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The Journal of Bone & Joint Surgery British Volume
Vol. 91-B, Issue 7 | Pages 928 - 934
1 Jul 2009
Palan J Gulati A Andrew JG Murray DW Beard DJ

Balancing service provision and surgical training is a challenging issue that affects all healthcare systems. A multicentre prospective study of 1501 total hip replacements was undertaken to investigate whether there is an association between surgical outcome and the grade of the operating surgeon, and whether there is any difference in outcome if surgeons’ assistants assist with the operation, rather than orthopaedic trainees. The primary outcome measure was the change in the Oxford hip score (OHS) at five years. Secondary outcomes included the rate of revision and dislocation, operating time, and length of hospital stay.

There was no significant difference in ΔOHS or complication rates between operations undertaken by trainers and trainees, or those at which surgeons’ assistants and trainees were the assistant. However, there was a significant difference in the duration of surgery, with a mean reduction of 28 minutes in those in which a surgeons’ assistant was the assistant.

This study provides evidence that total hip replacements can be performed safely and effectively by appropriately trained surgeons in training, and that there are potential benefits of using surgeons’ assistants in orthopaedic surgery.


The Journal of Bone & Joint Surgery British Volume
Vol. 94-B, Issue 3 | Pages 359 - 364
1 Mar 2012
Pumberger M Chiu Y Ma Y Girardi FP Mazumdar M Memtsoudis SG

Increasing numbers of posterior lumbar fusions are being performed. The purpose of this study was to identify trends in demographics, mortality and major complications in patients undergoing primary posterior lumbar fusion. We accessed data collected for the Nationwide Inpatient Sample for each year between 1998 and 2008 and analysed trends in the number of lumbar fusions, mean patient age, comorbidity burden, length of hospital stay, discharge status, major peri-operative complications and mortality. An estimated 1 288 496 primary posterior lumbar fusion operations were performed between 1998 and 2008 in the United States. The total number of procedures, mean patient age and comorbidity burden increased over time. Hospital length of stay decreased, although the in-hospital mortality (adjusted and unadjusted for changes in length of hospital stay) remained stable. However, a significant increase was observed in peri-operative septic, pulmonary and cardiac complications. Although in-hospital mortality rates did not change over time in the setting of increases in mean patient age and comorbidity burden, some major peri-operative complications increased. These trends highlight the need for appropriate peri-operative services to optimise outcomes in an increasingly morbid and older population of patients undergoing lumbar fusion.


The Journal of Bone & Joint Surgery British Volume
Vol. 94-B, Issue 8 | Pages 1036 - 1044
1 Aug 2012
Penny JO Brixen K Varmarken JE Ovesen O Overgaard S

It is accepted that resurfacing hip replacement preserves the bone mineral density (BMD) of the femur better than total hip replacement (THR). However, no studies have investigated any possible difference on the acetabular side.

Between April 2007 and March 2009, 39 patients were randomised into two groups to receive either a resurfacing or a THR and were followed for two years. One patient’s resurfacing subsequently failed, leaving 19 patients in each group.

Resurfaced replacements maintained proximal femoral BMD and, compared with THR, had an increased bone mineral density in Gruen zones 2, 3, 6, and particularly zone 7, with a gain of 7.5% (95% confidence interval (CI) 2.6 to 12.5) compared with a loss of 14.6% (95% CI 7.6 to 21.6). Resurfacing replacements maintained the BMD of the medial femoral neck and increased that in the lateral zones between 12.8% (95% CI 4.3 to 21.4) and 25.9% (95% CI 7.1 to 44.6).

On the acetabular side, BMD was similar in every zone at each point in time. The mean BMD of all acetabular regions in the resurfaced group was reduced to 96.2% (95% CI 93.7 to 98.6) and for the total hip replacement group to 97.6% (95% CI 93.7 to 101.5) (p = 0.4863). A mean total loss of 3.7% (95% CI 1.0 to 6.5) and 4.9% (95% CI 0.8 to 9.0) of BMD was found above the acetabular component in W1 and 10.2% (95% CI 0.9 to 19.4) and 9.1% (95% CI 3.8 to 14.4) medial to the implant in W2 for resurfaced replacements and THRs respectively. Resurfacing resulted in a mean loss of BMD of 6.7% (95% CI 0.7 to 12.7) in W3 but the BMD inferior to the acetabular component was maintained in both groups.

