The relationship between spinopelvic measurements and patient-reported outcome scores in patients with multiple myeloma of the spine.

AIMS: Patients with multiple myeloma (MM) develop deposits in the spine which may lead to vertebral compression fractures (VCFs). Our aim was to establish which spinopelvic parameters are associated with the greatest disability in patients with spinal myeloma and VCFs. PATIENTS AND METHODS: We performed a retrospective cross-sectional review of 148 consecutive patients (87 male, 61 female) with spinal myeloma and analysed correlations between spinopelvic parameters and patient-reported outcome scores. The mean age of the patients was 65.5 years (37 to 91) and the mean number of vertebrae involved was 3.7 (1 to 15). RESULTS: The thoracolumbar region was most commonly affected (109 patients, 73.6%), and was the site of most posterior vertebral wall defects (47 patients, 31.8%). Poorer Oswestry Disability Index scores correlated with an increased sagittal vertical axis (p = 0.006), an increased number of VCFs (p = 0.035) and sternal involvement (p = 0.012). Poorer EuroQol visual analogue scale scores correlated with posterior vertebral wall defects in the thoracolumbar region (p = 0.012). The sagittal vertical axis increased with the number of fractures and kyphosis in the thoracolumbar (p = 0.009) and lumbar (p < 0.001) regions. CONCLUSIONS: In MM, patients with VCFs have poorer clinical scores at presentation in the presence of sagittal imbalance. Outcome is particularly affected by multiple fractures in the thoracolumbar and lumbar regions and by failure to prevent kyphosis. Patients with MM should be screened for spinal lesions early. Cite this article: Bone Joint J 2016;98-B:1234-9.

A review of the surgical management of sacral chordoma.

BACKGROUND: Sacral chordomas are rare low-to-intermediate grade malignant tumours, which arise from remnants of the embryonic notochord. This review explores prognostic factors in the management of sacral chordomas and provides guidance on the optimal treatment regimens based on the current literature. PATIENTS AND METHODS: Electronic searches were performed using MEDLINE, Embase and the Cochrane library to identify studies on prognostic factors in the management of sacral chordomas published between January 1970 and December 2013. The literature search and review process identified 100 articles that were included in the review article. This included both surgical and non-surgical studies on the management of sacral chordomas. RESULTS: Sacrectomy with wide resection margins forms the mainstay of treatment but is associated with high risk of disease recurrence and reduced long-term survival. Adequate resection margins may require sacrifice of adjacent nerve roots, musculature and ligaments leading to functional compromise and mechanical instability. Large tumour size (greater than 5-10 cm in diameter), dedifferentiation and greater cephalad tumour extension are associated with increased risk of disease recurrence and reduced survival. Chordomas are poorly responsive to conventional radiotherapy and chemotherapy. CONCLUSION: Operative resection with wide resection margins offers the best long-term prognosis. Inadequate resection margins, large tumour size, dedifferentiation, and greater cephalad chordoma extension are associated with poor oncological outcomes. Routine long-term follow-up is essential to enable early detection and treatment of recurrent disease.

Instability of the sternoclavicular joint: current concepts in classification, treatment and outcomes.

The sternoclavicular joint (SCJ) is a pivotal articulation in the linked system of the upper limb girdle, providing load-bearing in compression while resisting displacement in tension or distraction at the manubrium sterni. The SCJ and acromioclavicular joint (ACJ) both have a small surface area of contact protected by an intra-articular fibrocartilaginous disc and are supported by strong extrinsic and intrinsic capsular ligaments. The function of load-sharing in the upper limb by bulky periscapular and thoracobrachial muscles is extremely important to the longevity of both joints. Ligamentous and capsular laxity changes with age, exposing both joints to greater strain, which may explain the rising incidence of arthritis in both with age. The incidence of arthritis in the SCJ is less than that in the ACJ, suggesting that the extrinsic ligaments of the SCJ provide greater stability than the coracoclavicular ligaments of the ACJ. Instability of the SCJ is rare and can be difficult to distinguish from medial clavicular physeal or metaphyseal fracture-separation: cross-sectional imaging is often required. The distinction is important because the treatment options and outcomes of treatment are dissimilar, whereas the treatment and outcomes of ACJ separation and fracture of the lateral clavicle can be similar. Proper recognition and treatment of traumatic instability is vital as these injuries may be life-threatening. Instability of the SCJ does not always require surgical intervention. An accurate diagnosis is required before surgery can be considered, and we recommend the use of the Stanmore instability triangle. Most poor outcomes result from a failure to recognise the underlying pathology. There is a natural reluctance for orthopaedic surgeons to operate in this area owing to unfamiliarity with, and the close proximity of, the related vascular structures, but the interposed sternohyoid and sternothyroid muscles are rarely injured and provide a clear boundary to the medial retroclavicular space, as well as an anatomical barrier to unsafe intervention. This review presents current concepts of instability of the SCJ, describes the relevant surgical anatomy, provides a framework for diagnosis and management, including physiotherapy, and discusses the technical challenges of operative intervention.

