Long-term outcome of total hip arthroplasty in patients with haemophilia.

INTRODUCTION: Besides the target joints (elbow, knee and ankle), the hip is one of the commonly affected joints in haemophilic arthropathy. Hip arthroplasty is the therapy of choice after failure of conservative treatment. There are only limited data on long-term results after primary total hip arthroplasty (THA). AIM: The aim of this retrospective study was to analyse clinical outcome and complication rate after total hip replacement in patients with severe haemophilic arthropathy. METHODS: Forty-three patients with haemophilia (PWH), one patient with von Willebrand disease and one patient with a Factor-VII-deficiency undergoing 49 total hip arthroplasties, were evaluated in a retrospective study. Harris hip score (HHS), range of motion (ROM), pain status (visual analogue scale, VAS) complication rate and patient satisfaction were assessed at a mean follow-up of 11.5 years (range: 3-32). RESULTS: HSS, ROM and VAS improved significantly combined with high patient satisfaction. In total, three (6.1%) periprosthetic infections and five (10.2%) aseptic implant loosenings occurred after THA leading to revision arthroplasty. In two (4.1%) cases, a pseudotumour and one (2.0%) periarticular ossification had to be resected after THA. CONCLUSION: Total hip replacement in PWH leads to a significant increase of function, reduction of pain and a high satisfaction. Due to the relatively high complication rate (infections and aseptic loosening) compared to patients without haemophilia, an individual assessment of the risk-benefit ratio from surgical and haemostaseological point of view is needed.

Lower limb reconstruction in tumor patients using modular silver-coated megaprostheses with regard to perimegaprosthetic joint infection: a case series, including 100 patients and review of the literature.

PURPOSE AND OBJECTIVE: Bone resection regarding adequate surgical margins is the treatment of choice for malignant bone tumors. In the case of metastasis-related complications, so-called skeletal-related events, it is highly important to achieve pain relief and a stable joint situation to re-mobilize the patients immediately following surgery. To bridge the often large osseous defect zones after tumor resection, both cemented and uncemented modular endoprosthetic systems are widely used. Patients undergoing tumor-related endoprosthetic orthopedic surgery are facing high risk for developing a periprosthetic joint infection (PJI). The immunocompromised condition due to anti-neoplastic treatment and long operation time with large exposure of tissue contributes to a high risk of infection. METHODS: The authors present a case series of 100 patients (31% primary bone tumor and 69% metastasis-related surgery) undergoing tumor-related lower limb salvage surgery with special regard to periprosthetic joint infection and the management of this "difficult to treat" situation. Furthermore, a review of the current literature regarding infection following bone tumor resection and endoprosthetic reconstruction is performed and discussed. RESULTS: The median follow-up was 24 months (range 12-108 months). Ten patients (10%) suffered from a periprosthetic joint infection. We recorded six acute infections (type I) <4 weeks after surgery, one infection >4 weeks after surgery (type II), and three late infections (type III). According to the definition of Laffer et al., three of our patients (30%) are probably free of infection, one patient died of PJI-associated sepsis, and five patients were free of infection, but without restoration of the affected joint. CONCLUSION: In conclusion, our own results show that perimegaprosthetic joint infection among silver-coated implants, in patients undergoing tumor-related surgery of the lower limb, is lower compared to non-silver-coated implants. Due to heterogeneity of patients and potential treatment options, the treatment regime should be tailored for the patients' individual situation.

Perioperative management and outcome of fracture treatment in patients with haemophilia without inhibitors.

