Glenoid baseplate positioning for reverse total shoulder replacements (rTSR) is key for stability and longevity. 3D planning and image-derived instrumentation (IDI) are techniques for improving implant placement accuracy. This is a single-blinded randomised controlled trial comparing 3D planning with IDI jigs versus 3D planning with conventional instrumentation.

Eligible patients were enrolled and had 3D pre-operative planning. They were randomised to either IDI or conventional instrumentation; then underwent their rTSR. 6 weeks post operatively, a CT scan was performed and blinded assessors measured the accuracy of glenoid baseplate position relative to the pre-operative plan.

47 patients were included: 24 with IDI and 23 with conventional instrumentation. The IDI group were more likely to have a guidewire placement within 2mm of the preoperative plan in the superior/inferior plane when compared to the conventional group (p=0.01). The IDI group had a smaller degree of error when the native glenoid retroversion was >10° (p=0.047) when compared to the conventional group. All other parameters (inclination, anterior/posterior plane, glenoids with retroversion <10°) showed no significant difference between the two groups.

Both IDI and conventional methods for rTSA placement are very accurate. However, IDI is more accurate for complex glenoid morphology and placement in the superior-inferior plane. Clinically, these two parameters are important and may prevent long term complications of scapular notching or glenoid baseplate loosening.

Image-derived instrumentation (IDI) is significantly more accurate in glenoid component placement in the superior/inferior plane compared to conventional instrumentation when using 3D pre-operative planning. Additionally, in complex glenoid morphologies where the native retroversion is >10°, IDI has improved accuracy in glenoid placement compared to conventional instrumentation. IDI is an accurate method for glenoid guidewire and component placement in rTSA.

The Pelvic Support Osteotomy (PSO) or Ilizarov Hip Reconstruction(IHR) is well described for the treatment of septic sequelae of infancy. The purpose of this study was to clinically, functionally and radiographically assess our short-term results of this procedure.

25 patients (16 boys, 9 girls) who had undergone a IHR using the Ilizarov/TSF construct over a period of 10 years for a variety of pathologies were reviewed. The mean age at surgery was 15 years 4 months. The pre-operative diagnoses were SCFE(10), hip sepsis (6), DDH (6) and Perthes (3). All had significant leg length discrepancies, 16 had a painful stiff hip, 6 had a painful mobile hip and 3 had a painless unstable hip. At surgery, a mean measured proximal valgus angle of 51° and a mean extension angle of 15° was achieved. Distal femoral lengthening averaged 4.2 cm and distal varus correction was a mean of 8°. The mean fixator time was 173 days.

At a mean of 2 years and 7 months follow-up the lower-extremity length discrepancy had improved from a mean of 5.6 cm apparent shortening to 2.3 cm. Trendelenberg sign was eliminated in 18/25 cases. Improvements in range of hip movements and gait parameters were observed. Stance time asymmetry, step length asymmetry, pelvic dip and trunk lurch improved significantly. One patient had conversion to a total hip replacement after 7 years, 4 patients required re-do PSO due to remodelling of the proximal osteotomies, two had heterotopic ossification and two had significant knee stiffness due to lack of compliance.

The early results of IHR are encouraging to equalise limb lengths, negate trendelenburg gait, provide a mobile hip with a reasonable axis and the possibility of conversion to THR in the future if needed. Complications need to be anticipated and the effects of remodelling and maintaining adequate knee range of motion must be emphasised.

PURPOSE OF THE STUDY

To assess if prolonged use of Bisphosphonates in Osteogenesis Imperfecta alters the pattern of femoral fractures.

Osteogenesis Imperfecta (OI) has been treated with Bisphosphonates for many years with some clear clinical benefits. In adult cohorts there are reports of a new pattern of atraumatic subtrochanteric fractures seen with Bisphosphonate treatment.

Background: Activity advice and prescription are commonly used in the management of low back pain (LBP). However, no research has assessed whether objective measurements of physical activity predict outcome, recovery and course of LBP.

Methods: One hundred and one patients with acute LBP were recruited into a longitudinal cohort study. Each participant completed the Roland Morris Disability Questionnaire (RMDQ), Visual Analogue Scale and a “simple” activity question, detailing whether they had resumed full “normal” activities (Y/N), at baseline (T0) and 3 months (T1); Baecke Physical Activity Questionnaire, Fear-Avoidance Beliefs Questionnaire and the 12-item General Health Questionnaire at T0. Physical activity was measured for 7 days at T0 and T1 with an RT3 accelerometer and the seven day physical activity recall questionnaire (7d-PAR).

Results: The only significant predictor of RMDQ change was RMDQ score at T0 (p < .0001). Physical activity change did not predict RMDQ change in both univariate (p = 0.82) and multivariate analysis (p = 0.84). Paired t-tests found a significant change in RMDQ (p < .0001) and return to full “normal” activities (p < .0001) from T0 to T1, but no significant change in activity levels measured with the RT3 (p = 0.56) or the 7d-PAR (p = 0.43). RMDQ change (OR 1.72, p = 0.01) and RMDQ at T1 (OR 0.65, p = 0.04) predicted return to full “normal” activities at T1.

