There is believed to be a correlation between congenital idiopathic scoliosis and congenital heart disease (CHD). Clinical and cardiological data was recorded for 3538 adolescents suffering from CHD. Data collected included the type of scoliosis; the direction of the curve; the Cobb angle; the number of curves and the presence or not of previous corrective cardiac surgery. Over 30% of the study group were found to suffer from scoliosis and a positive correlation with specific syndromes was also identified. The mean age of the patients was 34.0 +/− 14.0 years. The maximum Cobb angle was 107 degrees while the median was 7.6 degreees. Scoliosis was present in 37/188 (19.7%) was Eisenmenger syndrome (with R-L shunt) and 60/158= 38% with complex cardiac anatomy. There were also 20/103= 19.4% patients with univentricular (Fontan) circulation. Scoliosis was not necessarily related to previous corrective cardiac surgery, contrary to the current assumption in the literature. The hypothesis of common genetic pathway defects expressed both in cardiovascular and musculoskeletal organogenesis was raised and the TGF-beta pathway involvement is speculated.

Abstract: It is known that the treatment of intra or extraspinal paediatric tumours with surgery and radiotherapy or radiotherapy alone can lead to the onset of progressive spinal deformity the management of which can be extremely challenging. We review our series of patients who have developed a spinal deformity in these circumstances.

Methods: A review of all patients seen between 1996 and 2007 in the spinal department who have developed a significant spinal deformity following treatment for an intra or extra spinal tumour.

Results: 14 patients were identified. The age of presentation to the spinal service was between 2 years 6 months and 15 years 3 months. The underlying diagnoses were Wilms Tumour treated with surgery and radiotherapy in 3, 1 extraspinal sarcoma treated with surgical resection, radiotherapy and chemotherapy, 1 extraspinal neuro-blastoma treated with surgery, radiotherapy, chemotherapy and stem cell rescue and 9 intra spinal tumours (PNET, astrocytoma, ganglioneuroblastoma and der-moid) all managed with resection with or without radiotherapy. The spinal deformities that have developed were thoracic kyphoscoliosis, thoracolumbar kyphosis and lumber hyperlordosis. The spinal management of these deformities has been conservative in 12 with regular assessment to allow intervention if indicated. 2 patients have undergone surgery, a vascularised fibular strut graft and anterior instrumentation in 1 and a non vascularised rib graft in 1 for progressive deformity felt to lead to neurological dysfunction or lung hypoplasia. The cases managed operatively were complicated by poor posterior soft tissues following previous surgery and radiotherapy requiring an anterior approach. In all patients who were treated with radiotherapy platyspondyly was always seen in the vertebral bodies anteriorly and this corresponded to the apex of the deformity.

Conclusion: In all children who undergo spinal surgery and or radiotherapy for paediatric tumours there must be ongoing surveillance for the development of a spinal deformity. We feel that this is as much a result of anterior growth arrest secondary to radiotherapy as to posterior laminectomy for intra canal tumour excision. The surgical management of this problem is complex and may require innovative solutions.

Introduction: The natural history of scoliosis in the presence of a cord syrinx, either treated conservatively, or post surgically, is disputed. It is generally believed to be associated with a greater likelihood of rapid deformity progression pre-operatively and a much greater likelihood of intraoperative neural injury.

In this study we aimed to retrospectively assess the local experience by reviewing patients, treated over the last 10 years, in whom scoliosis has been established, by means of MR imaging, to be associated with a cord syrinx.

Methods: A retrospective cohort study was undertaken of paediatric patients attending the Leeds Spinal Unit between the years of 1997 and 2007. The entry criteria for this study were spinal deformity with MRI proven cord syrinx, in a patient without underlying tumour or other cord anomaly. Given the association with Chiari malformation this was a measured parameter rather than exclusion criteria. Other parameters assessed were mode of presentation, progression of scoliosis, details of the identified syrinx, chosen mode of treatment for the syrinx and the scoliosis (including conservative) and outcome measures (neurological function and sequelae, change in Cobb angle/deformity correction).

Results: A total of 46 patients were identified with scoliosis and an associated syrinx. The age range was from 3 to 18 years. Only 12 were male. The syrinx was associated with an Arnold Chiari malformation in 24% of patients, and located at the apex of, or local to, the maximum deformity in 73%.

The syrinx was treated surgically in 10 patients, with 80% of these achieving either deformity arrest, or no longer requiring surgical deformity correction. In the 2 patients from the same subset who did undergo deformity correction there was no neurological sequelae. Of the conservatively managed syrinxes, deformity correction with intraoperative cord monitoring was nevertheless undertaken in 31%, all without neurological sequelae. In just 4 patients (of 69%) who did not proceed to deformity correction, surgery was precluded by the inherent risks in the presence of an untreated syrinx.

Conclusion: This small series does not lend support to the literature and anecdotal evidence for significantly increased surgical risk in deformity correction without treatment for syrinx first. However, this may reflect the fact that all syrinxes likely to compromise the surgical procedure were assessed as such and treated first. In the cohort of patients whose syrinxes were treated conservatively, a significant proportion did not require subsequent deformity surgery. Identifying a syrinx by, the mandatory, MR imaging of a patient with a deformity before considering surgical correction, appears to identify a significant proportion of syrinxes which neither significantly accelerate the progression deformity, or which do not lead to poor outcome after deformity surgery.

INTRODUCTION. We conducted a review of the Leeds Regional Bone Tumour Registry for primary bone tumours of the spine since establishment in 1958 until year 2000.

AIM. To analyze the incidence of primary tumours of the spine and to record the site of occurrence, sex distribution, survival and pathology of these tumours.

Summary of the Background Data. Primary tumours of the spine are particularly rare, accounting for between 4% and 13% of published series of primary bone tumours.

