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

We performed a retrospective review of 27 scoliotic patients with syringomyelia using MRI. Their mean age at the first MRI examination was 10.9 years, and at the final review 15.8 years. The mean ratio of the diameter of the syrinx to the cord on the midsagittal MRI (S/C ratio) decreased from 0.49 to 0.24; 14 patients showed a decrease of 50% or more (reduction group). In this reduction group, the cerebellar tonsillar herniation decreased from a mean of 11.3 mm to 6.0 mm, and some improvement in dissociated sensory disturbance was seen in nine of 13 patients. The scoliosis improved by 5° or more in six patients in the reduction group. Our results indicate that spontaneous shrinkage of syringomyelia in children is not unusual and is associated with improvement in the tonsillar herniation, the scoliosis and the neurological deficit

Our aim was to review the efficacy of the wound vacuum-assisted closure (VAC) system in the treatment of deep infection after extensive instrumentation and fusion for spinal deformity in children and adolescents.

A total of 14 patients with early deep spinal infection were treated using this technique. Of these, 12 had neuromuscular or syndromic problems. Clinical and laboratory data were reviewed. The mean follow-up was 44 months (24 to 72). All wounds healed. Two patients required plastic surgery to speed up the process. In no patient was the hardware removed and there was no loss of correction or recurrent infection.

We believe that the wound VAC system is a useful tool in the armamentarium of the spinal surgeon dealing with patients susceptible to wound infections, especially those with neuromuscular diseases. It allows for the retention of the instrumentation and the maintenance of spinal correction. It is reliable and easy to use.

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. Methods. Paediatric patients attending a speciality outpatient clinic were recruited through consecutive sampling. Two groups of patients were included: idiopathic scoliosis, and paediatric orthopaedic upper and lower limb condition without scoliosis. Patients were asked to complete PedsQL 4.0 generic core scales, Youth version of 5-level EuroQol-5-dimension questionnaire, and Refined Scoliosis Research Society 22-item (SRS-22r) questionnaire. Statistical analyses included scores comparison between scoliosis and limb pathology patients using independent-samples t-test, and correlation tests of PedsQL and SRS-22r. Results. A total of 566 paediatric patients were recruited: 357 (63.0%) having idiopathic scoliosis, and 209 (37.0%) with limb conditions. Patients with limb pathology had lower functioning scale, summary, and total scores of PedsQL than scoliosis patients (p < 0.05 to p < 0.001). No floor or ceiling effects (< 15%) were detected for PedsQL Psychosocial Health Summary and total scores in both groups. PedsQL was sensitive in differentiating patients with/without problems in their daily lives (p < 0.05 to p < 0.01). PedsQL summary and total scores correlated with SRS-22r total score. Conclusion. PedsQL is an effective HRQoL measure for both paediatric orthopaedic groups with minimal ceiling and floor effects, and is capable of detecting worse HRQoL in patients with limb pathology. The multidimensional PedsQL is sensitive in differentiating among those with daily life problems, especially for scoliosis patients. Cite this article: Bone Joint J 2020;102-B(7):890–898

Aims

Reimers migration percentage (MP) is a key measure to inform decision-making around the management of hip displacement in cerebral palsy (CP). The aim of this study is to assess validity and inter- and intra-rater reliability of a novel method of measuring MP using a smart phone app (HipScreen (HS) app).

