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Rehabilitation following Limb-Salvage Surgery in Sarcoma

November 15, 2016

Volume 2 | Issue 2 | May-Aug 2016 | Page 19-22 | Vincent S Paramanandam1, Anuradha A Daptardar1, Ashish Gulia2 Authors: Vincent S Paramanandam[1], Anuradha A Daptardar[1], Ashish Gulia[2] [1]Physiotherapy Department, Tata Memorial Hospital, Mumbai [2]Orthopedic Oncology Services, Department of Surgical Oncology, Tata Memorial Hospital, Mumbai. Address of Correspondence Dr. Paramanandam V Technichal Officer C, […]

Limb salvage with megaprosthesis in extremity osteosarcoma –a case-based approach

November 15, 2016

Volume 2 | Issue 2 | May-Aug 2016 | Page 13-18 | Gurpal Singh1, Mark Edward Puhaindran1 Authors: Gurpal Singh[1], Mark Edward Puhaindran[1] [1] Division of Musculoskeletal Oncology, University Orthopaedics, Hand and Reconstructive Microsurgery Cluster, National University Health System, Singapore. Address of Correspondence Dr. Gurpal Singh Division of Musculoskeletal Oncology, University Orthopaedics, Hand & Reconstructive […]

Rehabilitation following Limb-Salvage Surgery in Sarcoma

November 15, 2016

Limb salvage with megaprosthesis in extremity osteosarcoma –a case-based approach

November 15, 2016

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Rehabilitation following Limb-Salvage Surgery in Sarcoma

November 15, 2016

Volume 2 | Issue 2 | May-Aug 2016 | Page 19-22 | Vincent S Paramanandam1, Anuradha A Daptardar1, Ashish Gulia2

Authors: Vincent S Paramanandam[1], Anuradha A Daptardar[1], Ashish Gulia[2]

[1]Physiotherapy Department, Tata Memorial Hospital, Mumbai
[2]Orthopedic Oncology Services, Department of Surgical Oncology, Tata Memorial Hospital, Mumbai.

Address of Correspondence
Dr. Paramanandam V
Technichal Officer C, Physiotherapy Department, Tata Memorial Hospital, Mumbai
Email: vinsu24@gmail.com

Abstract

Introduction: Limb salvage after tumor resection has become a norm in today’s era. There are number of biological and non biological reconstruction options available for the reconstruction of these bone defects. The success story of these surgical procedures is mainly based on their excellent functional outcome. Post surgical rehabilitation plays an important role in achieving optimal functional outcome and good quality of life. The rehabilitation protocol following limb salvage surgery is complex and it differs with type of reconstruction procedure. Present articles discusses in detail the various rehabilitation protocols required to achieve above goals.

Keywords:

Introduction
Until 1970, amputation was the primary surgical treatment offered to bone and soft tissue sarcomas. However, from that time the treatment options have evolved dramatically and now approximately 90% of these cases undergo Limb Salvage Surgery (LSS)[1]. LSS has become the main line of treatment option for bone and soft tissue sarcomas along with adjuvant and/or neo adjuvant treatment modalities (Chemotherapy/ radiotherapy). The overall survival rate has been estimated as 55%-65%, based on the age of diagnosis, and it is considered to be comparable to that of amputation.
LSS is considered to be less invasive, provides better function and quality of life than amputation [2]. Moreover, it has been proposed that patients’ acceptability of LSS is high in view of the fact that it restores the body image better than amputation[3]. Nevertheless, LSS, unlike amputation, is associated with more peri-operative complications, prolonged hospital stay and requires repeated surgeries due to various reasons such as infection and prosthetic failure. LSS demands high surgical skills, whereas, amputation is a simple surgical procedure. Additionally, recent progress in prosthetic limbs, for example microprocessor based joints and endo-skeletal prosthetic reconstructions, have improved the functional outcome and cosmetic outlook following amputation [4].
A systematic review conducted by Bekkering et al [5] reported that the quality of life outcome from current available evidence is inconclusive in supporting LSS or amputation. Another recent systematic review and meta-analysis concluded that both surgical procedures provides similar functional recovery and quality of life [6].Despite the fact that early physical rehabilitation is the key to achieve good functional outcome and quality of life after LSS, rehabilitation techniques following LSS is largely neither tested nor documented in detail [7]. Lack of adequate early rehabilitation measures following LSS could be one of the rationales for conflicting interests reported by various studies examining the quality of life in LSS vs amputation.Hence, we have attempted to summarise basic principles and site specific considerations one must utilise to develop individual case specific rehabilitation protocol.

