The ankle is one of the most frequent joint affected by haemophilia, in the second decade of life it can be considered as the most common site for haemophilic arthropathy. The aim of this study is to evaluate the results of 15 surgical interventions of the hindfoot performed on 11 patients. Twenty-seven of 70 male patients suffering from haemophilia were treated for haemophilic arthropathy of the hindfoot. We performed 15 operations on 11 patients with a mean age of 33.5 (range: 10-53) years. The surgical treatment consisted of 15 operations, 12 of which concerned the talocrural joint (six synovectomies, three radiosynoviorthesis, four arthrodeses, one achilles tendon lengthening) and one the talocalcaneonavicular joint (one triplearthrodesis). The arthrodeses of the talocrural joint were undertaken by screw, the triplearthrodesis by clamp fixation. The synovectomies were all late synovectomies and performed by open means. The mean follow-up period was 32.0 (range: 12-84) months. The mean age at surgery was 29.6 (range: 8-51) years. No perioperative complications were registered when adequate replacement therapy was carried out. The consolidation of the arthrodeses was accomplished within 8-12 weeks. All synovectomies stopped or reduced significantly recurrent joint bleeding. With both procedures we achieved pain relief and walking ability improvement. The aim of synovectomy is to reduce bleeding episodes. If synovectomy can halt the progression of the haemophilic arthropathy is controversially discussed, especially when surgery was carried out in the presence of an established arthropathy. If synovectomy fails, the arthrodesis proves to be a good treatment option.

This study compares the predictive value of age at surgery in high tibial osteotomy. Twenty-seven high-tibial osteotomies in patients who are 65 years or older (mean age at surgery 68+/-4 years, follow up 12+/-2 years) were compared to 67 osteotomies in patients younger than 65 years (mean age at surgery 56+/-6 years, follow up 13+/-3) with respect to the outcome by Cox regression analysis. Failure, i.e. endpoint, was defined as implantation of a knee endoprosthesis and assessed by Kaplan-Meier analysis. There is a significantly higher risk for failure of high tibial osteotomies in patients of 65 years or more compared to younger patients (failure rate 38.4+/-11.3% vs. 23.1+/-5.8%) resulting in a relative risk of 1.5 (P=0.0461). The hazard of failure increased 7.6% per year of age. We conclude that in regard to the increasing risk of failure per year of age and the higher failure rate in older patients, high-tibial osteotomy should not be performed on patients older than 65 years.

Resection of a primary sarcoma of the diaphysis of a long bone creates a large defect. The biological options for reconstruction include the use of a vascularised and non-vascularised fibular autograft. The purpose of the present study was to compare these methods of reconstruction. Between 1985 and 2007, 53 patients (26 male and 27 female) underwent biological reconstruction of a diaphyseal defect after resection of a primary sarcoma. Their mean age was 20.7 years (3.6 to 62.4). Of these, 26 (49 %) had a vascularised and 27 (51 %) a non-vascularised fibular autograft. Either method could have been used for any patient in the study. The mean follow-up was 52 months (12 to 259). Oncological, surgical and functional outcome were evaluated. Kaplan-Meier analysis was performed for graft survival with major complication as the end point. At final follow-up, eight patients had died of disease. Primary union was achieved in 40 patients (75%); 22 (42%) with a vascularised fibular autograft and 18 (34%) a non-vascularised (p = 0.167). A total of 32 patients (60%) required revision surgery. Kaplan-Meier analysis revealed a mean survival without complication of 36 months (0.06 to 107.3, sd 9) for the vascularised group and 88 months (0.33 to 163.9, sd 16) for the non-vascularised group (p = 0.035). Both groups seem to be reliable biological methods of reconstructing a diaphyseal bone defect. Vascularised autografts require more revisions mainly due to problems with wound healing in distal sites of tumour, such as the foot.

Background and objectivesTreatment of pelvic tumors remains challenging due to complex anatomy, poor oncological outcome and high complication rates. We sought to investigate the long-term oncological and surgical outcome of these patients.MethodsBetween 1980 and 2012, 147 patients underwent surgical treatment for pelvic sarcoma. Histological diagnosis was Chondrosarcoma in 54, Ewing’s Sarcoma/PNET in 37, Osterosarcoma in 32 and others in 24 patients. Statistical analysis for the evaluation of oncological and surgical outcome was performed by applying Cox proportional hazards regression and Fine-Gray regression models for competing risk (CR) endpoints.ResultsThe estimated overall survival (OS) to death was 80%, 45% and 37% at 1, 5 and 10 years, respectively. Univariate analyses revealed a statistically significant unadjusted influence of age age (p = 0.038; HR = 1.01), margin (p = 0.043; HR = 0.51) and grade (p = 0.001; HR = 2.27) on OS. Considering the multivariable model, grade (p = 0.005; HR = 3.04) and tumor volume (p = 0.014; HR = 1.18) presented themselves as independent prognostic factors on OS. CR analysis showed a cumulative incidence for major complication of 31% at 5 years. Endoprosthetic reconstruction had a higher risk for experiencing a major complication (p<0.0001) and infection (p = 0.001).ConclusionsPelvic resections are still associated with a high incidence of complications. Patients with pelvic reconstruction and high volume tumors are especially at risk. Consequently, a cautious decision-making process is necessary when indicating pelvic reconstruction, although a restrictive approach to pelvic reconstruction is not necessarily reasonable when the other option is major amputation.