The purpose of the study was to identify risk factors that are associated with re-displacement of the hip after surgical reconstruction in cerebral palsy. Retrospective review of children with cerebral palsy who had hip reconstruction with proximal femoral varus derotation osteotomy (VDRO) and Dega-type pelvic osteotomy, between 2005–2012, at a UK and European institution, was performed. Patient demographics, GMFCS, clinical and radiological outcome were assessed as well as the presence of pelvic obliquity and significant scoliosis (Cobb angle > 10 degrees). Redisplacement was defined as Reimer's Migration Index (MI) >30% at final follow-up. Logistic regression analysis was used to assess which factors were predictive of redisplacement and adjusted for clustered variables (α = 0.05). Eighty hips were identified in 61 patients. The mean age at surgery was 8.8 years (± 3.3). Mean MI pre-op was 68% (± 23%) and post-op was 8% (± 12%). At a mean follow-up, of 3.2 years (± 2.0), 23 hips had a MI >30%. Of these; five were symptomatic, and one had required a salvage procedure. Metalwork removal was undertaken in 14 hips. Logistic regression demonstrated that the pre-operative MI and the percentage of acute correction were significant predictors of re-displacement. If the pre-operative MI was greater than 65 percent, the odds ratio (OR) for redisplacement was 5.99 (p = 0.04). If correction of the MI was less than 90% of the pre-operative MI, the OR for re-displacement was 4.6 (p = 0.03). Age at the time of surgery, GMFCS, pelvic obliquity and scoliosis were not predictive of re-displacement. These results, firstly, highlight the importance of hip surveillance in children with cerebral palsy to allow timely intervention to ensure adequate radiological outcomes. Secondly, as in developmental hip dysplasia, full concentric reduction is essential to reduce the risk of re-displacement, with its associated clinical consequences

In a prospective trial we performed MRI of the spine and hind brain in 31 patients with scoliosis of onset between the ages of four and 12 years. In eight patients (26%) there was a significant neuroanatomical abnormality; there were six cases of Chiari-1 malformation associated with a syrinx, one isolated Chiari-1 malformation and one astrocytoma of the cervical spine. Four of these patients had left-sided curves. There were no clinical features which could reliably identify those patients with abnormalities on MRI. In particular, the unilateral absence of abdominal reflexes was found to be non-specific (1 of 8 of patients with neuroanatomical abnormalities (12.5%) v 2 of 23 with normal scans (8.7%)). In view of the established risks of surgical correction of scoliosis in the presence of undecompressed syringomyelia and the possible improvement that may follow decompression of the foramen magnum, we feel that MRI of all patients with scoliosis of juvenile onset should be obligatory

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

Background. The association between idiopathic scoliosis and intrinsic abnormalities of the spinal cord are well known. MRI is the most sensitive imaging modality available to diagnose an intraspinal anomaly. The use of a routine ‘screening MRI’ prior to scoliosis correction is controversial. Purpose. This study sought to quantify the frequency of previously unidentified cord anomalies identified by a routine pre-operative MRI, in patients planned for surgical scoliosis correction. Methods and results. The patient group comprised 206 patients with idiopathic scoliosis who underwent deformity correction from 1998 to 2008. Of these, 20 (9.7 %) patients were diagnosed with an unexpected intraspinal anomaly (isolated syrinx-7, syrinx with Chiari malformation-4, isolated Chiari malformation-4, syrinx with tethered cord-2, split spinal cord-1, intrinsic spinal cord tumour-1, AV fistula-1) on routine pre-operative MRI. In all cases a neurosurgical opinion was sought prior to further intervention. Of the 20 patients, 11 underwent a neurosurgical procedure (de-tethering of cord, decompression of Chiari malformation, decompression of syrinx). No statistically significant difference was identified between individuals with or without a neurosurgical anomaly with respect to the age of presentation, gender, side and degree of curve. Conclusion. The high frequency of previously unidentified cord abnormalities detected, and the frequent need for subsequent neurosurgical intervention, suggests that MRI assessment prior to deformity correction is important in the management of idiopathic scoliosis

