Congenital Talipes Equinovarus (CTEV) occurs in approximately 1 in 1000 live births. Most cases occur as an isolated birth defect and are considered idiopathic. The widespread adoption of the Ponseti technique of serial casting followed by Achilles tenotomy and long term bracing has revolutionised the outcomes in CTEV. In most cases, plantigrade, flexible, pain-free feet may be produced without the need for extensive surgery. It is estimated that about 10% of cases of CTEV are not idiopathic. These feet are stiffer and more challenging to treat. In particular, there is little evidence in the literature concerning the efficacy of the Ponseti method in these cases.

In our institution, a dedicated weekly Ponseti clinic has operated since 2005. To date 140 patients have been treated. We prospectively enter all details regarding their management onto an independent international database.

The aim of this study was to audit the non-idiopathic cases of CTEV and to assess the effectiveness of the Ponseti technique in these challenging cases. Outcome measures included the Pirani score and eventual need for surgical intervention.

We identified 29 cases (46 feet) with non-idiopathic CTEV. This comprises 21% of our workload. Seventeen were bilateral. The commonest diagnoses were neuromuscular conditions such as spina bifida (5 cases) and cerebral palsy (3 cases). There were 4 cases of Trisomy 21. Other causes included Nail Patella syndrome, Moebius syndrome, Larsen syndrome and Ito syndrome. In approximately 12% of cases, the underlying disorder remained undiagnosed despite thorough medical and genetic testing.

In cases of non-idiopathic CTEV, the mean starting Pirani score was 5.5 (out of 6). After serial casting and Achilles tenotomy, the average score was 2.0. Twenty-one of 46 feet (46%) ultimately required further surgical intervention (mostly posteromedial release). We found that certain conditions were more likely to be successfully treated with the Ponseti method – these included conditions characterised by ligamentous laxity such as Trisomy 21 and Ehlers Danlos syndrome. All patients showed some improvement in Pirani score after serial casting.

We believe that it is essential to attempt the Ponseti method of serial casting in all cases of CTEV. More than half of all non-idiopathic cases will not require further surgical intervention – and those that do are not as stiff thanks to the effects of serial casting. Thus, the surgery required is not as complex as it might otherwise have been. This is the largest series of its kind in the current medical literature.

Introduction: Percutaneous epiphysiodesis is a well established procedure in the treatment of leg length discrepancy. Many techniques have been described ranging from an open technique to the more recently described percutaneous technique. This study assesses the percutaneous single portal technique, in combined distal femoral and proximal tibial lower limb epiphysiodesis, performed by a single surgeon.

Methods: We performed a retrospective review of cases performed in a single institution by a single surgeon from 1994 to present. A total of 45 combined epiphysiodesis were performed. 40 patients qualified for the study group with at least 2 years follow up. There were 19 female and 21 male patients, with the operative side equally shared between left and right.

Results: The mean predicted leg length discrepancy using the Mosley Straight Line Graph was 2.43 cm. The mean final leg length discrepancy, at an average follow up of 31 months, was 1.5 cm with a range of 0 to 2.81 cm. There were no angular deformities at follow up. One female patient had a knee effusion which resolved spontaneously. One male patient complained of anterior knee pain initially post surgery however this resolved at final follow up without treatment. The majority of patients (n=34) were inpatients, however more recently this procedure has been successfully carried out as a day case (n=6).

Discussion: Percutaneous epiphysiodesis has been accepted as a standard technique to treat leg length discrepancy of 2 cm to 5 cm. Several techniques have been described in the literature with varying complication rates. This study shows that single portal combined epiphysiodesis is successful and has a relatively low complication rate.

Introduction: Bone transport, or distraction osteogenesis, is a recognised technique to reconstruct extensive bony defects resulting from excision of bony tumours. Ilizarov demonstrated bone formation under tension allowing the movement of a free segment of living bone to fill intercalary defects. This study assesses the use of bone transport in the management of patients with resectable long bone tumours.

Methods: We retrospectively reviewed patients who underwent bone transport in two institutions, performed by a single surgeon. A total of 14 patients were included in the study. There were 11 males and 3 females. Histological results demonstrated osteosarcoma (n=7), Ewing’s sarcoma (n=6), and parosteal chondrosarcoma (n=1). The site of the tumour was the femur and tibia in 8 and 6 cases respectively.

Results: Bone transport was fully completed in 9 patients. Of the 5 patients remaining, 3 are currently in cast, 1 is currently undergoing tibial lengthening, and 1 patient died from local recurrence and distant spread of disease. The average length of bone resected in the tibia was 11 cm (range 8–15 cm), while in the femur the average was higher at 16.5 cm (range 12–27 cm). All patients underwent autologous bone grafting of their docking site from either the anterior or posterior iliac crest on the ipsilateral side. The average time in frame was 24.8 months. One patient undergoing tibial bone transport fell and sustained an ipsilateral supracondylar femoral fracture which was successfully treated with an external ring fixator.

