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.

The credibility and creativity of an author may be gauged by the number of scientific papers he or she has published, as well as the frequency of citations of a particular paper reflecting the impact of the data on the area of practice. The object of this study was to identify and analyse the qualities of the top 100 cited papers in orthopaedic surgery. The database of the Science Citation Index of the Institute for Scientific Information (1945 to 2008) was used. A total of 1490 papers were cited more than 100 times, with the top 100 being subjected to further analysis. The majority originated in the United States, followed by the United Kingdom. The top 100 papers were published in seven specific orthopaedic journals.

Analysis of the most-cited orthopaedic papers allows us a unique insight into the qualitites, characteristics and clinical innovations required for a paper to attain ‘classic’ status.

We evaluated the long-term outcome of patients with an osteosarcoma who had undergone prior manipulative therapy, a popular treatment in Asia, and investigated its effects on several prognostic factors. Of the 134 patients in this study, 70 (52%) patients had manipulative therapy and 64 (48%) did not. The age, location, and size of tumour were not significantly different between the groups. The five-year overall survival rate was 58% and 92% in the groups with and without manipulative therapy (p = 0.004). Both the primary and overall rates of lung metastasis were significantly higher in the manipulative group (primary: 32% vs 3%, p = 0.003; overall lung metastasis rate: 51.4% vs 18.8%, p < 0.001). Patients who had manipulative therapy had higher local recurrence rates in comparison to patients who did not (29% vs 6%, p = 0.011). The prognosis for patients with osteosarcoma who had manipulative therapy was significantly poorer than those who had not. Manipulative therapy was an independent factor for survival.

This form of therapy may serve as a mechanism to accelerate the spread of tumour cells, and therefore must be avoided in order to improve the outcome for patients with an osteosarcoma.

Massive endoprostheses using a cemented intramedullary stem are widely used to allow early resumption of activity after surgery for tumours. The survival of the prosthesis varies with the anatomical site, the type of prosthesis and the mode of fixation. Revision surgery is required in many cases because of aseptic loosening. Insertion of a second cemented endoprosthesis may be difficult because of the poor quality of the remaining bone, and loosening recurs quickly.

We describe a series of 14 patients with triplate fixation in difficult revision or joint-sparing tumour surgery with a minimum follow-up of four years. The triplate design incorporated well within a remodelled cortex to achieve osseomechanical integration with all patients regaining their original level of function within five months.

Our preliminary results suggest that this technique may provide an easy, biomechanically friendly alternative to insertion of a further device with an intramedullary stem, which has a shorter lifespan in revision or joint-sparing tumour surgery. A short segment of bone remaining after resection of a tumour will not accept an intramedullary stem, but may be soundly fixed using this method.

Coccydynia is a painful disorder characterised by coccygeal pain which is typically exaggerated by pressure. It remains an unsolved mystery because of the perceived unpredictability of the origin of the pain, some psychological traits that may be associated with the disorder, the presence of diverse treatment options, and varied outcomes. A more detailed classification based on the aetiology and pathoanatomy of coccydynia helps to identify patients who may benefit from conservative and surgical management.

This review focuses on the pathoanatomy, aetiology, clinical features, radiology, treatment and outcome of coccydynia.

Haemophilia is an x-linked inherited bleeding disorder which can cause severe arthropathy. We have reviewed the results of 70 primary total knee replacements (TKR) performed in 57 haemophilic patients between 1983 and 2007. The functional results were assessed using the Hospital for Special Surgery (HSS) knee scoring system and Kaplan-Meier survivorship analysis. Six patients died. HSS scores were available for 60 TKRs at a mean follow-up of 9.2 years (2 to 23); 57 (95%) had good or excellent results. Deep infection was recorded in one patient. Kaplan-Meier analysis using infection and aseptic loosening as endpoints showed the survival rate at 20 years to be 94.0%.

A reduction in infection, spontaneous haemarthrosis and improvement in the quality of life were noted to justify surgery in our series of patients with a mean age of 43 (25 to 70). We have found that using the latest techniques of continuous infusion of clotting Factor have significantly helped to reduce the complication rates and have achieved results which match those of the non-haemophilic population undergoing TKR.

We describe the clinical outcome of a technique of surgical augmentation of chronic massive tears of the rotator cuff using a polyester ligament (Dacron) in 21 symptomatic patients (14 men, seven women) with a mean age of 66.5 years (55.0 to 85.0). All patients had MRI and arthroscopic evidence of chronic massive tears. The clinical outcome was assessed using the Constant and Murley and patient satisfaction scores at a mean follow-up of 36 months (30 to 46).

The polyester ligament (500 mm × 10 mm) was passed into the joint via the portal of Neviaser, medial to the tear through healthy cuff. The two ends of the ligament holding the cuff were passed through tunnels made in the proximal humerus at the footprint of the insertion of the cuff. The ligament was tied with a triple knot over the humeral cortex.

All the patients remained free from pain (p < 0.001) with improvement in function (p < 0.001) and range of movement (p < 0.001). The mean pre-operative and post-operative Constant scores were 46.7 (39.0 to 61.0) and 85.4 (52.0 to 96.0), respectively (p < 0.001). The mean patient satisfaction score was 90%. There were two failures, one due to a ruptured ligament after one year and the other due to deep-seated infection. The MR scan at the final follow-up confirmed intact and thickened bands in 15 of 17 patients.