Common rehabilitation principles in LSS
In a recent paper, Shehadeh et al. [7] attempted to standardize the rehabilitation protocol for LSS following high grade bone and soft tissue sarcomas. They reported that following a standardized rehabilitation protocol produced improved functional outcome in group of 59 patients with LSS. Their conclusion, however, is based on small observational study with heterogeneous population who received different type of LSS for different anatomical sites. Following set protocol in LSS, unlike general orthopaedic procedure, will be counterproductive. In general orthopaedic procedures, more or less,
specific anatomical structures are involved with minimal damage to the bone, joint and
soft tissue structures. In contrast, in LSS
following sarcomas, these structures are
extensively resected and may not be
identical between two individuals
undergoing similar procedures for a particular site. For example, resection length for distal end of femur osteogenic sarcoma may depend on the extent of disease in two individuals [4].
Following are common rehabilitation prospectives that need to be considered to formulate comprehensive rehabilitation protocol for LSS.
Bone and joint reconstructions: Stability and mobility following LSS largely depends on the bone and joint structure loss and the type of reconstruction. For example, megaprosthetic distal femur replacement with cementing will allow the patient to be ambulated full weight bearing (FWB), whereas, if it is a bone graft , like in most of biological reconstructions, weight bearing needs to be delayed till the osteosynthesis is confirmed by radiographical evaluation.
Neuromuscular loss: Oncology resection demands large resections, which will also include a part of uninvolved soft tissue cover as surgical margin. Large resection may require additional rotational or free flaps for soft tissue coverage. In addition, nearby neuro-vascular bundle may need to be excised or repaired, hence, complete evaluation of neuro-motor loss would be necessary to plan the dynamic strength training and external support requirements.
Skin involvement: Donor sites of free flaps often receive split thickness skin graft which may hinder the early mobilisation of nearby joint. Moreover, scar development following open biopsy and LSS may need special attention from the rehabilitation team to prevent any future functional loss.
External supports: Temporary or permanent external support in the form of static or dynamic splinting may be required to provide support to the limb. To exemplify, prophylactic use of abduction brace along with derotation boot to prevent hip dislocation following proximal femur replacement and dynamic cock-up splint for radial nerve palsy needs to be the integral part of rehabilitation service.
Oncology treatments: Deranged blood count often hinders with the intensity of rehabilitation; hence, it prolongs the overall rehabilitation(8). Radiation induced fibrosis could cause severe restrictions in the joint range of motion. Thus, rehabilitation professionals must plan around the chemotherapy cycles and add prophylactic measures to prevent any impending radiation induced joint and soft tissue dysfunction.
Multidisciplinary approach: Limb salvage surgery is complex and demands close concordance in treatment specific outcomes between various health professional working in the rehabilitation team. This team may comprise team of surgeons, medical oncologists, radiation therapists, nurses, physiotherapists, occupational therapists, prosthetics orthotics and medical social workers. Prehabilitation, rehabilitation even before starting the primary cancer therapy and surgery, such as crutch muscles strengthening, would be greatly beneficial in post-treatment functional outcome. Although in the field of LSS evidence of prehabilitation is lacking, there are considerable evidence to show beneficial effects in overall rehabilitation following cancer therapies [9–11].
Rehabilitation prescriptions and follow-up: Rehabilitation protocol for LSS must be tailor made considering the general principles and site specific modification, hence specific and also progressive. However, some negative effects of adjuvant therapies, such as the deranged blood counts and infection, may alter the course of rehabilitation process. Thus, frequent follow-up and close monitoring may be required during adjuvant therapy till they are functionally independent.
Rehabilitation consideration for specific sites
Site specific rehabilitation principles following LSS have been presented below for few common sites.
Mega prosthetic replacement for distal femoral resection:
Distal femur is the commonest site for primary high grade sarcoma and giant cell tumors. Overall strengthening other than the affected site, in all possibility, should begin preoperatively. Limb elevation and ankle toe movement should be encouraged from post operative day one to prevent deep vein thrombosis. Cemented and semiconstrained(allows rotations and flexion/extension) knee joint endoprosthetic replacement permits early joint mobilization and FWB walking. Unlike other centres [4,7], in our centre knee joint mobilisation starts from day one with the help of continuous passive motion units (Figure1) and active assisted methods within the pain tolerance level unless tight suturing. Close communication between the surgical team and the rehabilitation team helps in personalising the rehabilitation protocol as per the patients’ requirements. With adequate pain relief through appropriate medical management, active exercises could be started from day one to three. Full weight bearing walking could be started from day one initially with walker and later without any support if patient could effectively extend the knee “locking the knee”. Prior to ambulation, one leg standing and spot marching must be encouraged with appropriate support. After acute inflammation subsides slow progressive muscle strengthening exercises must be encouraged with the goal of achieving 900knee flexion, complete knee extension and muscle strength equivalent to the contra lateral lower limb by end of three months. Active passive motion devices, such as the one shown in Figure 2, may help in joint mobilisation and strengthening. Summary of rehabilitation protocol is tabulated in Table 1.