METHOD. The Leeds Bone Tumour Registry was reviewed and a total of 2750 cases of bone tumours and tumour-like cases were analyzed. Consultants in orthopaedic surgery, neurosurgery, oncology and pathology in North and West Yorkshire and Humberside contribute to the Registry.

RESULTS. Primary bone tumours of the osseous spine constitute only 126 of the 2,750 cases (4.6%). Chordoma was the most frequent tumour in the cervical and sacral regions, while the most common diagnosis overall was multiple myeloma and plasmacytoma. Osteosarcoma ranked third. The mean age of presentation was 42 years and pain was the most common presenting symptom, occurring in 95% of malignant and 76% of benign tumours. Neurological involvement occurred in 52% of malignant tumours and usually meant a poor prognosis,

CONCLUSIONS. The establishment of Bone Tumour Registries is the only way that sufficient data on large numbers of these rare tumours can be accumulated to provide a valuable and otherwise unavailable source of information for research, education and clinical follow-up.

Purpose: We conducted a review of the Leeds Regional Bone Tumour Registry for primary bone tumours of the axial skeleton since establishment in 1958 until year 2000 to analyze the incidence of primary tumours of the axial skeleton and to record their site of occurrence, sex distribution, survival and pathology.

Method: Primary tumours of the axial skeleton are particularly rare, accounting for between 4% and 13% of published series of primary bone tumours. The Leeds Bone Tumour Registry was reviewed and a total of 2750 cases of bone tumours and tumour-like cases were analyzed. Consultants in orthopaedic surgery, neurosurgery, oncology and pathology in North and West Yorkshire and Humberside contribute to the Registry.

Results: Primary bone tumours of the axial skeleton constitute only 126 of the 2,750 cases (4.6%). Chordoma was the most frequent tumour in the cervical and sacral regions, while the most common diagnosis overall was myeloma. Osteosarcoma ranked third. Mean age of presentation was 42 years. Pain was the most common presenting symptom, occurring in 95% of malignant and 76% of benign tumours. Neurological involvement occurred in 52% of malignant tumours and usually meant a poor prognosis,

Conclusions: The establishment of Bone Tumour Registries is the only way that sufficient data on large numbers of these rare tumours can be accumulated to provide a valuable and otherwise unavailable source of information for research, education and clinical follow-up.

Introduction: Given the timing and nature of adolescent-onset idiopathic scoliosis (AIS), this progressively deforming condition is highly likely to have a significant psychosocial impact. Body image dissatisfaction is a frequent finding in AIS patients, which is of concern, as there is a well-documented causative link between body image disturbance and the formation of disordered eating behaviour, reflected in the theoretical models for this area of psychopathology.

However, although AIS patients have frequently been observed to exhibit disturbed body image, there has been no previous attempt to assess indications of disordered eating behaviour. Given the prevalence of AIS in adolescent females and the possible medical consequences of disordered eating, this study aimed to investigate whether AIS patients have an increased likelihood of low body weight.

Methods and Results: Patients were recruited over a four month period from the regional scoliosis out-patient clinic at St James’ University Hospital; 44 female scoliosis patients participated, with a mean age of 16 (range 13 to 19). All those meeting the inclusion criteria (diagnosed with AIS, not diagnosed with any other serious medical condition), and attending clinic over the data collection period were asked to participate.

Weight, height, and BMI (weight (kg)/height(m)2) measurements taken from AIS participants were compared to age and gender-adjusted normative data. No uncoiling correction was made for the scoliosis in terms of body height. The International Classification of Diseases (ICD-10) body mass criterion for eating pathology was used to determine how many AIS participants were within the range considered eating disordered.

Independent-sample t-tests revealed that, when compared to the normative data, the AIS group did not differ significantly in terms of height (p=0.646). However, they were significantly lighter (p< 0.001), and had significantly lower BMI scores (p< 0.001); 25% of the sample had a BMI score within the range considered anorectic. Of these low-BMI patients, the mean index score was 15.6 (range 12.9–17.5). The mean weight was 40.25 kg (6st 4lbs), with a range from 31.5 to 49 kg (4st 13lbs – 7st 11lbs). The body mass data for this low-BMI group, both in terms of range and severity, is not within ‘normal’ body shape variation, and would not be expected in healthy adolescent females.

Conclusion: The relationship between a diagnosis of AIS and low body weight may indicate disordered eating behaviour and is thus a cause for considerable concern. This is of particular relevance in the light of the well-established relationship between eating psychopathology and osteoporosis, which may result if disordered eating produces a reduced peak bone mass. Organic health consequences may need to be added to a matter previously considered to be one of cosmetic deformation.

We investigate the hypothesis that normal spinal shape is genetically determined raising the possibility that individuals with scoliosis may inherit an ‘at risk’ spinal shape leading to the clustering of scoliosis within families.

A large scale study of spinal topography (Quantec system) in straight-backed sibling pairs of schoolchildren was undertaken. All children had no family or past history of spinal pathology and passed a modified Adams forward bend test. 223 sibling pairs were scanned including 28 heterozygote and 11 homozygote pairs. Control groups were constructed by breaking the sibling pair and replacing the removed sibling with an age, sex matched, unrelated counterpart. A sagittal spine line was extracted and analysed using principal component analysis to produce four scores accounting for 97.5% of the cumulative variation in shape. The scores were analysed by intraclass correlation coefficients (ICC).

The results for the second score were:-

All sibling groups showed greater correlation of sagittal profile for the second principal component than unrelated controls. Significant correlations in shape were seen for both twin groups where no correlation was seen with controls. A significant correlation was observed in same sex pairings. This work suggests that some elements of spinal profile may be familial but also shows correlation with sex. Both of these observations may be important in the aetiology of idiopathic scoliosis.