INTRODUCTION: Fractures in persons with haemophilia (PWH) are not uncommon and require an interdisciplinary approach to maintain haemostasis during surgical treatment. AIM: The aim of this study was to evaluate the perioperative management and outcome in PWH following fracture fixation compared to a matched non-haemophilic control group. METHODS: A cohort of 44 PWH who underwent 46 surgical fracture fixations was retrospectively compared to 46 non-haemophilic patients (matched-pair controls). Patients were classified according to the fracture localization: (i) proximal upper extremity (PrUEx; n = 7), (ii) distal upper extremity (DiUEx; n = 12), (iii) proximal lower extremity (PrLEx; n = 13) and (iv) distal lower extremity (DiLEx; n = 14). Both groups were assessed for length of hospital stay, duration of surgery, drainage use and complication rates. RESULTS: There was no significant difference regarding the duration of the preoperative hospital stay between PWH and controls. Only PWH who were operated at the DiUEx stayed significantly longer in hospital (4.8 ± 3.7 days) than controls (2.2 ± 2.3 days; P = 0.039). Operation time was significantly longer in PWH with fractures treated at the DiLEx (64.9 ± 26.6 min) compared to the controls (49.8 ± 37.9 min; P = 0.035). Neither frequency nor duration of surgical drainage placement differed significantly between the two groups. The overall complication rate in both groups was low without a statistically significant difference. CONCLUSION: An optimal interdisciplinary perioperative management provided the surgical treatment of fractures in PWH can be performed safely with a low complication rate.

Outcome after total knee arthroplasty in haemophilic patients with stiff knees.

INTRODUCTION: Advanced haemophilic arthropathy of the knee is associated with progressive joint stiffness. Results after total knee arthroplasty (TKA) in stiff knees are considered to be inferior compared to those with less restricted preoperative range of motion (ROM). There is only very limited data on the results of primary TKA in haemophilic patients with stiff knees. AIM: The purpose of this retrospective study was to evaluate the clinical outcome after TKA performed in haemophilic patients with preoperative ROM of 50° or less. METHODS: Twenty one patients (23 knees) undergoing TKA with stiff knees were retrospectively evaluated. Mean follow-up was 8.3 years (range, 2-25). Clinical assessment included ROM, degree of flexion contracture and complication rate. Functional evaluation and pain status were assessed using the Knee Society's Scoring System (KSS). RESULTS: Range of motion improved from 26.7° preoperatively to 73.0° postoperatively. Flexion contracture decreased from 21.7° to 8.3°. KSS increased from 22.9 to 72.9 points. Evaluation of pain revealed a decrease from 8.4 points preoperatively to 2.1 points postoperatively. All these differences were statistically significant (P < 0.005). The complication rate was 8.7% including one late periprosthetic infection, and one aseptic implant loosening. Nine patients who required VY-quadricepsplasty for knee exposure developed a mean postoperative extensor lag of 7°. CONCLUSION: Total knee arthroplasty in haemophilic patients presenting with stiff knees results in significant improvement of function and reduction in pain. Although the clinical outcome is inferior compared to nonstiff knees reported in the literature, joint replacement surgery can be successfully performed in this particular group of patients.

Total ankle replacement in patients with haemophilia and virus infections--a safe alternative to ankle arthrodesis?

Despite reliable results of ankle fusion for advanced haemophilic arthropathy, total ankle replacement (TAR) may be functionally advantageous. There is only very limited literature data available on TAR in patients with haemophilia. The objective of this study is to evaluate the short- and mid-term results after TAR in patients with end-stage haemophilic ankle arthropathy and concomitant virus infections. In a retrospective study, results after eleven TAR in 10 patients with severe (n = 8) and moderate (n = 2) haemophilia (mean age: 49 ± 7 years, range, 37-59) were evaluated at a mean follow-up of 3.0 years (range, 1.2-5.4). Nine patients were positive for hepatitis C, five were HIV-positive. Range of motion (ROM), AOFAS-hindfoot-score, pain status (visual analogue scale, VAS) as well as patient satisfaction were evaluated. In two cases deep prosthesis infection occurred leading to the removal of the implant. In the remaining eight patients the mean AOFAS score improved significantly from 21.5 to 68.0 points (P < 0.0005), the VAS score decreased significantly from 7.6 to 1.9 points (P < 0.0005). ROM increased from 23.2 to 25.0 degrees (P = 0.51). At final follow-up all patients without any complications were satisfied with the postoperative results. Radiographic examination did not reveal any signs of prosthetic loosening. TAR is a viable surgical treatment option in patients with end-stage ankle osteoarthritis due to haemophilia. It provides significant pain relieve and high patient satisfaction. However, due to the increased risk of infection and lack of long-term results, TAR particularly in patients with severe haemophilia and virus infections should be indicated carefully.