Discussion: Bone transport is a recognised method of reconstructing extensive bony defects and is beneficial for patients with a good prognosis. It is a specialised technique and requires a multidisciplinary approach. Other techniques can be less time consuming however distraction osteogenesis avoids the complications associated with prosthetic or allograft replacements.

Hurler syndrome is an autosomal recessive metabolic storage disease, with specific musculoskeletal abnormalities termed dysostosis multiplex. Haematopoietic Stem Cell Transplant (HSCT) increases life expectancy, but its effects on the progression of dysostosis multiplex are less certain. We detail the ongoing follow up of 23 patients (range 2.6 – 20.7 years) at a mean of 8.5 years after successful HSCT, the largest series reported in the literature to date.

All patients were clinically examined at an annual multidisciplinary clinic, and serial radiological studies were reviewed to assess development and management of hip dysplasia and genu valgum.

All patients demonstrated characteristic acetabular dysplasia and failure of ossification of the superolateral femoral head. Thirteen patients have undergone hip containment, including eight bilateral combined pelvic osteotomy and femoral derotation, at a mean of 4.4 years. Mean preoperative acetabular angle was 34 ± 5°. Long term follow up of older patients (> 8 years, mean 9.9 years after surgery) demonstrated adequate femoral head cover, with mean centre-edge angle of 40 ± 5° (range 32 – 48°). More recently, isolated innominate osteotomy has been used.

Genu valgum of variable severity due to failure of ossification of the lateral aspect of the proximal tibial metaphysis was more variable, and seven patients underwent medial epiphyseal stapling at a mean of 7.8 years, decreasing tibiofemoral angle by a mean of 7°. Staple dislodgment, however, was seen in four children. All patients remain independently mobile, but hip stiffness and valgus knees contribute to the early fatigue and hip discomfort seen in older children.

Based on our series, we conclude that hip containment surgery has been successful at least into early adolescence, with overall mobility being well preserved. We recommend plating of the proximal tibial epiphysis. Further follow up will monitor the effectiveness of orthopaedic intervention.

Introduction: A parameter in surface topography was developed to measure left-right differences in back surface of different scoliosis patterns, and to relate these to biological asymmetry and the evolution of deformity. Because of the close association between scoliosis and growth, the hypothesis that scoliosis is growth, that it affects not just the spine but the whole body and that it falls into well-described biological patterns of asymmetry, was explored.

Methods: The new measure compares the positions of three points (mid way between the first thoracic vertebra and axilla, and one and two thirds from axilla to posterior superior iliac spines) on either side of the mid-line, reflecting right onto left and expressing the displacement along Cartesian axes in millimetres. The purpose is to measure size and growth differences at diagnosis and during follow-up. Statistical analysis was of prospectively collected topographic, radiographic and clinical data. There were three groups, all female: 1. mild asymmetry (N=84, no radiograph); 2. thoracic (N=65, mean Cobb angle 61.4°±19.5) and 3. thoracolumbar or lumbar (N=40, mean Cobb angle was 51.8°±23.0). Comparisons were made between each group and theoretically perfect symmetry (test value zero). Correlations with Cobb angle change over time were analysed.

Results: Groups one and three showed directional asymmetry in the coronal plane only, and were not statistically different from each other. Group two showed directional asymmetry at all levels, the side of the scoliosis convexity being larger in all three dimensions (left-right, antero-posterior and cranio-caudal). Changes in Cobb angle correlated with statistical significance with change in the vertical height of the convex side.

Conclusions: This topographic measure was developed specifically to quantify the asymmetry of the back surface, to assign it to a biological pattern and to observe how it might change during growth and scoliosis evolution. All levels of asymmetry, the minor as well as the true scoliosis, showed directional asymmetry (normal distribution of left-right differences about a mean that is not zero, genetically determined) which suggests an origin of scoliosis lying in the biology of growth and the evolution of morphology, rather than in a particular disease process. This asymmetry does not cause scoliosis: it is the result of asymmetric growth processes, it is scoliosis. The relevance of this view is that it obviates the need for an identifiable disease process, as scoliosis is a non-specific developmental response to physiological stress. It is the destabilising of the genetic control “programme” that operates in the growing organism to produce an adult phenotype which is an accurate expression of its genotype. This interpretation can explain observations of natural history that currently cause problems viz. the association with growth and development, lateralisation, increased incidence with other medical conditions, and female predominance, the recurrence of deformity after surgical correction and perhaps even the difficulty in reaching a final conclusion on the efficacy of brace treatment.