Figure 1: Continuous passive motion

Figure 2: Active passive motion unit

Table 1: Rehabilitation protocol following LSS

Mega prosthetic replacement for proximal tibial resection:
Proximal tibia and knee joint is the second most common site for primary high grade sarcoma and giant cell tumors. It is indeed a challenging location for rehabilitation in view of the fact that the extensor mechanism have to be reconstructed in these cases [4]. In most of the cases a gastrocnemius flap is done to provide a dynamic anchorage and direct anchorage to prosthesis provide a static attachment to help reattaching extensor mechanism to proximal tibial prosthesis. Protecting the extensor mechanism reconstruction till it attaches through fibrosis along with the surrounding soft tissue structures are crucial to prevent quadriceps lag. Hence, knee bending and quadriceps strengthening will be delayed for six weeks until then the knee is protected with the help of long knee brace. Re-attached gastrocnemius flap could lead to protective muscle spasm of plantar flexors and if not mobilised early it may create plantar flexors contracture. Thus, achieving/maintaining dorsi flexion of the ankle joint in the early post operative period is crucial for appropriate weight bearing.

Table 2: Rehabilitation protocol following Mega prosthetic replacement for proximal tibial resection

Mobilisation of knee joint and quadriceps strengthening could be started after six weeks; however, therapist must consider that immobilisation of the knee joint in long knee brace leads to severe restriction of patella mobility. Unless adequate patella mobility is achieved, especially the medial lateral movement, knee flexion exercises could prevent smooth gliding of patella over the femoral condyle. This will increase strain on the reconstructed patella ligament. Therefore, our institute follows a unique mobilisation protocol following proximal tibia and knee replacement which is depicted in the Table 2. In few cases quadriceps lag could be evident due to patella tendon overstretch/avulsion, this could be due to improper patella mobilisation or forceful knee bending. Figure 3 a, b and c depicts the patella tendon overstretch.

Figure 3: a) Patella position following proximal tiabia and knee joint replacement b) Patellar tendon stretch after 3months c) Patella mal position (over ride) on patient.

Mega prosthetic replacement for proximal and total femoral resection:
Resection of proximal femur and prosthetic replacement may be done for proximal femur tumor or as a part of total femoral resection and reconstruction. Partial or complete loss of joint capsule and dynamic stabilisers of hip joint during tumor resection may leave the hip joint vulnerable to dislocation. This may get potentiated with certain combination movements, if these joint movements are allowed beyond a certain limit. This restriction largely depends upon the surgical approach. Postero-lateral approach being most common in the LSS of this site, hip rotations, especially internal rotation and, flexion more than 600 and adduction of the hip joint needs to be prevented up to 6 weeks [4,7]. These movement restrictions could be achieved using hip abduction pillow/brace and de-rotation splint. Before the patient gets discharged from the hospital, training them for bed transfer, supine to standing, standing to supine and sitting in a chair/commode becomes paramount important in the early phase of
rehabilitation. Knee joint mobilisation must be started early either by the edge of bed with hip joint well supported or in side lying with pillows between legs. Any restriction of knee joint range would adversely affect the overall function since hip joint function of the ipsilateral leg has already been compromised. Early FWB ambulation could be started from post operative day one initially with walker, then with walking stick. Later most of them would be trained to walk without any walking aid. Total femoral resections may require more intense rehabilitation with additional emphasis on knee strengthening as discussed earlier (Table 2). Patients may life long need to use walking aids in view of the fact that large motor loss in these cases.
Mega prosthetic replacement for proximal humeral resections:
Proximal humerus and the shoulder girdle are the third common place for primary bone sarcomas [4]. Despite endoprosthetic replacements for functional shoulder girdle structures, such as reverse glenoid prosthesis, are available, lack of muscular structures post excision and damage to the axillary nerve often prevent their use. Frequently, the proximal end of the humerus is replaced with the endoprosthesis and suspended by the remaining muscles and soft tissue structures by suturing around the proximal end of the prosthesis. The objective of the procedure is to achieve a stable shoulder to facilitate good elbow and hand function.
To prevent the weight of the endoprosthesis and the limb acting on the newly formed pseudo joint, shoulder sling and elbow pouch are provided for 4 – 6 weeks. Early post operative rehabilitation consists of elbow and hand range of motion (ROM) and strengthening exercises within pain limit. Again these exercises must be performed in supine position only to avoid undue stress on the shoulder. After six weeks, shoulder joint limited ROM exercise in the form of pendular movements and vigorous strengthening of shoulder girdle, elbow and hand complex should be commenced. Additionally, postural correction must be included in the rehabilitation program.

