer was 0.98, 0.99 and 0.97, respectively, while the interobserver intraclass correlation coefficient was 0.98 for the first and 0.97 for the second set of calculations.

We also reviewed retrospectively 96 radiographs of children with Perthes’ disease, who were part of a multicentre trial which followed them to skeletal maturity. We found that the deformity index at two years correlated well with the Stulberg grading at skeletal maturity. A deformity index value above 0.3 was associated with the development of an aspherical femoral head. Using a deformity index value of 0.3 to divide groups for risk gives a sensitivity of 80% and specificity of 81% for predicting a Stulberg grade of III or IV.

We conclude that the deformity index at two years is a valid and reliable radiological outcome measure in unilateral Perthes’ disease.