Background: Adolescent idiopathic scoliosis has been intensively studied, but is still not understood. It is the paradoxical co-existence of rude health and gross deformity in the same individual that needs to be explained. The essence of scoliosis is asymmetry, and bilateral asymmetries in many anatomical features have been described in association with it. Measurement of asymmetry in back surface made possible by surface topography can explore this aspect and throw light on the evolution of the deformity as the Cobb angle changes.

Objective: To quantify the asymmetry of the back surface in scoliosis and the lesser non-scoliosis deformities.

Methods: Routine clinical material (patient demographics, radiography and surface topography) was analysed. Changes in body symmetry were quantified, using a topographic measure that calculates the difference, in three dimensions and at three levels, between the left and right sides of the back across the mid-line (natal cleft to first thoracic vertebra). Girls only (to eliminate any effect from sexual dimorphism) with all presenting degrees of deformity from barely failing the forward bend test through mild scoliosis unconfirmed by radiograph (Group 1, N=311) to documented scoliosis (Cobb angle => 10°), apex at T12 or below (Group 2 and apex above T12 (Group 3).

Results: All groups showed significant departures from bilateral symmetry. Groups 1 and 2 were similar, in that the left side was taller but narrower than the left. In Group 3, the side of curve convexity was taller than the concave side. This was reversed in left thoracic scoliosis patterns and was seen to increase over time with progression of the Cobb angle.

Discussion: It has long been acknowledged that scoliosis and growth are inseparable, but studies have failed to demonstrate a disease process or endocrine imbalance. These findings suggest that it is not a disorder superimposed on growth, but that growth itself causes the deformity. The spine, the whole trunk, in fact, is crooked because it grew that way. Only a small discrepancy in left-right symmetry is sufficient, over time and during periods of rapid growth, to produce both the curve and the rotation.

Conclusion: Scoliosis is neither a disease nor a mechanically induced aberration. It results from asymmetrical growth, which occurs at the cellular and molecular level.

A 2002 study by Goldberg et al showed that surgery before age 10 for infantile onset idiopathic scoliosis (diagnosis < 4 years, Cobb angle => 10°) preserved neither respiratory function nor cosmesis, and has not been contradicted. In 2005, Mehta re-emphasised scoliosis correction by serial cast-bracing, while Thompson et al reported satisfactory results with growing rods. An analysis of the status quo of a cohort of patients with infantile idiopathic scoliosis (other diagnoses and syndromes excluded), managed by cast-bracing, was undertaken, asking whether interim progress was acceptable or demanded a change of protocol.

Of 35 patients born between October 1993 and December 2002,15 have completely resolved, age at diagnosis 1.6 ± 0.96 years, Cobb angle 20.3°±11.9, RVAD 11.1°±13.8, latest age 4.1± 2.3. 20 were prescribed cast-bracing, age at diagnosis 1.8±0.9 years, Cobb angle 47.3°±12.6, RVAD 29.6±24.5, age at treatment was 2.1±1.0 years. Cobb angle (p< 0.001) and RVAD (p=0.001) were larger in the treated group, but age at presentation was the same (p=0.473). Surgery was performed on 3 children unresponsive to initial casting, at ages 3.2, 3.6 and 3.7, and in 3 at ages 8.6, 10.1 and 11 years. 3 children, aged 6.0, 8.1 and 11.3 are out of brace with straight spines and 11 are stable in brace.

Infantile idiopathic scoliosis seems programmed to resolve or progress according to initial severity and in line with growth rate. Those who respond to casting in infancy generally remain stable until near puberty when surgery is uncontroversial. Those who progress relentlessly and immediately in cast remain the issue, as reports of newer methods include a wide range of ages and diagnoses and give their outcome in terms of Cobb angle only. It has not yet been shown that any treatment will alter their prognosis so constant analysis of all outcome parameters is essential.

It is customary to analyse scoliosis as a mechanical failure: first there is a straight spine (=normal), then an habitual and collapsing posture (=disease) and finally, structural remodelling (Hueter-Volkmann effect = scoliosis). This hypothesis makes two practical predictions:

There is a disease process causing the pathological posture. The purpose of gatherings such as this is to identify this pathology, thus far without success.

The failure of school scoliosis screening to achieve this end is well documented, but the consequence for the underlying hypothesis has not been analysed. Screening failed, not because it was unable to detect scoliosis, but because scoliosis did not behave as the hypothesis predicted.

