Aims. The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. Patients and Methods. A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation. Results. Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). Conclusion. Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563–1569

This study aimed to define the rates of lower limb angular correction using temporary hemiepiphysiodesis in differing skeletal pathologies. A retrospective review of 61 children (36M:25F) with angular deformities about the knee who underwent 8-plate hemiepiphysiodesis (mean age 10.8y) was undertaken. The children were divided into 9 groups based on their underlying pathology (lower limb hypoplasia, Blount's disease, skeletal dysplasia, rickets, metabolic disease, acquired growth disturbance, vascular malformation, steroid use and complex genetic disorders). Radiographic measurements of each limb segment was undertaken using the TraumaCad® digital templating software based on standing long-leg radiographs - mechanical lateral distal femoral angle (mLDFA) and mechanical medial proximal tibial angle (mMPTA). The rate of correction of each parameter was calculated as a function of the time lapse between the operation date and first radiographic evidence of full correction of the mechanical axis (zone 1). A total of 144 limb segments (80 distal femoral, 64 proximal tibial physes) were analysed. 62.5% of children had mechanical axes outside the knee joint at the time of operation; 63.2% achieved full correction. The rate of angular correction at the distal femur (mLDFA) was quickest in those with acquired growth disturbance (1.15°/month), complex genetic disorders (1.12°/month) and rickets (0.93°/month). It was slowest in those with vascular malformation (0.40°/month), lower extremity hypoplasia (0.44°/month) and metabolic disease (0.49°/month). At the proximal tibia, mMPTA correction was quickest in those with acquired growth disturbance (0.77°/month) and skeletal dysplasia (0.57°/month); whilst being slowest in those with metabolic disease (0.22°/month) and Blount's disease (0.29°/month). The rate of angular correction about the knee varies with the underlying pathology with correction rates varying up to 3-fold. This study demonstrated the differential rate of correction of angular deformities in children with different skeletal pathologies, which would help guide the timing of hemiepiphysiodesis

Aim. To determine the rate of recurrence of coronal plane deformity in children treated with ‘guided growth’ using 8-plates, from the time of implant removal to skeletal maturity. Methods. Over a consecutive 5 year period between April 2008 and April 2013 we analysed our results of guided growth treatment using 8-plates to correct coronal plane lower limb deformity. Patients with neuromuscular disorders such as cerebral palsy were excluded. Deformity planning was performed using standardised techniques. Our standard practice is to remove the 8-plate and screws once deformity is corrected both clinically and radiologically. Patients were followed up until either skeletal maturity or recurrence, which necessitated reapplication of the 8-plate. We are aware of no study in which children treated with guided growth using 8-plates are followed up to skeletal maturity. Results. 267 patients were treated with 8-plates in our unit over this 5 year period. Of the patients in whom deformity was corrected and had subsequent plates removed, we identified 41 patients who have either reached skeletal maturity or had recurrence of deformity. Six patients required reapplication of the 8-plates implant. These were young and had skeletal dysplasia. Deformity parameters were analysed both clinically and radiologically in patients who have reached skeletal maturity and showed no recurrence, which necessitated further intervention. Conclusion. A higher proportion of younger patients, especially a sub-group with skeletal dysplasia had recurrence of deformity necessitating reapplication of the 8-plate device. In this group we recommend removal of only the metaphyseal screw once deformity is corrected. This would allow ease of reapplication if recurrence were to reoccur. Level of evidence: III