Management of peri-prosthetic fracture of the humerus with severe bone loss and loosening of the humeral component after total shoulder replacement.

There is little information about the management of peri-prosthetic fracture of the humerus after total shoulder replacement (TSR). This is a retrospective review of 22 patients who underwent a revision of their original shoulder replacement for peri-prosthetic fracture of the humerus with bone loss and/or loose components. There were 20 women and two men with a mean age of 75 years (61 to 90) and a mean follow-up 42 months (12 to 91): 16 of these had undergone a previous revision TSR. Of the 22 patients, 12 were treated with a long-stemmed humeral component that bypassed the fracture. All their fractures united after a mean of 27 weeks (13 to 94). Eight patients underwent resection of the proximal humerus with endoprosthetic replacement to the level of the fracture. Two patients were managed with a clam-shell prosthesis that retained the original components. The mean Oxford shoulder score (OSS) of the original TSRs before peri-prosthetic fracture was 33 (14 to 48). The mean OSS after revision for fracture was 25 (9 to 31). Kaplan-Meier survival using re-intervention for any reason as the endpoint was 91% (95% confidence interval (CI) 68 to 98) and 60% (95% CI 30 to 80) at one and five years, respectively. There were two revisions for dislocation of the humeral head, one open reduction for modular humeral component dissociation, one internal fixation for nonunion, one trimming of a prominent screw and one re-cementation for aseptic loosening complicated by infection, ultimately requiring excision arthroplasty. Two patients sustained nerve palsies. Revision TSR after a peri-prosthetic humeral fracture associated with bone loss and/or loose components is a salvage procedure that can provide a stable platform for elbow and hand function. Good rates of union can be achieved using a stem that bypasses the fracture. There is a high rate of complications and function is not as good as with the original replacement.

The outcome of scapulothoracic fusion for painful winging of the scapula in dystrophic and non-dystrophic conditions.

Scapulothoracic fusion (STF) for painful winging of the scapula in neuromuscular disorders can provide effective pain relief and functional improvement, but there is little information comparing outcomes between patients with dystrophic and non-dystrophic conditions. We performed a retrospective review of 42 STFs in 34 patients with dystrophic and non-dystrophic conditions using a multifilament trans-scapular, subcostal cable technique supported by a dorsal one-third semi-tubular plate. There were 16 males and 18 females with a mean age of 30 years (15 to 75) and a mean follow-up of 5.0 years (2.0 to 10.6). The mean Oxford shoulder score improved from 20 (4 to 39) to 31 (4 to 48). Patients with non-dystrophic conditions had lower overall functional scores but achieved greater improvements following STF. The mean active forward elevation increased from 59° (20° to 90°) to 97° (30° to 150°), and abduction from 51° (10° to 90°) to 83° (30° to 130°) with a greater range of movement achieved in the dystrophic group. Revision fusion for nonunion was undertaken in five patients at a mean time of 17 months (7 to 31) and two required revision for fracture. There were three pneumothoraces, two rib fractures, three pleural effusions and six nonunions. The main risk factors for nonunion were smoking, age and previous shoulder girdle surgery. STF is a salvage procedure that can provide good patient satisfaction in 82% of patients with both dystrophic and non-dystrophic pathologies, but there was a relatively high failure rate (26%) when poor outcomes were analysed. Overall function was better in patients with dystrophic conditions which correlated with better range of movement; however, patients with non-dystrophic conditions achieved greater functional improvement.

Custom rotating-hinge total knee replacement in patients with spina bifida and severe neuromuscular dysfunction.