[Periprosthetic fractures of the proximal femur]. / Periprothetische Frakturen des proximalen Femurs.

The incidence of periprosthetic fractures of the proximal femur is increasing due to the growing numbers of hip replacements in old age. The treatment of these fractures is a challenge for the treating physician and standardized procedures are necessary. The unified classification system (UCS) is a suitable tool to establish treatment strategies: On the basis of the fracture localization and extent, bone quality, stability of the prosthesis and the presence of further implants, the fractures can be classified according to the UCS and a treatment algorithm can be derived. This article gives an overview of the diagnostics, classification and characteristics of the various periprosthetic fracture types as well as the treatment.

[The Role of a Modular Universal Tumour and Revision System (MUTARS®) in Lower Limb Endoprosthetic Revision Surgery - Outcome Analysis of 25 Patients]. / Tumorprothesen in der endoprothetischen Revisionschirurgie der unteren Extremität ­ Ergebnisse von 25 Patienten nach Versorgung mit einem modularen Tumor- und Revisionssystem (MUTARS®).

Introduction/Background: With extended life expectancy, the number of primary joint arthroplasties has also increased. Revision surgery is also more often necessary, due to aseptic or septic loosening of the prosthesis or periprosthetic fracture. Large bone defects often occur in these patients and several non-modular, conventional implants are available to handle this difficult situation. Custom made implants offer an individually designed and defect-adapted shape with perfect covering of the lesion, but may delay the operation. The Modular Universal Tumor And Revision System (MUTARS®) offers the possibility to vary the length and angle of the femoral neck and the antetorsion angle. Thus, it permits intraoperative adaption to the individual patient's defect. The aim of our study is to present clinical, functional results and the rate of complications in a cohort of patients undergoing revision surgery for failed endoprosthetic replacement or failed trauma surgery, using the Modular Universal Tumor And Revision System with short-to midterm follow-up. Patients and Methods: Between August 2005 and September 2014, 25 patients (17 female, 8 male) with an average age of 72 years (min. 56, max. 92 years) were included in a retrospective study using the MUTARS® system. The patients underwent surgical revision of osseous defects that were not susceptible to conventional care. The mean follow-up was 16 months (min. 12, max. 72 months). The indications which led to revision surgery were as follows: loosened metal-on-metal prosthesis with an acetabular defect caused by a metal-induced granuloma (n = 1), pseudoarthrosis after implantation of femur nail (n = 2), cut-out of a femur nail (n = 1), aseptic loosening of an implanted endoprosthesis (n = 4), septic loosening of an implanted endoprosthesis (n = 1), periprosthetic fracture (n = 6) and periprosthetic infection after two stage revision surgery (infection consolidated Girdlestone situation) (n = 10). All patients were followed up at regular intervals, both clinically and radiologically. Apart from comorbidities, clinical and functional parameters were measured, including the Harris Hip Score. An outcome analysis was also performed with respect to peri- and postoperative complications. Serial plain X-rays were followed-up. Results: In all patients, stable reconstruction without the use of an allograft was possible. In total, 23 patients underwent surgery by using a proximal femur prosthesis; three patients also received a custom made acetabular component. One patient was treated by using a distal femur and one patient was treated by implantation of a total femur prosthesis. The average length of hospital stay was 25 days (min.11, max. 47 days). The Harris Hip Score improved from 28 points preoperatively to 81 points after surgery.In total we recorded 24 % of complications after surgery. Two patients suffered recurrent periprosthetic infection and the prosthesis had to be revised in a further two stage exchange; one patient had a tractus gap and was revised by surgery. Two patients suffered periprosthetic fracture due to trauma and the patient with the total femur prosthesis suffered recurrent periprosthetic infection of the silver - coated mega-prosthesis and committed suicide triggered by an episode of major depression. Conclusion: Because of its modular nature, the Modular Tumor And Revision System (MUTARS®) can be used for highly variable intraoperative defect adaption. Good clinical and functional results were achieved in patients with excessive bone defects. However, the high rate of infection, even with silver coated mega-implants, is still a problem and should be studied further.