Aim. To study the results of using recombinant human bone morphogenic protein-2 (rhBMP-2) to achieve bone union in children with complex orthopaedic problems including skeletal dysplasia, congenital limb deficiencies and complex trauma. Materials & Methods. Between November 2006 to April 2010, rhBMP-2 was used to achieve bone healing in a total of 19 children. The indications were delayed union or non-union in 16 long bones where previous surgeries had failed and 4 posterior spinal fusions. All children had underlying complex orthopaedic problems predisposing them to delayed bone healing. The average age at the time of surgery was 13 years and 8 months (Range: From 4 years to 19 years and 4 months). There were 7 boys and 12 girls. Results. Average follow-up period was 9.6 months (Range: From 3 months to 2 years and 2 months). Out of the 16 long bone non-unions treated with rhBMP-2, 13 united without further interventions but 3 required further intervention. The average time for clinical union was 10 weeks (Range: from 6 weeks to 18 weeks). The average time for radiological union was 15 weeks (Range: from 7 weeks to 27 weeks). Two children who underwent instrumented posterior spinal fusion and two children who underwent uninstrumented spinal fusion achieved radiologically evident spinal fusion at an average of 16 weeks. No local or systemic complications attributable to BMP were noted in any of the children. Conclusions. rhBMP-2 can be used successfully to achieve bone healing in long bone nonunion and delayed union and posterior spinal fusion in children with complex orthopaedic problems including skeletal dysplasia, congenital limb deficiencies and complex trauma. Its use in children has shown no unwanted effects in the short term. Significance. rhBMP-2 has been approved by Food and Drug Administration (FDA) as a bone graft substitute in adults. Its use in adults has been widely reported but there are only a few case reports of its use in children. This study shows that rhBMP-2 can be safely

Aims

The aim of this retrospective cohort study was to assess and investigate the safety and efficacy of using a distal tibial osteotomy compared to proximal osteotomy for limb lengthening in children.

Purpose. To document the incidence and nature of complications associated with hemiepiphysiodesis using a screw and plate device (8-plate, Orthofix). Methods. We reviewed case notes and radiographs of 71 children (130 segments) with lower limb deformities treated with temporary hemiepiphyseal arrest using the 8-plate. 96% of deformities were in the coronal plane, 4% sagittal. 72% of coronal deformities demonstrated valgus malalignment. We defined three types of complications:. early (perioperative) complications eg infection and/or wound breakdown, bleeding, neurological impairment. implant related complications such as soft tissue irritation, plate breakage or migration,. complications involving the growth plate including rebound deformity, early physeal closure or iatrogenic deformity. Complications were related to variables of patient age, gender, diagnosis, location of deformity and associated surgery. Results. The average age at surgery was 10 years (range 3-16). There were 41 boys, 30 girls. 3/70 patients were treated for an idiopathic deformity: the remainder were affected by a wide range of pathologies (20% skeletal dysplasia, 20% metabolic bone disease). Full correction has occurred in 38% of patients. The remaining 45 children are all showing improvement in their deformity. 10 patients (14%) have had a complication: 3 patients had early complications (wound infection, wound breakdown and intra-operative hypotensive crisis) and 2 patients have had implant related complications (irritation and migration). In a further 5 patients there have been physis related problems: 1 over-correction, 1 recurrence and 3 cases in which the timing of the procedure or the ill-health of the physis did not allow correction to occur. Conclusion. The 8-plate is an effective device in the management of deformity secondary to pathological conditions. 10% of patients had complications: the majority were minor but care should be taken regarding the timing of the procedure related to the growth potential of the pathological physis

Purpose. To define the orthopaedic problems associated with pseudoachondroplasia (PSACH) and their functional impact. Methods. We reviewed the medical records of 12 consecutive patients presenting to our unit. Radiographic analysis of deformity included assessment of mechanical axis and dysplasia at hip, knee and ankle measured by acetabular index (AI), Reimer's migration percentage (MP), neck-shaft angle, distal lateral femoral (aDFLA) and proximal (mMPTA) and distal tibial angles. The paediatric/adolescent PODCI questionnaires and the SF36 were used to assess quality of life issues. Results. 12 patients (9 female) were reviewed at median age 18yrs (range 12-43yrs). Most symptoms related to walking tolerance, joint discomfort and deformity: 9 patients had genu varum, 7 tibial torsion, 2 patella instability and 3 significant low back pain. All patients had hip dysplasia. 10 had medial displacement of the mechanical axis, with a mean mDFLA 105 deg (88 -128) and mMPTA 75deg (51-90). 2 patients have been treated only with growth hormone; 10 patients have undergone a total of 9 distal femoral, 19 proximal tibial and 2 supramalleolar osteotomies. 6 procedures were performed using an external fixator. 7 limb segments have been treated by guided growth and in all these cases alignment has improved. One patient has had bilateral hip arthroplasties (age 29), a second patient has had bilateral patellectomies. These 10 patients have undergone a mean 3.8 operative procedures on a mean 2.4 occasions. Patients scored less well than their peer groups in all domains of the PODCI assessment. All have maintained some independent mobility. Conclusions. PSACH is a severe skeletal dysplasia with deformity at all levels of the lower limb affecting patient satisfaction and quality of life. Knee deformities are those which most frequently require surgical intervention. Significance. The genetic defect in PSACH differs from that in achondroplasia, joint degeneration is more common and maintenance of limb alignment is essential