Biological reconstructions
Wherever feasible, biological reconstructions are preferred over endoprosthetic implants in order to provide a stable and permanent solution for reconstruction of defects after tumor resection. However, rehabilitation following biological reconstructions needs careful considerations regarding weight bearing and joint mobilization. Utmost importance to surgical notes and communication with operative surgeon is of prime importance. Early joint mobilisation is the key to prevent joint stiffness and functional loss; nevertheless, often protective functional braces may be required to prevent damage. For example, curettage and bone grafting of the lower end of femur close to the joint demands hinge knee brace to avoid varus and valgus stress. Strengthening exercises also could be started early with functional knee brace (Fig. 4).

Figure 4: Hinge knee brace following bone graft.

Patients are taught to walk non-weight bearing with brace generally from post operative day one with the help of axillary crutches up to 8 weeks. Once, osteosynthesis is confirmed through radiological evaluation, progressive weight bearing walking could be started. FWB walking and complete joint range and strength are expected to be achieved by the end of 3 months to 4 months.

conclusion

Although limb salvage surgery for primary malignant tumours have achieved commendable advancement in surgical techniques and endo-prosthetic design and manufacturing, without optimal and timely peri and post-operative physical rehabilitation, achieving the desired quality of life outcome may not be feasible. This paper has highlighted few important rehabilitation principles and we have summarised rehabilitation protocol for specific area. Most of the oncology resection and reconstruction vary from one individual to another even in one particular site and needs tailor made rehabilitation protocol; nevertheless, this summary will be a guide for necessary foundation to design individual rehabilitation program.

References

1. Chopra BK. Health related quality of life studies in Indian patients after limb salvage surgery: Need of the hour. Med J Armed Forces India. 2013 Jul;69(3):209–10.
2. Ottaviani G, Robert RS, Huh WW, Palla S, Jaffe N. Sociooccupational and physical outcomes more than 20 years after the diagnosis of osteosarcoma in children and adolescents. Cancer. 2013;119(20):3727–36.
3. Frieden RA, Ryniker D, Kenan S, Lewis MM. Assessment of patient function after limb-sparing surgery. Arch Phys Med Rehabil. 1993 Jan;74(1):38–43 6p.
4. Oren R, Zagury A, Katzir O, Kollender Y, Meller I. Principles of rehabilitation after limb-sparing surgery for cancer. In: Musculoskeletal Cancer Surgery [Internet]. Springer; 2001 [cited 2014 Sep 3]. p. 583–93. Available from: http://link.springer.com/chapter/10.1007/0-306-48407-2_36
5. Bekkering WP, Vliet Vlieland TPM, Fiocco M, Koopman HM, Schoones JW, Nelissen RGHH, et al. Quality of life, functional ability and physical activity after different surgical interventions for bone cancer of the leg: A systematic review. SurgOncol. 2012 Jun;21(2):e39–47.
6. Mei J, Zhu X-Z, Wang Z-Y, Cai X-S. Functional outcomes and quality of life in patients with osteosarcoma treated with amputation versus limb-salvage surgery: a systematic review and meta-analysis. Arch Orthop Trauma Surg. 2014 Nov;134(11):1507–16.
7. Shehadeh A, Dahleh ME, Salem A, Sarhan Y, Sultan I, Henshaw RM, et al. Standardization of rehabilitation after limb salvage surgery for sarcomas improves patients’ outcome. HematolOncol Stem Cell Ther. 2013 Sep;6(3–4):105–11.
8. Schmitz KH, Courneya KS, Matthews C, Demark-Wahnefried W, Galvão DA, Pinto BM, et al. American College of Sports Medicine roundtable on exercise guidelines for cancer survivors. Med Sci Sports Exerc. 2010 Jul;42(7):1409–26.
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11. Silver JK. Cancer prehabilitation and its role in improving health outcomes and reducing health care costs. SeminOncolNurs. 2015 Feb;31(1):13–30.

How to Cite this article:

Dr. Paramanandam V

Dr. Anuradha A Daptardar

Dr. Ashish Gulia

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Limb salvage with megaprosthesis in extremity osteosarcoma –a case-based approach

November 15, 2016

Volume 2 | Issue 2 | May-Aug 2016 | Page 13-18 | Gurpal Singh1, Mark Edward Puhaindran1

Authors: Gurpal Singh[1], Mark Edward Puhaindran[1]

[1] Division of Musculoskeletal Oncology, University Orthopaedics, Hand and Reconstructive Microsurgery Cluster, National University Health System, Singapore.