Conclusions: These results question the role of physical activity in LBP recovery and the assumption that activity levels change as LBP symptoms resolve.

Conflicts of Interest: None

Sources of Funding: This research was supported by a University of Otago Establishment Grant

Introduction: This study examined the cohort of patients selectively screened over a 5 year period with ultrasonography according to our risk factors (positive Ortolani or Barlow manoeuvre, breech presentation, first degree affected relative and talipes equinovarus) for developmental hip dysplasia (DDH). The aims were to evaluate the success of those managed in a Pavlik harness and identify predictive factors for those that failed treatment.

Methods: 1181 patients were selectively screened between 1999 and 2004. Of those, 128 patients (189 hips) were identified as having hip instability. Failure was defined as inability to achieve or maintain hip reduction in a Pavlik harness. A proforma was designed to document patients’ risk factors and ultrasound findings at time of initial dynamic ultrasound scan where the senior radiographer and treating consultant were present. Each hip was classified according to Graf type. Acetabular indices were recorded prior to discharge.

Results: All 128 patients with hip instability were managed in a Pavlik harness. This was abandoned for surgical treatment in 9 patients (10 hips) giving a failure rate of 5.3 % (0.17 per 1000 live births). All those successfully managed had an acetabular index of less than 30 degrees at follow up (6 – 48 months). 7 hips in the series were classified as Graf type IV, of these, 6 went on to fail management in a Pavlik harness. 67% of those that failed were also breech presentation compared to 22% of those managed successfully. There were no complications associated with management in a Pavlik harness. 16 patients born within our region presented after 12 weeks of age with DDH resulting in a late presentation rate of 0.3 per 1000 live births.

Discussion: Our overall rate of selective screening is 14 per 1000 live births with a subsequent treatment rate of 2.3 per 1000 is comparable with other centres. Our rate of failure for DDH in a Pavlik harness 0.17 per 1000 live births is an improvement on any previously published results. Irreducible hips, Graf type IV hips and breech presentation correlated with a high likelihood of treatment failure.

Background: Information on embryological hip development has been obtained from post mortem examination

. There is less information on normal foetal hip

AIM: To assess the value of MRI of the foetus and neonate to provide information on normal and abnormal hip development.

To establish normal patterns of hip development.

To obtain charts that could be used to detect abnormality earlier.

There are three aspects to this study:

Validation – analysing MRI scans of babies hips prior to post mortem (the gold standard) would verify MRI as a valid tool for such studies.

PATIENT SELECTION: 30 patients for each aspect of this pilot study, 90 in total (3).

For the initial validation process, parents who had consented to post mortem were asked to consider additionally an MR scan of their neonate’s hips, a total of 30 cases.

Method: MR images in axial and coronal planes were obtained using a high resolution T2 weighted sequences (4).

Measurements were made, by two independent observers, of the width and depth of the acetabulum and the radius & diameter of the femoral head, volume and area were calculated. Inter-observer variation was assessed.

Results: The babies ranged in gestation from 17 – 42 weeks

With the exception of the acetabular width each dimension showed little development until week 20 when the line of growth rose exponentially. The acetabular width showed only a slow rate of growth despite the changes seen in the femoral head. Levels of observer agreement were high (ICCs = 0.98) for all but depth (ICCs = 0.86). The measurements for all dimensions were in line with previous post mortem studies.

CONCLUSION: MRI is a valid and acceptable alternative to post mortem in the assessment of hip development eventually allowing early detection of abnormal hip development.

To review the results of limb lengthening and deformity correction in fibular hemimelia, fifty-five patients with fibular hemimelia underwent limb reconstruction at Sheffield Children’s Hospital. According to Achter-man and Kalamchi classification, twenty-six were classified as Type IA, six as Type IB and twenty-three as Type II fibular hemimelia. All patients had at least some shortening of ipsilateral femur but forty-nine had sig-nificant femoral deficiency. Lengthening of tibia and in significant cases femur was done using De Bastiani or Vilarrubias or Ilizarov methods. Ankle valgus and heel valgus were corrected through osteotomies either in the supramalleolar region or heel. Equinus was corrected by lengthening of tendoachelis with posterior soft tissue release and in severe cases using Ilizarov technique.

The average length gained was 4.2 cm (range 1 to 8) and the mean percentage of length increase was 15.82 (range 4.2 to 32.4). Mean bone healing index was 54.23 days/cm. Significant complications included knee subluxation, transient common paroneal nerve palsy, and recurrence of equinus and valgus deformity of foot. Overall alignment and ambulation improved in all patients. Knee stiffness due to cruciate deficient subluxations needed prolonged rehabilitation. Presence of 3-ray foot gives a better functional result and cosmetic acceptance by patients. The Ilizarov frame has the advantage to cross joints and lengthen at the metaphysis.

Limb reconstruction in fibular hemimelia using limb lengthening and deformity correction techniques improve functional status of involved lower limb.