Aims

Perthes’ disease (PD) often results in femoral head deformity and leg length discrepancy (LLD). Our objective was to analyze femoral morphology in PD patients at skeletal maturity to assess where the LLD originates, and evaluate the effect of contralateral epiphysiodesis for length equalization on proximal and subtrochanteric femoral lengths.

Aims

The study was undertaken to compare the efficacy of Woodcast splints and plaster-of-Paris casts in maintaining correction following sequential manipulation of idiopathic clubfeet.

Aims

Guided growth using eight-plates is commonly used for correction of angular limb deformities in growing children. The principle is of tethering at the physeal periphery while enabling growth in the rest of the physis. The method is also applied for epiphysiodesis to correct limb-length discrepancy (LLD). Concerns have been raised regarding the potential of this method to create an epiphyseal deformity. However, this has not been investigated. The purpose of this study was to detect and quantify the occurrence of deformities in the proximal tibial epiphysis following treatment with eight-plates.

Aims

Our aim was to investigate the predictive factors for the development of a rebound phenomenon after temporary hemiepiphysiodesis in children with genu valgum.

We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity.

We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis.

Two types of fracture, early and late, have been reported following limb lengthening in patients with achondroplasia (ACH) and hypochondroplasia (HCH).

We reviewed 25 patients with these conditions who underwent 72 segmental limb lengthening procedures involving the femur and/or tibia, between 2003 and 2011. Gender, age at surgery, lengthened segment, body mass index, the shape of the callus, the amount and percentage of lengthening and the healing index were evaluated to determine predictive factors for the occurrence of early (within three weeks after removal of the fixation pins) and late fracture (> three weeks after removal of the pins). The Mann‑Whitney U test and Pearson’s chi-squared test for univariate analysis and stepwise regression model for multivariate analysis were used to identify the predictive factor for each fracture. Only one patient (two tibiae) was excluded from the analysis due to excessively slow formation of the regenerate, which required supplementary measures. A total of 24 patients with 70 limbs were included in the study.

There were 11 early fractures in eight patients. The shape of the callus (lateral or central callus) was the only statistical variable related to the occurrence of early fracture in univariate and multivariate analyses. Late fracture was observed in six limbs and the mean time between removal of the fixation pins and fracture was 18.3 weeks (3.3 to 38.4). Lengthening of the tibia, larger healing index, and lateral or central callus were related to the occurrence of a late fracture in univariate analysis. A multivariate analysis demonstrated that the shape of the callus was the strongest predictor for late fracture (odds ratio: 19.3, 95% confidence interval: 2.91 to 128). Lateral or central callus had a significantly larger risk of fracture than fusiform, cylindrical, or concave callus.

Radiological monitoring of the shape of the callus during distraction is important to prevent early and late fracture of lengthened limbs in patients with ACH or HCH. In patients with thin callus formation, some measures to stimulate bone formation should be considered as early as possible.

Cite this article: Bone Joint J 2014;96-B:1269–73.

Objectives

The development of tibiofemoral angle in children has shown ethnic variations. However this data is unavailable for our population.

We undertook a retrospective analysis of 306 procedures on 233 patients, with a mean age of 12 years (1 to 21), in order to evaluate the use of somatosensory evoked potential (SSEP) monitoring for the early detection of nerve compromise during external fixation procedures for limb lengthening and correction of deformity. Significant SSEP changes were identified during 58 procedures (19%). In 32 instances (10.5%) the changes were transient, and resolved once the surgical cause had been removed. The remaining 26 (8.5%) were analysed in two groups, depending on whether or not corrective action had been performed in response to critical changes in the SSEP recordings. In 16 cases in which no corrective action was taken, 13 (81.2%, 4.2% overall) developed a post-operative neurological deficit, six of which were permanent and seven temporary, persisting for five to 18 months. In the ten procedures in which corrective action was taken, four patients (40%, 1.3% overall) had a temporary (one to eight months) post-operative neuropathy and six had no deficit.