[Elastic Stable Intramedullary Nailing (ESIN) of Metatarsal Fractures]. / Elastisch stabile intramedulläre Nagelung (ESIN) von Mittelfußfrakturen.

AIM: In the treatment of metatarsal fractures, the objective is early restoration of the physiological painless function of the foot. While undisplaced metatarsal fractures can be treated non-surgically, displaced fractures are a valid indication for reduction and internal fixation. Whereas plate fixation may lead to soft tissue irritation involving tendon adhesions and scar formation, retrograde percutaneous pinning may harm the intact metatarsophalangeal joint and lead to joint stiffness. We have therefore used the technique of elastic stable intramedullary nailing (ESIN) with titanium elastic nails (TEN) to achieve minimally invasive, antegrade splinting of short metatarsal shaft and neck fractures. METHOD: Within 7 years, ESIN was performed in 22 patients. The surgical technique is presented and the functional results and complications were retrospectively evaluated using the AOFAS Midfoot Score. RESULTS: Nineteen patients were analysed after an average follow-up of 25.6 ± 21.3 months (range: 3-72 months). The mean AOFAS score was 93.9 ± 10.4 (range 62-100) points. One case of skin irritation required TEN shortening. Pseudarthrosis, secondary fracture dislocation and nail breakage were not observed. After TEN removal 13.4 ± 12.9 (range: 5-52) weeks after implantation, no refracture occurred. CONCLUSION: Antegrade, minimally invasive, elastic stable intramedullary nailing of short metatarsal shaft and neck fractures using titanium nails (TEN) is a safe surgical procedure and achieves primary functional stability. It reliably leads to fracture healing and produces good functional results.

[Impact of Preoperative Knee Stiffness on the Postoperative Outcome after Total Knee Arthroplasty in Patients with Haemophilia]. / Einfluss der präoperativen Kniesteife auf das postoperative Ergebnis nach Knieendoprothetik bei Patienten mit Hämophilie.

INTRODUCTION: Total knee arthroplasty (TKA) is an effective treatment option for patients with end-stage haemophilic arthropathy of the knee. However, the procedure is technically challenging, as knee motion is often restricted before the operation and complication rates are then thought to be higher than for patients with a normal range of motion (ROM). There is very limited information on the outcome of TKA in haemophilic patients presenting with stiff knees. The objective of the present study was to retrospectively analyse and compare the clinical results after TKA in haemophiliacs with stiff and non-stiff knees. PATIENTS AND METHODS: The results of 50 TKA procedures in 41 haemophilic patients were retrospectively evaluated at a mean follow-up of 7.2 ± 4.9 years (range 2-25 years). 20 patients presenting with 23 stiff knees - defined by a preoperative ROM of 50° or less - were compared with 21 patients with 27 non-stiff knees. Knee motion (ROM, flexion, extension), Knee Society Score (KSS/KSS function), pain status (visual analogue scale, VAS), number of bleedings and patient satisfaction were evaluated. RESULTS: The complication rate was 12 %, including two haematomas, one aseptic loosening, and three periprosthetic infections. The overall mean ROM increased from 58.6 ± 34.2° (range 0-120°) preoperatively to 85.9 ± 23.4 (35-130°) postoperatively (p < 0.005). Mean KSS and KSS function improved from 30.6 ± 11.0 points (range 10-49) and 43.4 ± 9.3 points (range 15-65) to 79.3 ± 9.6 points (range 49-95) and 68.9 ± 11.0 points (45-90), respectively (p < 0.005). The mean VAS score decreased significantly from 7.9 ± 0.8 points (range 6-9) to 1.8 ± 1.1 points (range 0-4; p < 0.005). In comparison to the non-stiff group, patients with stiff knees showed a significantly greater mean improvement in ROM (46.3 ± 21.8° [range - 10-85°] vs. 9.4 ± 16.9° [range - 30-35°]), flexion (32.8 ± 19.6° [range - 10-85°] vs. 5.2 ± 16.2° [range - 40-35°]), and flexion contracture (13.5 ± 9.6° [range 5-30°] vs. 5.9 ± 6.7° [range 5-20°]). Both KSS and KSS function were significantly inferior in stiff knees than with non-stiff knees. Nine patients with knee stiffness who underwent additional v-y quadricepsplasty to lengthen the extensor mechanism developed a mean extensor lag of 7-0° ± 4-8° (range 5-15°). At final follow-up, 37/41 patients were satisfied or very satisfied with the surgical result. CONCLUSION: TKA in haemophilic patients presenting with haemophilic arthropathy of the knee results in significant improvements in function and reduced pain. Although the ultimate clinical outcome in stiff knees is inferior to that with non-stiff knees, joint replacement surgery can be successfully performed in patients with restricted preoperative range of motion. Vy-quadricepsplasty for to facilitate exposure is associated with the development of a postoperative extensor lag and should therefore be performed restrictively. Patient satisfaction after TKA was equally high in the two groups.