Disease process: All theories presume some form of neurological or muscular deficit as the final pathway but while the variety is wide, e.g. (historically) anterior poliomyelitis; more recently proprioceptive defect, melatonin or calmodulin disorder, there is no clear evidence for such a deficit in adolescent idiopathic scoliosis (AIS). Of 1342 screening referrals to this centre, 10 had a neurological diagnosis (most of which were already known to the patients) and 598 had radiologically confirmed AIS. In contrast, 1707 referrals to the general clinics included 410 syndromic cases and 420 AIS. Patients with a neurological problem, by and large, find their own way to medical attention. The hypothesis does not explain the natural history or the aetiology, and awkward observations, such as the association with growth (Goldberg et al Spine.18(5):529–535.1993, Eur Spine J.2:29–36.1993 and, most recently, Ylikoski M. Journal of Pediatric Orthopaedics B.14:320–324, 2005) or the higher incidence in ballet dancers (Warren et al. New England Journal of Medicine.314(21):1348–1353.1986) and rhythmic gymnasts (Tanchev et al. Spine.25(11):1367–1372.2000) are ignored.

Screening: Screening programmes (e.g. Goldberg et al., Spine.20(12):1368–1374, 1995) showed that there was no precise demarcation between “scoliosis” and “normal,” and that there was no benefit in terms of the need for surgical correction from screening or bracing, (Goldberg et al. Spine.26(1):42–47, 2001).

Discussion: his information has been in the public domain for some years and, in the meanwhile, there have been huge advances in biology and medicine which must have relevance. When the predictions of a hypothesis are not confirmed, that hypothesis must at least be re-examined, and it is not necessary to wait until a replacement can be suggested. The undisputed aspects of scoliosis, such as association with growth rate and maturation, lateralisation, gender predominance, normal distribution of Cobb angle and asymmetry over the wider population, essential health and normality of those with even severe deformity, increased incidence in other conditions, all suggest a different model. This is an opportune time to pause and reconsider the underlying model of scoliosis in the light of what we have learned about scoliosis and what is now known in other disciplines about how morphology is determined and evolved.

Background: It is commonly observed that a good correction of the Cobb angle at scoliosis surgery is accompanied by an acute asymmetry of shoulder height. Kuklo et al in 2002 described (Spine. 26(18):1966–1975) spontaneous reversal of this, using radiographic measures and patient questionnaires.

Objective: To determine the incidence and extent of shoulder-imbalance before posterior spinal surgery and to ascertain its outcome, using radiographic and topographic measures.

Methods: Patients with right thoracic adolescent idiopathic scoliosis who had undergone corrective posterior spinal fusion by one surgeon were identified. Pre- and all postoperative spinal radiographs and surface topography were evaluated and correlated. Any effect from concomitant anterior release procedures was sought.

Results: Sixty six patients were identified, 56 girls and 10 boys. Their pre-operative major Cobb angle was 73°±14.0 and mean correction was 38.8°±12.333 (56%). Before surgery, surface topography showed the mid-point of the right shoulder to be at a mean or 18.3mm.±10.9 higher than the equivalent left point; eight days later, the difference was −6.7 mm. ±9.68, a mean change of 25.9mm±11.8. At six months, it was −5.1 ±6.86, statistically unchanged. At two years, it was −2.16 (p=0.051) and at three years, 1.76± 6.53 and indistinguishable from zero or perfect balance. The difference between pre-operative and final shoulder level difference was 19.54mm.±9.09. The Cobb angle of the compensatory upper thoracic curve was not significantly changed throughout. There was no statistically significant difference in shoulder height between patients undergoing single or two-stage surgery, either before or at any stage after.

Discussion and conclusion: Correction of post-operative shoulder imbalance does occur spontaneously, as reported by Kuklo et al. and is not a function of spinal accommodation to the new anatomy.

Introduction: Therapeutic bone marrow transplantation has increased survival in Hurler syndrome, but the effects on musculoskeletal development remain unclear. Long term reports on mobility are poor, with many patients gradually losing walking ability in later childhood secondary to hip subluxation and joint contractures. As previous cohorts are small, data is limited.

Methods: We detail the follow up of twenty patients over a mean of 94 months (range 1 – 17.4 years). Radiographs were assessed for hip dysplasia using acetabular angle of Sharp, centre edge angle of Wiberg and tibiofemoral shaft angle. Clinical examination was performed at an annual multidisciplinary assessment by one clinician and compared against age matched controls. 3D gait analysis was performed on eight older children, and deviance in kinematic variables was plotted against controls with Mann-Whitney U test for statistical analysis.

Results: All patients demonstrated characteristic ace-tabular dysplasia. Fourteen patients have undergone containment surgery at a mean of 4.4 years. Innominate osteotomy is an essential part of this. Mean preoperative acetabular angle was reduced from 34 ± 4° to 22 ± 3°. Femoral head containment is maintained, with mean centre edge angle in older patients 39 ± 7°. Genu valgum is observed early, and five patients underwent medial epiphyseal stapling at a mean of 7.8 years, decreasing tibiofemoral angle by a mean of 8.0°. All patients are currently independently mobile, with restriction of internal hip rotation being the only significant clinical finding (P< 0.001). Joint contractures were not noted. Walking speed and stride length were comparable to controls, but endurance is reduced by about one quarter. Gait analysis demonstrates a characteristic pattern, with anterior pelvic tilt secondary to thoracolumbar gibbus, relative hip flexion throughout the gait cycle, valgus knees and compensatory pronated feet; all measured deviations were significant (P< 0.001).