The study describes a technique of tibial autograft to augment posterior instrumented spinal fusion in a population of paediatric patients with severe idiopathic, neuromuscular or syndromic scoliosis who are at a higher risk of postoperative pseudarthrosis and reports patient outcomes in terms of union rate, donor site morbidity and cost. Patients were identified from a review of waiting list and operating room records between 2007–2014. Surgery was performed by the senior author. Information on patient demographics, underlying diagnosis, age at surgery, revision surgery and length of follow-up was obtained from clinic notes. Parents of children were followed up with a structured telephone questionnaire regarding ambulatory status, post-operative pain, infection, further surgery and general satisfaction. Four hundred and nine patients underwent posterior instrumented spinal fusion, during the study period. Forty-two patients’ fusions were augmented with tibial graft, 40 of whom participated in the study. There were no cases of donor site infection, compartment syndrome, tibial fracture or perioperative mortality. In 85% of cases leg pain had resolved within 6 weeks, and 100% within 6 months of surgery. There were 6 cases of revision spinal surgery, 3 for infection, 2 for sacroiliac screw removal and 1 for sacroiliac screw revision. There were no clinical cases of spinal pseudarthrosis in this series. All parents were satisfied by the clinical outcome of both the tibial and spinal surgeries. Spinal fusion utilising tibial autograft is advocated as a simple, safe and cost-effective method of providing significant structural autograft to support fusion for a population of patients with high risk of junctional pseudarthrosis. With the exception of transient post-operative pain, the procedure was without any serious donor site morbidity. The outcomes of this study were as expected and in keeping with previous reports

The standard approach of diagnosing and monitoring scoliosis involves using the Cobb angle from posteroanterior (PA) radiograph. This approach has two key limitations: 1) It involves exposing the patients to ionising radiation during a period of heightened radiosensitivity. 2) The 2D x-ray image is a projection image of a 3D deformity and the Cobb angle represents only lateral rotation. 3DUS would overcome both these limitations. We developed a 3DUS system by combining motion capture technology, a conventional 2D ultrasound scanner and bespoke software. An ex vivo experiment and a pilot clinical study were carried out to demonstrate the system's ability in identifying vertebrae landmarks and quantifying the curvature. For the ex vivo validation, a spine phantom was created by 3D-printing a segmented abdo-pelvis CT scan. The spine phantom was then scanned using 3DUS and the level of agreement in the dimensions measured using 3DUS and CT was assessed. An 11 year old female with adolescent idiopathic scoliosis (AIS) was scanned with 3DUS. The SP co-ordinates were projected on a plane of best-fit to compare the curvature angle from 3DUS with the Cobb angle from the x-ray image. The spinous (SP), transverse processes and the laminae demonstrated high echogenicity and were easily identifiable. The difference between the spine phantom inter-SP dimension measurements made in 3DUS and CT was <2.5%. The PA x-ray of the AIS patient revealed 47° (L4-T11) and 52° (T6-T11) curves. 3DUS was able to represent the deformity in 3D revealing complex curvatures in all planes. The curvature angle from derived from 3DUS for the L4-T11 and T6-T11 curves were 132° (48°) and 125° (55°) respectively. The results of this pilot study demonstrate 3DUS as a promising tool for imaging spine curvature

Aims

The Fassier Duval (FD) rod is a third-generation telescopic implant for children with osteogenesis imperfecta (OI). Threaded fixation enables proximal insertion without opening the knee or ankle joint. We have reviewed our combined two-centre experience with this implant.

Aims

The aim of this study was to assess whether supine flexibility predicts the likelihood of curve progression in patients with adolescent idiopathic scoliosis (AIS) undergoing brace treatment.

Introduction. Albania is one of the poorest countries in Western European with a GDP per capita standing at 26 percent of the EU average in 2010. Whilst there is government-funded universal free provision of healthcare, it is accepted that delivery is patchy, not accessible to all and lacking expertise for more complex paediatric orthopaedic conditions. With the sponsorship of a UK-based charity, we have set up and completed 5 visits to Albania (3 assessment and 2 operative) to provide additional expertise for paediatric orthopaedic disorders running parallel to and utilising currently available local services. We present the results of this treatment and training programme to date. Patients and methods. Between 2008 and 2011, we assessed 204 children and adolescents with paediatric orthopaedic disorders in Tirana and Durres on 3 separate visits. Of these, 28 were listed for surgical procedures whilst the rest were treated non-operatively. Of the listed patients, 14 patients underwent surgical intervention (total of 18 procedures). Results. The most common diagnoses were developmental dysplasia of the hip, club feet, cerebral palsy and scoliosis. Most patients were treated non-operatively with advice and/or reassurance. Of those listed for surgery, the reasons for cancellation included problems with access to the treating hospital and failure to establish patient contact on the day of admission. Of the operated patients, the procedures performed, the perioperative challenges and significant complications (2/18) will be discussed. Conclusion. Although it is viable to establish parallel service delivery of paediatric orthopaedic surgical services in countries such as Albania, the perioperative and social challenges must be considered