These results suggest that the ability of a resurfacing hip replacement to preserve BMD only applies to the femoral side.


The Journal of Bone & Joint Surgery British Volume
Vol. 91-B, Issue 1 | Pages 31 - 36
1 Jan 2009
de Jong PT de Man FHR Haverkamp D Marti RK

We report the long-term outcome of a modified second-generation cementing technique for fixation of the acetabular component of total hip replacement. An earlier report has shown the superiority of this technique assessed by improved survival compared with first-generation cementing. The acetabular preparation involved reaming only to the subchondral plate, followed by impaction of the bone in the anchorage holes.

Between 1978 and 1993, 287 total hip replacements were undertaken in 244 patients with a mean age of 65.3 years (21 to 90) using a hemispherical Weber acetabular component with this modified technique for cementing and a cemented femoral component.

The survival with acetabular revision for aseptic loosening as the endpoint was 99.1% (95% confidence interval 97.9 to 100 after ten years and 85.5% (95% confidence interval 74.7 to 96.2) at 20 years. Apart from contributing to a long-lasting fixation of the component, this technique also preserved bone, facilitating revision surgery when necessary.


The Journal of Bone & Joint Surgery British Volume
Vol. 94-B, Issue 1 | Pages 122 - 127
1 Jan 2012
Streitbuerger A Ahrens H Gosheger G Henrichs M Balke M Dieckmann R Hardes J

The aim of this study was to define the treatment criteria for patients with recurrent chondrosarcoma. We reviewed the data of 77 patients to examine the influence of factors such as the intention of treatment (curative/palliative), extent of surgery, resection margins, status of disease at the time of local recurrence and the grade of the tumour. A total of 70 patients underwent surgery for recurrent chondrosarcoma. In seven patients surgery was not a viable option. Metastatic disease occurred in 41 patients, appearing synchronously with the local recurrence in 56% of cases. For patients without metastasis at the time of local recurrence, the overall survival at a mean follow-up after recurrence of 67 months (0 to 289) was 74% (5 of 27) compared with 19% (13 of 50) for patients with metastasis at or before the development of the recurrence. Neither the type/extent of surgery, site of tumour, nor the resection margins for the recurrent tumour significantly influenced the overall survival.

With limited survival for patients with metastatic disease at the time of local recurrence (0% for patients with grade III and de-differentiated chondrosarcoma), palliative treatment, including local radiation therapy and debulking procedures, should be discussed with the patients to avoid long hospitalisation and functional deficits. For patients without metastasis at the time of local recurrence, the overall survival of 74% justifies an aggressive approach including wide resection margins and extensive reconstruction.


The Journal of Bone & Joint Surgery British Volume
Vol. 89-B, Issue 9 | Pages 1234 - 1238
1 Sep 2007
Foster L Dall GF Reid R Wallace WH Porter DE

We have reviewed the data from our regional Bone Tumour Registry on patients with osteosarcoma diagnosed between 1933 and 2004 in order to investigate the relationship between survival and changes in treatment. There were 184 patients with non-metastatic appendicular osteosarcoma diagnosed at the age of 18 or under. Survival was calculated using Kaplan-Meier curves, and multivariate analysis was performed using the Cox regression proportional hazards model.

The five-year survival improved from 21% between 1933 and 1959, to 62% between 1990 and 1999. During this time, a multi-disciplinary organisation was gradually developed to manage treatment. The most significant variable affecting outcome was the date of diagnosis, with trends in improved survival mirroring the introduction of increasingly effective chemotherapy. Our experience suggests that the guidelines of the National Institute for Clinical Excellence on the minimum throughput of centres for treatment should be enforced flexibly in those that can demonstrate that their historical and contemporary results are comparable to those published nationally and internationally.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 11 | Pages 1513 - 1517
1 Nov 2011
Singh JA Sperling JW Cofield RH