Spina bifida (SB) is a congenital disorder which may result in a number of musculoskeletal problems. Total knee replacement (TKR) in this patient group is technically demanding due to bone deformity, soft tissue contracture, muscle tone abnormality and ligament insufficiency. This is a retrospective review of three patients with SB and disabling knee arthritis who were managed with a custom rotating-hinge (RHK) total knee system. All patients reported an improvement in knee pain and stability at mean follow-up 47 months (43-53). Mean Oxford Knee score improved from 21 preoperatively to 32 at final follow-up. One patient required revision of tibial and patella components at 37 months for lateral patella instability and excessive wear. Custom RHK for patients with SB, severe neuromuscular dysfunction and bone deformity relieves pain, restores stability and improves early knee function; however there is a significant risk of extensor mechanism complications and functional outcome is worse than primary TKR in the general population.

Custom-made rotating hinge total knee replacement in a patient with congenital tibial deficiency avoids the need for amputation.

We report a unique case of a patient with type 2 congenital tibial deficiency and disabling knee osteoarthritis in whom a custom-made rotating hinge knee replacement was successfully performed, allowing continued mobilisation with a below-knee prosthesis, thereby avoiding the need for an above-knee amputation. Level of evidence Therapeutic study, Level IV.

Custom rotating-hinge primary total knee arthroplasty in patients with skeletal dysplasia.

Patients with skeletal dysplasia are prone to developing advanced osteoarthritis of the knee requiring total knee replacement (TKR) at a younger age than the general population. TKR in this unique group of patients is a technically demanding procedure owing to the deformity, flexion contracture, generalised hypotonia and ligamentous laxity. We retrospectively reviewed the outcome of 11 TKRs performed in eight patients with skeletal dysplasia at our institution using the Stanmore Modular Individualised Lower Extremity System (SMILES) custom-made rotating-hinge TKR. There were three men and five women with mean age of 57 years (41 to 79). Patients were followed clinically and radiologically for a mean of seven years (3 to 11.5). The mean Knee Society clinical and function scores improved from 24 (14 to 36) and 20 (5 to 40) pre-operatively, respectively, to 68 (28 to 80) and 50 (22 to 74), respectively, at final follow-up. Four complications were recorded, including a patellar fracture following a fall, a tibial peri-prosthetic fracture, persistent anterior knee pain, and aseptic loosening of a femoral component requiring revision. Our results demonstrate that custom primary rotating-hinge TKR in patients with skeletal dysplasia is effective at relieving pain, with a satisfactory range of movement and improved function. It compensates for bony deformity and ligament deficiency and reduces the likelihood of corrective osteotomy. Patellofemoral joint complications are frequent and functional outcome is worse than with primary TKR in the general population.

Intercalary diaphyseal endoprosthetic reconstruction for malignant tibial bone tumours.

The best method of reconstruction after resection of malignant tumours of the tibial diaphysis is unknown. In the absence of any long-term studies analysing the results of intercalary endoprosthetic replacement, we present a retrospective review of 18 patients who underwent limb salvage using a tibial diaphyseal endoprosthetic replacement following excision of a malignant bone tumour. There were ten men and eight women with a mean age of 42.5 years (16 to 76). Mean follow-up was 58.5 months (20 to 141) for all patients and 69.3 months (20 to 141) for the 12 patients still alive. Cumulative patient survival was 59% (95% confidence interval (CI) 32 to 84) at five years. Implant survival was 63% (95% CI 35 to 90) at ten years. Four patients required revision to a proximal tibial replacement at a mean follow-up of 29 months (10 to 54). Complications included metastases in five patients, aseptic loosening in four, peri-prosthetic fracture in two, infection in one and local recurrence in one. The mean Musculoskeletal Tumor Society score and the mean Toronto Extremity Salvage Score were 23 (17 to 28) and 74% (53 to 91), respectively. Although rates of complication and revision were high, custom-made tibial diaphyseal replacement following resection of malignant bone tumours enables early return to function and provides an attractive alternative to other surgical options, without apparent compromise of patient survival.

Femoral diaphyseal endoprosthetic reconstruction after segmental resection of primary bone tumours.