Address of Correspondence
Dr. Gurpal Singh
Division of Musculoskeletal Oncology, University Orthopaedics, Hand & Reconstructive Microsurgery Cluster, National University Health System, 1E Kent Ridge Road, Singapore 119228.
Email: gurpal_singh@nuhs.edu.sg

Abstract

Introduction: Musculoskeletal oncology is an evolving field in orthopaedic surgery and surgical management for patients with osteosarcoma has changed fundamentally over the past three decades. Osteosarcoma is a rare tumour, but it is the most common type of primary bone cancer with a biomodal peak. Primary cases tend to occur within the first two decades of life and secondary osteosarcomas affect older patients. Common causes of secondary osteosarcoma include Paget’s disease of bone and radiation exposure. Due to advances in chemotherapeutic regimes, imaging modalities, surgical techniques, material and prosthesis designs, amputation is no longer considered as treatment of choice for most patients. Limb salvage surgery including endoprosthetic reconstructions is also constantly evolving, addressing surgical challenges such as margin control, reconstructive procedures and soft tissue management.
In this review, we focus our discussion on the management of patients with osteosarcoma undergoing megaprosthetic reconstruction. Based on three real cases (osteosarcoma of the distal femur, pelvic osteosarcoma involving the hip joint and osteosarcoma of the proximal tibia), this paper aims to highlight surgical challenges that sometimes need to be overcome in this very challenging field.

Keywords: Limb salvage surgery; megaprosthetic reconstruction; osteosarcoma; musculoskeletal oncology.

Introduction
Musculoskeletal oncology is a rapidly evolving field in orthopaedic surgery and surgical management for patients with osteosarcoma has progressed significantly over the past three decades. Due to advances in neoadjuvant treatment, imaging modalities, surgical techniques and material and prosthesis designs, amputation is no longer considered as standard of care in most cases. Literature is currently reporting limb salvage surgeries in 85% to 95% without compromising oncological principles when compared to amputation [1–5]. Main objectives of limb salvage surgery include maximized functional outcome, satisfactory wound coverage for adjuvant therapy and optimized aesthetic outcome without compromising oncologic principles [6]. The reconstructive options for limb salvage surgery can be thought of as biological (autograft, allograft etc.) or endoprosthetic. Sometimes, a combination of both may be necessary.
When considering treatment options for patients with osteosarcoma, several factors need to be considered, including the response to neoadjuvant chemotherapy, evidence of periprosthetic fracture, anatomic site, involvement of soft tissue, joint and neurovascular structures as well as the evidence of metastases at diagnosis. Adequate margin control during surgery is crucial. Surgery represents only one component of the multidisciplinary treatment protocol of osteosarcoma patients. Since the 1970s, the role of neoadjuvant therapy has evolved and intense multi-agent chemotherapy has improved the prognosis significantly by eradicating accompanying micrometastases and also reducing the reactive zone around the tumour. The main drawback of chemotherapy prior to surgery includes immunosuppression. Therefore, a multidisciplinary approach between medical oncologists and the surgical team is essential in order to optimise treatment for the patient. After completion of neoadjuvant chemotherapy, re-staging is performed to assess response to chemotherapy and verify resectability and evaluate margins prior to proceeding with definite surgery. Limb salvage surgery for osteosarcoma is typically described as consisting of three parts, starting with en bloc resection of the tumour. Based on the exact location, intra- or extra-articular resection may be considered. In skeletally immature patients, the possibility of physis preservation should be considered. Thereafter, the bone defect is reconstructed. The third part of the surgery consists of soft tissue coverage and functional re-establishment. Treatment protocols for osteosarcoma further include the administration of adjuvant chemotherapy and subsequent long-term surveillance.
After limb salvage surgery, overall survival rates of 60% to 70% at 5 years have been reported in literature [1–4, 9, 15]. A recent systematic review and meta-analysis by Stokke et al. evaluating the quality of life among paediatric, adolescent and young adult bone tumour survivors suggested that quality of time improves over time. According to their results, female patients and patients at older age are more likely to have a poor quality of life. Interestingly, there was no difference in outcomes between patients who underwent limb salvage surgery versus amputation for local control [16].
Failure rates in patients undergoing reconstruction with megaprostheses are generally higher compared to patients treated with conventional arthroplasties [7]. However, a direct comparison of study findings is difficult due to different settings and definitions used. Failure rates between 40% to 73% at 5 to 15 years have been reported from factors due to the underlying disease contributing to unfavourable early and late surgical outcome, complications can be specifically related to endoprosthetic reconstruction including aseptic loosening, periprosthetic fracture, infection, implant failure, dissociation of modular components and wear [1, 2, 5, 8, 9]. Implant survival rates for fixed-hinge prostheses have been reported to be approximately 70%, whereas rotating hinge prostheses survival between almost 80 to 100% have been reported [1, 7, 10–13]. A classification system proposed by Henderson et al. for megaprosthesis failure indicates 5 causes of failure, including soft tissue failure (Type 1), aseptic loosening (Type 2), structural fracture (Type 3), infection (Type 4) and local tumour recurrence (Type 5) [14].