Aims

To monitor the performance of services for developmental dysplasia of the hip (DDH) in Northern Ireland and identify potential improvements to enhance quality of service and plan for the future.

Aims

Using 90% of final height as a benchmark, we sought to develop a quick, quantitative and reproducible method of estimating skeletal maturity based on topographical changes in the distal femoral physis.

Aims

We report the use of the distal radius and ulna (DRU) classification for the prediction of peak growth (PG) and growth cessation (GC) in 777 patients with idiopathic scoliosis. We compare this classification with other commonly used parameters of maturity.

Aims

This study compared the long-term results following Salter osteotomy and Pemberton acetabuloplasty in children with developmental dysplasia of the hip (DDH). We assessed if there was a greater increase in pelvic height following the Salter osteotomy, and if this had a continued effect on pelvic tilt, lumbar curvature or functional outcomes.

The accurate assessment of skeletal maturity is essential in the management of orthopaedic conditions in the growing child. In order to identify the time of peak height velocity (PHV) in adolescents, two systems for assessing skeletal maturity have been described recently; the calcaneal apophyseal ossification method and the Sanders hand scores.

The purpose of this study was to compare these methods in assessing skeletal maturity relative to PHV. We studied the radiographs of a historical group of 94 healthy children (49 females and 45 males), who had been followed longitudinally between the ages of three and 18 years with serial radiographs and physical examination. Radiographs of the foot and hand were undertaken in these children at least annually between the ages of ten and 15 years. We reviewed 738 radiographs of the foot and 694 radiographs of the hand. PHV was calculated from measurements of height taken at the time of the radiographs.

Prior to PHV we observed four of six stages of calcaneal apophyseal ossification and two of eight Sanders stages. Calcaneal stage 3 and Sanders stage 2 was seen to occur about 0.9 years before PHV, while calcaneal stage 4 and Sanders stage 3 occurred approximately 0.5 years after PHV.

The stages of the calcaneal and Sanders systems can be used in combination, offering better assessment of skeletal maturity with respect to PHV than either system alone.

Cite this article: Bone Joint J 2015;97-B:1710–17.

Pelvic obliquity is a common finding in adolescents with cerebral palsy, however, there is little agreement on its measurement or relationship with hip development at different gross motor function classification system (GMFCS) levels.

The purpose of this investigation was to study these issues in a large, population-based cohort of adolescents with cerebral palsy at transition into adult services.

The cohort were a subset of a three year birth cohort (n = 98, 65M: 33F, with a mean age of 18.8 years (14.8 to 23.63) at their last radiological review) with the common features of a migration percentage greater than 30% and a history of adductor release surgery.

Different radiological methods of measuring pelvic obliquity were investigated in 40 patients and the angle between the acetabular tear drops (ITDL) and the horizontal reference frame of the radiograph was found to be reliable, with good face validity. This was selected for further study in all 98 patients.

The median pelvic obliquity was 4° (interquartile range 2° to 8°). There was a strong correlation between hip morphology and the presence of pelvic obliquity (effect of ITDL on Sharpe’s angle in the higher hip; rho 7.20 (5% confidence interval 5.59 to 8.81, p < 0.001). This was particularly true in non-ambulant adolescents (GMFCS IV and V) with severe pelvic obliquity, but was also easily detectable and clinically relevant in ambulant adolescents with mild pelvic obliquity.

The identification of pelvic obliquity and its management deserves closer scrutiny in children and adolescents with cerebral palsy.

Cite this article: Bone Joint J 2015;97-B:1435–40.