This technique of augmentation gives consistent relief from pain with improved shoulder movement in patients with symptomatic massive tears of the rotator cuff.

The management of joint replacement in lysosomal storage diseases has not been well reported. We present three patients with progressive degenerative changes of the hips who required bilateral total hip replacement in early childhood. The stature of the patients make it essential to have access to appropriately scaled prostheses. Consideration has to be given to associated disorders of the skeleton which must be carefully screened to ensure safety in providing appropriate anaesthesia as well as ensuring that there is no cardiac abnormality. In one patient, a periprosthetic fracture was sustained in one hip in the early post-operative course requiring internal fixation.

The patient made a full recovery and all six hips were clinically and radiologically satisfactory at mid-term review.

Smoking is known to have an adverse effect on wound healing and musculoskeletal conditions. This case-controlled study looked at whether smoking has a deleterious effect in the outcome of autologous chondrocyte implantation for the treatment of full thickness chondral defects of the knee.

The mean Modified Cincinatti Knee score was statistically significantly lower in smokers (n = 48) than in non-smokers (n = 66) both before and after surgery (p < 0.05). Smokers experienced significantly less improvement in the knee score two years after surgery (p < 0.05). Graft failures were only seen in smokers (p = 0.016). There was a strong negative correlation between the number of cigarettes smoked and the outcome following surgery (Pearson’s correlation coefficient −0.65, p = 0.004).

These results suggest that patients who smoke have worse pre-operative function and obtain less benefit from this procedure than non-smokers. The counselling of patients undergoing autologous chondrocyte implantation should include smoking, not only as a general cardiopulmonary risk but also because poorer results can be expected in smokers following this procedure.

We describe a 63-year-old man who had xanthomatosis of the right tendo Achillis. He had undergone excision of the left tendo Achillis 17 years earlier without reconstruction for the same condition. The neurological history and examination were normal. Blood investigations showed hypercholestrolaemia, for which he was being treated with statins.

He was referred with pain in the right tendo Achillis and problems with footwear.

He was treated by excision of the right tendo Achillis, the xanthomatous nodules and the involved skin, followed by reconstruction with a cadaver bone-tendon graft. At follow-up eight months postoperatively, the scar had healed well. He walked without pain and could wear any type of shoe. Plain radiographs showed that the bone graft had healed. The American Orthopaedic Foot and Ankle Society hindfoot score was 95/100. The patient’s subjective evaluation of the result was very good.

Fractures of the proximal humerus can lead to malalignment of the humeral head, necrosis and post-traumatic osteoarthritis. In such cases surface replacement might be a promising option.

A total of 28 shoulders with glenohumeral arthritis subsequent to a fracture underwent surface replacement arthroplasty of the humeral head in patients with a mean age of 60 years (35 to 83). On the basis of the inclination of the impacted head, post-traumatic arthritis was divided into three types: type 1, an impacted fracture of the head in an anatomical position (seven cases); type 2, a valgus impacted fracture (13 cases); type 3, a varus impacted fracture (eight cases). The outcome was measured by means of the Constant score.

According to the Boileau classification of the sequelae of fractures of the proximal humerus, all 28 patients had a final result of intra-capsular category 1. The mean Constant score for the 28 shoulders increased from 23.2 points (2 to 45) pre-operatively to 55.1 points (20 to 89) at a mean of 31 months (24 to 66) post-operatively. Valgus impacted fractures had significantly better results (p < 0.039).

Surface replacement arthroplasty can provide good results for patients with post-traumatic osteoarthritis of the shoulder. Their use avoids post-operative complications of the humeral shaft, such as peri-prosthetic fractures. Further surgery can be undertaken more easily as the bone stock is preserved.

Idiopathic calcium pyrophosphate deposition disease (pseudogout) has a variable presentation. Many joints are usually affected; single joint disease is uncommon. We present a case report of primary monoarticular pseudogout affecting the hip. The diagnosis was made on the appearance and analysis of specimens obtained at arthroscopy. Monoarticular pseudogout is rare, but should be considered in the differential diagnosis of any presentation of joint pain.

We retrospectively reviewed 11 consecutive patients with an infected reverse shoulder prosthesis. Patients were assessed clinically and radiologically, and standard laboratory tests were carried out. Peroperative samples showed Propionbacterium acnes in seven, coagulase-negative Staphylococcus in five, methicillin-resistant staphylococcus aureus in one and Escherichia coli in one. Two multibacterial and nine monobacterial infections were seen. Post-operatively, patients were treated with intravenous cefazolin for at least three days and in all antibiotic therapy was given for at least three months. Severe pain (3 of 11) or severe limitation of function (3 of 11) are not necessarily seen. A fistula was present in eight, but function was not affected. All but one patient were considered free of infection after one-stage revision at a median follow-up of 24 months, and without antibiotic treatment for a minimum of six months. One patient had a persistent infection despite a second staged revision, but is now free of infection with a spacer. Complications included posterior dislocation in one, haematoma in one and a clavicular fracture in one. At the most recent follow-up the median post-operative Constant-Murley score was 55, 6% adjusted for age, gender and dominance.