Three case examples of patients with osteosarcoma
Case 1 – Osteosarcoma of the distal femur
A 16 year old male patient presented with right knee pain at the lateral aspect of the knee and night pain for about one month. There was no known trauma to the right knee. Clinical findings revealed tenderness over the medial and lateral aspect of the knee and a decreased range of motion 10-110°. Plain radiograph, magnetic resonance imaging, computed tomography and bone scan for local and distal staging were performed, showing an aggressive right distal femoral tumour, with extraosseous soft tissue extension abutting the quadriceps and gastrocnemius muscles, iliotibial band, and medial and lateral gutters of the knee joint (Figure 1a). No fracture or neurovascular involvement was detected. There was no evidence of distant osseous or pulmonary metastases. An open biopsy confirmed the diagnosis of a high-grade conventional osteosarcoma.

Figure 1A: Anteroposterior radiograph of the right knee demonstrating a gross cortical breach at the lateral aspect of the distal metaphysis.

After completion of neoadjuvant chemotherapy, the patient developed an extensive local fungal infection on the lateral aspect of the thigh, surrounding the biopsy incision site. Systemic antifungal therapy was given for two weeks, directed by a dermatologist and surgery was delayed by two weeks. Given the small window of opportunity between neoadjuvant and adjuvant chemotherapy and to minimise further delay in treatment, the surgical team in the outlined case decided to proceed with tumour resection. In view of the recent tinea corporis with lesions on the lateral aspect of the right thigh, the decision was made to proceed with an anteromedial approach with the excision of the biopsy tract laterally. Local tumour recurrence as a result of spread of malignant cells during biopsy has been reported [17, 18]. Consequently, the biopsy tract is considered as contaminated and conventionally excised during tumour removal. However, more recent studies have shown preliminary evidence for the safety of limb salvage surgery without biopsy tract excision following a diagnostic core needle biopsy and fine-needle aspiration, respectively [19, 20].
In the outlined case, a medial dissection via interval between vastus medialis and rectus femoris was performed for en bloc resection of the tumour. Femoral vessels were identified and traced into the popliteal fossa via adductor canal. The femur osteotomy was made at pre-templated 150 mm from the joint line. The sciatic and peroneal nerve were identified and preserved. Histopathological analysis of frozen sections and tumour specimen showed negative margins. After change of instruments, distal femur reconstruction was continued. Femur and tibia were prepared and a rotating-hinge Global Modular Reconstructive System (GMRS) (Stryker Inc, Rutherford, NJ, USA) knee prosthesis was used for reconstruction (Figure 1b). The vastus medialis was mobilised and brought down to cover the defect medially. The extensor mechanism was reconstructed over the prosthesis. No post-operative complications occurred, all wounds healed satisfactorily. Six months after surgery, the patient is walking without any support.

Figure 1B: Anteroposterior radiograph showing the defect reconstructed with a rotating-hinge Global Modular Reconstructive System (GMRS) prosthesis.

This case highlights the necessity of non-conventional surgical approaches when necessary. Although the main tumour burden was on the lateral aspect of the knee joint, given the location of the previous treated fungal infection, the decision for a medial approach was made. Extra- versus intraarticular resection needs to be discussed in the appropriate context. Preliminary evidence is hinting towards an overestimation of neoplasm seeding in the biopsy tract, especially when the biopsy is performed by the operating surgeon himself. The point to emphasize in this case is the close communication between medical oncologist and the surgeon and balancing the risks of delaying adjuvant chemotherapy versus an “unconventional” surgical approach. This patient underwent an intraarticular resection of the distal femur. In cases where the knee joint is found to be contaminated or being directly invaded by the tumour, an extraarticular resection of the entire joint with en-bloc removal is recommended. Usually, this approach is associated with poorer functional results.