Our objective was to examine the rate of revision and its predictive factors in patients undergoing total shoulder arthroplasty (TSA). We used prospectively collected data from the Mayo Clinic Total Joint Registry to examine five-, ten- and 20-year revision-free survival following TSA and the predictive factors. We examined patient characteristics (age, gender, body mass index, comorbidity), implant fixation (cemented versus uncemented), American Society of Anesthesiologists class and underlying diagnosis. Univariate and multivariable adjusted hazard rates were calculated using Cox regression analysis. A total of 2207 patients underwent 2588 TSAs. Their mean age was 65.0 years (19 to 91) and 1163 (53%) were women; osteoarthritis was the underlying diagnosis in 1640 shoulders (63%). In all, 212 TSAs (8.2%) were revised during the follow-up period. At five, ten and 20 years, survival rates were 94.2% (95% confidence interval (CI) 93.2 to 95.3), 90.2% (95% CI 88.7 to 91.7) and 81.4% (95% CI 78.4 to 84.5), respectively. In multivariable analyses men had a higher hazard ratio of revision of 1.72 (95% CI 1.28 to 2.31) (p < 0.01) compared with women, and those with rotator cuff disease had a hazard ratio of 4.71 (95% CI 2.09 to 10.59) (p < 0.001) compared with patients with rheumatoid arthritis. We concluded that male gender and rotator cuff disease are independent risk factors for revision after TSA. Future studies are needed to understand the biological rationale for these differences.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 7 | Pages 970 - 974
1 Jul 2011
Giannoudis PV Kanakaris NK Dimitriou R Mallina R Smith RM

Isolated fractures of the anterior column and anterior wall are a relatively rare subgroup of acetabular fractures. We report our experience of 30 consecutive cases treated over ten years. Open reduction and internal fixation through an ilioinguinal approach was performed for most of these cases (76.7%) and percutaneous techniques were used for the remainder. At a mean follow-up of four years (2 to 6), 26 were available for review. The radiological and functional outcomes were good or excellent in 23 of 30 patients (76.7%) and 22 of 26 patients (84.6%) according to Matta’s radiological criteria and the modified Merlé d’Aubigné score, respectively.

Complications of minor to moderate severity were seen in six of the 30 cases (20%) and none of the patients underwent secondary surgery or replacement of the hip.


The Journal of Bone & Joint Surgery British Volume
Vol. 90-B, Issue 2 | Pages 203 - 208
1 Feb 2008
Chandrasekar CR Wafa H Grimer RJ Carter SR Tillman RM Abudu A

We investigated whether our policy of routine re-excision of the tumour bed after an unplanned excision of a soft-tissue sarcoma was justified.

Between April 1982 and December 2005, 2201 patients were referred to our hospital with the diagnosis of soft-tissue sarcoma, of whom 402 (18%) had undergone an unplanned excision elsewhere. A total of 363 (16.5%) were included in this study. Each patient was routinely restaged and the original histology was reviewed. Re-excision was undertaken in 316 (87%). We analysed the patient, tumour and treatment factors in relation to local control, metastasis and overall survival.

Residual tumour was found in 188 patients (59%). There was thus no residual disease in 128 patients of whom 10% (13) went on to develop a local recurrence. In 149 patients (47%), the re-excision specimen contained residual tumour, but it had been widely excised. Local recurrence occurred in 30 of these patients (20%). In 39 patients (12%), residual tumour was present in a marginal resection specimen. Of these, 46% (18) developed a local recurrence. A final positive margin in a high-grade tumour had a 60% risk of local recurrence even with post-operative radiotherapy.

Metastases developed in 24% (86). The overall survival was 77% at five years. Survival was related to the grade of the tumour and the finding of residual tumour at the time of re-excision.

We concluded that our policy of routine re-excision after unplanned excision of soft-tissue sarcoma was justified in view of the high risk of finding residual tumour.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 11 | Pages 1518 - 1523
1 Nov 2011
Lakkol S Bhatia C Taranu R Pollock R Hadgaonkar S Krishna M

Recurrence of back or leg pain after discectomy is a well-recognised problem with an incidence of up to 28%. Once conservative measures have failed, several surgical options are available and have been tried with varying degrees of success. In this study, 42 patients with recurrent symptoms after discectomy underwent less invasive posterior lumbar interbody fusion (LI-PLIF). Clinical outcome was measured using the Oswestry Disability Index (ODI), Short Form 36 (SF-36) questionnaires and visual analogue scales for back (VAS-BP) and leg pain (VAS-LP). There was a statistically significant improvement in all outcome measures (p < 0.001). The debate around which procedure is the most effective for these patients remains controversial.