Conclusions This large group maintained successful hip containment and good mobility throughout childhood. Innominate osteotomy alone has been used recently. Despite plain film appearance, genu valgum is a functional problem in gait, and we would anticipate greater use of corrective stapling in the future. This is the first report of gait analysis in Hurler syndrome, and features specific to the condition are described.

Introduction: There is little epidemiological data on childhood injury in Ireland, despite large numbers of referrals to fracture clinics particularly in the summer months. Information is difficult to obtain retrospectively, and our aim was to quantify paediatric injury referrals to our clinics and analyse trends in injury patterns.

Methods: A prospective injury surveillance system was initiated in our department. Parents were asked to record demographic information and a brief description of the injury at fracture clinics or admission to the ward. Diagnosis and treatment was completed by the attending doctor. Details were transferred to a customised database for analysis.

Results: Overall compliance was excellent. Of 397 recorded referrals, 66% had confirmed fractures, and 20% of these were admitted for operative management. There was an equal sex distribution, and mean age at presentation was 9.1 years. The peak hour of injury was 7 – 8 pm, with fairly even distribution throughout the week. 62% of injuries were due to falls. 39% of injuries occured in or about the home, including 61% of all falls greater than 1 metre, most often from walls and slides. Other common locations for injury were school (16%) and sportsfields (14%). Gaelic football and soccer were the predominant sports causing injury. Fractures occuring during unsupervised sport were more likely to need surgery. Road traffic accidents were an uncommon cause of injury. Home ‘bouncy castles’ and trampolines were a notable cause of injury, causing 6% of all fractures, particularly of the upper limb. Predictably, 41% of all fractures involved the radius. Fractures of the distal humerus, diaphyses of radius, ulna and tibia were most likely to need operative management.

Discussion Analysis yielded a timely insight into the local epidemiology of childhood injuries. In comparison with other studies, sports related injuries were frequent and road accidents were unusually few in our group. Many injuries occurring late in the evening needed early reduction, with almost two thirds of surgical procedures performed out of hours with significant implications on theatre and radiology staffing. A large proportion of higher energy trauma occured in or about the home, representing a potential area for injury prevention stratgies.

Background: A sharp, localised, thoracolumbar gibbus is pathognomonic of the mucopolysaccharidosis (MPS) group of disorders, the most common of which is Hurlers syndrome (MPS I). Untreated patients with this disease run an inevitable course of neurological and physical degeneration until death within the first decade. Haemopoietic stem cell transplantation (HSCT) has resulted in considerable improvement in survival with amelioration of many of the symptoms and signs which characterise this disease. Data, however, is disappointing in relation to the impact of HSCT on skeletal dysplasia. This study reviews the natural history of spinal deformity in Hurler’s syndrome after HSCT in infancy.

Methods: Twenty three patients (12 male and 11 female), transplanted at a mean age of 0.9 years ± 0.47, (range 0.27 – 1.8yrs) were investigated, of whom 19 were at least two years post-HSCT and were included. HLA identical donor sources included unaffected or heterozygote family members, unrelated adults or cord blood. Mean age at review was 9.4 years ± 4.57, (range 2.5 – 18.4yrs). Serial measurements of the thoracolumbar spines incorporated clinical records, radiographs and surface topography. The thoracolumbar gibbus was measured on lateral spinal radiograph using the standard adaptation of the Cobb method. Two segments of the spine were documented: the gibbus itself and the thoracic profile above it. Clinical assessment and surface topography were contrasted with this.

Results: At presentation, all showed the characteristic gibbus at the thoracolumbar junction, with a flat and stiff thoracic spine above. Three patients underwent surgery to correct or maintain the gibbus, which was unsuccessful in two; the third is stable, but still young. Two patients have developed scoliosis: one in the juvenile period and one in infancy. Three female patients are now post-menarchal and have shown no progression of their gibbus. One male patient, now aged 19 years, had significant progression of his gibbus at puberty, but is now stable, untreated and cosmetically acceptable. The remainder are still pre-pubertal but their deformities are not currently progressive.

Conclusion: The fate of the spinal deformity in untreated MPS-I has been poorly documented, as the condition was invariably fatal from cardiorespiratory failure during the first decade. These interim results suggest that, while the deformity persists and may become more pronounced during growth and adolescence, it does not significantly impact on quality of life. The considerations which usually dictate intervention in other spinal deformities of childhood may not necessarily apply and should be approached with caution. The more recent availability of recombinant human -L- iduronidase adds further interest to the management of these patients and warrants cautious expectation , in the context of experience gained in these groups of patients. In conclusion atients with MPS I have complex multisystem disorders, independent of their orthopaedic status. While monitoring their spinal deformity is indicated, over-intrusive investigation and treatment may be counterproductive.