Case 2 – Pelvic osteosarcoma involving the hip joint
A 24 year old female patient presented with a left neck of femur fracture with underlying osteosarcoma. Prognostic and treatment implications of pathological fractures at presentation have been widely debated. In the past, pathological fractures have been considered as indication for amputation due to the risks of local recurrence from contamination caused by the fracture hematoma. However, a recent systematic review and meta-analysis by Salunke et al. is demonstrating similar rates of local recurrence in patients with limb salvage surgeries compared to amputation, provided carefully selection of patients has been performed [21]. Our own 18-year experience of high-grade osteosarcoma with a pathological fracture at initial presentation showed no differences in the survival and recurrence rates of patients with pathological fractures compared to those with no fractures. Moreover, we did not see any difference in survival between amputated and salvaged patients with fractures, provided margin control attempts are aggressive.

Figure 2A: Anteroposterior radiograph of the pelvis 6 years after pelvic osteosarcoma removal and reconstruction of the proximal femur.

In the outlined case, the patient was treated according to protocol with neoadjuvant chemotherapy. Given the complexity of the case, but still aiming for limb salvage, the decision was made for using endoprosthetic and biologic reconstructive options, namely the combination of a hemipelvectomy with an extra-articular proximal femur resection followed by proximal femur replacement and acetabular column plating/deep frozen bone (Figure 2a). Biologic reconstruction options such as allografts, autografts and re-implantation of sterilized tumour bone replace the defect with a biologic construct, providing the theoretical advantage of incorporation of the biological graft. However, retrieval studies have shown that grafts become necrotic and may act as a spacer rather than a stable replacement. Typical failures include graft-fracture, non-union between the graft and host-bone and increased infection-rates. Drawbacks also include the dependence of graft incorporation for rehabilitation. A reliable tissue bank and associated logistics are sine qua non, and harvesting the autograft or preparing the allograft and subsequently increasing surgical time need to be taken into account. Since a long-term study showed that less than 50% of allografts lasted less than 10 years [22], this technique is mostly preserved for special occasions only, such as osteosarcomas in skeletally immature patients without involving the growth plates. Combined usage of endoprosthetic and biologic reconstruction is described by the allograft prosthetic composite. The concept behind includes restoration of the bone stock by allograft and using endoprosthetic components to restore articulating surfaces, theoretically offering a higher durability in the long-term when compared to pure biologic reconstruction. In the outlined case, the patient’s own pre-treated tumour bone was used for part of the recontruction. Sterilization of the bone can be performed in various ways including pasteurizing, autoclaving, radiotherapy, and freezing. Similarly to the use of allografts, non-union, infection and fracture belong to the main complications in biologic reconstruction with autografts. As described in the outlined case, liquid nitrogen was used for pre-treatment of the bone. Cryosurgery in combination with autograft-prosthesis composites shows promising results with excellent functional outcomes, low complication rates and improved union rates, especially when used with the pedicle-freezing technique [23–25].
After completion of adjuvant chemotherapy and being in remission, the patient in the outlined case developed a hematogenous methicillin susceptible staphylocococcus aureus (MSSA) infection of the endoprosthesis six years postoperatively. Infection is still the most common complication in megaprosthetic reconstruction and in general, it is more likely to happen when adequate wound coverage is missing. In primary megaprosthesis infection rates between 2% to 20% have been reported, with the numbers increasing to more than 40% in revision cases [1, 2, 5, 10, 14, 26–31]. The classic approach in periprosthetic infection is a two-stage exchange revision surgery, including the removal of original endoprosthetic components, thorough debridement, administration of intravenous antibiotics, and implantation of a spacer followed by the re-implantation of implants.
In the outlined case, this approach was not practical. A consensus with the multidisciplinary musculoskeletal oncology team and the patient was made for management with ultrasound-guided drainage of the left hip joint and suppressive antibiotics with follow-ups by our colleagues for infectious disease. The patient was discharged with peripherally inserted central catheter line in situ. She continued to develop a collection in the left hip and anterior thigh. A formal arthrotomy with extensive debridement and removal of all necrotic tissue was performed. A large bore chest drain was left in situ, exited through quadriceps anteriorly, plus two additional drains subcutaneously. An urostomy pouch was applied and the patient was kept on long term drainage, allowing the sinus track to the left lateral thigh to mature. In the following, kept with suppressive antibiotics (ciprofloxacin and rifampicin), the patient was under close follow-up with a regular change of the urostomy bag. Erythema and tenderness regressed and drainage successively decreased (Figure 2b). The chest drain was removed two months after surgery. On examination, 29 months after debridement the left thigh is non-tender and showed no erythema. Scars to the left tight (sinus track) are well healed and she had no fever, chills or rigor and denied any pain. She is walking with a slight limp and was returned to work recently. She remains on long-term suppressive antibiotics as guided by the infectious disease physician.