Our results show that LI-PLIF is as effective as any other surgical procedure. However, given that it is less invasive, we feel that it should be considered as the preferred option.


The Journal of Bone & Joint Surgery British Volume
Vol. 88-B, Issue 7 | Pages 860 - 864
1 Jul 2006
McCullough CJ Remedios D Tytherleigh-Strong G Hua J Walker PS

Between June 1991 and January 1995, 42 hydroxyapatite-coated CAD-CAM femoral components were inserted in 25 patients with inflammatory polyarthropathy, 21 of whom had juvenile idiopathic arthritis. Their mean age was 21 years (11 to 35). All the patients were reviewed clinically and radiologically at one, three and five years. At the final review at a mean of 11.2 years (8 to 13) 37 hips in 23 patients were available for assessment.

A total of four femoral components (9.5%) had failed, of which two were radiologically loose and two were revised. The four failed components were in patients aged 16 years or less at the time of surgery. Hydroxyapatite-coated customised femoral components give excellent medium- to long-term results in skeletally-mature young adults with inflammatory polyarthropathy. Patients aged less than 16 years at the time of surgery have a risk of 28.5% of failure of the femoral component at approximately ten years.


The Journal of Bone & Joint Surgery British Volume
Vol. 89-B, Issue 12 | Pages 1574 - 1580
1 Dec 2007
Hallan G Lie SA Furnes O Engesaeter LB Vollset SE Havelin LI

Primary uncemented femoral stems reported to the Norwegian arthroplasty register between 1987 and 2005 were included in this prospective observational study. There were 11 516 hips (9679 patients) and 14 different designs of stem. Kaplan-Meier survival probabilities and Cox regression were used to analyse the data.

With aseptic loosening as the end-point, all currently used designs performed excellently with survival of 96% to 100% at ten years. With the end-point as stem revision for any cause, the long-term results of the different designs varied from poor to excellent, with survival at 15 years ranging between 29% and 97%. Follow-up for longer than seven years was needed to identify some of the poorly-performing designs. There were differences between the stems; the Corail, used in 5456 hips, was the most frequently used stem with a survival of 97% at 15 years. Male gender was associated with an increased risk of revision of × 1.3 (95% confidence interval 1.05 to 1.52), but age and diagnosis had no influence on the results. Overall, modern uncemented femoral stems performed well.

Moderate differences in survival between well-performing stems should be interpreted with caution since the differences may be caused by factors other than the stem itself.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 3 | Pages 399 - 403
1 Mar 2011
Griffiths D Gikas PD Jowett C Bayliss L Aston W Skinner J Cannon S Blunn G Briggs TWR Pollock R

Between 1997 and 2007, 68 consecutive patients underwent replacement of the proximal humerus for tumour using a fixed-fulcrum massive endoprosthesis. Their mean age was 46 years (7 to 87). Ten patients were lost to follow-up and 16 patients died. The 42 surviving patients were assessed using the Musculoskeletal Tumor Society (MSTS) Score and the Toronto Extremity Salvage Score (TESS) at a mean follow-up of five years and 11 months (one year to ten years and nine months). The mean MSTS score was 72.3% (53.3% to 100%) and the mean TESS was 77.2% (58.6% to 100%).

Four of 42 patients received a new constrained humeral liner to reduce the risk of dislocation. This subgroup had a mean MSTS score of 77.7% and a mean TESS of 80.0%. The dislocation rate for the original prosthesis was 25.9; none of the patients with the new liner had a dislocation at a mean of 14.5 months (12 to 18).

Endoprosthetic replacement for tumours of the proximal humerus using this prosthesis is a reliable operation yielding good results without the documented problems of unconstrained prostheses. The performance of this prosthesis is expected to improve further with a new constrained humeral liner, which reduces the risk of dislocation.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 4 | Pages 531 - 536
1 Apr 2011
Pradhan A Grimer RJ Spooner D Peake D Carter SR Tillman RM Abudu A Jeys L

The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing’s sarcoma. We identified 300 patients with new primary Ewing’s sarcoma diagnosed between 1980 and 2005 from the centres’ local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing’s sarcomas. Although patients with skeletal Ewing’s were younger (mean age 16.8 years) than those with extra-skeletal Ewing’s sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing’s sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not.