After appropriate intervention in response to SSEP changes, the incidence and severity of neurological deficits were significantly reduced, with no cases of permanent neuropathy. SSEP monitoring showed 100% sensitivity and 91% specificity for the detection of nerve injury during external fixation. It is an excellent diagnostic technique for identifying nerve lesions when they are still highly reversible.

Perthes’ disease is an osteonecrosis of the juvenile hip, the aetiology of which is unknown. A number of comorbid associations have been suggested that may offer insights into aetiology, yet the strength and validity of these are unclear. This study explored such associations through a case control study using the United Kingdom General Practice Research database. Associations investigated were those previously suggested within the literature. A total of 619 cases of Perthes’ disease were included, as were 2544 controls. The risk of Perthes’ disease was significantly increased with the presence of congenital anomalies of the genitourinary and inguinal region, such as hypospadias (odds ratio (OR) 4.04 (95% confidence interval (CI) 1.41 to 11.58)), undescended testis (OR 1.83 (95% CI 1.12 to 3.00)) and inguinal herniae (OR 1.79 (95% CI 1.02 to 3.16)). Attention deficit hyperactivity disorder was not associated with Perthes’ disease (OR 1.01 (95% CI 0.48 to 2.12)), although a generalised behavioural disorder was (OR 1.55 (95% CI 1.10 to 2.17)). Asthma significantly increased the risk of Perthes’ disease (OR 1.44 (95% CI 1.17 to 1.76)), which remained after adjusting for oral/parenteral steroid use.

Perthes’ disease has a significant association with congenital genitourinary and inguinal anomalies, suggesting that intra-uterine factors may be critical to causation. Other comorbid associations may offer insight to support or refute theories of pathogenesis.

It has been reported that there is an association between Perthes’ disease and poverty. We examined the demographic data of a group of 240 children (263 hips) who presented with Perthes’ disease in Greater Glasgow, where the mean deprivation scores are substantially greater than in the rest of Scotland, to see if this association applied and whether other clues to the aetiology of Perthes’ disease could be found. There were 197 boys and 43 girls; 39 (16.25%) had a family history of Perthes’ disease. Bone age in this series was heavily skewed towards the lower percentiles. The mean number of siblings was 1.9, with 31 (12.9%) being an only child. Maternal age at the birth of the first child showed no preponderance of older mothers. Maternal smoking during and after pregnancy was noted in 132 (55%), which compared with the 52% reported in the population of Greater Glasgow in general. Of the children in our series, 60 (25%) were in social class IV and V. However, this applies to more than half of the population of Greater Glasgow. There was no significant evidence of a preponderance of Perthes’ disease in the most deprived groups. The aetiology of Perthes’ disease is likely to be multifactorial and may include a genetic or deprivation influence resulting in delayed bone age.

We reviewed the medical records of 115 patients with 130 hips with developmental dysplasia with complete dislocation in the absence of a neuromuscular disorder, spontaneous reduction with a Pavlik harness, and a minimum of 14 years’ follow-up. The mean age at the time of harness application was 4.8 months (1 to 12) and the mean time spent in the harness was 6.1 months (3 to 12). A total of 108 hips (83.1%) were treated with the harness alone and supplementary surgery for residual acetabular dysplasia, as defined by an acetabular index > 30°, was performed in 22 hips (16.9%).

An overall satisfactory outcome (Severin grade I or II) was achieved in 119 hips (91.5%) at a mean follow-up of 16 years (14 to 32) with a follow-up rate of 75%. Avascular necrosis of the femoral head was noted in 16 hips (12.3%), seven of which (44%) underwent supplementary surgery and nine (56%) of which were classified as satisfactory. The acetabular index was the most reliable predictor of residual acetabular dysplasia.