Figure 2B: Photograph of the left thigh, 23 months after debridement showing externally healed sinus track. Mild erythema was noted surrounding the sinus track secondary to occlusive dressing. Previous dressing was dry and intact.

This case highlights how limb salvage surgery has pushed the boundaries over the last decades. Combining different reconstructive options may be considered for complex cases. This case also illustrates the option of a relatively conservative management strategy for periprosthetic infection in a compliant patient, achieving a satisfactory result.

Case 3 – Osteosarcoma of the proximal tibia
A 16 year old female patient was diagnosed with non metastatic osteosarcoma of the left proximal tibia (stage IIb). She underwent left tibial osteosarcoma resection, reconstruction with megaprosthesis, extensor mechanism reconstruction, medial gastrocnemius flap and split skin graft (Figure 3a and 3b).

Figure 3A: Intraoperative photograph of the osteosarcoma of the left proximal tibia after en-block removal.

Figure 3B: Anteroposterior radiograph showing megaprosthetic reconstruction after proximal tibia osteosarcoma excision.

Proximal tibia resection presents unique local anatomical features which need to be addressed during limb salvage surgery. Neurovascular structures in the popliteal fossa as well as the peroneal nerve between the biceps femoris and the lateral head of the gastrocnemius muscle need to be preserved as much as possible. Displacement of these structures due to a large tumour mass are likely, therefore careful preparation in this area is required. In most cases, biological borders such as perivascular fat or the popliteus muscle are separating the tumour from the neurovascular bundle. Intraoperatively, overstretching these structures due to overdistraction of the femur and tibia (after resecting the tumour) needs to be avoided in order to prevent neuropraxia and endothelial injury. Involvement of tissue adjacent to the tibiofibular joint is usually of concern and this joint is typically included in the resection. Once the patellar tendon has been divided, reconstruction of the extensor mechanism is obligatory. Various techniques to attach the tendon to the endoprostetic implant have been described in a systematic review by Ek et al. including direct fixation using screw/washer or sutures, synthetic soft tissue augments such as tubes, sutures, cerclage wires and non-absorbable tapes and biological augmentation such as graft from the biceps or satorius tendons or gastrocnemius flaps with or without synthetic materials [32]. The authors report a trend towards improved outcomes with biologic reconstructive options. However, there is no clear evidence for a single technique. Extensor lag is of major concern in all surgeries requiring reconstruction of the extensor mechanism. Therefore, postoperative management in the outlined case included immobilization with a long-leg cast and a long-leg brace respectively for six weeks to allow healing. Obtaining full extension and allowing the extensor mechanism to heal was given priority compared to knee flexion in view of its impact on ambulation and there was a strong emphasis on extensor mechanism strengthening during rehabilitation.
The close proximity of the proximal tibia to the skin makes the wound more susceptible to infectious complications. Keeping thick flaps during preparation at the beginning of the surgery minimize necrosis of the skin. Typically, a primary medial gastrocnemius flap is used to improve wound coverage with the advantage of offering a larger muscular material and also bypassing a longer distance when compared to the lateral gastrocnemius [33, 34]. This technique has been first described by Dubousset et al. [35]. Skin grafting provides an additional supportive procedure while relieving tension during skin closure and therefore preventing skin flap necrosis. Currently, split-thickness skin graft is considered as gold standard in any major skin loss. Typically, the graft is meshed in order for expansion while keeping the morbidity to the donor side as low as possible. Postoperative management in patients with skin grafts includes a close observation of the donor side for scarring, pain and signs suggestive of infection.
This case illustrates a conventional proximal tibia resection, highlighting surgical considerations with regards to anatomical characteristics and postoperative management.

Conclusion
Medical management, surgical techniques and prosthetic design have improved significantly during the last three decades. Amputation is no longer considered as standard of care in patients with osteosarcoma in most cases. However, limb salvage with megaprosthesis remains challenging. Complication rates are high, with infection occurring most commonly. A good interaction within a multidisciplinary team in a preferably high-volume centre is required for optimal management. Given the unique features of these patients, “unconventional” approaches and combinations of reconstructive options may be considered sometimes, provided oncologic principles are not compromised.

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How to Cite this article: Singh G, Puhaindran ME. ELimb salvage with megaprosthesis in extremity osteosarcoma –a case-based approach. Journal of Bone and Soft Tissue Tumors May- Aug 2016;2(2):13-18 .

Dr. Gurpal Singh

Dr. Mark Edward Puhaindran

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Rehabilitation following Limb-Salvage Surgery in Sarcoma

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Rehabilitation following Limb-Salvage Surgery in Sarcoma

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