To review the results of reconstruction of pseudoar-throsis and/or significant varus with retroversion of proximal femur in congenital longitudinal lower limb deficiencies, twenty-three of ninety-five patients with lower limb deficiencies underwent proximal femoral reconstruction at the Sheffield Children’s Hospital. All twenty-three underwent valgus derotation osteotomies to correct coxa vara and retroversion of femur. Seven patients also had pseudoarthrosis of the neck of femur. Three of these were treated with valgus derotation osteotomy and cancellous bone grafting, two with fibular strut grafts, one King’s procedure and one with excision of fibrous tissue and valgus derotation osteotomy. A variety of internal fixation devices and external fixator were used.

Seventeen of the twenty-three patients had valgus osteotomies repeated more than once (average 2.3) for recurrence of varus deformity. Average initial neck-shaft angle was 72 degrees, which improved to an average of 115 degrees after reconstruction.

All seven patients with pseudoarthroses underwent multiple procedures (average 3.3) to achieve union. Cancellous bone grafting was repeated twice in two patients to achieve union but all three with cancellous bone grafting underwent repeat osteotomies to correct residual varus. Two patients achieved union after fibu-lar strut grafting. One patient, who underwent excision of pseudoarthrosis, achieved union but had to undergo further valgus osteotomy. No particular advantage of any one-fixation device over the others was noted in achieving correction.

Early axis correction using valgus derotation oste-otomy is important in limb reconstruction when there is significant coxa vara and retroversion, although recurrence may require repeated osteotomies. Pseudoarthro-ses needed more aggressive surgery to achieve union.

Purpose: The aim of this part retrospective and part prospective study was to establish the incidence of pinsite infections and assess evolution of changes in practice

Methods: Data was collected retrospectively and prospectively for pin site infections from the inception of limb reconstruction service viz. 1985 to January 2002. There were 812 patients, 1042 limb segments, and 9935 pins. The various external fixators used were limb reconstruction system (LRS) 549; Ilizarov 397; Sheffield ring fixator (SRF); Dynamic axial fixator (DAF) 35; LRS/Sequoia 8; LRS/Garche 7; and Pennig 5.

Results: The pin site infections were graded from 0 to 6 ( Saleh & Scott). There were no infections in 206 segments. The infection grade is shown below:

We changed our pin tract care practice from 1996. We had a significant decrease in pin tract infections since then (p< 0.0001). We also found that using Ilizarov wires had significantly less infections than with half pins used with monolateral fixators (p< 0.0001; linear trend, p= 0.0338). There were 48 patients that required hospital admissions for IV antibiotics. and of these 10 patients required debridement. There were no residual long lasting infections or chronic osteomyelitis.

Conclusion: Attention to detail in insertion of wires and half pins is crucial to avoid pin site infections. This audit supports the fact that external fixation is a safe method from the point of view of infection contrary to general belief.

Introduction: This study examined the cohort of patients selectively screened over a 5 year period with ultrasonography according to our risk factors (positive Ortolani or Barlow manoeuvre, breech presentation, first degree affected relative and talipes equinovarus) for developmental hip dysplasia (DDH). The aims were to evaluate the success of those managed in a Pavlik harness and identify predictive factors for those that failed treatment.

Methods: 728 patients were selectively screened between 1999 and 2004. Of those, 128 patients (189 hips) were identified as having hip instability. Failure was defined as inability to achieve or maintain hip reduction in a Pavlik harness. A proforma was designed to document patients’ risk factors and ultrasound findings at time of initial dynamic ultrasound scan where the senior radiographer and treating consultant were present. Each hip was classified according to Graf type. Acetabular indices were recorded prior to discharge.

Results: All 128 patients with hip instability were managed in a Pavlik harness. This was abandoned for surgical treatment in 9 patients (10 hips) giving a failure rate of 5.3 %. All those successfully managed had an acetabular index of less than 30 degrees at follow up (6 – 48 months). 7 hips in the series were classified as Graf type IV, of these, 6 went on to fail management in a Pavlik harness. 67% of those that failed were also breech presentation compared to 22% of those managed successfully. There were no complications associated with management in a Pavlik harness.

Discussion: Our overall rate of selective screening is 14 per 1,000 with a subsequent treatment rate of 2.3 per 1,000, which is comparable with other centres. Our rate of failure for DDH in a Pavlik harness (5.3 %) is extremely encouraging. Graf type IV hips and breech presentation correlated with a high likelihood of treatment failure.

Background The ossific nucleus of the femoral head is usually present ultrasonographically around 12 weeks of age. It has been considered that the presence of an ossific nucleus in the femoral head is an indication of hip stability. In the ultrasound scan clinic for the assessment of developmental dysplasia of the hip at Sheffield Children’s Hospital, we have identified unstable hips with ossific nuclei, as well as, the appearance of ossific nuclei at as early as 2 weeks of age. These observations suggested the need to clarify the initial considerations that the ossific nucleus appeared ultrasonographically around 12 weeks of age and was an indicator of hip stability.

Aim To determine the relationship, if any, between the presence of the ossific nucleus of the femoral head ultrasonographically and stability of the hip.

Patient selection We have included in our study all the children who have had an ultrasound scan of their hips from 1996 to 1999 at Sheffield Children’s Hospital for suspected developmental dysplasia.