Elective total knee arthroplasty in haemophilic patients. Proposal for a clinical pathway.

UNLABELLED: Total knee arthroplasty (TKA) provides significant pain relief and better function in patients with end-stage haemophilic knee arthropathy. Peri- and postoperative care tends to be more complex than in non-haemophilic patients undergoing TKA and requires a multidisciplinary team approach. AIM: The implementation of standardized clinical pathways in non-haemophilic patients undergoing TKA has been shown to increase quality of care and to reduce postoperative complication rates. Consequently, the use of clinical pathways in haemophilic patients undergoing TKA may be beneficial to this particular subpopulation of patients. METHODS: A clinical pathway for TKA for haemophilic patients was designed in a consensus process involving all participating departments. RESULTS: We propose a specifically adjusted clinical pathway for TKA for haemophilic patients to show that standardization of elective orthopaedic surgery in haemophilia is feasible. CONCLUSION: The authors emphasize that there are limitations on categorizing haemophilic patients and stress that individual interdisciplinary treatment should take precedence over a standardized approach.

Treatment options for haemophilic arthropathy of the elbow after failed conservative therapy. A single centre experience.

UNLABELLED: After ankle and knee, the elbow is the most frequent joint affected by haemophilic arthropathy. The objective of this retrospective single centre study is to evaluate the results of treatment of elbow arthropathy after failed conservative therapy. PATIENTS, METHODS: In 21 consecutive patients, 11 radiosynoviortheses (RSO), four arthroscopic and six open synovectomies were performed, among them four with additional resection of the radial head. The mean duration of follow-up was 4.8 (RSO) and 5.3 years (surgery), respectively. Pain status (visual analogue scale, VAS), bleeding frequency, range of motion (ROM) as well as patient satisfaction were evaluated. RESULTS: Both, RSO and surgical synovectomy, achieved a significant reduction of pain and bleeding frequency (p < 0.05). Surgical synovectomies were associated with a marked yet not statistically significant increase of postoperative ROM. Radial head resection improved forearm rotation in all cases. No complications occurred. 20 out of 21 patients were satisfied or highly satisfied with the result of the treatment and would undergo the respective procedure again. CONCLUSION: Due to the effectiveness and safety RSO is considered to be the primary treatment option in haemophilic arthropathy of the elbow after failed conservative therapy. Arthroscopic synovectomy should be considered if RSO shows inadequate effect or in the presence of contraindications. Open synovectomy with resection of the radial head yields good results in the case of advanced arthropathy with radial head impingement.

Targeted gene correction: a new strategy for molecular medicine.

Advances, over the past 20 years, in the genetic manipulation of mammalian cells form the scientific basis of gene therapy. A number of strategies are presently being used to replace or augment a dysfunctional gene with a correct copy of itself. Now, a novel approach to correct the dysfunctional gene in the chromosome is being developed. Data obtained from biochemical, cell-based and animal studies suggest that the era of gene repair is dawning. It is now conceivable that inherited and non-inherited disorders might be treated with a small molecular tool designed to fix the mutation directly. Here, the conceptualization of the technique and its barriers to success are discussed.