Background: In adolescents at or near skeletal maturity, correction of severe scoliosis may be facilitated by first mobilising the spine anteriorly before the definitive posterior fusion and instrumentation. There is no dispute that this is effective, but it is significantly more invasive, and carries greater risks. The benefits have been measured in greater reduction in the Cobb angle, but the patient’s real concern is with cosmesis. Surface topography can measure this aspect.

Methods: Retrospective comparison of topographic parameters (before surgery and at 7 days, 6 months, and 2 years after, and at latest review, if more than two years) after one-stage (Group 5, N=10) and two stage (Group 3, N=39) with normal adolescents (Group 1, N=63). Patients operated for adolescent idiopathic scoliosis by one surgeon (FED) were compared with girls referred and then judged normal from the screening programme. Topographic parameters (spinal angle, saggittal profile, asymmetry and trunk balance) and Cobb angles were compared by t-test.

Results: Prior to surgery, both treatment groups differed significantly from the index group on all parameters except saggittal profile; from each other, they differed only in mean Cobb (Group 3: 73.6°, Group 5: 59°) and spinal angles and Suzuki hump sum. After surgery, both groups showed significant mean reduction in most parameters and in final Cobb angle (Group 3: 32.7° (−40.9°), Group 5: 29° (−30°) postoperatively) excepting rib hump, and were not statistically distinguishable. Over two years, there was continued improvement in trunk balance and re-establishment of lumbar lordosis in both groups. Group 5 (single stage) showed a slight recurrence of some asymmetry parameters that was statistically (but perhaps not clinically) significant. All patients had a solid fusion post-operatively.

Conclusion: Ideally, this study would be done prospectively, on a controlled, double-blind, randomised basis, but the numbers required and time involved make this impractical. These two surgical groups were pre-selected on the basis of curve severity, and these results may show that the anterior procedure is necessary to bring Group 3 to the same end-point as Group 5. Alternatively, while the anterior procedure improves the reduction of the Cobb angle, it might be the posterior fusion that rearranges the shape of the back and hence brings about the cosmetic improvement. In conclusion reducing the Cobb angle has been the standard of surgical assessment, but the cosmetic result does not necessarily correlate with this. The possibility that less invasive surgery may give as good a cosmetic outcome is worth discussing, as the savings in time, money and risk would be enormous.

Background: Scoliosis occurring during the growing years of childhood, while less common, has a greater potential for severe deformity than that with adolescent onset. Treatment is therefore more urgent, and the untreated natural history more difficult to determine. Orthotic treatment and the more recently improved surgical techniques may halt or even reverse the natural history, but the length of time needed for adequate follow-up makes this hard to verify. This report examines the outcome for a historical group of these patients, treated and not, to establish a bench-mark against which results can be measured.

Methods: Retrospective analysis of records derived from the scoliosis database. Subjects were patients presenting with non-congenital scoliosis before the age of ten years and who were at least ten years old when last reviewed. Outcome measures were treatment protocols, the age and incidence of surgery, and the radiological and cosmetic outcome.

Results: 243 children were included, being 38 infantile idiopathic scoliosis (IIS: 20 male, 18 female); 86 juvenile idiopathic scoliosis (JIS: 19 male, 67 female); 119 symdromic scoliosis (Syn:46 male, 71 female) Depending on age, perceived progression potential and individual factors, treatment was either jacket and brace, or observation unless surgery was deemed advisable. In all, 81 children were braced and 162 were not; 129 have had surgery (25 IIS, 48 JIS; 56 Syn.). The individual groups showed no statistical advantage to non-operative treatment in preventing surgery, but in the whole group it appears that a significantly greater proportion (Z=2.7269, p< 0.01) of those braced were subsequently operated. Mean age at surgery was 7.3 years for IIS, 12.97 for JIS and 8.3 for Syn. Recurrence of deformity post-operatively was always observed in those operated before puberty, regardless of the surgical technique.

Conclusion: Ten years of age was taken as the minimum for inclusion, although it is significantly earlier than skeletal maturity, because it has been practice to offer surgery well before this age, and some short term effects may already be apparent by the tenth birthday. This was not a trial of treatment between similar groups, so the appearance of increased surgery in the braced children suggests that, while the clinicians were well able to identify those with a worse prognosis, orthotic treatment was not effective in altering this prognosis. A recently published study¹ demonstrated the failure of past surgical techniques to prevent progressive deformity and respiratory compromise in infantile-onset scoliosis. Here it was found that the older the patient at corrective surgery, the better the result, that methods supposed to prevent post-operative recurrence in skeletally immature children failed to do so, and, while non-operative treatment may be effective at least in postponing surgery, even preventing it in some cases, this was not demonstrated statistically. Treatment of spinal deformity in pre-adolescent children warrants debate as a separate subject, and is a more serious problem than that occurring in adolescence.