A one-stage revision arthroplasty reduces the cost and duration of treatment. It is reliable in eradicating infection and good functional outcomes can be achieved.

A pronation deformity of the forearm following an obstetric brachial plexus injury causes functional and cosmetic disability. We evaluated the results of pronator teres transfer to correct their deformity in 14 children treated over a period of four years. The mean age at surgery was 7.6 years (5 to 15). The indication for surgery in each case was impairment of active supination in a forearm that could be passively supinated provided that there was no medial contracture of the shoulder and normal function of the hand. The median follow-up was 20.4 months (8 to 42). No patient was lost to follow-up. Qualitative results were also assessed. The median active supination improved from 5° (0° to 10°) to 75° (70° to 80°) with no loss of pronation.

A passively correctible pronation contracture can be corrected safely and effectively by the transfer of pronator teres.

We prospectively evaluated 61 patients treated arthroscopically for anterior instability of the shoulder at a mean follow-up of 44.5 months (24 to 100) using the Rowe scale. Those with post-operative dislocation or subluxation were considered to be failures. Logistic regression analysis was used to identify patients at increased risk of recurrence in order to develop a suitable selection system.

The mean Rowe score improved from 45 pre-operatively to 86 at follow-up (p < 0.001). At least one episode of post-operative instability occurred in 11 patients (18%), although their stability improved (p = 0.018), and only three required revision. Subjectively, eight patients were dissatisfied. Age younger than 28 years, ligamentous laxity, the presence of a fracture of the glenoid rim involving more than 15% of the articular surface, and post-operative participation in contact or overhead sports were associated with a higher risk of recurrence, and scored 1, 1, 5 and 1 point, respectively. Those patients with a total score of two or more points had a relative risk of recurrence of 43% and should be treated by open surgery.

In our database of 7935 patients referred for investigation of a soft-tissue mass, only 100 were found to have a soft-tissue metastasis (1.3%). Our aim was to define the clinical features of such patients and to identify the site of their primary tumour.

The most common presentation was a painful lump, deep to the fascia, ranging between 2 cm and 35 cm (mean 8.3 cm) with 78% of the lumps located deep to the fascia. The mean age of the patients at presentation was 64 years (22 to 84) and there were almost equal numbers of men and women. Of 53 patients with a history of malignancy, 52 had metastases from the same primary (lung in 12, melanoma in ten, kidney in nine, gastrointestinal track in four, breast in five, bladder in four, and others in eight). The other 47 had no history of malignancy and the metastasis was the first presentation. The primary sites in these cases were the lung in 19, gastro-intestinal track in four, kidney in two, melanoma in nine, other in three, and unknown (despite investigations) in ten. There was no correlation between the site of the metastases and the primary tumour.

Of the 7935 patients, 516 had a history of malignancy. Of these, only 10% had a soft-tissue metastasis, 29% had a benign diagnosis, 55% a soft-tissue sarcoma and 6% another malignancy. Patients with soft-tissue metastases have similar clinical features to those with soft-tissue sarcomas and should be considered for assessment at appropriate diagnostic centres for patients with suspicious soft-tissue lumps.

We evaluated 56 patients for neurological deficit after enucleation of a histopathologically confirmed schwannoma of the upper limb. Immediately after the operation, 41 patients (73.2%) had developed a new neurological deficit: ten of these had a major deficit such as severe motor or sensory loss, or intolerable neuropathic pain. The mean tumour size had been significantly larger in patients with a major neurological deficit than in those with a minor or no deficit. After a mean 25.4 months (12 to 85), 39 patients (70%) had no residual neurological deficit, and the other 17 (30%) had only hypoaesthesia, paraesthesiae or mild motor weakness.

This study suggests that a schwannoma in the upper limb can be removed with an acceptable risk of injury to the nerve, although a transient neurological deficit occurs regularly after the operation. Biopsy is not advised. Patients should be informed pre-operatively about the possibility of damage to the nerve: meticulous dissection is required to minimise this.

This paper outlines the recent development of an exchange Travelling Fellowship scheme between the British and American Orthopaedic Research Societies.

We prospectively studied 26 consecutive patients with clinically documented sensory or motor deficiency of a peripheral nerve due to trauma or entrapment using ultrasound, and in 19 cases surgical exploration of the nerves was undertaken. The ultrasonographic diagnoses were correlated with neurological examination and the surgical findings. Reliable visualisation of injured nerves on ultrasonography was achieved in all patients. Axonal swelling and hypoechogenity of the nerve was diagnosed in 15 cases, loss of continuity of a nerve bundle in 17, the formation of a neuroma of a stump in six, and partial laceration of a nerve with loss of the normal fascicular pattern in five. The ultrasonographic findings were confirmed at operation in those who had surgery.

Ultrasound may be used for the evaluation of peripheral nerve injuries in the upper limb. High-resolution ultrasound can show the exact location, extent and type of lesion, yielding important information that might not be obtainable by other diagnostic aids.