Segmental resection of malignant bone disease in the femoral diaphysis with subsequent limb reconstruction is a major undertaking. This is a retrospective review of 23 patients who had undergone limb salvage by endoprosthetic replacement of the femoral diaphysis for a primary bone tumour between 1989 and 2005. There were 16 males and seven females, with a mean age of 41.3 years (10 to 68). The mean overall follow-up was for 97 months (3 to 240), and 120 months (42 to 240) for the living patients. The cumulative patient survival was 77% (95% confidence interval 63% to 95%) at ten years. Survival of the implant, with failure of the endoprosthesis as an endpoint, was 85% at five years and 68% (95% confidence interval 42% to 92%) at ten years. The revision rate was 22% and the overall rate of re-operation was 26%. Complications included deep infection (4%), breakage of the prosthesis (8%), periprosthetic fracture (4%), aseptic loosening (4%), local recurrence (4%) and metastases (17%). The 16 patients who retained their diaphyseal endoprosthesis had a mean Musculoskeletal Tumour Society score of 87% (67% to 93%). They were all able to comfortably perform most activities of daily living. Femoral diaphyseal endoprosthetic replacement is a viable option for reconstruction following segmental resection of malignant bone disease. It allows immediate weight-bearing, is associated with a good long-term functional outcome, has an acceptable complication and revision rate and, most importantly, does not appear to compromise patient survival.

Total femoral endoprosthetic replacement following excision of bone tumours.

We undertook a retrospective review of 33 patients who underwent total femoral endoprosthetic replacement as limb salvage following excision of a malignant bone tumour. In 22 patients this was performed as a primary procedure following total femoral resection for malignant disease. Revision to a total femoral replacement was required in 11 patients following failed segmental endoprosthetic or allograft reconstruction. There were 33 patients with primary malignant tumours, and three had metastatic lesions. The mean age of the patients was 31 years (5 to 68). The mean follow-up was 4.2 years (9 months to 16.4 years). At five years the survival of the implants was 100%, with removal as the endpoint and 56% where the endpoint was another surgical intervention. At five years the patient survival was 32%. Complications included dislocation of the hip in six patients (18%), local recurrence in three (9%), peri-prosthetic fracture in two and infection in one. One patient subsequently developed pulmonary metastases. There were no cases of aseptic loosening or amputation. Four patients required a change of bushings. The mean Musculoskeletal Tumour Society functional outcome score was 67%, the mean Harris Hip Score was 70, and the mean Oxford Knee Score was 34. Total femoral endoprosthetic replacement can provide good functional outcome without compromising patient survival, and in selected cases provides an effective alternative to amputation.

Non-invasive extendible endoprostheses for limb reconstruction in skeletally-mature patients.

We describe the application of a non-invasive extendible endoprosthetic replacement in skeletally-mature patients undergoing revision for failed joint replacement with resultant limb-length inequality after malignant or non-malignant disease. This prosthesis was developed for tumour surgery in skeletally-immature patients but has now been adapted for use in revision procedures to reconstruct the joint or facilitate an arthrodesis, replace bony defects and allow limb length to be restored gradually in the post-operative period. We record the short-term results in nine patients who have had this procedure after multiple previous reconstructive operations. In six, the initial reconstruction had been performed with either allograft or endoprosthetic replacement for neoplastic disease and in three for non-neoplastic disease. The essential components of the prosthesis are a magnetic disc, a gearbox and a drive screw which allows painless lengthening of the prosthesis using the principle of electromagnetic induction. The mean age of the patients was 37 years (18 to 68) with a mean follow-up of 34 months (12 to 62). They had previously undergone a mean of six (2 to 14) open procedures on the affected limb before revision with the non-invasive extendible endoprosthesis. The mean length gained was 56 mm (19 to 107) requiring a mean of nine (3 to 20) lengthening episodes performed in the outpatient department. There was one case of recurrent infection after revision of a previously infected implant and one fracture of the prosthesis after a fall. No amputations were performed. Planned exchange of the prosthesis was required in three patients after attainment of the maximum lengthening capacity of the implant. There was no failure of the lengthening mechanism. The Mean Musculoskeletal Tumour Society rating score was 22 of 30 available points (18 to 28). The use of a non-invasive extendible endoprosthesis in this manner provided patients with good functional results and restoration of leg-length equality, without the need for multiple open lengthening procedures.

The early results of joint-sparing proximal tibial replacement for primary bone tumours, using extracortical plate fixation.