This large series has shown that the oncological outcomes of Ewing’s sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.


The Journal of Bone & Joint Surgery British Volume
Vol. 88-B, Issue 10 | Pages 1293 - 1298
1 Oct 2006
Allami MK Fender D Khaw FM Sandher DR Esler C Harper WM Gregg PJ

Using the Trent regional arthroplasty register, we analysed the survival at ten years of 1198 consecutive Charnley total hip replacements carried out across a single health region of the United Kingdom in 1990. At ten years, information regarding outcome was available for 1001 hips (83.6%). The crude revision rate was 6.2% (62 of 1001) and the cumulative survival rate with revision of the components as an end-point was 93.1%. At five years, a review of this series of patients identified gross radiological failure in 25 total hip replacements which had previously been unrecognised. At ten years the outcome was known for 18 of these 25 patients (72%), of whom 13 had not undergone revision.

This is the first study to assess the survival at ten years for the primary Charnley total hip replacement performed in a broad cross-section of hospitals in the United Kingdom, as opposed to specialist centres. Our results highlight the importance of the arthroplasty register in identifying the long-term outcome of hip prostheses.


The Journal of Bone & Joint Surgery British Volume
Vol. 88-B, Issue 12 | Pages 1584 - 1590
1 Dec 2006
Hook S Moulder E Yates PJ Burston BJ Whitley E Bannister GC

We reviewed 142 consecutive primary total hip replacements implanted into 123 patients between 1988 and 1993 using the Exeter Universal femoral stem. A total of 74 patients (88 hips) had survived for ten years or more and were reviewed at a mean of 12.7 years (10 to 17). There was no loss to follow-up.

The rate of revision of the femoral component for aseptic loosening and osteolysis was 1.1% (1 stem), that for revision for any cause was 2.2% (2 stems), and for re-operation for any cause was 21.6% (19 hips). Re-operation was because of failure of the acetabular component in all but two hips.

All but one femoral component subsided within the cement mantle to a mean of 1.52 mm (0 to 8.3) at the final follow-up. One further stem had subsided excessively (8 mm) and had lucent lines at the cement-stem and cement-bone interfaces. This was classified as a radiological failure and is awaiting revision. One stem was revised for deep infection and one for excessive peri-articular osteolysis. Defects of the cement mantle (Barrack grade C and D) were found in 28% of stems (25 hips), associated with increased subsidence (p = 0.01), but were not associated with endosteal lysis or failure.

Peri-articular osteolysis was significantly related to the degree of polyethylene wear (p < 0.001), which was in turn associated with a younger age (p = 0.01) and male gender (p < 0.001).

The use of the Exeter metal-backed acetabular component was a notable failure with 12 of 32 hips (37.5%) revised for loosening. The Harris-Galante components failed with excessive wear, osteolysis and dislocation with 15% revised (5 of 33 hips). Only one of 23 hips with a cemented Elite component (4%) was revised for loosening and osteolysis.

Our findings show that the Exeter Universal stem implanted outside the originating centre has excellent medium-term results.


The Journal of Bone & Joint Surgery British Volume
Vol. 89-B, Issue 5 | Pages 599 - 603
1 May 2007
Robertsson O Stefánsdòttir A Lidgren L Ranstam J

Patients with osteoarthritis undergoing knee replacement have been reported to have an overall reduced mortality compared with that of the general population. This has been attributed to the selection of healthier patients for surgery. However, previous studies have had a maximum follow-up time of ten years. We have used information from the Swedish Knee Arthroplasty Register to study the mortality of a large national series of patients with total knee replacement for up to 28 years after surgery and compared their mortality with that of the normal population. In addition, for a subgroup of patients operated on between 1980 and 2002 we analysed their registered causes of death to determine if they differed from those expected.