Methodology We have retrospectively reviewed reports of ultrasound scans performed for developmental dysplasia of the hip between 1996 and 1999. We have looked at the report for both the hips of each child. We have collected and analyzed data with regard to the age of the child at the time of the scan, the depth of the acetabulum, the shape of the femoral head, the presence or absence of an ossific nucleus, the dynamic stability of the hips and the congruity of the joint as reported on the ultrasound report. We report the findings in the first 318 hips of the 627 available patients in the study period.

Results The ossific nucleus can appear as early as 2 weeks and yet may not be visible until 24 weeks. In the 318 hips examined the ossific nucleus was present in 46 (14.47%). The age range for these scans was 1–40 weeks after birth. Of the 318 hips 252 (79.24%) were stable on dynamic screening, 274 (86.16%) had a normal(spherical) appearance of the femoral head, 209 (65.72%) had normal acetabular development and 263 (82.7%) demonstrated congruence of the hip joint. These data have been analyzed using Microsoft excel at confidence intervals of 0.8, which suggest no relationship between the presence of the ossific nucleus and hip stability.

Conclusion The limited early results of this study have shown that the ossific nucleus of the femoral head can appear from a very early age, may not appear until well after 12 weeks of age and is not an indicator of hip stability. Its presence on ultrasound scan does not exclude developmental dysplasia of the hip.

Background Information on embryological hip development has been obtained from post mortem examination (1). There is less information on normal fetal hip (2). Magnetic resonance imaging (MRI) allows development to be followed in the healthy baby.

Aim To assess the value of MRI of the foetus and neonate to provide information on normal and abnormal hip development.

To establish normal patterns of hip development.

To obtain charts that could be used to detect abnormality earlier.

There are three aspects to this study:

Validation – analysing MRI scans of babies hips prior to post mortem (the gold standard) would verify MRI as a valid tool for such studies.

Patient selection 30 patients for each aspect of this pilot study, 90 in total (3).

For the initial validation process, parents who had consented to post mortem were asked to consider additionally an MR scan of their neonate’s hips, a total of 30 cases.

Method MR images in axail and coronal planes were obtained using a high resolution T2 weighted sequences (4).

Measurements were made, by two independent observers, of the width and depth of the acetabulum and the radius & diameter of the femoral head, volume and area were calculated. Inter-observer variation was assessed.

Results The babies ranged in gestation from 17 – 42 weeks

With the exception of the acetabular width each dimension showed little development until week 20 when the line of growth rose exponentially. The acetabular width showed only a slow rate of growth despite the changes seen in the femoral head. Levels of observer agreement were high (ICCs 95% = 0.98) for all but depth (ICCs 95% = 0.86). The measurements for all dimensions were in line with previous post mortem studies.

Conclusion MRI is a valid and acceptable alternative to post mortem in the assessment of hip development eventually allowing early detection of abnormal hip development.

Introduction and Aims: To review the existing classifications in characterising the pathologic morphology of congenital lower limb deficiencies (CLLLD) and their usefulness in planning limb reconstruction.

Method: Ninety-five patients undergoing limb reconstruction were classified using existing classifications. Predominantly femoral deficiencies were classified using Aitken, Amstutz, Hamanishi, Gillespie and Torode, Fixsen and Lloyd-Roberts, Kalamchi, and Pappas systems and fibular deficiencies were classified using Coventry and Johnston, Achterman and Kalamchi, and Birch systems.

Results: All patients with predominant deficiency of one segment (femoral or fibular) also had associated shortening of the other segment in the same limb. Acetabular dysplasia, knee instability due to cruciate insufficiency and lateral femoral condylar hypoplasia were found in both femoral and fibular deficiencies. None of the existing classification systems were able to represent the complete pathologic morphology in any given patient. Due consideration of alignment, joint stability and length discrepancy of affected limb as a whole at the planning stage of reconstruction could not be ascertained using these classification systems. Instead, it was useful to characterise the morphology of the involved limb using the following method:

Acetabulum: Dysplastic/Non-dysplastic

Conclusion: Existing classifications do not represent the complete morphology of the entire involved lower limb in CLLLD and therefore a systematic method of characterising the morphology of the lower limb is more useful in planning limb reconstruction.

Nine children with knee and foot deformities were treated by Ilizarov external fixation from 1989 to 2000 at the Sheffield Children’s Hospital. Sixteen cases of arthrogryposis were identified. Progressive correction was combined with soft tissue release, soft tissue distraction or bony correction. Clinical outcomes were assessed and comparisons made between the different treatment modalities. Three fixed flexion deformities of the knee treated with progressive correction and soft tissue distraction were corrected initially, but recurred some time after the removal of fixators. Out of five clubfoot deformities treated with an Ilizarov frame with progressive soft tissue distraction alone, three recurred despite long-term splinting. Eight clubfoot deformities were treated with a bony procedure combined with gradual correction in the circular frame, and all corrections were maintained at follow-up.

The mean treatment time in the fixator was 17 weeks (12 to 50), and the mean follow-up time was 36 months. Complications included four cases of pin-tract sepsis, one case of osteitis requiring a sequestrectomy, one of transient neuropraxia and one fracture following removal of the fixator.