This paper describes the preliminary results of a proximal tibial endoprosthesis which spares the knee joint and enables retention of the natural articulation by replacing part of the tibial metaphysis and diaphysis. In eight patients who had a primary malignant bone tumour of the proximal tibia, the distal stem, which had a hydroxyapatite-coated collar to improve fixation, was cemented into the medullary canal. The proximal end had hydroxyapatite-coated extracortical plates which were secured to the remaining proximal tibial metaphysis using cortical screws. The mean age of the patients at operation was 28.9 years (8 to 43) and the mean follow-up was for 35 months (4 to 48). The mean Musculoskeletal Tumour Society score was 79% (57% to 90%), the mean Oxford Knee score was 40 points of 48 (36 to 46) and the mean knee flexion was 112 degrees (100 degrees to 120 degrees). In one patient, revision to a below-knee amputation through the prosthesis was required because of recurrence of the tumour. Another patient sustained a periprosthetic fracture which healed with a painful malunion. This was revised to a further endoprosthesis which replaced the knee. In the remaining six patients the prosthesis allowed preservation of the knee joint with good function and no early evidence of loosening. Further follow-up is required to assess the longevity of these prostheses.

Endoprosthetic reconstruction of the distal tibia and ankle joint after resection of primary bone tumours.

Endoprosthetic replacement of the distal tibia and ankle joint for a primary bone tumour is a rarely attempted and technically challenging procedure. We report the outcome of six patients treated between 1981 and 2007. There were four males and two females, with a mean age of 43.5 years (15 to 75), and a mean follow-up of 9.6 years (1 to 27). No patient developed a local recurrence or metastasis. Two of the six went on to have a below-knee amputation for persistent infection after a mean 16 months (1 to 31). The four patients who retained their endoprosthesis had a mean musculoskeletal tumour society score of 70% and a mean Toronto extremity salvage score of 71%. All were pain free and able to perform most activities of daily living in comfort. A custom-made endoprosthetic replacement of the distal tibia and ankle joint is a viable treatment option for carefully selected patients with a primary bone tumour. Patients should, however, be informed of the risk of infection and the potential need for amputation if this cannot be controlled.

Outcome of intralesional curettage for low-grade chondrosarcoma of long bones.

BACKGROUND: Different treatment strategies for low-grade chondrosarcomas are reported in the literature with variable outcomes. The aim of this study was to assess the oncological and functional outcomes associated with intralesional curettage and cementation of the lesion as a treatment strategy. PATIENTS AND METHODS: We performed a retrospective review of 39 consecutive patients with intramedullary low-grade chondrosarcoma of long bones treated by intralesional curettage and cementation at our institution between 1999 and 2005. RESULTS: There were 10 males and 29 females with a mean age of 55.5 years (32-82), and a mean follow-up of 5.1 years (3-8.7). Local recurrence occurred in two patients (5%) within the first two years following index surgery. Both were treated by re-curettage and cementation of the resultant defects. A second local recurrence developed a year later in one of these two patients, for which a further curettage followed by local liquid nitrogen treatment was performed. Overall, there were no cases of post-operative complications or metastases. The patients were assessed using the Musculoskeletal Tumour Society scoring system (MSTS) to determine limb function. The average score achieved was 94% (79-100%). CONCLUSION: Intralesional curettage is an effective treatment strategy for low-grade intramedullary chondrosarcoma of long bones, with excellent oncological and functional results. Careful case selection with stringent clinical and radiographic follow-up is recommended.

Below-knee amputation through a joint-sparing proximal tibial replacement for recurrent tumour.

We report a case which highlights the progression of osteofibrous dysplasia to adamantinoma and questions whether intralesional curettage is the appropriate treatment. The role of a joint-sparing massive endoprosthesis using cortical fixation is demonstrated and we describe a unique biomedical design which resulted in the manufacture of an end cap to allow amputation through a custom-made proximal tibial replacement, rather than an above-knee amputation following recurrence.

Foramen magnum decompression and expansile duroplasty for acquired Chiari type I malformation in craniometaphyseal dysplasia.

Craniometaphyseal dysplasia (CMD) is a rare genetic bone remodelling disorder characterized by undertubulation of the long bones and sclerosis and hyperostosis of the skull base with cranial nerve impairment. To our knowledge there are no previous reported cases of autosomal dominant CMD and acquired Chiari type 1 malformation with cervicomedullary compression. We outline the clinical and radiological features of this condition and describe the surgical management.