There is little data regarding the epidemiology of childhood injury in Ireland. This is difficult to obtain retrospectively. The aim of this study was to prospectively evaluate paediatric trauma referrals to our department, describe their epidemiology, and identify potentially preventable injuries in children. Our unit at the National Childrens Hospital is located in a growing suburban area in South Dublin. Injury surveillance was conducted on orthopaedic referrals by distributing a form to parents of children attending fracture clinics or admitted acutely for surgery. Parents were asked for demographic information, and a brief description of the injury in terms of location, mechanism and circumstance of injury. Diagnosis was completed by the attending doctor and data was transferred to a computerised database. We analyzed data from the 397 referrals in the first month of this study. The mean age of injury was 9.1 years and the male: female ratio was 1.3:1. The peak hour of injury was 7 – 8 pm. Only 33% of injuries occured during the weekend. 62% of injuries were due to falls, usually form the standing position. The most common location for injury was in or about the home (39%), and other notable locations were school (16%) and sportsfield (14%). 61% of falls greater than 1 metre occurred at home, mostly from walls and childrens slides. 20% of injuries occurred while participating in organized sport, including Gaelic football, soccer and hurling. Injuries occuring during unsupervised sport were more likely to need surgery. Domestic ‘bouncing castles’ and trampolines, increasingly popular in our area, were a notable cause of significant trauma to the upper limb. 7% of injuries occurred by falling from a bicycle, but vehicular road traffic accident was an uncommon cause of injury. 263 children had confirmed fractures, other injuries consisting largely of sprains to the ankle, elbow and wrist. Predictably, the bones most commonly fractured were the radius (41%), phalanges (15%) and humerus (11%). 20% of fractures needed operative management, mostly forearm manipulation under anaesthesia. 63% of operative cases were performed outside of normal working hours. Several countries utilise injury surveillance as a means of development and evaluation of injury prevention strategies. In our initial study, basic surveillance has outlined local characteristics of chilhood trauma, and some trends were noted. In particular, we suggest home injuries need further attention in out catchment area.

Thirty four patients underwent 34 single entry percutaneous physiodesis (SEPP) of both distal femur and proximal tibia between July 1996 and June 2004. Twenty six patients had attained maturity and the rest continue to be followed up. There were 10 females and 16 males. The mean ages were12.8yrs (range11–14yrs) and 13.8yrs (range11–15) respectively.

All patients underwent at least three assessments of limb length discrepancy(lld) using CT Scannograms. The Mosely’s straight line graph was then used to predict lld and timing of correction. The procedure was performed under image intensifier control using a 6.5mm drill passed through a small incision. The drill was passed in three directions through a single entry . The physis was curetted.

The mean lld at SEPP was 3.36cm(range1.5–5.9cm). The prediction of lld at maturity after SEPP was a mean of 1.4cm(range0.2–2.5cm) and final lld was a mean of 1.38cm (range0.3–2.5cm). The accuracy of prediction was found to have a mean of 0.44cm (range0–0.7cm). One patient (6%) complained of knee pain for about 2 weeks that settled.

The rest had no complications. We feel that this technique is minimally invasive with a cosmetic scar, has a shorter hospital stay, low complications and is reliable for phuseal ablation. This technique aided by the CT scannogram and a Mosely’s straight line graph provides a reliable and effective method in the management of small amounts of lld.

The aim of this study is to assess the success of posterior lumbar interbody fusion in the treatment of degenerative spinal instability. Methods: Historical prospective study containing sixty-five consecutive patients who underwent posterior lumbar interbody fusion (PLIF) using carbon cages and pedicle fixation between 1993 and 2000.

Clinical outcome was assessed by the postoperative symptomatic relief, complications rate and the fusion rate. The fusion rate was assessed using plain radiographs and the Brantigan and Steffee scoring system. Functional outcome was measured by the improvement in the Oswestry disability index, PROLO score, return to work and satisfaction with the surgical outcome. The determinants of functional relief were analysed against the improvement in disability using multiple regression analysis. Results: Overall fusion rate was ninety eight percent. There was a significant improvement in Oswestry disability index P< 0.001. There was 85% satisfaction with the surgical procedure and 58% return to pre-disease activity level. We found preoperative level of disability to be best the determinant of functional recovery irrespective of age or the degree of psychological morbidity (p< 0.0001). Conclusion: The combination of posterior lumbar interbody fusion (PLIF) and posterior instrumented fusion is a safe and effective method of achieving segmental fusion with sustained functional relief and high satisfaction rate. Direct relationship between preoperative level of disability and functional recovery suggests that spinal fusion should be performed to alleviate disability caused by degenerative spine.