The treatment of joint deformities in arthrogryposis remains challenging and complications occur. Combining the Ilizarov device with a bony procedure seems to give better results, with fewer recurrence of deformities than pure progressive soft tissue correction.

Introduction We have reviewed the clinical outcome and complications of Monofocal and Bifocal Callotasis for lower limb lengthening in children with Achondroplasia.

Methods Between August 1986 and January 1999, 57 children with Achondroplasia had lower limb lengthening. Monofocal callotasis had been carried out in 147 Segments of 44 children and bifocal callotasis in 38 segments of 17 children. Complications were noted and final outcomes recorded.

Results The 29 children who completed the programme gained an average of 20 cms in height. For all patients, the mean length gained per segment was roughly nine centimetres. Average Bone Healing Index in the mono-focal lengthening group was 39.9 days/cm and in the bifocal lengthening group 33.6 days/cm. Complications were staged and graded and the average was 2.8 complications per lengthened segment. Most were pin-site related and occurred during stage of distraction. Twenty percent of the segments required further axis corrections. Most patients regained their pre-operative range of motion. Serious irreversible complications were seen in only two patients and included a physeal bar and psychological disturbances. Functional outcome analyses are planned.

Conclusions Limb lengthening for short stature due to Achondroplasia can be confidently undertaken with favourable results in most cases. Bifocal lengthening is an alternative technique with quicker consolidation time.

The treatment of acetabular dysplasia in adolescents (age> 12) is difþcult and various complex pelvic osteotomies have been described. The aim of surgery being improvement in pain and to delay the onset of secondary osteoarthrosis. Methods: We present our experience of using the Tonnis triple pelvic osteotomy for treatment of acetabular dysplasia in the adolescent and adult age groups (range 13–27 years). This retrospective analysis includes 25 patients operated on over a nine year period (1991–2000) with an average followup of four years (range 2–8 years). More than 50% of the patients had had a previous open reduction or femoral osteotomy for CDH. Results: Radiographic analysis (pre & post op) included CE angle of Wiberg, Sharp-Ullmann index and the acetabular angle of the weight bearing zone. All parameters showed an improvement in the post operative analysis with an improvement in pain and range of movement in all patients. Discussion: The Tonnis triple pelvic osteotomy has the advantage of allowing the operator a direct þeld of view at all times and achieving a great deal of lateral rotation and medial displacement of the acetabulum due to the proximity of the osteotomy to the acetabulum. The ischial ramus and its ligaments to the sacrum are left intact, leading to greater stability of the pelvis and spine. Conclusion: Though technically difþcult and needing a long learning curve, it does improve acetabular alignment and symptoms in the early postoperative years. However long term studies are required to document its effect on the rate of secondary osteoarthrosis.

Aims: To review the results of reconstruction of pseudoarthrosis and/or significant varus with retroversion of proximal femur in congenital longitudinal lower limb deficiencies. Methods: 23 of 95 patients had proximal femoral reconstruction. 7 had pseudoarthrosis of the neck of femur and the remaining had significant coxa vara with retroversion of femur. 3 patients with pseudoarthroses were treated with valgus derotation osteotomy and cancellous bone grafting, 2 with fibular strut grafts, 1 King’s procedure and 1 with excision of fibrous tissue and valgus derotation osteotomy. Remaining patients underwent valgus derotation osteotomies. A variety of internal fixation devices and external fixators were used. Results: All patients with pseudoarthroses underwent multiple procedures to achieve union. 3 with cancellous bone grafting underwent repeat osteotomies to correct residual varus and 2 had grafting repeated twice. 2 patients achieved union after fibular strut grafting. One patient, who underwent excision of pseudoarthrosis, achieved union but had to undergo further valgus osteotomy. The remaining 17 patients with coxa vara and retroversion of femur also had valgus osteotomies repeated more than once (average 2.3) for recurrence of varus deformity. There were significant numbers of implant failures. Average initial neck-shaft angle of 72 degrees improved to 115 degrees after reconstruction. Conclusion: Achieving union of pseudoarthrosis and early axis correction using valgus derotation osteotomy with a view to later lengthening is important in limb reconstruction. Recurrence may require repeated osteotomies and pseudoarthrosis may need more aggressive surgery to achieve union. Muscle slides and soft tissue releases decrease the stress on implant and maintain correction.

Aims: To review the results of limb lengthening and deformity correction in fibular hemimelia. Methods: Fifty-five patients with fibular hemimelia underwent limb reconstruction at Sheffield Children’s Hospital. According to Achterman and Kalamchi classification, twenty-six were classified as Type IA, six as Type IB and twenty-three as Type II fibular hemimelia. All patients had at least some shortening of ipsilateral femur but forty-nine had significant femoral deficiency. Lengthening of tibia and in significant cases femur was done using either De Bastiani, Vilarrubias or Ilizarov methods. Ankle valgus and heel valgus were corrected through osteotomies either in the supramalleolar region or heel. Equinus was corrected by lengthening of tendoachelis with posterior soft tissue release and in severe cases using Ilizarov technique. Results: The average length gained was 4.2 cm (range 1 to 8) and the mean percentage of length increase was 15.82 (range 4.2 to 32.4). Mean bone healing index was 54.23 days/cm. Significant complications included knee subluxation, transient common paroneal nerve palsy, and recurrence of equinus and valgus deformity of foot. Overall alignment and ambulation improved in all patients. Knee stiffness due to cruciate deficient subluxations needed prolonged rehabilitation. Presence of 3-ray foot gives a better functional result and cosmetic acceptance by patients. The Ilizarov frame has the advantage to cross joints and lengthen at the metaphysis. Conclusion: Limb reconstruction in fibular hemimelia using limb lengthening and deformity correction techniques improve functional status of involved lower limb.