We found a reduced overall mortality during the first 12 post-operative years after which it increased and became significantly higher than that of the general population. Age-specific analysis indicated an inverse correlation between age and mortality, where the younger the patients were, the higher their mortality. The shift at 12 years was caused by a relative over-representation of younger patients with a longer follow-up. Analysis of specific causes of death showed a higher mortality for cardiovascular, gastrointestinal and urogenital diseases. The observation that early onset of osteoarthritis of the knee which has been treated by total knee replacement is linked to an increased mortality should be a reason for increased general awareness of health problems in these patients.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 1 | Pages 68 - 72
1 Jan 2011
Motosuneya T Maruyama T Yamada H Tsuzuki N Sakai H

We reviewed 75 patients (57 men and 18 women), who had undergone tension-band laminoplasty for cervical spondylotic myelopathy (42 patients) or compression myelopathy due to ossification of the posterior longitudinal ligament (33 patients) and had been followed for more than ten years. Clinical and functional results were estimated using the Japanese Orthopaedic Association score. The rate of recovery and the level of postoperative axial neck pain were also recorded. The pre- and post-operative alignment of the cervical spine (Ishihara curve index indicating lordosis of the cervical spine) and the range of movement (ROM) of the cervical spine were also measured.

The mean rate of recovery of the Japanese Orthopaedic Association score at final follow-up was 52.1% (sd 24.6) and significant axial pain was reported by 19 patients (25.3%). Axial pain was reported more frequently in patients with ossification of the posterior longitudinal ligament than in those with cervical spondylotic myelopathy (p = 0.027). A kyphotic deformity was not seen post-operatively in any patient. The mean ROM decreased post-operatively from 32.8° (sd 12.3) to 16.2° (sd 12.3) (p < 0.001). The mean ROM ratio was 46.9% (sd 28.1) for all the patients. The mean ROM ratio was lower in patients with ossification of the posterior longitudinal ligament than in those with cervical spondylotic myelopathy (p < 0.001). Compared to those with cervical spondylotic myelopathy, patients with ossification of the posterior longitudinal ligament had less ROM and more post-operative axial neck pain.


The Journal of Bone & Joint Surgery British Volume
Vol. 92-B, Issue 10 | Pages 1351 - 1355
1 Oct 2010
Veitch SW Whitehouse SL Howell JR Hubble MJW Gie GA Timperley AJ

We report the outcome of the flangeless, cemented all-polyethylene Exeter acetabular component at a mean of 14.6 years (10 to 17) after operation. Of the 263 hips in 243 patients, 122 prostheses are still in situ; 112 patients (119 hips) have died, 18 hips have been revised, and three patients (four hips) were lost to follow-up. Radiographs at the final review were available for 110 of the 122 surviving hips. There were acetabular radiolucent lines in 54 hips (49%). Two acetabular components had migrated but neither patient required revision. The Kaplan-Meier survivorship at 15 years with 61 hips at risk with revision for any cause as the endpoint was 89.9% (95% confidence interval (CI) 84.6 to 95.2) and for aseptic loosening of the acetabular component or lysis 91.7% (95% CI 86.6 to 96.8). In 210 hips with a diagnosis of primary osteoarthritis, survivorship with revision for any cause as the endpoint was 93.2% (95% CI 88.1 to 98.3), and for aseptic loosening of the acetabular component 95.0% (95% CI 90.3 to 99.7).

The cemented all-polyethylene Exeter acetabular component has an excellent long-term survivorship.


The Journal of Bone & Joint Surgery British Volume
Vol. 92-B, Issue 6 | Pages 880 - 884
1 Jun 2010
Hassan A Whately C Letts M

Stüve-Wiedemann syndrome is an autosomal-recessive disorder characterised by bowing of the long bones, progressive scoliosis, episodic hyperthermia and respiratory distress, usually resulting in death in infancy. We reviewed five children with the condition who had been followed since birth and who survived into childhood with a mean age at operation of 7.8 years (5 to 14). There was marked functional impairment with dysplasia of the long bones and scoliosis. Treatment of the triplanar deformities of the femora involved the use of the Ilizarov technique with the Taylor Spatial Frame.

Walking was preserved and improved in three children along with considerable enhancement of the appearance. Early insertion of a growing rod to control the progressive juvenile scoliosis was beneficial. The use of the Taylor Spatial Frame is strongly recommended to address the major complex deformities of the lower limbs which are encountered in this condition and to prevent their progression.