Background and ObjectivesThe proximal tibia (pT) is a common site for bone tumors. Improvements in imaging, chemotherapy and surgical technique made limb salvage surgery the treatment of choice. Yet, reconstructions of the pT have been associated with less favorable outcome compared to other parts of the extremities. The aim of this study was to evaluate the outcome of patients with a modular endoprosthetic reconstruction of the pT.MethodsEighty-one consecutive patients with an average age of 29 years underwent endoprosthetic reconstruction of the pT. Postoperative complications were categorized according to the ISOLS classification, and revision-free survival until first complication (any Type 1–5), soft tissue failure (Type 1), aseptic loosening (Type 2), structural failure (Type 3), infection (Type 4), and local tumor progression (Type 5) was estimated by using a Fine-Gray model for competing risk analyses for univariate and multivariable regression with Firth’s bias correction.ResultsA total of 45 patients (56%) had at least one complication. Cumulative incidence for complication Types 1 to 5 at 5 years with death and amputation as competing events revealed a risk of 41% for the first complication, 14% for Type 1, 16% for Type 2, 11% for Type 3, 17% for Type 4, and 1% for Type 5.ConclusionDespite inclusion of amputation and death as strong competing events, pT replacements are still associated with a high risk of postoperative failures. The results suggest that infection and soft tissue failures (Type 1 and 5) seem to depend from each other. Sufficient soft tissue reconstruction and closure allow better function and reduce the risk of infection as the most prominent complication. The use of a rotating hinge design has significantly reduced structural failures over time.

Data on prognostic biomarkers in soft tissue sarcomas are scarce. The aim of the study was to define prognostic markers in patients with a liposarcoma, a subtype of sarcoma derived from adipose tissue. We restrospectively reviewed 85 patients with liposarcoma treated at our department from May 1994 to October 2011. Kaplan-Meier curves, uni-, and multivariable Cox proportional hazard models and competing risk analysis were performed to evaluate the association between putative biomarkers with diseasespecific and overall survival. We observed a significant association between both alkalic phosphatase (ALP; subhazard ratio [SHR] per 1 unit increase: 1.35; 95%CI 1.10-1.65; p ¼ 0.005) and C-reactive protein (CRP; SHR per 1 mg/dl increase: 2,57; 95%CI 1.36-4,86; p ¼ 0.004) with disease-specific survival. Hemoglobin (Hb) (HR per 1 g/dl increase: 065; 95%CI 0.48-0.87; p ¼ 0.003) was associated with overall survival. These associations prevailed after multivariable adjustment for AJCC tumor stage. This study identifies CRP and ALP as novel independent predictors of disease-specific survival in patients with liposarcoma. ß

Purpose Haemophilic pseudotumour was defined by Fernandez de Valderrama and Matthews as a progressive cystic swelling involving muscle, produced by recurrent haemorrhage into muscles adjacent to the bone. The pseudotumour mainly occurs in the long bones and the pelvis. The treatment of the haemophilic pseudotumour poses a challenge, and extensive clinical experience is essential to appropriately address this serious complication in patients with haemophilia. Consequently, the aim of this study is to present our own clinical experience and treatment results of the haemophilic pseudotumour. Methods We retrospectively reviewed the records of 87 patients with bleeding disorders treated between 1967 and 2011 for musculoskeletal complications of congenital bleeding disorders. We identified six patients with a haemophilic pseudotumour who were treated at our department. Results The mean age at surgery was 45.9 (range, 40-61) years. The iliac bone was affected in three patients (one right, two left), the right tibia (distal diaphysis) in one, the right thigh in two and the right ulna (proximal part) in one patient. One patient had two pseudotumours. The perioperative course was easily controllable with adequate factor VIII substitution. At the latest follow-up after 8.4 (range, 4-24) years, normal healing with no recurrence was observed. Conclusions The haemophilic pseudotumour is a rare but severe complication of hereditary bleeding disorders. In the international literature the resection and postoperative course are described as challenging and difficult, requiring detailed preoperative planning. It is advisable to perform such operations in specialised centres with close cooperation between surgeons and haematologists.

Low serum albumin levels and impaired kidney function have been associated with decreased survival in patients with a variety of cancer types. In a retrospective cohort study, we analyzed 84 patients with liposarcoma treated at from May 1994 to October 2011. Uni-and multivariable Cox proportional hazard models and competing risk analyses were performed to evaluate the association between putative biomarkers with disease-specific and overall survival. The median age of the study population was 51.7 (range 19.6-83.8) years. In multivariable analysis adjusted for AJCC tumor stage, serum creatinine was highly associated with diseasespecific survival (Subdistribution Hazard ratio (SHR) per 1 mg/dl increase ¼ 2.94; 95%CI 1.39-6.23; p ¼ 0.005). High albumin was associated with improved overall and disease-specific survival (Hazard Ratio (HR) per 10 units increase ¼ 0.50; 95%CI 0.26-0.95; p ¼ 0.033 and SHR ¼ 0.64; 95%CI 0.42-1.00; p ¼ 0.049). The serum albumin-creatinine-ratio emerged to be associated with both overall and disease-specific survival after adjusting for AJCC tumor stage (HR ¼ 0.95; 95%CI 0.92-0.99; p ¼ 0.011 and SHR ¼ 0.96; 95%CI 0.93-0.99; p ¼ 0.08). Our study provides evidence for a tumor-stage-independent association between higher creatinine and lower albumin with worse disease-specific survival. Low albumin and a high albumin-creatinine-ratio independently predict poor overall survival. Our work identified novel prognostic biomarkers for prognosis of patients with liposarcoma. ß