Aims: To review the hip subluxations or dislocations occurring during femoral lengthening in patients with congenital longitudinal lower limb deficiencies. Methods: Sixty-three patients with congenital longitudinal lower limb deficiencies underwent femoral lengthening using either De Bastiani, Villarubias or Ilizarov technique. Acetabular index, medial joint space, CE angle of Wiberg, acetabular angle of Sharp and neck-shaft angle were measured on anteroposterior radiographs of hip before, during and after lengthening. Acetabulum was considered dysplastic when the Sharp angle was more than 45 degrees. Hip was considered to be subluxed when the medial joint space increased during lengthening. Results: During femoral lengthening, eleven hips subluxed as measured by the increase in medial joint space and one hip dislocated. All these hips had a pre-operative acetabular index more than 25 degrees, CE angle less than 20 degrees and Sharp angle more than 45 degrees. The average neck-shaft angle was 75 degrees. Following subluxation, lengthening was stopped and the hips were reduced in hip spica after adductor and sartorius tenotomies. In one patient femoral shortening and acetabuloplasty had to be done to reduce the sub-luxation. No case of avascular necrosis or chondrolysis was noted. Conclusions: Hip subluxation during femoral lengthening of congenital longitudinal lower limb deficiencies tends to occur when there is associated ace-tabular dyplasia and femoral coxa vara. Careful preoperative assessment, if need be hip reconstruction prior to lengthening and close monitoring during lengthening is recommended.

Upper limbs are commonly involved in Arthrogyposis Multiplex Congenita. They may be involved in isolation or in combination with lower limbs. There are two patterns of involvement in upper limbs. The most common (type I) pattern presents with adduction and internal rotation at the shoulder, extension at the elbow, pro-nation of the forearm and flexion deformity of the wrist, indicating involvement of the C5 and C6 segments. These deformities can be quite disabling and may require surgery to help improve function. We present our long-term results with pectoralis major transfer procedure (as modified by senior author MJB) to restore elbow flexion in seven patients (ten procedures).

Results: Early results in all our patients were quite encouraging. Six patients retained useful power in transferred pectoralis major muscle and maintained the arc of flexion, which was attained following tricepsplasty. However, as children were followed up a gradually increasing flexion deformity and decreasing flexion arc were observed in eight elbows. The onset and progression of flexion deformity was gradual and progressive. The flexion deformity reached ninety degrees or more in all cases.

Conclusions: Results of pectoralis major transfer to treat extension contracture of the elbow in arthrogryposis deteriorate with time due to development of recalcitrant flexion deformity of the elbow. Presently we recommend this procedure on one side only in cases of bilateral involvement because if one procedure is carried out it would be possible for this hand to get to the mouth for feeding and the other unoperated side would be able to look after the perineal hygiene

Children with osteogenesis imperfecta(OI) have multiple long bone fractures with subsequent deformities. The mainstay of treatment is correction with multiple osteotomies and intramedullary fixation. The Shefffield intramedullary telescoping rod system has been successful in the treament of long bone fractures and deformities (Wilkinson et al ,JBJS-B,1998) Bisphosphonates (Pamidronate -1- 1.5mg/kg/day)have been used as adjuvant therapy in the treatment of OI since the last five years. The perceived benefits include reduction in fracture frequency, improvement in bone density and a general feeling of well being.

We present our experience of five cases of OI who developed infections around thier Sheffield telescoping rods while on Pamidronate therapy. There was only one case of sepsis over a ten year period(over eighty patients)in a previously reported series from our centre.

The time interval between the start of Pamidronate therapy and the diagnosis of infection varied between 12–36 months ie. between 4–12 cycles of Pamidronate (parenteral administration over a three day period at three month intervals). All patients had their intramedullary rods in situ from anywhere between 2–7 years. The infections were low grade with a 2–3 month period of dull ache prior to actual presentation. Intrestigly though all patients had multiple rods in situ, only one of their femoral rods was affected and they did not have any other infective focus at the time of diagnosis. Three patients presented with thigh abcesses while the other two presented with ipsilateral knee pain and effusion. All had raised inflammatory markers, radiological signs of sepsis with Staph Aureus the commonest infecting organism.

Those cases presenting with abcesses were treated by drainage and rod removal, however only antibiotics were sufficient in the rest. The relationship between Pamidronate therapy and these infections is not absolutely clear and has not been reported previously. The possible links are discussed and a high degree of suspicion is recommended for those cases of OI on bisphosphonate

Objective: To review the hip subluxations or dislocations occurring during femoral lengthening in patients with congenital longitudinal lower limb deficiencies.