Back pain screening clinics are established to clinically screen patients with back pain for organic lumbar pathology. The aim of this study is to assess the relationship between clinical signs of organic pathology and the level of disability as measured by functional outcome scores. Methods: Notes from 581 consecutive patients who were seen in the back screening clinic was analyzed. Sixty-nine patients who were found to have clinical signs of organic pathology and 69 age and sex-matched patients from 512 patients who were found to have no signs of organic pathology in the same time period in the back pain screening clinic were selected. The Oswestry disability, Short form-36 and visual analogue (pain) scores between the two groups were statistically analyzed.

The correlation between the level of psychological morbidity, length of symptoms and presence of past history of symptoms against the level of disability was statistically assessed. Results: Although there was a signiþcant increase in the level of disability in the referred group with each score (Oswestry Disability Score P< 0.001, SF-36 physical component score P=0.014, Visual analogue pain score P< 0.001). We also found a strong relationship between psychological disability and the duration of back symptoms. Conclusions: High level of disability is associated with organic pathology. Acute back pain should be treated promptly to reduce it impact on the psychological disability.

Objective: To assess the treatment outcome at a minimum age of 15 years in patients who had presented with idiopathic scoliosis in infancy.

Design: Patients were recalled for full pulmonary function testing (spirometry, lung volumes and gas diffusion) and surface topography. Results were correlated with history and clinical radiographs.

Subjects: The records showed 32 patients, of whom 23 could be contacted and agreed to take part in the study. Thirteen had no other abnormality, and 9 had a variety of additional problems not thought to directly precipitate their spinal deformity. There were 13 female and 9 male and age at testing ranged from 15.2 to 30.2 years.

Outcome measures: Spirometry (forced vital capacity (FVC), forced expiratory volume in one minute (FEV₁)), lung volumes ( total lung capacity (TLC), residual volume (RV)) and gas diffusion (carbon monoxide diffusion (DLCO), and alveolar volume (VA)) were correlated with the most recent Cobb angle, surface topography and age at surgery where applicable.

Results: Those who were successfully managed without recourse to surgery (N=6) had normal cosmesis and pulmonary function (mean FEV₁ = 98.7%, mean FVC = 96.6%). When surgery had been postponed until after age 10 (N=6, mean age at surgery 12.9 years) pulmonary function showed some restriction (mean FEV₁ = 79%, mean FVC = 68.3%). Those who underwent corrective surgery before age 10 years (N=11, mean age at surgery 4.1 years) had significant recurrence of deformity and diminished respiratory function (mean FEV₁= 41%, range 14 – 72%, mean FVC = 40.8%, range 12 – 67%). There was statistically significant correlation (p< 0.01 or less) between respiratory measures on the one hand and age at surgery (where applicable), surface topography measures and latest Cobb angle.

Conclusions: It has been reported that only in early-onset scoliosis is the growth of lung tissue and the multiplication of alveoli impeded[1,2] and treatment is directed at preserving both pulmonary function and cosmesis. Early surgery is recommended on the assumption that the Cobb angle can be controlled and normal pulmonary development enabled in those whose scoliosis did not respond to conservative methods. Methods have changed since the earlier cases in this series were treated, and it is hoped that later results will be different. However, caution requires that, in monitoring these patients, cosmesis and, more importantly, respiratory function be considered before a conclusion is drawn.

Langerhans-cell histiocytosis (LCH) is a reactive proliferative disease characterized by the accumulation of abnormal histiocytes. The disease is broadly divided into two groups, unisystem and multisystem disease. The aetiology of LCH is unknown; the disease is currently accepted to be a reactive process rather than a malignancy. Localized LCH of bone is a benign tumour-like condition, which is characterized by a clonal proliferation of Langerhan’s-type histocytes, which infiltrate bone and cause osteolytic lesions. The common bones involved include – skull, pelvis, and diaphysis of long bones.

We wanted to determine whether patient demographics at the time of presentation could help determine the clinical course and eventual outcome of the disease. We prospectively reviewed 68 patients with a primary diagnosis of LCH.

Forty-six patients had unisystem disease, 22 had multisystem disease. There was a statistically significant difference in the age of presentation between the two groups. There were 6 deaths, all had multisystem disease. Of the 46 patients with unisystem disease, 31 (67.3%) underwent orthopaedic surgical intervention, 26 open biopsies and 5 curettage and bone grafting of lesions of the humeras (2), skin, clavicle and skull (1 each). There was a statistically significant difference in the average length of follow-up, between the 2 groups.

We recommend closed and prolonged multidisciplinary follow-up of patient initially presenting with multisystem disease. Patients with unisystem disease can safely be discharged after a short follow-up period.