Aims. The aim of this study is to define a core outcome set (COS) to allow consistency in outcome reporting amongst studies investigating the management of orthopaedic treatment in children with spinal dysraphism (SD). Methods. Relevant outcomes will be identified in a four-stage process from both the literature and key stakeholders (patients, their families, and clinical professionals). Previous outcomes used in clinical studies will be identified through a systematic review of the literature, and each outcome will be assigned to one of the five core areas, defined by the Outcome Measures in Rheumatoid Arthritis Clinical Trials (OMERACT). Additional possible outcomes will be identified through consultation with patients affected by SD and their families. Results. Outcomes identified in these stages will be included in a two-round Delphi process that will involve key stakeholders in the management of SD. A final list including the identified outcomes will then be summarized in a consensus meeting attended by representatives of the key stakeholders groups. Conclusion. The best approach to provision of orthopaedic care in patients with SD is yet to be decided. The reporting of different outcomes to define success among studies, often based on personal preferences and local culture, has made it difficult to compare the effect of treatments for this condition. The development of a COS for orthopaedic management in SD will enable meaningful reporting and facilitate comparisons in future clinical trials, thereby assisting complex decision-making in the clinical management of these children. Cite this article: Bone Jt Open 2022;3(1):54–60

The purpose of this cohort study is to determine the incidence of all congenital vertebral anomalies detected antenatally through ultrasound. We also reported on the early mortality rate for this patient cohort, as well as the frequency and type of associated congenital anomalies. The East Midlands and South Yorkshire Anomalies Register consists of data on all voluntary reports of congenital anomalies, from an annual baseline birth rate of 67000 births. We analysed all registered congenital anomalies reported over a 10 year period. Between January 1997 and January 2007, 108 vertebral anomalies were reported, excluding spinal dysraphism (incidence 0.01%). 61 of these were detected antenatally (56%), 17 were detected postnatally (16%) and in 30 patients, the precise time of diagnosis was unclear (28%). At the time of analysis January 2007, 45 of 108 patients had died, either in utero or soon after delivery (42%). 12 fetuses remained in utero and 51 infants were alive. The mortality rate for antenatally diagnosed patients was 41% and the majority were electively terminated (72%). 2 fetuses electively terminated had vertebral anomalies in isolation. There is a relatively high incidence of elective termination of pregnancy as a result of antenatal anomaly ultrasound screening. We have evidence to suggest that foetuses with potentially minor congenital anomalies are being electively terminated at approximately 18 weeks gestation. A structured and timely spinal counselling should be offered once antenatal vertebral anomalies have been identified

Study Design: Retrospective case series. Objective: To evaluate the clinical outcome, radiographic results and complications associated with single rod anterior instrumentation in neuromuscular thoracolumbar scoliosis. Methods: Retrospective study with mean follow up of 35 months. Subjects: Nine patients (6F, 3M), mean age 15 years, were operated on between 1994–2000. This heterogeneous patient group consisted of five cases of spinal dysraphism, one prune belly syndrome, one arthrogryposis, one myotonic dystrophy and one congenital myopathic dystrophy (muscle-eye-brain-syndrome). All patients were ambulatory and had minimal pelvic obliquity (< 15degrees). Outcome measures: Pre-operative, post-operative and final follow-up measurements of Cobb angles, apical vertebral translation (AVT), thoracic kyphosis, lumbar lordosis, sagittal and coronal balance were recorded along with operative complications, pseudarthrosis, metalwork failure and loss of correction. Results: There was one rod breakage and one case of proximal thoracic curve progression requiring supplementary posterior surgery. For the remaining 7 patients, the average corrections for Cobb angle was 62% (52 to 20 degrees), AVT was 53% (5.7 to 2.7cms), and both thoracic kyphosis and lumbar lordosis remained unchanged. No pseudarthrosis, vascular or neurological complications were encountered. Subjectively results were excellent in six and good in one. Conclusions: Selective anterior instrumentation for neuromuscular scoliosis using a single rod resulted in acceptable clinical and radiographic outcomes in this highly selected series. Advantages include preservation of distal lumbar motion segments whilst maintaining sagittal and coronal alignment. We believe that this method of scoliosis correction has a definite yet select role in patients who are ambulatory, have minimal pelvic obliquity (< 15degrees), non-progressive pathology and near normal mental function

Objective: To evaluate the subjective clinical outcomes, radiographic results and complications associated with single solid rod anterior instrumentation in neuromuscular scoliosis. Design: Retrospective clinical case series with a mean follow up of 30 months (range 24 – 42 months). Subjects: 9 consecutive cases (6F, 3M) with a mean age 15 years (range 11 – 24 years), underwent single solid rod anterior instrumentation of their neuromuscular thoracolumbar scoliosis between 1994 and 2000. The heterogeneous patient group consisted of 5 spinal dysraphism, and 1 each of prune belly syndrome, arthrogryposis, myotonic dystrophy and congenital myopathic dystrophy (muscle eye brain syndrome). All patients were ambulatory and had minimal pelvic obliquity (< 15 degrees). Outcome measures: Pre-operative, post-operative and final follow up measurements were collected for 1) Cobb angles, 2) apical vertebral translation (AVT), 3) thoracic kyphosis (T5-12) and 4) lumbar lordosis (L1-5). Operative complications, pseudarthrosis, metalwork failure and loss of correction were also recorded. Results: There was 1 each of rod breakage and upper thoracic curve progression requiring supplementary posterior surgery. For the remaining 7 patients, the average follow-up corrections for Cobb angle was 56% (49 to 22 degrees), AVT was 49% (5.1 to 2.6 cms), and both the thoracic kyphosis and lumbar lordosis remained unchanged. No significant loss in correction occurred during the post-operative period to final follow-up in all the above parameters. No pseudarthrosis, vascular or neurological complications were encountered. Subjectively, there were 6 excellent and 1 good results. Conclusions: In this limited case review, selective anterior instrumentation for neuromuscular scoliosis using a single solid rod system resulted in acceptable clinical and radiographic outcomes. Our results appear to compare favourably with those published for the recommended method of posterior instrumentation. Advantages include preservation of distal lumbar motion segments whilst maintaining segmental saggital and coronal alignment. We believe that this method of scoliosis correction has a definite yet select role in patients who are ambulatory, have minimal pelvic obliquity (< 15 degrees), non-progressive pathology and near normal mental function

Aims

Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.