Methods: Sixty-three patients with congenital longitudinal lower limb deficiencies underwent femoral lengthening using either De Bastiani, Villarubias or Ilizarov technique. Acetabular index, medial joint space, CE angle of Wiberg, acetabular angle of Sharp and neck-shaft angle were measured on anteroposterior radiographs of hip before, during and after lengthening. The Acetabulum was considered dysplastic when the Sharp angle was more than 45 degrees. Hip was considered to be subluxed when the medial joint space increased during lengthening.

Results: During femoral lengthening, eleven hips sub-luxed as measured by the increase in medial joint space and one hip dislocated. All these hips had a preoperative acetabular index more than 25 degrees, CE angle less than 20 degrees and Sharp angle more than 45 degrees. The average neck-shaft angle was 75 degrees. Following subluxation, lengthening was stopped and the hips were reduced in hip spica after adductor and sartorius tenotomies. In one patient femoral shortening and acetabulo-plasty had to be done to reduce the subluxation. No case of avascular necrosis was noted.

Conclusion: Hip subluxation during femoral lengthening of congenital longitudinal lower limb deficiencies tends to occur when the acetabular index is more than 25 degrees, Sharp angle is more than 45 degrees, CE angle is less than 20 degrees and when there is associated femoral coxa vara. Careful preoperative assessment is required, and if need be hip reconstruction prior to lengthening. Close monitoring during lengthening is recommended.

Increased incidence of complications has been reported when lengthening limbs with underlying bone disorders such as dysplasias and metabolic bone diseases. There is a paucity of literature on limb lengthening in Osteogenesis Imperfecta (OI), probably due to the concern that the bone containing abnormal collagen may not tolerate the external fixators for a long term and there may not be adequate regenerate formation from this abnormal bone.

We performed limb lengthening and deformity correction of nine lower limb long bones in six children with OI. Four children were type I and two were type IV OI as per Sillence classification. The mean age was 14.7 years. All six children had lengthening for femoral shortening and three of them also had lengthening for tibial shortening on the same side. Angular deformities were corrected during lengthening. Five limb segments were treated using a monolateral external fixator and four limb segments were treated using an Ilizarov external fixator. In three children, previously inserted femoral intramedullary nails were left in situ during the course of femoral lengthening. The average lengthening achieved was 6.26 cm. Limb length discrepancies were corrected to within 1.5 cm of the length of the contralateral limb in five children. In one child with fixed pelvic obliquity and spinal scoliosis, functional leg length was achieved. The mean healing index was 33.25 days/cm of lengthening. Among the complications significant ones included, one deep infection, one fracture through the midshaft of the femur, and development of anterior angulation deformity after the removal of the fixator in one tibia. Abnormal bone of OI tolerated the external fixator throughout the period of lengthening without any incidence of migration of wires and screws through the soft bone when distraction forces were applied. The regenerate bone formed within the time that is normally expected in limb lengthening procedures performed for other conditions. We conclude that despite abnormal bone characteristics, limb reconstruction to correct limb length discrepancy and angular deformity can be done safely in children with OI.

We studied the quantity and rate of formation of new bone during lengthening of 17 limb segments in 10 patients using dual-energy X-ray absorptiometry (DEXA), ultrasonography and radiography. Whereas new bone was detected by both DEXA and ultrasonography within 1 to 2 weeks of distraction, it was not visible on the radiographs until 4 to 8 weeks. Limb alignment and gap measurement were accurately assessed by DEXA without the need for standard radiographs or scanograms. With ultrasound the distraction gap appeared as an echolucent window which narrowed progressively producing a hyper-reflecting line after which further consolidation could not be assessed. As measured by DEXA the density of the new bone at this stage was approximately 45% of control values and did not represent normal cortication. Whereas ultrasound could be used to identify defects in mineralisation and to determine when to dynamise the fixator system, DEXA could measure the quantity and rate of formation of bone throughout lengthening.

We measured the extent and rate of new bone formation over an 18-month period before, during and after the lengthening of ten leg segments in six patients aged between 8 and 18 years, using dual-energy X-ray absorptiometry (DEXA). New bone formation could be identified within one week of the start of distraction. As lengthening proceeded, the bone density of the gap fell, reaching minimum values at the time of maximal distraction. Consolidation of the regenerating bone was started 1 to 2 weeks later in the tibia, and 2.5 to 3.0 weeks later in the femur. The rate of mineral accretion in new bone was significantly greater in the tibia than in the femur (16 +/- 1.86%/month, and 11 +/- 1.1%/month respectively; mean +/- SEM). There was significant osteoporosis distal to the osteotomy, more in the tibia than in the femur, particularly on the side of the fixator. The bone mineral density of the distal segment remained low at the time of fixator removal (44.2 +/- 5.58% and 61.0 +/- 4.2% of the control values at the tibia and femur respectively) and was only partially reversed by subsequent weight-bearing. We conclude that dual-energy X-ray absorptiometry provides an objective and quantitative assessment of new bone formation during leg lengthening. The technique also allows the measurement of the distraction gap and the assessment of leg alignment from the high-resolution images. Its use may decrease the requirements for conventional radiography.

Five patients with Boyd type II congenital pseudarthrosis of the tibia underwent excision of the pseudarthrosis and double onlay bone grafting. Stability was maintained by extending intramedullary rods. Clinical union was achieved in all cases at a mean of 8.6 months (range six to 11). The rods extended by 15.7% (range 2% to 31.4%) as growth occurred. One rod was removed because of infection and a vascularised free fibular graft was subsequently performed. The extending rods provided stability while union occurred and did not require revision as the legs grew. The rods can be removed easily and have not jeopardized further surgical options.

We made a prospective study of 111 children with acute hip pain to assess whether ultrasound can replace traditional radiography. An effusion was diagnosed in 71% by ultrasound but in only 15% by radiography. This effusion persisted for a mean of nine days; symptoms lasted for five days. Two patients found to have Perthes' disease had longer-lasting effusion and symptoms. Patients without an effusion had no obvious cause for their pain, so the pressure of an effusion from a transient synovitis does not account for all patients with irritable hips. Patients with an effusion persisting for over 24 days (the mean + 2 s.d. of our series) had more symptoms, a significantly larger effusion and greater limitation of movement. They may be more at risk for avascular necrosis. We found that radiographic examination influenced the immediate management of only two patients, those with Perthes' disease. We therefore propose a protocol of management for irritable hip, using ultrasonography at the first presentation of certain categories of patients. This would reduce the number of early radiographs by 75%.

We report the results of using 83 expanding intramedullary rods in 24 children with osteogenesis imperfecta after a mean follow-up of five years three months. In all, 62% of the rods have expanded after one primary operation. Thirty-four additional operations were necessary; 11 for the correction of rotation or angulation deformities and 23 for revision of the rod or T-piece. All these revisions were successful. Complications were more frequent in children who required very small rods. Problems with Bailey-Dubow rods led to the development of the Sheffield rod system; 17 bones treated with these rods are included in the series. Before surgery only eight of the 24 children were able to walk but at review 20 children were walking, 15 without walking aids. Elongating intramedullary rods should be available to all children with osteogenesis imperfecta as they improve walking capability, reduce the number of fractures, prevent deformity and allow integration of the child into society.

We report nine cases of irreducible congenital dislocation of the knee which were treated by early operation with good results. All were resistant to conservative measures and operation was performed at an average age of nine months. The essential abnormality was a short quadriceps muscle together with subluxation of the hamstring muscles to lie anterior to the axis of knee flexion. The quadriceps tendon was lengthened by VY-plasty and in six cases additional length was gained by proximal mobilisation of the muscle. After operation all the patients were able to walk.

Seven pectoralis major transfers in children suffering from bilateral paralysis of elbow flexion due to arthrogryposis or to trauma are reported. A technique is described in which the muscle is mobilised from the clavicle to allow the tendon of insertion to be attached to the biceps tendon at the elbow. The biceps tendon was found to be present and could be mobilised forwards in all the arthrogrypotic elbows. Subjectively, the results were considered by patients or parents to be very good in six cases and fair in one. Elbow flexion power against gravity and against some resistance was achieved in all patients except one. The overall function was very good in one elbow, good in two, fair in three and poor in only one. The merits of the various procedures described for the restoration of elbow flexion in arthrogryposis are discussed. It is concluded that total pectoralis major transfer by the method described here has given the best results.

Excellent results can be achieved by plating fractures of the shaft of the humerus in patients with multiple injuries. This helps in nursing care and in the management of other injuries. In 38 patients admitted to a regional trauma centre, 39 humeral shaft fractures were plated. There were 27 men and 11 women, with an average age of 31.5 years. Fourteen of the humeral fractures were compound and 20 had significant comminution; 23 were fixed by a plate on the day of admission and all 39 by the twentieth day. Follow-up of 34 fractures showed that all had united, 33 primarily. All patients but one had a fully functional shoulder and no patient with a fractured humerus alone had lost any elbow movement. Complications were rare--one case each of non-union, fixation failure and infection. No permanent nerve injuries were produced at operation. The plating of fractures of the humerus in these circumstances has been shown to produce excellent results and has a place in the management of the patient with multiple injuries.

We report the ulnar impingement syndrome, which is caused by a shortened ulna impinging on the distal radius and causing a painful, disabling pseudarthrosis. Of the 11 cases reported, 10 were due to excision of the distal ulna after injury to the wrist; the other was a result of a growth arrest after a fracture of the distal ulna in a child. The symptoms are a painful, clicking wrist and a weak grip; clinical examination reveals a narrow wrist with pain on compression of the radius and ulna and on forced supination. Radiographs in the majority of cases show scalloping of the distal radius corresponding to the site of impingement. The mechanism by which ulnar impingement occurs after radio-ulnar convergence is illustrated. The plan of management for the young patient with traumatic dysfunction of the distal radio-ulnar joint is discussed; excision of the lower end of the ulna is not advised in such patients.